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Merkel Cell Carcinoma of Left Groin: A Case Report and Literature Review

Merkel Cell Carcinoma of Left Groin: A Case Report and Literature Review Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2013, Article ID 431743, 6 pages http://dx.doi.org/10.1155/2013/431743 Case Report Merkel Cell Carcinoma of Left Groin: A Case Report and Literature Review 1 2 3 Ahmed Abu-Zaid, Ayman Azzam, Ahmed Al-Wusaibie, 4 5 3 Maraei Bin Makhashen, Abdulaziz Jarman, and Tarek Amin College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh 11533, Saudi Arabia Department of General Surgery, Faculty of Medicine, Alexandria University, Alexandria 21526, Egypt Department of Surgical Oncology, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Department of Plastic Surgery, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Correspondence should be addressed to Ahmed Abu-Zaid; aabuzaid@alfaisal.edu Received 31 March 2013; Accepted 9 May 2013 Academic Editors: Y.-F. Jiao and Y. Yamada Copyright © 2013 Ahmed Abu-Zaid et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Merkel cell carcinoma (MCC) is an uncommon highly aggressive skin malignancy with an increased tendency to recur locally, invade regional lymph nodes, and metastasize distally to lung, liver, brain, bone, and skin. The sun-exposed skin of head and neck is the most frequent site of involvement (55%). We report the case of a 63-year-old Caucasian male patient who presented with a recurrent left inguinal mass for the third time aeft r surgical resection with safe margins and no postoperative radio- or chemotherapy. eTh presented mass was excised, and pathological diagnosis revealed recurrent MCC. The patient underwent postoperative radiation therapy, and 6 months later, he developed a right groin mass which was resected and pathological diagnosis confirmed metastatic MCC. Six months later, patient developed an oropharyngeal mass which was unresectable, and pathological biopsy confirmed metastatic MCC. Patient was oeff red palliative radio- and chemotherapy. In this paper, we also present a brief literature review on MCC. 1. Introduction most importantly skin biopsy for pathological examination. Majority of MCC patients present with localized disease Merkel cell carcinoma (MCC) is an uncommon highly (70–80%). eTh clinical course of MCC is highly aggressive aggressive skin malignancy that originates from the neu- with an increased predisposition to recur locally (26–60%), roendocrine and mechanoreceptor Merkel cells in the skin invade regional lymph nodes (45–91%), and metastasize [1]. Clinical features of MCC are summed up by the acro- distally (18–52%) [4] to lung, liver, brain, bone, and skin [5]. nym AEIOU: asymptomatic/nontender tumor, expanding Management and prognosis of MCC are largely dependent rapidly, immune system suppression, older than 50 years, on tumor staging at the time of presentation. Management and ultraviolet-exposed/fair-skinned location [2]. The sun- modalities include utilization of surgical excision with safe exposed skin of head and neck is the most frequent loca- margins, lymphadenectomy, radiotherapy, and chemother- tion of involvement (55%) [3]. Due to its rarity and early apy [4]. Generally, prognosis of MCC is extremely poor with asymptomatic clinical course, diagnosis of MCC is fairly a high mortality rate [3]. challenging, oen ft delayed, or even missed [ 4]. Definitive Herein, we report a 63-year-old Caucasian male patient diagnosis requires a high index of clinical suspicion and who presented with an unusual recurrent mass in the left 2 Case Reports in Oncological Medicine groin (nonsun-exposed site) for the third time aer ft surgical resection and subsequently developed regional metastasis to the contralateral groin, as well as distant metastasis to the oropharynx—an exceedingly unusual site of metastasis. 2. Case Report A 63-year-old Caucasian male patient was referred to our hospital for further management of a recurrent big mass in the left inguinal region. Past medical history was remarkable for severely uncontrolled diabetes mellitus and hypertension. Past surgical history was remarkable for two surgical resec- tions (with safe margins) of recurrent left inguinal masses andwithoutpostoperativeradio-orchemotherapy.Patholog- Figure 1: Computed tomography (CT) scan with contrast showing ical diagnosis of both resected masses revealed Merkel cell an 8.5× 10.5 cm, heterogeneous, lobulated, and large mass in the left carcinoma. On physical examination, the left inguinal mass groin, compressing the left common femoral vein and inseparable was oval, measuring around 9× 11cm,lobulated,nontender, from the vein as well as from the adductor muscles ventrally. eTh firm, fixed to underlying tissue, and with no overlying skin mass is associated with local lymphadenopathy, multiple small sub- changes. The patient was admitted for further tumor workup. cutaneous nodules, and an enlarged left external iliac lymph node. Upon admission, all laboratory tests including complete blood count, renal, bone, hepatic, and coagulation profiles, carcinoembryonic antigen (CEA), alfa-fetoprotein (AFP), andCA12–5werenormal. Computed tomography (CT) scan with contrast revealed a large multilobulated mass with heterogeneous enhance- ment at the left groin. The mass was compressing the left common femoral vein and remained inseparable from the vein as well as from the adductor muscles ventrally. The mass was associated with local