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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2013, Article ID 131695, 3 pages http://dx.doi.org/10.1155/2013/131695 Case Report Merkel Cell Carcinoma of the Retroperitoneum with No Identifiable Primary Site Daniele Rossini, Salvatore Caponnetto, Vittoria Lapadula, Lucilla De Filippis, Gabriella Del Bene, Alessandra Emiliani, and Flavia Longo Department of Clinical Oncology A, Sapienza University of Rome, Policlinico Umberto Primo, Viale Regina Elena 324, 00161 Rome, Italy Correspondence should be addressed to Daniele Rossini; email@example.com Received 24 May 2013; Accepted 1 August 2013 Academic Editors: B. I. Razzouk and D. Yin Copyright © 2013 Daniele Rossini 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 extremely rare primary neuroendocrine neoplasm of the skin that shows aggressive behavior and a poor prognosis. We report a case of a 67-year-old male with a Merkel cell carcinoma which initially presented itself as a large retroperitoneal mass. Pathological and immunohistochemical analysis revealed tissue consistent with neuroendocrine carcinoma. Despite complete medical workup, no other primary MCC could be detected. While being an atypical presentation, the tumor mass showed an excellent response to the combination of chemotherapy followed by radiotherapy. 1. Introduction example, according to Albores-Saavedra et al. , the 10-year relative survival rate of localized stage MCC is 71%, whereas Merkel cell carcinoma (MCC) is an aggressive neuroen- the 10-year relative survival rates for regional and distant docrine malignancy arising from the skin, with estimated stages are 47.8% and 20.1%, respectively . incidence of 0.6 per 100,000 person/years . The incidence Most Merkel cell carcinomas develop within the skin of appearstobeonthe rise,due likely to improved diagnos- the head and neck or on the extremities. In a review of 661 tic techniques, increased exposure to known risk factors, cases conducted in 2000, only 2 percent presented with no immunosuppression, prolonged sun exposure, and a history apparent primary lesion . of prior malignancy [2–4]. The natural history of MCC is unpredictable, ranging from a localized indolent course to a regionally more aggres- 2. Case Presentation sive course with widespread metastases. Several large reviews document the development of local recurrence in 25–30% of A67-year-old man, with ahistory of hypertension,obe- all cases of MCC, regional disease in 52–59% of all cases, and sity, hypercholesterolemia, and paroxysmal tachycardia, pre- distant metastatic disease in 34–36% of all cases [5, 6]. sentedwithafirmandpalpablemassintherightinguinalarea On examination, the clinician should focus on the lym- without any skin lesions. The ultrasound findings showed a phatic and integumentary systems . When symptoms massive and well-circumscribed nodular hypoechoic lymph lead to suspicion of recurrence, appropriate imaging studies node formation with a diameter of 4.7× 3.5 cm. A second should be performed. The aggressive nature of this disease similar formation, large 3.8 × 3.1 cm, was at the root of necessitates frequent followup, and the presence of risk fac- thethigh.Anexcisionbiopsywas performed: focalareas tors including tumors larger than 2 cm, truncal location, male due to residual nodal structure, site of metastases from sex, age over 65, nodal or distant disease at presentation, and malignantneoplasmcomposedofnests,cords,and sheets duration of disease before presentation  should determine of elements of medium size with rounded nuclei, finely the appropriate frequency. Also, in this type of tumor, the dispersed chromatin and scant cytoplasm. In the tissue there stage of disease stratiefi s the rate of survival groups. For is also the presence of large necrotic areas with images 2 Case Reports in Oncological Medicine (a) (b) (c) Figure 1: CT scan of pelvis showing right mass at diagnosis, aer ft -chemotherapy and aer ft -radiotherapy. of vascular invasion. Phenotype of neoplastic cells was as Radiotherapy on the right abdominal inguinal region follows: panCK (MNF116) + (do-like), CK20 + (dot-like), (200 cGy/day for 5 days/week) + boost on the previous area neurofilament + (dot-like), synaptophysin +/ −,NCAM+/−, neoplastic to a total dose of 68 Gy was then performed. chromogranin−,CK7−,CD99−, TTF1−,and CD45−.These Areevaluationwithtotal-bodyCT(Figure 1)showed findings were consistent with the diagnosis of a