Case Study D: Evaluation of Multiple Merkel Cell Carcinomas in a Single Patient

A 69-year-old otherwise healthy Caucasian woman was evaluated for a small, asymptomatic, erythematous papule on her right infraorbital cheek. After 4 months, she developed a second red plaque on her left distal medial calf. Her past medical history includ

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Iris Ahronowitz and Siegrid S. Yu

A 69-year-old otherwise healthy Caucasian woman was evaluated for a small, asymptomatic, erythematous papule on her right infraorbital cheek. After 4 months, she developed a second red plaque on her left distal medial calf. Her past medical history included a left arm squamous cell carcinoma excised 5 years prior, hypertension, and dyslipidemia. She had no history of immunosuppression. Medications included lisinopril, hydrochlorothiazide, and lovastatin. She had a family history of a grandmother with melanoma and a grandfather with bladder carcinoma. By the time of her initial presentation for care (9 months after she first noted the cheek lesion), the right infraorbital lesion had grown to a domeshaped nodule approximately 3 × 3 cm in size. The patient had no lymphadenopathy on exam. The nodule was excised, and histopathology showed small malignant cells with hyperchromatic nuclei and scant cytoplasm (typical of neuroendocrine carcinoma) distributed diffusely in the dermis and in subjacent soft tissue and skeletal muscle. Immunohistochemical stains for I. Ahronowitz Department of Dermatology, University of California San Francisco, 1701 Divisadero Street, Third Floor, San Francisco, CA 94115, USA e-mail: [email protected] S.S. Yu (*) Department of Clinical Dermatology, UCSF Dermatologic Surgery & Laser Center, 1701 Divisadero Street, Third Floor, San Francisco, CA 94115-0316, USA e-mail: [email protected]

neurofilament and CK20 antibody, chromogranin, and synaptophysin were positive, and MART1, TTF-1 and S-100, CD20, CD45, MP63, and factor I were all negative, effectively eliminating the possibility of metastasis of a visceral neuroendocrine tumor or melanoma. The patient presented for further evaluation 2 weeks after her initial excision. At this time, the left leg nodule was noted to have increased in size to 2.2 cm. The leg lesion was biopsied, with pathology demonstrating irregularly shaped masses of neoplastic cells in the dermis (including many mitotic figures and necrotic cells) with scant cytoplasm and large round nuclei with inconspicuous nucleoli. Perinuclear dot-like expression of Cam 5.2 and CK20, positivity for neuron-specific enolase, and negativity for S100 and TTF-1 were demonstrated by immunostaining, confirming again the diagnosis of MCC. In light of the near-synchronous appearance of her right cheek and left leg nodules, our multidisciplinary tumor board felt that her presentation was suggestive of two distinct primary tumors. Therefore wide local excision and sentinel lymph node biopsy of both cheek and leg lesions was recommended. Preoperative full-body FDG-PET/ CT demonstrated positive uptake at the site of the tumor of the left lower extremity, but no evidence of other metabolically active distant metastatic disease. The cheek lesion underwent wide local re-excision, which was found to be free of residual carcinoma. Right parotid sentinel lymph node biopsy showed a single negative node. The calf lesion also underwent wide local excision and

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