lymphadenopathy, multiple small subcutaneous nodules, and an enlarged left external iliac lymph node measuring around 1× 1.5 cm (Figure 1). Positron emission tomography (PET) scan revealed hypermetabolic, heterogeneous, and lobulated lesion seen in the left groin that measured approximately 9.6 × 9cm in its transverse and anteroposterior diameters. In the vicinity, there were few nodal lesions with moderate activity, mostly relatedtolocal metastatic disease(Figure 2). Afterwards, the patient underwent left inguinal dissec- tion with excision of the tumor. Macroscopic examination revealed a large, solid, rm, fi yellow-tanned, and lobulated Figure 2: Positron emission tomography (PET) scan showing left mass measuring 11× 10.5× 9.5 cm (Figure 3(a)). Microscopic inguinal hypermetabolic, heterogeneous, and lobulated mass lesion examination showed a tumor composed of small uniformly with few nodal lesions in the same vicinity consistent with the sizedblueneoplasticcells with roundtoovalnuclei, scant knownMerkelcellcarcinoma. cytoplasm, distinct nuclear membranes, nel fi y dispersed nuclear chromatin, and inconspicuous nucleoli (Figure 3(b)). Mitotic gur fi es and individually necrotic cells were present. In addition, nests of neoplastic cells metastasizing to the left right groin, and the patient was admitted to the hospital. femoral lymph node were noted (Figure 3(c)). The neoplastic Local resection of the mass was done with safe margins. cells expressed cytokeratin 20 (CK20) in a perinuclear dot- The postoperative period was uneventful. Pathology analysis like fashion (Figure 3(d)). Further, the neoplastic cells also revealed metastatic Merkel cell carcinoma. eTh patient was expressed CD56 showing cytoplasmic and membranous pos- discharged in good shapeand startedonradiotherapy1 itivity (Figure 3(e)). The neoplastic cell stained negative for month after hospital discharge. LCA, S-100, CK7, and TTF-1. Based on the immunohisto- Another 6 months aer ft hospital discharge, the patient chemical stains, diagnosis of Merkel cell carcinoma (MCC) presented to the emergency department complaining of dys- was established. Subsequently, the radiation oncology team phagiawithsolidfoodandassociatedwithmuffledsoundand was consulted, and the plan was to start radiotherapy 3 weeks throat pain. Consultation with ear, nose, and throat (ENT) after the operation. team was done, and CT scan was ordered which revealed large Six months aeft r hospital discharge and during the exophyticmasslesioninthe oropharynx arisingfromthe left followup period, a rapidly growing mass appeared on the side base of the tongue indicative of malignant tumor with Case Reports in Oncological Medicine 3 (a) (b) (c) (d) (e) Figure 3: Merkel cell carcinoma. (a) Macroscopic examination of the resected mass showing a large, yellow-tanned, and lobulated mass. (b) H&E stain showing sheets of small blue cells with scant cytoplasm, irregular nuclei, mitoses, and individually necrotic cells. (c) H&E stain showing invasion of femoral lymph node by nests of neoplastic cells. (d) eTh neoplastic cells stain positive for CK20 in a perinuclear dot-like fashion. (e) eTh neoplastic cells stain positive for CD 56. enlarged left-sided group 2 and 3 cervical lymphadenopathy carcinoma of skin, trabecular carcinoma of skin, and cuta- with necrosis. PET scan revealed interval development of neous APUDoma [6]. MCC is an exceedingly rare and highly multiple u fl orodeoxyglucose- (FDG-) avid lesions involv- aggressive cutaneous malignancy arising from uncontrolled ing left thigh, left inguinal region, left hip, and left lower proliferation of the neuroendocrine and mechanoreceptor abdomen, consistent with progression of the MCC disease. Merkel cellsthatare locatedinthe stratumbasalelayer of Furthermore, activity was also noted in the lungs and neck epidermal skin [1]. bilaterally, suggestive of MCC metastases. Under general Numerous etiological factors contribute to development anesthesia, biopsy was taken from the large exophytic mass of MCC. These factors include exposure to ultraviolet (UV) lesion in the oropharynx which proved to be metastatic radiation [7],infectionwithMerkelcellpolyomavirus(MCV) Merkel cell carcinoma, and it was not amenable for resection. [8], and statues of chronic immunosuppression [2, 9, 10]. Tracheostomy and feeding gastrostomy tubes were inserted. MCC is mainly a malignancy of UV-exposed and fair- The decision of the medical oncology consultation team was skinned elderly Caucasians [2]. It is most frequently found on to start the patient on palliative radio- and chemotherapy. skin-damaged and sun-exposed areas particularly face, head, and neck (55%), followed by extremities (40%) and lastly followed by truncal structures (5%) [3]. MCC occurring in 3. Discussion nonsun-exposed sites, such as groins, is extremely uncom- Merkel cell carcinoma (MCC) is also known as primary small mon. Although infection with MCV and its subsequent cell carcinoma of skin, primary neuroendocrine carcinoma of monoclonal integration into the genome accounts for approx- skin, primary undifferentiated carcinoma of skin, anaplastic imately80% of allcases of MCC[11], interestingly, our patient 4 Case Reports in Oncological Medicine tested negative for anti-MCV antibodies. Immunosuppressed lymph nodes (IA: less than 2 cm, and IB: more than 2 cm); individuals who are at an increased risk of developing MCC Stage II: evidence of regional lymph node invasion; Stage include solid organ transplant recipients [9], HIV-infected III: evidence of distant metastatic disease. Management of AIDS patients [10], and B-cell lymphoma individuals [2]. MCC primarily depends on the staging of disease at time Clinically, MCC presents as a painless, nontender, rfi m, of diagnosis: for Stages I and II, the aim of management is glossy, bluish-red or bluish-purple, rapidly growing nodule of therapeutic, whereas for Stage III, the aim of management is less than 2cmindiameteratthe time of clinical presentation geared towards palliative care. [12]. Overlying skin may exhibit acneiform, telangiectatic, or For a localized disease (Stage I), an extensive surgical ulcerative characteristics. In addition, associations with sev- excision (3 cm wide and 2 cm deep), or alternatively, Mohs eral satellite lymphadenopathies secondary to MCC invading micrographic surgery [4, 19], along with adjuvant locore- dermal lymphatics are also possible [13]. Majority of MCC gional radiation therapy has been shown to yield an overall cases present as localized disease (70%–80%), followed by improved survival rather than surgery alone [20]. For patients invasion of regional lymph nodes (9%–26%) and lastly with positive regional lymphadenopathy (Stage II), manage- followed by extra nodal distant metastasis (1%–4%) [4]. ment is dependent on resectability of regional lymph nodes. Due to the low incidence rate of MCC and its distinc- Patients with possibly resectable lymph nodes are managed tive early symptom-free clinical course, diagnosis is highly with regional lymphnodedissectionfollowedbyadjuvant challenging and therefore oeft n delayed, or even missed [ 4]. radiation therapy. On the contrary, patients with unresectable Diagnosis is based on a hybrid of light microscopy, electron metastatic lymph nodes are treated with neoadjuvant radio- microscopy, and immunohistochemistry. Microscopically, and/or chemotherapy followed by lymph node dissection [4]. MCC frequently originates in the dermis and occasionally Lymphadenectomy is highly recommended in sentinel lymph extends into subcutaneous tissues and muscles; the overlying node- (SLN-) positive biopsies as SLN positivity is highly epidermis is usually preserved [14]. Histologically, the car- extrapolative of an increased risk of potential local/regional cinoma is composed of small round blue cells, with sparse recurrence and distant metastasis [21]. On the contrary, cytoplasm, medium- to large-sized hyperchromatic nuclei, prophylactic lymphadenectomy is neither recommended as a multiple small nucleoli, delicately granular chromatin, abun- standard management scheme nor in SLN-negative biopsies; dant mitoses, and numerous apoptotic gfi ures [ 4]. Ultrastruc- however, it is recommended in MCC malignancies that are turally, electron microscopy demonstrates distinctive intracy- at an increased risk of potential recurrence and aggressive toplasmic neurosecretory/neuroendocrine granules [1, 2]and metastasis [22]. collection of intermediate filaments organized in a globular For an MCC metastatic disease (Stage III), radio- and paranuclear configuration [ 15]. chemotherapy are employed with a palliative intent [23]. MCC is occasionally mistaken for other histologically MCC is predominantly a radio-sensitive malignancy, and uti- related cutaneous tumors, such as malignant melanoma, lization of radiation therapy is highly advised [24]. However, lymphoma, small cell lung carcinoma, and extraskeletal the role of chemotherapy is still debatable and does not seem primitive neuroendocrine tumors (PNET/Ewing’s sarcoma) to yield survival benefit [ 3]. Tumor regression and remission [13]. Immunohistochemistry is a valuable method to establish rates can be as high as 70%; however, disease rapidly recurs a definitive differentiation between MCC and other closely within a couple of months, and response does not appear related skin neoplasms [16]. Generally, MCC cells express to signicfi antly prolong survival [ 3]. The most frequently both epithelial and neuroendocrine markers. Specifically, used chemotherapeutic regimens include the combination MCCcellsstainpositivelyforepithelialmarkerssuchasCK20 of cyclophosphamide, doxorubicin, and vincristine, as well in a peculiar “dot-like” fashion (negative in extraskeletal as the combination of cisplatin or carboplatin plus/minus PNET/Ewing’s sarcoma) and stain negatively for epithelial etoposide [3, 25]. markers such as LCA (positive in malignant lymphoma), S- Generally, prognosis of MCC is unfortunate with a 100 (positive in malignant melanoma), and CK7 and TTF- high mortality rate in which nearly one-third of patients 1 (positive in small cell lung carcinoma) [17]. Furthermore, pass away from MCC within 36 months from the time of MCC cells stain positively for neuroendocrine markers such diagnosis [3]. The following clinical and histological factors as chromogranin A, synaptophysin, NSE, neurofilament, and are predictive of poor prognosis in MCC: male gender, age CD 56. more than 65 years, existence of comorbid immunocom- MCC is a highly aggressive cutaneous tumor with an promised/immunosuppressant status, presence of metastatic increased tendency to recur locally (27%–60%), invade disease, tumor situated in head and neck regions, tumor size regional lymph nodes (45%–91%), and metastasize distally more than 2 cm in diameter, tumor with more than 10 mitoses (18%–52%) [4]. MCC distant metastases are not uncommon per high-power field (HPF), evidence of vascular invasion, and typically involve lung, liver, bone, brain, and