Case report

From the lymph nodes to the muscles: an unusual case of refractory familial Mediterranean classic Kaposi sarcoma Maria Ioannidou1, MD, Msc, Georgios Panayiotakopoulos2, MD, PhD, Dimosthenis Skarlos1, MD, PhD, Kalliopi Petraki3, MD, PhD, Nikolaos Doulas4, MD, Konstantinos Mileounis5, MD, and Christos Christodoulou1, MD, PhD

1 Department of Second Oncology, Metropolitan Hospital, 2Department of Infectious Diseases, Metropolitan Hospital, 3 Department of Pathology, Metropolitan Hospital, 4Department of Angiosurgery, Metropolitan Hospital, and 5Department of Dermatology, Metropolitan Hospital, Athens, Greece

Correspondence Maria Ioannidou, MD, Msc Ethnarhou Makariou & El. Venizelou 1, 18547 Neo Phaliro Greece E-mail: [email protected] Conflicts of interest: No conflicts of interest.

doi: 10.1111/ijd.12497

Case report A 59-year-old man was admitted to our department for the first time in March 2011 with left lower extremity edema, irregular-shaped skin patches, and enlargement of the left superficial inguinal lymph nodes. His family history revealed familial Mediterranean classic Kaposi sarcoma (CKS) on his mother’s side (HLA-DR5 positive). Subsequent blood sample analysis, polymerase chain reaction for human herpesvirus-8, was positive. Computed tomography of the chest and abdomen showed disseminated enlargement of the lymph nodes. The skin lesions were assessed by a dermatologist and were determined to be CKS lesions. Biopsies from the lymph nodes (axillary and inguinal) revealed infiltration of aggressive KS, with moderate nuclear atypia, high mitotic activity, presence of tumor necrosis, and immunophenotype with CD34(+), CD31(+), D2-40 focal expression, factor VIII( ), and human herpesvirus-8(+). The patient was treated with chemotherapy as per disseminated KS. In May 2011, he started first-line chemotherapy with liposomal doxorubicin, 20 mg/m2, every two weeks for five cycles. After receiving the first cycle of ª 2014 The International Society of Dermatology

chemotherapy, he demonstrated a significant decrease in the lymphedema of the lower extremity and of the left inguinal lymph nodes. After the fifth cycle of liposomal doxorubicin, the patient developed severe palmoplantar erythrodysesthesia. A month after receiving the fifth cycle of chemotherapy, he was readmitted with a progressive purple epidermal nodular lesion on the inside of the left thigh (Fig. 1). Biopsy of the skin lesion and underlying tissues to the muscle was performed. Histopathology revealed focal infiltration of the skin, subcutaneous fat, and striated muscle by spindle cell lesions, measuring 0.1–0.5 cm each, with morphological and immunophenotypical characteristics of KS (Fig. 2). Second-line chemotherapy with paclitaxel 80 mg/m2 weekly was initiated in August 2011. After the first dose of chemotherapy, he showed decrease in the lymphedema (Fig. 3). He received paclitaxel for 12 weeks. As a result, the lymphedema of the left lower extremity almost disappeared, and the computed tomography examinations showed elimination of the enlarged lymph nodes. He remained progression-free until April 2012, when he developed extensive KS-like skin lesions on the left thigh.

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Case report

Unusual case of Mediterranean CKS

Ioannidou et al.

(a)

Figure 2 Infiltration of the striated muscle by spindle cell lesions, with the typical characteristics of classic Kaposi sarcoma (hematoxylin and eosin stain, 920).

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Figure 1 Purple epidermal lesion of the internal area of the left thigh (a) and edema of the left lower extremity (b) a month after receiving the fifth cycle of chemotherapy.

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After receiving paclitaxel for eight weeks, he had an excellent response. Intending to consolidate the good chemotherapy response, he received electron beam radiotherapy to the left thigh (total 34 Gy for 17 fractions). In October 2012, the KS-like lesions reappeared on the left thigh, within and outside the irradiated area. He was treated with vinorelbine per os, but he had mixed response. Hence, in December 2012, he again received paclitaxel for 12 weeks, and he has had good response. At present, the patient remains asymptomatic. Discussion CKS is most often diagnosed in patients whose origin is from the Mediterranean basin, Middle East, or eastern Europe.1–3 In our case, CKS was initially diagnosed by International Journal of Dermatology 2014

Figure 3 Before (a) and after (b) receiving the second dose of weekly chemotherapy with paclitaxel, he had decrease of the lymphedema of the left lower extremity and progressive decolorization of the internal area of the left thigh. The incision of the biopsy is not infected. ª 2014 The International Society of Dermatology

Ioannidou et al.

lymph node biopsy. When recurrence was suspected, biopsy of the skin lesions and underlying tissues, including muscles, revealed infiltration of all of these tissues by aggressive CKS. No muscle infiltration of CKS was reported except for two cases of AIDS-associated KS.4 Our case is the first reported in the literature to present a CKS involving the underlying muscle. No optimal therapeutic strategies have been developed for cKS. Chemotherapy is generally applied in cases with disseminated, aggressive CKS and in CKS refractory to local therapy. Many physicians prefer pegylated liposomal doxorubicin as first-line chemotherapy.5 Concerning second-line chemotherapy, several small studies have evaluated taxanes as a treatment for aggressive or refractory life-threatening CKS.6 It is of note that paclitaxel given as a weekly schedule has antiangiogenic properties, in addition to its cytotoxic activity.7 References 1 Jakob L, Metzler G, Chen KM, et al. Non-AIDS associated Kaposis sarcoma: clinical features and treatment outcome. PLoS one 2011; 6: e18397.

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Unusual case of Mediterranean CKS

Case report

2 Ohe EM, Padihla MH, Enokihara MM, et al. Fatal outcome in classic Kaposis sarcoma. An Bras Dermatol 2010; 85: 375–379. 3 Hiatt KM, Nelson AM, Lichy JH, et al. Classic Kaposi sarcoma in the United States over the last two decades: a clinicopathologic and molecular study of 438 non-HIV-related Kaposi sarcoma patients with comparison to HIV-related Kaposi sarcoma. Mod Pathol 2008; 21: 572–582. 4 Caponetti G, Dezube BJ, Restrepo CS, et al. Kaposi sarcoma of the musculoskeletal system: a review of 66 patients. Cancer 2007; 109: 1040–1052. 5 Di Lorenzo G, Kreuter A, Di Trolio R, et al. Activity and safety of pegylated liposomal doxorubicin as first-line therapy in the treatment of non-visceral classic Kaposis sarcoma: a multicenter study. J Invest Dermatol 2008; 128: 1578–1580. 6 Brambilla L, Romanelli A, Bellinvia M, et al. Weekly paclitaxel for advanced aggressive classic Kaposi sarcoma: experience in 17 cases. Br J Dermatol 2008; 158: 1339– 1344. 7 Bocci G, Di Paolo A, Danesi R. The pharmacological bases of the antiangiogenic activity of paclitaxel. Angiogenesis 2013; 16: 481–492.

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From the lymph nodes to the muscles: an unusual case of refractory familial Mediterranean classic Kaposi sarcoma.

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