© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd

J Cutan Pathol 2014: 41: 680–685 doi: 10.1111/cup.12329 John Wiley & Sons. Printed in Singapore

Journal of Cutaneous Pathology

Cutaneous scalp metastases from retroperitoneal leiomyosarcoma: a case report A 71-year-old woman presented with five scalp nodules that were clinically suspicious for pilar cysts. Histopathologic examination showed a proliferation of mitotically active pleomorphic spindle cells arranged into intersecting fascicles in the dermis and subcutis. Tumor cells displayed deeply eosinophilic cytoplasm and expressed desmin but were negative for S100 protein by immunohistochemistry. Before 10 years, the patient was diagnosed with high-grade retroperitoneal leiomyosarcoma and underwent resection with intraoperative radiation. Metastatic disease involving the lungs, liver and soft tissue developed, requiring treatment with resections, radiation and chemotherapy. Owing to the presentation of multiple scalp nodules with microscopic features of leiomyosarcoma in conjunction with the clinical history of retroperitoneal leiomyosarcoma, a diagnosis of metastatic leiomyosarcoma was made. Scalp metastasis from retroperitoneal leiomyosarcoma is extremely rare and portends a poor prognosis. To our knowledge, only two other cases have been reported in the English literature, and a further search discovered only nine additional cases of scalp metastasis from soft tissue leiomyosarcoma of any non-gynecologic anatomic site. This case highlights the striking microscopic similarity between primary cutaneous and metastatic leiomyosarcoma and illustrates the necessity of adequate clinical information and an appropriate index of suspicion in excluding the possibility of cutaneous metastases of leiomyosarcoma from somatic soft tissue. Keywords: cutaneous metastasis, leiomyosarcoma, scalp, skin tumors Baltz RG, Kaley JR, Hull CA, Gardner JM. Cutaneous scalp metastases from retroperitoneal leiomyosarcoma: a case report. J Cutan Pathol 2014; 41: 680–685. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd

Leiomyosarcoma is a rare smooth muscle cell malignancy with an annual incidence of less than two per million people.1 The most common origins of primary leiomyosarcoma include gynecologic, gastrointestinal and somatic soft tissue, particularly that of the retroperitoneum.1 It may also arise as a primary tumor of the skin and subcutis. Usually the route of metastasis is hematogenous, with the

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Rebekah G. Baltz1 , Jennifer R. Kaley2 , Cheryl A. Hull3 and Jerad M. Gardner2 1

College of Medicine, University of Arkansas for Medical Sciences, Little Rock, AR, USA, 2 Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA, and 3 Hull Dermatology, PA, Rogers, AR, USA

Jerad M. Gardner, MD, Department of Pathology, University of Arkansas for Medical Sciences, 4301 West Markham St., #517, Little Rock, AR 72205, USA Tel: +1 501 6868884 Fax: +1 501 2961184 e-mail: [email protected]; Twitter:@JMGardnerMD Accepted for publication March 3, 2014

lung, kidney and liver being the most common sites; however, cutaneous metastases also occur but are very rare, usually arise late in the course of the disease, and demonstrate a poor prognosis.1,2 In this report, we present an unusual case of retroperitoneal leiomyosarcoma with multiple locations of metastases occurring throughout the disease course, the most recent being cutaneous scalp metastases.

Leiomyosarcoma scalp metastases

Fig. 1. Cutaneous metastasis of leiomyosarcoma presented clinically as smooth-surfaced papules on the scalp.

Fig. 2. Cutaneous metastasis of leiomyosarcoma presented clinically as smooth-surfaced papules on the scalp.

Case report A 71-year-old woman presented to dermatology with a 1 month history of five lesions at various locations on her scalp. Clinically, the lesions were described as skin-colored nodules ranging in size from 3 to 6 mm and resembling pilar cysts (Figs. 1 and 2). The nodules were not pruritic but were mildly tender to firm palpation. Prior to seeking treatment she had tried various remedies such as changing shampoos, but eventually became concerned that the nodules could be metastatic from a primary retroperitoneal leiomyosarcoma diagnosed over 10 years earlier. Ten years prior to her current presentation, the patient presented with a 5.9 × 4.0 × 1.8 cm mass in the medial aspect of the lower half of the right kidney. Histopathologic examination of a biopsy specimen revealed high-grade leiomyosarcoma. She