skin [5]. absence of an inflammatory reaction, and high expression of MCC distant metastases to oropharyngeal structures are Ki-67 (proliferation index marker) and p63 (antiproliferative exceedingly rare. and apoptosis-inducing marker) [1, 26]. eTh re is no universally agreed consensus on staging of The most significant unfavorable/poor prognostic factor MCC [4]. However, the most broadly used staging system of long-term survival and also associated with an increased has been proposed by Yiengpruksawan et al. [18]based on risk of yielding metastatic disease is an evidence of lymph clinical manifestations at the time of diagnosis, as follows: node invasion [19]. Presence of regional lymph node invasion Stage I: localized skin tumor with no evidence of regional markedly drops the overall survival rate from 90% to 50%, Case Reports in Oncological Medicine 5 and it occurs in almost 50%–70% of all patients within 24 [5] P.Peloschek,C.Novotny,C.Mueller-Mangetal.,“Diagnostic imaging in Merkel cell carcinoma: lessons to learn from 16 cases months from the time of clinical diagnosis [21]. Presence with correlationofsonography, CT,MRI andPET,” European of distant metastases signifies a deadly condition, and the Journal of Radiology,vol.73, no.2,pp. 317–323, 2010. anticipated survival is frequently less than 10% within a [6] M. L. Haag, L. F. Glass, and N. A. Fenske, “Merkel cell carci- frametimeof3years[27], and death mostly ensues within noma: diagnosis and treatment,” Dermatologic Surgery,vol.21, 10 months from time of diagnosis of metastatic disease no. 8, pp. 669–683, 1995. [28]. The literature has shown that neither chemotherapeutic [7] M.T.Fernandez-Figueras, ´ L. Puig, E. Musulen ´ et al., “Expres- nor immunotherapeutic or molecular-targeted therapeutic sion profiles associated with aggressive behavior in Merkelcell tacticshaverevealedfavorable impact on themanagementof carcinoma,” Modern Pathology,vol.20, no.1,pp. 90–101,2007. metastatic MCC disease [29]. [8] H.Feng, M. Shuda, Y. Chang, andP.S.Moore,“Clonal integra- tion of a polyomavirus in human Merkel cell carcinoma,” 4. Conclusion Science,vol.319,no. 5866,pp. 1096–1100, 2008. [9] V. Koljonen, H. Kukko, E. Tukiainen et al., “Incidence of Merkel Merkel cell carcinoma (MCC) occurring in nonsun-exposed cell carcinoma in renal transplant recipients,” Nephrology Dial- sites, such as inguinal regions, is exceptionally rare. How- ysis Transplantation,vol.24, no.10, pp.3231–3235,2009. ever, they should always be considered in the differential [10] E. A. Engels, M. Frisch, J. J. Goedert, R. J. Biggar, and R. W. diagnosis in any elderly (above 50 years old), fair-thinned Miller, “Merkel cell carcinoma and HIV infection,” The Lancet , and immunocompromised patient presenting with a non- vol. 359, no. 9305, pp. 497–498, 2002. tender and rapidly expanding inguinal mass. Skin biopsy [11] R. Houben, M. Shuda, R. Weinkam et al., “Merkel cell polyo- for pathological examination is necessary for a definitive mavirus-infected Merkel cell carcinoma cells require expression diagnosis. Management of MCC is largely dependent on of viral T antigens,” Journal of Virology,vol.84, no.14, pp.7064– tumor staging at the time of presentation: curative intent for 7072, 2010. locoregional disease and palliative intent for distant disease. [12] M. H. Swann and J. Yoon, “Merkel cell Carcinoma,” Seminars in Optimal management modalities with varying results include Oncology, vol. 34, no. 1, pp. 51–56, 2007. surgical excision of primary tumor with safe margins, radio- [13] P. S. Gorjo´n,A.C.M.Mart´ın, P. B. Pe´rez,J.L.G.Gonzalez, ´ J. therapy, chemotherapy, immunotherapy, molecular-targeted C. delPozodeDios, andA.Melgar, “Merkelcellcarcinoma:a therapy, and lymphadenectomy to control regional and dis- presentation of 5 cases and a review of the literature,” Acta Oto- rrinolaringolog ´ ica Espano ˜ la,vol.62, no.4,pp. 306–310, 2011. tant disease. Despite management, MCC has an exception- ally increased tendency to recur locally (26–60%), invade [14] O. Bayrou,M.F.Avril,P.Charpentier,B.Caillou,J.C.Guil- regional lymph nodes (45–91%), and eventually metasta- laume, and M. Prade, “Primary neuroendocrine carcinoma of the skin. Clinicopathologic study of 18 cases,” Journal of the size distally (18–52%) to liver, lung, bone, brain, and skin. American Academy of Dermatology,vol.24, no.2,pp. 198–207, Hence, frequent short- and long-term followups are highly recommended. Broadly, MCC has a very poor prognosis, and [15] I.Hierro, A. Blanes,A.Matilla, S. Muno ˜ z, L. Vicioso, and F. F. generally one-third of patients pass away from MCC within Nogales, “Merkel cell (neuroendocrine) carcinoma of the vulva. 36 months from time of diagnosis. A case report with immunohistochemical and ultrastructural findings and review of the literature,” Pathology Research and Acknowledgment Practice,vol.196,no. 7, pp.503–509,2000. [16] K. Gessner, G. Wichmann, A. Boehm et al., “Therapeutic eTh authors sincerely acknowledge the editorial assistance of options for treatment of Merkel cell carcinoma,” European Arch- Ms. Tehreem Khan and Ms. Ranim Chamseddin, College of ives of Oto-Rhino-Laryngology,vol.268,no.3,pp.443–448,2011. Medicine, Alfaisal University, Riyadh, Saudi Arabia. [17] P. Nghiem and N. Jaimes, “Chapter 120: Merkel cell carcinoma,” in Fitzpatrick’s Dermatology in General Medicine,K.Wol,ff S. Katz,L.Goldsmith,B.Gilchrest,D.Leeff ll, andA.Paller, Eds., References chapter120,McGraw-Hill,New York,NY, USA, 7thedition, [1] R. Gollard, R. Weber, M. P. Kosty, H. T. Greenway, V. Massullo, and C. Humberson, “Merkel cell carcinoma review of 22 [18] A. Yiengpruksawan,D.G.Coit,H.T.Thaler,C.Urmacher, and cases with surgical, pathologic, and therapeutic considerations,” W. K. Knapper, “Merkel cell carcinoma: prognosis and manage- Cancer, vol. 88, no. 8, pp. 1842–1851, 2000. ment,” Archives of Surgery,vol.126,no. 12,pp. 1514–1519, 1991. [2] M. Heath, N. Jaimes, B. Lemos et al., “Clinical characteristics of [19] T. Y. Eng, M. G. K. Boersma, C. D. Fuller, S. X. Cavanaugh, Merkel cell carcinoma at diagnosis in 195 patients: the AEIOU F. Valenzuela, and T. S. Herman, “Treatment of Merkel cell features,” Journal of the American Academy of Dermatology,vol. carcinoma,” American Journal of Clinical Oncology,vol.27, no. 58,no. 3, pp.375–381,2008. 