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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2013, Article ID 839618, 4 pages http://dx.doi.org/10.1155/2013/839618 Case Report Eitan R. Friedman, Lesley Farquharson, Jessica Warsch, Ran Huo, Clara Milikowski, and Margarita Llinas University of Miami Miller School of Medicine/Jackson Memorial Hospital, 1611 NW 12th Avenue, Central 600-D, Miami, FL 33136, USA Correspondence should be addressed to Eitan R. Friedman; firstname.lastname@example.org Received 23 April 2013; Accepted 14 May 2013 Academic Editors: R. Palmirotta, M. Ryberg, and K. Tanaka Copyright © 2013 Eitan R. Friedman 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. Kaposi’s sarcoma (KS) is a multicentric vascular neoplasm associated with the Kaposi’s sarcoma-associated herpes virus (KSHV). KS can occur in immunocompromised patients as well as certain populations in Africa or in the Mediterranean. Less than 5% of KS cases can present with lymphangioma-like kaposi sarcoma (LLKS), which can occur in all KS variants. KS presents with characteristic skin lesions that appear as brown, red, blue, or purple plaques and nodules. eTh lesions are initially flat and if untreated will become raised. LLKS presents similarly to KS but is associated with severe lymphedema and soft tissue swelling as well as bulla-like vascular lesions. We present the case of an 85-year-old Lebanese, HIV negative, man who presented with a swollen and painful right lower extremity accompanied by necrotic lesions. Wound cultures were positive, and we began the work-up for secondarily infected gangrene. However, skin biopsy results revealed that he in fact had lymphangioma-like Kaposi sarcoma, which allowed us to shift our management. Advanced Kaposi’s sarcoma can present similar to gangrene. It is important to recognize the typical skin lesions of KS and not to overlook Kaposi’s sarcoma or LLKS within the differential. 1. Introduction 2. Case Presentation Kaposi’s sarcoma is a vascular neoplasm that develops sec- We present the case of an 85-year-old Lebanese man with ondary to infection with the KSHV virus. The KSHV virus a history of coronary artery disease, diabetes, dyslipidemia, is also knownasthe humanherpesvirus 8(HHV8).KSis hypertension, and hypothyroidism who presented to our oen ft seen in people of African or Mediterranean descent institution with a reported 2-week history of right foot who have a genetic susceptibility or immunocompromised edema, erythema, pain, and crusted lesions over the dorsum patients such as those receiving chemotherapy or in those of the right foot. eTh patient was referred to our facility infected with the HIV virus. Lymphangioma-like Kaposi’s aer ft receiving 2 days of IV antibiotics at a hospital in sarcoma is a rare subtype that presents similar to KS but Jordan, where he had resided for the past several years. He is also associated with severe lymphedema and soft tissue presented to that institution aer ft noticing red “bubbles,” swelling and bulla-like vascular lesions. These skin lesions which burst and crusted over with subsequent pain and can be confused with other dermatologic ndin fi gs including swelling. He denied fevers and chills. On examination in gangrene, especially if the lesion becomes superinfected and the emergency room, the patient was noted to have weakly if the disease progresses without treatment. It is important to palpable pulses in the left lower extremity and nonpalpable differentiate between LLKS and gangrene so that the patient pulses in the right lower extremity. His right lower extremity receives the correct intervention. We report a case of LLKS was edematous with blue/purple discolored lesions as well that wasinitially diagnosedasgangrene. as areas of necrosis (Figure 1). The rest of his physical exam 2 Case Reports in Oncological Medicine (a) (b) (c) Figure 1: (a) Patient’s right lower leg is seen here demonstrating the lymphedema compared to the opposite left leg. (b) and (c) Picture of the patient’s right foot showing the blue/purple KS skin lesions as well as areas of necrosis. was benign with negative lymphadenopathy and lungs that dermatology. Unfortunately, the patient was lost to followup were clear to auscultation, as well as negative cardiac and aer ft discharge. abdominal exams. He was initially evaluated by vascular surgery for suspected gangrene but, on arterial ultrasound 3. Discussion with Doppler, was noted to have adequate