underwent pre-operative chemotherapy and radiation before resection, and intraoperative radiation was performed. On resection, the tumor appeared to arise from peri-renal soft tissue, as no invasion of the renal cortex, ureter, renal artery or inferior vena cava was identified. Nine months after resection, lung metastases were identified. Over the 10 years that followed, the patient had five thoracotomies for lung metastasectomy along with several surgeries for removal of various other leiomyosarcoma metastases including a right hepatectomy and resection of soft tissue nodules of the medial right knee, right chest wall and left buttocks. She received a variety of regimens of chemotherapy and radiation during this period. At the time of presentation for the scalp nodules, she was undergoing chemotherapy (Nexavar and Femara) and had stable lung metastases and nodules in the right hip and thigh. Owing to her history of primary leiomyosarcoma and an appropriate degree of clinical suspicion, she underwent excision of three of the five scalp nodules. Microscopically, all three punch biopsies had nearly identical features and displayed a nodular proliferation of markedly atypical pleomorphic spindle cells within the subcutis. These nodules were well circumscribed, with smooth rather than infiltrative borders, and were centered in the subcutis with only minimal involvement of the deep dermis. The tumor cells showed marked nuclear atypia with increased mitoses including atypical mitotic figures. The cytoplasm was deeply eosinophilic, and the spindle cells were arranged into intersecting fascicles. Central tumor necrosis was identified within one of the nodules (Figs. 3–5). The tumor cells strongly and diffusely expressed desmin (Fig. 6) but were negative for S100 protein by immunohistochemistry. The pathologic features of the scalp nodules, along with the clinical history of retroperitoneal leiomyosarcoma with subsequent distant metastases elsewhere, were all consistent with a diagnosis of metastatic leiomyosarcoma. At her 3-month follow-up after biopsy of the initial scalp nodules, the patient noted two new slow-growing scalp nodules similar to the previous nodules; these were not biopsied. Further care was provided by oncology including administration of zoledronic acid every 3 months. The patient’s internal metastases continued to progress despite chemotherapy, particularly multiple spine metastases for which she received palliative radiation. Interestingly, her scalp nodules did not progress, and no new skin lesions arose. Chemotherapy was discontinued 10 months after the initial scalp biopsies, and the patient is currently alive with extensive disease but receiving no further treatment aside from hospice care.

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Fig. 3. Cutaneous metastasis of leiomyosarcoma presented histopathologically as a nodular proliferation of eosinophilic spindle cells in the deep dermis/subcutis. The findings are nearly identical to primary cutaneous leiomyosarcoma. Hematoxylin & eosin, original magnification ×14.

Discussion Cutaneous metastases are infrequent overall when compared with other sites of metastases. Reports of cutaneous metastasis range from 0.7 to 9% of patients with cancer.3 Tumors may metastasize to cutaneous tissue by either lymphatic or hematogenous spread, with distant cutaneous metastases most commonly resulting from spread via hematogenous dissemination.1 Cutaneous metastases usually signify advanced disease with a poor prognosis.4 Survival from time of diagnosis of cutaneous metastasis averages from 3 to 12 months, with a graver prognosis with multiple vs. single skin metastases.1 Typically cutaneous metastases are diagnosed a period of time after the initial diagnosis of a primary malignancy; however, on occasion detection is made simultaneously or before the diagnosis of a primary.4 An appropriate level of suspicion should be maintained while evaluating cutaneous lesions, as cutaneous metastases may manifest in a variety of ways, including ulcerations, cellulitis-like lesions,

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Fig. 4. Another cutaneous metastasis of leiomyosarcoma in the same patient. Central necrosis is evident. Hematoxylin & eosin, original magnification ×9.

bullae or fibrotic processes, and most typically as painless firm nodules under an intact and normalappearing epidermis.1,2 As such lesions may be benign-appearing and even asymptomatic, patients and physicians may delay appropriate care. Carcinomas account for the majority of cutaneous metastases. Cutaneous metastasis of sarcomas is a very rare occurrence, with all types of sarcomas accounting for only 2–3% of all metastatic skin lesions.3,5 Various studies have reported a range of

Cutaneous scalp metastases from retroperitoneal leiomyosarcoma: a case report.

A 71-year-old woman presented with five scalp nodules that were clinically suspicious for pilar cysts. Histopathologic examination showed a proliferat...
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