5, pp.510–515,2004. [3] RockvilleMerkelcellCarcinoma Group, “Merkelcellcarci- [20] M. J. Veness, “Merkel cell carcinoma (primary cutaneous neu- noma: recent progress and current priorities on etiology, patho- roendocrine carcinoma): an overview on management,” Aus- genesis, and clinical management,” Journal of Clinical Oncology, tralasian Journal of Dermatology,vol.47, no.3,pp. 160–165, vol. 27, no. 24, pp. 4021–4026, 2009. [4] D. Pectasides, M. Pectasides, and T. Economopoulos, “Merkel [21] K. Mehrany, C. C. Otley, R. H. Weenig, P. K. Phillips, R. K. cell cancer of the skin,” Annals of Oncology,vol.17, no.10, pp. Roenigk, and T. H. Nguyen, “A meta-analysis of the prognostic 1489–1495, 2006. significance of sentinel lymph node status in Merkel cell 6 Case Reports in Oncological Medicine carcinoma,” Dermatologic Surgery,vol.28, no.2,pp. 113–117, [22] D. Ratner,B.R.Nelson, M. D. Brown, andT.M.Johnson, “Merkel cell carcinoma,” Journal of the American Academy of Dermatology,vol.29, no.2,part1,pp. 143–156, 1993. [23] M. Papamichail, I. Nikolaidis, N. 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Merkel Cell Carcinoma of Left Groin: A Case Report and Literature Review

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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2013, Article ID 431743, 6 pages http://dx.doi.org/10.1155/2013/431743 Case Report Merkel Cell Carcinoma of Left Groin: A Case Report and Literature Review 1 2 3 Ahmed Abu-Zaid, Ayman Azzam, Ahmed Al-Wusaibie, 4 5 3 Maraei Bin Makhashen, Abdulaziz Jarman, and Tarek Amin College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh 11533, Saudi Arabia Department of General Surgery, Faculty of Medicine, Alexandria University, Alexandria 21526, Egypt Department of Surgical Oncology, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Department of Plastic Surgery, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi Arabia Correspondence should be addressed to Ahmed Abu-Zaid; aabuzaid@alfaisal.edu Received 31 March 2013; Accepted 9 May 2013 Academic Editors: Y.-F. Jiao and Y. Yamada Copyright © 2013 Ahmed Abu-Zaid et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Merkel cell carcinoma (MCC) is an uncommon highly aggressive skin malignancy with an increased tendency to recur locally, invade regional lymph nodes, and metastasize distally to lung, liver, brain, bone, and skin. The sun-exposed skin of head and neck is the most frequent site of involvement (55%). We report the case of a 63-year-old Caucasian male patient who presented with a recurrent left inguinal mass for the third time aeft r surgical resection with safe margins and no postoperative radio- or chemotherapy. eTh presented mass was excised, and pathological diagnosis revealed recurrent MCC. The patient underwent postoperative radiation therapy, and 6 months later, he developed a right groin mass which was resected and pathological diagnosis confirmed metastatic MCC. Six months later, patient developed an oropharyngeal mass which was unresectable, and pathological biopsy confirmed metastatic MCC. Patient was oeff red palliative radio- and chemotherapy. In this paper, we also present a brief literature review on MCC. 1. Introduction most importantly skin biopsy for pathological examination. Majority of MCC patients present with localized disease Merkel cell carcinoma (MCC) is an uncommon highly (70–80%). eTh clinical course of MCC is highly aggressive aggressive skin malignancy that originates from the neu- with an increased predisposition to recur locally (26–60%), roendocrine and mechanoreceptor Merkel cells in the skin invade regional lymph nodes (45–91%), and metastasize [1]. Clinical features of MCC are summed up by the acro- distally (18–52%) [4] to lung, liver, brain, bone, and skin [5]. nym AEIOU: asymptomatic/nontender tumor, expanding Management and prognosis of MCC are largely dependent rapidly, immune system suppression, older than 50 years, on tumor staging at the time of presentation. Management and ultraviolet-exposed/fair-skinned location [2]. The sun- modalities include utilization of surgical excision with safe exposed skin of head and neck is the most frequent loca- margins, lymphadenectomy, radiotherapy, and chemother- tion of involvement (55%) [3]. Due to its rarity and early apy [4]. Generally, prognosis of MCC is extremely poor with asymptomatic clinical course, diagnosis of MCC is fairly a high mortality rate [3]. challenging, oen ft delayed, or even missed [ 4]. Definitive Herein, we report a 63-year-old Caucasian male patient diagnosis requires a high index of clinical suspicion and who presented with an unusual recurrent mass in the left 2 Case Reports in Oncological Medicine groin (nonsun-exposed site) for the third time aer ft surgical resection and subsequently developed regional metastasis to the contralateral groin, as well as distant metastasis to the oropharynx—an exceedingly unusual site of metastasis. 