metastatic a reduction in size of the pathological lymphadenopathy MCC. A subsequent CT Whole Body scan (Figure 1)showed previously reported in the right iliac (2.2 versus 3 cm). The at right common iliac level a new formation of pathological lymph node described at the root of the right thigh was tissue with irregular margins, with markedly heterogeneous thinner and had a lower contrast enhancement compared to contrast enhancement and areas of central necrosis of 45 mm the previous. The edematous thickening of adipose tissue and which has a longitudinal extent of about 67 mm. This lesion contextual small lymphadenopathy was also reduced. appears inseparable from the psoas muscle and the ipsilateral iliac vessels. At the height of the thigh, a package of 83× 3. Discussion 60 mm was observed which was associated with marked thickening of adipose tissue around the wound, edema, and According to the large population study by Albores-Saavedra multiple other small lymphadenopathies. Here, there was et al.ofthe majority,(97.6%) casesofMCC occurred in the also a left inguinal lymph node of probable nonspecific skin. Among the less common sites are the external surface of appearance of 20 mm. Thickening of adipose tissue combines the lip (0.4%), the parotid gland (0.4%), the submandibular multiple mesenteric lymph nodes of 15 mm in diameter, while gland (0.1%), the nasal cavity (0.1%) and the lymph nodes, other lymph nodes were in the para-aortic area. vulva, vagina, and esophagus (<0.1%) . Given the results obtained and the clinical state of the As far as we know, in the literature, there are rare and patient, it was decided to start treatment according to the occasional cases of Merkel cell tumors that originate from following schema: carboplatin (CBDCA) (300 mg/m (2) of the retroperitoneum [7, 9], but strong suspicion remains that AUC 5 on day 1) and etoposide (VP-16) (100 mg/m (2) on these are the expressions of a metastasized tumor whose days 1–3) every 3 weeks for a total of six cycles. After 3 doses, primitive declined. a new reevaluation with CT was performed: the pathological We have to remember in fact that it is difficult to lymphadenopathy reported markedly reduced in any size in diagnose MCCinalymphnodedue to itssimilaritytoother the right iliac between the psoas muscle and the iliac vessels, poorly differentiated small basophilic cell tumors, including in the surgical scar in the groin (30 mm versus 45 mm), metastatic melanoma, lymphoma, small cell carcinoma, and andatlevel of therootofthe rightthigh (46 × 25 mm, neuroendocrine carcinoma metastatic from other organs prev. 83 mm× 60 mm). Also, the edematous thickening of . Immunohistochemistry is necessary for the den fi itive adiposetissueappearedmodestlydecreased,and thesmall diagnosis. In this case, the tumor cells were positive for cytok- lymphadenopathy context was reduced in number and size. eratin 20 and CD56, which demonstrated both epithelial and eTh posttreatment CT of total body ( Figure 1)showedin neuroendocrine features. eTh histologic diagnosis was also the abdomen/pelvis a reduction in the size of the patholog- aided by the finding of a dot-like pattern with CK 20 and CK ical lymphadenopathy reported previously in the right iliac 7 stainings. betweenthe muscle psoasand iliacvessels (19versus21mm) The presentation of this abnormal neoplasia can be and at the level of the root of the right thigh (versus 26× 16. explained according to two main justifications, in agreement 46× 25 mm); localized lymphadenopathy in the groin at the with what has already been proposed by Boghossian et al. surgical scar was unchanged. eTh edematous thickening of : the mass could be the expression of a replacement adipose tissue and small contextual lymphadenopathy at this of alymph node with aprimitive neoplasia, or an initial level was further reduced. skin lesion could have spontaneously regressed leaving the Given the results obtained, a surgical evaluation was retroperitonealmassassinglesiteofmetastasis. Athird requested which excluded the surgery for cardiac comorbidi- hypothesis, although anecdotal, is explained in some studies ties. that suggest a possible onset of Merkel cell tumor in the Case Reports in Oncological Medicine 3 subcutaneous tissue [11, 12]. It is however interesting to  T.I.Tarantola,L.A.Vallow,M.Y.Halyardetal.etal.,“Unknown primary Merkel cell carcinoma: 23 new cases and a review,” note that our patient, despite the absence of a history of Journal