ankle-brachial indices. Venous ultrasound with Doppler was also done to Kaposi’s Sarcoma is a multicentric vascular neoplasm-associ- rule out deep venous thrombosis and this study was negative. ated with the Kaposi’s sarcoma associated herpes virus. eTh FurtherevaluationwithX-raysaswellasmagneticresonance KSHV virus is also known as the human herpes virus 8. imaging (MRI) of the lower extremities was done to rule Studies have shown that KSHV-infected cells of vascular out possible osteomyelitis, given his comorbidities, and these endothelial origin secrete cytokines, chemokines, and growth studies were also negative. After wound cultures were done, factors that allow for angiogenesis and further cancer patho- the patient was started on broad-spectrum antibiotics for genesis . The KSHV viral genome encodes several viral suspected soft tissue infection. Punch biopsy was also done genes. One of the viral genes encodes for a constitutively to rule out fungal or parasite infection. Aeft r 4 days with no active intramembranous viral G-protein coupled receptor clinical improvement, he was taken to the operating room (vGPCR). This vGPCR is present in only a small number of for debridement. Pathology from that debridement revealed infected cells and causes a signaling cascade which allows Kaposi’s sarcoma, lymphangioma type with positive human for the downstream transcription of genes which encode herpes virus-8 (HHV8) immunostain. eTh biopsy, which for cytokines, chemokines, and growth factors which are was done prior to surgery, also confirmed this diagnosis. ultimately secreted from the cell and have paracrine eeff cts on Multiple environmental pathogens, as well as Enterococcus neighboring cells causing further cell proliferation and cancer faecalis,werenoted on woundculture andassuch, thepatient growth . completed 2 weeks of ampicillin/sulbactam. There are four types of KS seen clinically. es Th e include Giventhe diagnosisofKaposi’ssarcoma,HIV testingwas classic variant KS, endemic KS, transplant-associated KS, done, which was negative, and so no other tests for immuno- and AIDS-associated KS. Classic variant KS is slow growing, suppression were performed. eTh patient also underwent often limited to the skin, and often found in people of computed tomography (CT) scans of his chest, abdomen, and Mediterranean descent. Endemic KS is found in Africa in pelvis to evaluate for metastatic disease. es Th e studies were HIV negative patients. This form is aggressive and can aeff ct done withoutcontrastasthe patientdeveloped acutekidney children. Transplant-associated KS is seen in patients who injury during his hospitalization, likely as a consequence are on immunosuppressive therapy. The last type is AIDS- of supratherapeutic vancomycin. Lastly, the patient received associated KS, which can develop in HIV positive patients upper and lower endoscopies, both of which were negative . It is unknown why people of Mediterranean descent or for lesions suspicious for malignancy. As there was no of African descent are susceptible to the KSHV; however, it evidence of metastases on this evaluation, the decision was is known that HIV positive patients and patients who are made to provide local therapy only, under direction from on chronic immunosuppressive drugs have a compromised Case Reports in Oncological Medicine 3 (a) (b) (c) Figure 2: (a) Patient’s skin biopsy showing immunohistochemical stai n for HHV8. There is a strong nuclear reactivity in the endothelial cells as well as some of the stromal spindle cell component. (b) and (c) H and E staining of skin biopsy from the patient. Within the dermis are markedly dilated vascular spaces lined by prominent endothelial cells. Within the stroma are few spindle cells without apparent atypia. This represents the lymphangioma-like variant of Kaposi sarcoma. immune system which can make them susceptible to the Often these patients also histologically show patterns asso- KSHV and subsequently to the development of KS. u Th s, ciated with the other forms of KS. eTh se patterns include it can be deduced that the classic and endemic variants are spindle and endothelial cell proliferation, red blood cell caused by genetic susceptibilities to the KSHV. extravasation, hemosiderin-laden macrophages, and other Our patient is of Mediterranean descent and is HIV nega- signs of an inflammatory reaction [ 5]. In addition, biopsy tive,and theclassic variantofKSinthese patients tendstobe samples can be stained for immunohistochemistry with anti- more indolent in nature, these patients are oeft n elderly as was HHV8 antibodies in order to detect the KSHV in the tumors our patients and occurs in men more oeft n than women in a themselves, which can be detected in all four KS variants. 