2. Case Report A 63-year-old Caucasian male patient was referred to our hospital for further management of a recurrent big mass in the left inguinal region. Past medical history was remarkable for severely uncontrolled diabetes mellitus and hypertension. Past surgical history was remarkable for two surgical resec- tions (with safe margins) of recurrent left inguinal masses andwithoutpostoperativeradio-orchemotherapy.Patholog- Figure 1: Computed tomography (CT) scan with contrast showing ical diagnosis of both resected masses revealed Merkel cell an 8.5× 10.5 cm, heterogeneous, lobulated, and large mass in the left carcinoma. On physical examination, the left inguinal mass groin, compressing the left common femoral vein and inseparable was oval, measuring around 9× 11cm,lobulated,nontender, from the vein as well as from the adductor muscles ventrally. eTh firm, fixed to underlying tissue, and with no overlying skin mass is associated with local lymphadenopathy, multiple small sub- changes. The patient was admitted for further tumor workup. cutaneous nodules, and an enlarged left external iliac lymph node. Upon admission, all laboratory tests including complete blood count, renal, bone, hepatic, and coagulation profiles, carcinoembryonic antigen (CEA), alfa-fetoprotein (AFP), andCA12–5werenormal. Computed tomography (CT) scan with contrast revealed a large multilobulated mass with heterogeneous enhance- ment at the left groin. The mass was compressing the left common femoral vein and remained inseparable from the vein as well as from the adductor muscles ventrally. The mass was associated with local lymphadenopathy, multiple small subcutaneous nodules, and an enlarged left external iliac lymph node measuring around 1× 1.5 cm (Figure 1). Positron emission tomography (PET) scan revealed hypermetabolic, heterogeneous, and lobulated lesion seen in the left groin that measured approximately 9.6 × 9cm in its transverse and anteroposterior diameters. In the vicinity, there were few nodal lesions with moderate activity, mostly relatedtolocal metastatic disease(Figure 2). Afterwards, the patient underwent left inguinal dissec- tion with excision of the tumor. Macroscopic examination revealed a large, solid, rm, fi yellow-tanned, and lobulated Figure 2: Positron emission tomography (PET) scan showing left mass measuring 11× 10.5× 9.5 cm (Figure 3(a)). Microscopic inguinal hypermetabolic, heterogeneous, and lobulated mass lesion examination showed a tumor composed of small uniformly with few nodal lesions in the same vicinity consistent with the sizedblueneoplasticcells with roundtoovalnuclei, scant knownMerkelcellcarcinoma. cytoplasm, distinct nuclear membranes, nel fi y dispersed nuclear chromatin, and inconspicuous nucleoli (Figure 3(b)). Mitotic gur fi es and individually necrotic cells were present. In addition, nests of neoplastic cells metastasizing to the left right groin, and the patient was admitted to the hospital. femoral lymph node were noted (Figure 3(c)). The neoplastic Local resection of the mass was done with safe margins. cells expressed cytokeratin 20 (CK20) in a perinuclear dot- The postoperative period was uneventful. Pathology analysis like fashion (Figure 3(d)). Further, the neoplastic cells also revealed metastatic Merkel cell carcinoma. eTh patient was expressed CD56 showing cytoplasmic and membranous pos- discharged in good shapeand startedonradiotherapy1 itivity (Figure 3(e)). The neoplastic cell stained negative for month after hospital discharge. LCA, S-100, CK7, and TTF-1. Based on the immunohisto- Another 6 months aer ft hospital discharge, the patient chemical stains, diagnosis of Merkel cell carcinoma (MCC) presented to the emergency department complaining of dys- was established. Subsequently, the radiation oncology team phagiawithsolidfoodandassociatedwithmuffledsoundand was consulted, and the plan was to start radiotherapy 3 weeks throat pain. Consultation with ear, nose, and throat (ENT) after the operation. team was done, and CT scan was ordered which revealed large Six months aeft r hospital discharge and during the exophyticmasslesioninthe oropharynx arisingfromthe left followup period, a rapidly growing mass appeared on the side base of the tongue indicative of malignant tumor with Case Reports in Oncological Medicine 3 (a) (b) (c) (d) (e) Figure 3: Merkel cell carcinoma. (a) Macroscopic examination of the resected mass showing a large, yellow-tanned, and lobulated mass. (b) H&E stain showing sheets of small blue cells with scant cytoplasm, irregular nuclei, mitoses, and individually necrotic cells. (c) H&E stain showing invasion of femoral lymph node by nests of neoplastic cells. (d) eTh neoplastic cells stain positive for CK20 in a perinuclear dot-like fashion. (e) eTh neoplastic cells stain positive for CD 56. enlarged left-sided group 2 and 3 cervical lymphadenopathy carcinoma of skin, trabecular carcinoma of skin, and cuta- with necrosis. PET scan revealed interval development of neous APUDoma [6]. MCC is an exceedingly rare and highly multiple u fl orodeoxyglucose- (FDG-) avid lesions involv- aggressive cutaneous malignancy arising from uncontrolled ing left thigh, left inguinal region, left hip, and left lower proliferation of the neuroendocrine and mechanoreceptor abdomen, consistent