of the American Academy of Dermatology,vol.68, no. immunosuppression and a presentation not compatible with 3, pp.433–440,2013. sun exposure, makes use of statins. The role of these drugs  E. J. Kim, H. S. Kim, H. O. Kim et al., “Merkel cell carcinoma seems controversial, but there are lines of evidence of a of the inguinal lymph node with an unknown primary site,” possible inu fl ence [ 13]. Journal of Dermatology,vol.36, no.3,pp. 170–173, 2009. According to the NCCN guidelines the use of chemother-  T. Gambichler, S. Kobus, A. Kreuter, U. Wieland, and M. apy with or without surgery and/or radiation therapy is indi- Stuc ¨ ker, “Primary merkel cell carcinoma clinically presenting catedfor stageIV, distantmetastaticdisease (M1) . The as deep oedematous mass of the groin,” European Journal of most common regimen used for regional disease is cisplatin Medical Research,vol.15, no.6,pp. 274–276, 2010. or carboplatin with or without etoposide . Although com- ´ ´ ´  S. Sanchez-Garcıa, C. Manzanares-Campillo, P. Menendez- bined treatment with radiotherapy and surgery has a better Sanc ´ hez, V. Muno ˜ z-Atienza, and J. Mart´ın-Fernandez, ´ “Merkel impact with significantly lower rates of local and regional cell carcinoma: case report and literature review,” Cirugia y recurrence of MCC than surgery alone , in the presented Cirujanos,vol.80, no.1,pp. 63–66, 2012. case,itwasnotpossibletoassociatethetreatmentsasrequired  H. Sahi, V. Koljonen, T. Bohlin ¨ g et al., “Increased incidence due to the history of paroxysmal tachycardia. Combination of Merkel cell carcinoma among younger statin users,” Cancer of carboplatin and etoposide followed by radiotherapy has Epidemiology,vol.36, no.5,pp. 421–424, 2012. shown, however, eeff ctive in inducing a reduction of the  National Comprehensive Cancer Network, “National Compre- tumor mass with an acceptable toxicological profile. hensive Cancer Network (NCCN) Clinical Practice Guide- In the situation described, it clearly emerges, albeit in a lines in Oncology: Merkel Cell Carcinoma. Version 1,” 2013, unusual presentation, that the treatment of Merkel cell cancer http://www.nccn.org/ . may benefit from systemic chemotherapy based on cisplatin  D. Pectasides, M. Pectasides, A. Psyrri et al., “Cisplatin-based or carboplatin plus etoposide combined with radiation ther- chemotherapy for Merkel cell carcinoma of the skin,” Cancer apy. Investigation,vol.24, no.8,pp. 780–785, 2006. Clearly, as there is yet no certainty about the best  P. Ghadjar, J. H. Kaanders, P. Poortmans et al., “eTh essential approach in Merkel cell tumors, further studies are needed to role of radiotherapy in the treatment of Merkel cell carcinoma: investigate the issue of unusual presentations of this cancer. astudy from therarecancer network,” International Journal of Radiation Oncology Biology Physics,vol.81, no.4,pp. e583–e591, References  J. Albores-Saavedra, K. Batich, F. Chable-Montero, N. Sagy, A. M. Schwartz, and D. E. Henson, “Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study,” Journal of Cutaneous Pathology,vol. 37,no. 1, pp.20–27,2010.  E.Ramahi, J. Choi,C.D.Fuller,and T. Y. Eng, “Merkelcell carcinoma,” American Journal of Clinical Oncology,vol.36, no. 3, pp. 299–309, 2013.  M. Agelli and L. X. Clegg, “Epidemiology of primary Merkel cell carcinoma in the United States,” Journal of the American Academy of Dermatology,vol.49, no.5,pp. 832–841, 2003.  J.A.Santamaria-Barria, G. M. Boland,B.Y.Yeap, V. Nardi, D. Dias-Santagata, and J. C. Cusack, “Merkel cell carcinoma: 30- year experience from a single institution,” Annals of Surgical Oncology,vol.20, no.4,pp. 1365–1373, 2013.  S. Akhtar, K. K. Oza, and J. Wright, “Merkel cell carcinoma: report of 10 cases and review of the literature,” Journal of the American Academy of Dermatology,vol.43, no.5,pp. 755–767,  H.Medina-Franco,M.M.Urist,J.Fiveash,M.J.Heslin, K. I. Bland, and S. W. Beenken, “Multimodality treatment of merkel cell carcinoma: case series and literature review of 1024 cases,” Annals of Surgical Oncology,vol.8,no. 3, pp.204–208,2001.  V.Boghossian, I. D. Owen,B.Nuli, andP.Q.Xiao, “Neuroen- docrine (Merkel cell) carcinoma of the retroperitoneum with no identifiable primary site,” World Journal of Surgical Oncology, vol. 5, article 117, 2007.  P.T.H.Tai,E.Yu, J. Tonita,and J. Gilchrist, “Merkelcell carcinoma of the skin,” Journal of Cutaneous Medicine and Surgery,vol.4,no. 4, pp.186–195,2000. 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