15 to 1ratio.Thecanceroeft nbeginsinthe handsorfeetand Figure 2 showsthe staining that wasperformed on our progresses towards the body, potentially taking several years patient’s biopsy sample. Figure 2(a) shows immunohisto- to decades before the cancer metastasizes to the viscera . chemical staining for HHV8, and Figures 2(b) and 2(c) show KS most oeft n metastasizes to lymph nodes, the gastrointesti- the classic lymphangioma-like spaces. nal tract, and to the lungs . The lesions begin as multiple Treatment of KS includes simple excision of a single lesion firm reddish/brown or purple/blue plaques and nodules [ 4]. and a combination of surgery, radiation, and chemotherapy eTh lesions are initially flat and if left untreated will become for multiple and metastasized KS. conu fl ent and raised. Although of Mediterranean descent, Herein we describe a patient with an extremity lesion our patient had a rare type of KS known as lymphangioma- that was initially believed to be a necrotic gangrenous foot. like KS, which can occur in each of the four KS variants and is Cultures from his left foot biopsy from one of his lesions grew less than 5% of all KS cases . eTh se patients usually present Gram-positive cocci and Gram-negative rods. We began the similar to KS, with blue/purple solid lesions; however, our patient on systemic broad-spectrum antibiotics to treat his patient also had severe lymphedema and soft tissue swelling superinfected gangrenous foot. It was only aeft r a biopsy was as well as bulla-like vascular lesions characteristic of LLKS attained and histological and immunohistochemical exam- . Bulla-like lesions are the most common clinical feature ination was performed that we discovered that the patient seen in LLKS. Also, LLKS usually presents in the lower had LLKS and it was at that time when the oncology team extremities, as was the case in our patient . was able to begin management with chemotherapy, radiation, The diagnosis of LLKS can be made based on clinical and surgery. It is important to recognize the typical skin presentation and on histological examination of the lesions. lesionsofKSand nottooverlookKaposi’ssarcoma within the LLKS is typified histologically by lymphangioma-like spaces. differential of a patient who presented as ours did, especially 4 Case Reports in Oncological Medicine in a patient who is of Mediterranean descent, elderly, and HIV negative. References  B. C. Jham, T. Ma, J. Hu et al., “Amplification of the angiogenic signal through the activation of the TSC/mTOR/HIF axis by the KSHV vGPCR in Kaposi’s sarcoma,” PLoS ONE,vol.6,no. 4, Article ID e19103, 2011.  T. Ma, B. C. Jham, J. Hu et al., “Viral G protein-coupled receptor up-regulates angiopoietin-like 4 promoting angiogenesis and vascular permeability in Kaposi’s sarcoma,” Proceedings of the National Academy of Sciences of the United States of America, vol. 107, no. 32, pp. 14363–14368, 2010.  D.P.Dittmer,K.L.Richards, andB.Damainia, “Treatment of Kaposi sarcoma-associated herpesvirus-associated cancers,” Frontiers in Microbiology, vol. 3, article 141, 2012.  K. Antman and Y. Chang, “Kaposi’s sarcoma,” The New England Journal of Medicine,vol.342,no. 14,pp. 1027–1038, 2000.  A. S. Fauci and H. C. Lane, “Human immunodeficiency virus disease: AIDS and related disorders,” in Harrison’s Principles of Internal Medicine,A.S.Fauci,E.Braunwald,D.L.Kasperetal., Eds.,vol.17, pp.1086–1087,McGraw-Hill,New York,NY, USA, 17th edition, 2008.  P. C. Garcia, A. Garcia-Cruz, I. Garcia-Doval, C. de la Torre, and M. J. Cruces, “Lymphangioma-like Kaposi sarcoma,” Dermatol- ogy Online Journal,vol.15, no.9,2009.  J. A. Ramirez, W. B. Laskin, and J. Guitart, “Lymphangioma-like Kaposi sarcoma,” JournalofCutaneous Pathology,vol.32, no.4, pp.286–292,2005.  S.Mohanna,J.Sanchez,J.C.Ferrufino,F.Bravo,andE.Gotuzzo, “Lymphangioma-like Kaposi’s sarcoma: report of four cases and review,” Journalofthe European AcademyofDermatology and Venereology,vol.20, no.8,pp. 1010–1011, 2006. 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Case Reports in Oncological Medicine – Hindawi Publishing Corporation
Published: May 25, 2013
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