with progression of the MCC disease. Merkel cellsthatare locatedinthe stratumbasalelayer of Furthermore, activity was also noted in the lungs and neck epidermal skin [1]. bilaterally, suggestive of MCC metastases. Under general Numerous etiological factors contribute to development anesthesia, biopsy was taken from the large exophytic mass of MCC. These factors include exposure to ultraviolet (UV) lesion in the oropharynx which proved to be metastatic radiation [7],infectionwithMerkelcellpolyomavirus(MCV) Merkel cell carcinoma, and it was not amenable for resection. [8], and statues of chronic immunosuppression [2, 9, 10]. Tracheostomy and feeding gastrostomy tubes were inserted. MCC is mainly a malignancy of UV-exposed and fair- The decision of the medical oncology consultation team was skinned elderly Caucasians [2]. It is most frequently found on to start the patient on palliative radio- and chemotherapy. skin-damaged and sun-exposed areas particularly face, head, and neck (55%), followed by extremities (40%) and lastly followed by truncal structures (5%) [3]. MCC occurring in 3. Discussion nonsun-exposed sites, such as groins, is extremely uncom- Merkel cell carcinoma (MCC) is also known as primary small mon. Although infection with MCV and its subsequent cell carcinoma of skin, primary neuroendocrine carcinoma of monoclonal integration into the genome accounts for approx- skin, primary undifferentiated carcinoma of skin, anaplastic imately80% of allcases of MCC[11], interestingly, our patient 4 Case Reports in Oncological Medicine tested negative for anti-MCV antibodies. Immunosuppressed lymph nodes (IA: less than 2 cm, and IB: more than 2 cm); individuals who are at an increased risk of developing MCC Stage II: evidence of regional lymph node invasion; Stage include solid organ transplant recipients [9], HIV-infected III: evidence of distant metastatic disease. Management of AIDS patients [10], and B-cell lymphoma individuals [2]. MCC primarily depends on the staging of disease at time Clinically, MCC presents as a painless, nontender, rfi m, of diagnosis: for Stages I and II, the aim of management is glossy, bluish-red or bluish-purple, rapidly growing nodule of therapeutic, whereas for Stage III, the aim of management is less than 2cmindiameteratthe time of clinical presentation geared towards palliative care. [12]. Overlying skin may exhibit acneiform, telangiectatic, or For a localized disease (Stage I), an extensive surgical ulcerative characteristics. In addition, associations with sev- excision (3 cm wide and 2 cm deep), or alternatively, Mohs eral satellite lymphadenopathies secondary to MCC invading micrographic surgery [4, 19], along with adjuvant locore- dermal lymphatics are also possible [13]. Majority of MCC gional radiation therapy has been shown to yield an overall cases present as localized disease (70%–80%), followed by improved survival rather than surgery alone [20]. For patients invasion of regional lymph nodes (9%–26%) and lastly with positive regional lymphadenopathy (Stage II), manage- followed by extra nodal distant metastasis (1%–4%) [4]. ment is dependent on resectability of regional lymph nodes. Due to the low incidence rate of MCC and its distinc- Patients with possibly resectable lymph nodes are managed tive early symptom-free clinical course, diagnosis is highly with regional lymphnodedissectionfollowedbyadjuvant challenging and therefore oeft n delayed, or even missed [ 4]. radiation therapy. On the contrary, patients with unresectable Diagnosis is based on a hybrid of light microscopy, electron metastatic lymph nodes are treated with neoadjuvant radio- microscopy, and immunohistochemistry. Microscopically, and/or chemotherapy followed by lymph node dissection [4]. MCC frequently originates in the dermis and occasionally Lymphadenectomy is highly recommended in sentinel lymph extends into subcutaneous tissues and muscles; the overlying node- (SLN-) positive biopsies as SLN positivity is highly epidermis is usually preserved [14]. Histologically, the car- extrapolative of an increased risk of potential local/regional cinoma is composed of small round blue cells, with sparse recurrence and distant metastasis [21]. On the contrary, cytoplasm, medium- to large-sized hyperchromatic nuclei, prophylactic lymphadenectomy is neither recommended as a multiple small nucleoli, delicately granular chromatin, abun- standard management scheme nor in SLN-negative biopsies; dant mitoses, and numerous apoptotic gfi ures [ 4]. Ultrastruc- however, it is recommended in MCC malignancies that are turally, electron microscopy demonstrates distinctive intracy- at an increased risk of potential recurrence and aggressive toplasmic neurosecretory/neuroendocrine granules [1, 2]and metastasis [22]. collection of intermediate filaments organized in a globular For an MCC metastatic disease (Stage III), radio- and paranuclear configuration [ 15]. chemotherapy are employed with a palliative intent [23]. MCC is occasionally mistaken for other histologically MCC is predominantly a radio-sensitive malignancy, and uti- related cutaneous tumors, such as malignant melanoma, lization of radiation therapy is highly advised [24]. However, lymphoma, small cell lung carcinoma, and extraskeletal the role of chemotherapy is still debatable and does not seem primitive neuroendocrine tumors (PNET/Ewing’s sarcoma) to yield survival benefit [ 3]. Tumor regression and remission [13]. Immunohistochemistry is a valuable method to establish rates can be as high as 70%; however, disease rapidly recurs a definitive differentiation between MCC and other closely within a couple of months, and response does not appear related skin neoplasms [16]. Generally, MCC cells express to signicfi antly prolong survival [ 3]. The most frequently both epithelial and neuroendocrine markers. Specifically, used chemotherapeutic regimens include the combination MCCcellsstainpositivelyforepithelialmarkerssuchasCK20 of cyclophosphamide, doxorubicin, and vincristine, as well in a peculiar “dot-like” fashion (negative in extraskeletal as the combination of cisplatin or carboplatin plus/minus PNET/Ewing’s sarcoma) and stain negatively for epithelial etoposide [3, 25]. markers such as LCA (positive in malignant lymphoma), S- Generally, prognosis of MCC is unfortunate with a 100 (positive in malignant melanoma), and CK7 and TTF- high mortality rate in which nearly one-third of patients 1 (positive in small cell lung carcinoma) [17]. Furthermore, pass away from MCC within 36 months from the time of MCC cells stain positively for neuroendocrine markers such diagnosis [3]. The following clinical and histological factors as chromogranin A, synaptophysin, NSE, neurofilament, and are predictive of poor prognosis in MCC: male gender, age CD 56. more than 65 years, existence of comorbid immunocom- MCC is a highly aggressive cutaneous tumor with an promised/immunosuppressant status, presence of metastatic increased tendency to recur locally (27%–60%), invade disease, tumor situated in head and neck regions, tumor size regional lymph nodes (45%–91%), and metastasize distally more than 2 cm in diameter, tumor with more than 10 mitoses (18%–52%) [4]. MCC distant metastases are not uncommon per high-power field (HPF), evidence of vascular invasion, and typically involve lung, liver, bone, brain, and skin [5]. absence of an inflammatory reaction, and high expression of MCC distant metastases to oropharyngeal structures are Ki-67 (proliferation index marker) and p63 (antiproliferative exceedingly rare. and apoptosis-inducing marker) [1, 26]. eTh re is no universally agreed consensus on staging of The most significant unfavorable/poor prognostic factor MCC [4]. However, the most broadly used staging system of long-term survival and also associated with an increased has been proposed by Yiengpruksawan et al. [18]based on risk of yielding metastatic disease is an evidence of lymph clinical manifestations at the time of diagnosis, as follows: node invasion [19]. Presence of regional lymph node invasion Stage I: localized skin tumor with no evidence of regional markedly drops the overall survival rate from 90% to 50%, Case Reports in Oncological Medicine 5 and it occurs in almost 50%–70% of all patients within 24 [5] P.Peloschek,C.Novotny,C.Mueller-Mangetal.,“Diagnostic imaging in Merkel cell carcinoma: lessons to learn from 16 cases months from the time of clinical diagnosis [21]. Presence with correlationofsonography, CT,MRI andPET,” European of distant metastases signifies a deadly condition, and the Journal of Radiology,vol.73, no.2,pp. 317–323, 2010. anticipated survival is frequently less than 10% within a [6] M. L. Haag, L. F. Glass, and N. A. Fenske, “Merkel cell carci- frametimeof3years[27], and death mostly ensues within noma: diagnosis and treatment,” Dermatologic Surgery,vol.21, 10 months from time of diagnosis of metastatic disease no. 8, pp. 669–683, 1995. [28]. The literature has shown that neither chemotherapeutic [7] M.T.Fernandez-Figueras, ´ L. Puig, E. Musulen ´ et al., “Expres- nor immunotherapeutic or molecular-targeted therapeutic sion profiles associated with aggressive behavior in Merkelcell tacticshaverevealedfavorable impact on themanagementof carcinoma,” Modern Pathology,vol.20, no.1,pp. 90–101,2007. metastatic MCC disease [29]. [8] H.Feng, M. Shuda, Y. Chang, andP.S.Moore,“Clonal integra- tion of a polyomavirus in human Merkel cell carcinoma,” 4. Conclusion Science,vol.319,no. 5866,pp. 1096–1100, 2008. [9] V. Koljonen, H. Kukko, E. Tukiainen et al., “Incidence of Merkel Merkel cell carcinoma (MCC) occurring in nonsun-exposed cell carcinoma in renal transplant recipients,” Nephrology Dial- sites, such as inguinal regions, is exceptionally rare. How- ysis Transplantation,vol.24, no.10, pp.3231–3235,2009. ever, they should always be considered in the differential [10] E. A. Engels, M. Frisch, J. J. Goedert, R. J. Biggar, and R. 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Pe´rez,J.L.G.Gonzalez, ´ J. therapy, chemotherapy, immunotherapy, molecular-targeted C. delPozodeDios, andA.Melgar, “Merkelcellcarcinoma:a therapy, and lymphadenectomy to control regional and dis- presentation of 5 cases and a review of the literature,” Acta Oto- rrinolaringolog ´ ica Espano ˜ la,vol.62, no.4,pp. 306–310, 2011. tant disease. Despite management, MCC has an exception- ally increased tendency to recur locally (26–60%), invade [14] O. Bayrou,M.F.Avril,P.Charpentier,B.Caillou,J.C.Guil- regional lymph nodes (45–91%), and eventually metasta- laume, and M. Prade, “Primary neuroendocrine carcinoma of the skin. Clinicopathologic study of 18 cases,” Journal of the size distally (18–52%) to liver, lung, bone, brain, and skin. American Academy of Dermatology,vol.24, no.2,pp. 198–207, Hence, frequent short- and long-term followups are highly recommended. Broadly, MCC has a very poor prognosis, and [15] I.Hierro, A. Blanes,A.Matilla, S. Muno ˜ z, L. Vicioso, and F. 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