A lipomatous variant of scrotal angiomyofibroblastoma – A case report

Abstract

Rosai-Dorfman disease is a rare non-malignant proliferation of histiocytes typically involving the cervical lymph nodes. A small subset of cases present with extranodal manifestations. Case series and individual case reports discuss occurrences of Rosai-Dorfman disease in virtually every anatomical site. We discuss a case of a 63-year-old male presenting with multiple raised nodular lesions on multiple cutaneous sites. Biopsy of these lesions ultimately suggested a diagnosis of cutaneous extranodal Rosai-Dorfman disease. We discuss the histopathological, clinical and immunohistochemical features that allowed for this diagnosis. This is a rare pathological diagnosis with variable manifestations. Clinical suspicion and recognition of its classical histological features are essential for correct diagnosis, and in avoiding unnecessary treatment. References 1. Komaragiri M, Sparber L, Dardik M. Extranodal Rosai-Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma. World J Surg Oncol 2013; 11: 63. 2. Mantilla J, Goldberg-Stein S, Wang Y. Extranodal Rosai-Dorfman disease: clinicopathologic series of 10 patients with radiologic correlation and review of the literature.Salivary gland neoplasia is a challenging area of diagnostic surgical pathology due to significant histomorphologic overlap between benign and malignant entities with the resultant risk of misdiagnosis and inappropriate treatment. Clear cell carcinoma is a rare infiltrative, low grade salivary gland neoplasm with an indolent clinical course, little metastatic potential and a propensity for local recurrence. This case reports a 32-year-old woman who presented with a 1year history of a mass on her hard palate. The clinical history, histomorphologic, immunophenotypic and molecular features are described and discussed with emphasis on diagnostic pitfalls.Angiomyofibroblastoma-like tumour of the male genital tract is a rare benign mesenchymal tumour arising in the scrotum, perineum or inguinal region. It is usually seen in females of reproductive age. Clinical presentation has no pathgnomic findings. In the largest series to date, Iwasa and Fletcher 1 reported 25 cases in men with the mean age being 52 years. This case report is of an 18-year-old male with a scrotal angiomyofibroblastoma that was 35 cm in maximum dimension, in the paratesticular tissue separate from testicular parenchyma. Macroscopically, the lesion was a fibrous, fatty and mucoid mass. Histologically, it showed a lobulated pattern of epithelioid and spindle cells arranged around various calibre vessels.Other differential diagnoses including myxoid liposarcoma and rhabdomyosarcoma were ruled out with the help of FISH studies for FOX 10 and PAX3. DDIT3 and FUS were negative. To date, in all open-access literature, there has only been 34 cases reported of this rare tumour, of which there is only one reported case <20 years of age; this will be the second case in the <20 age group in men. The poster will describe the clinical presentation, imaging, macroscopic findings, histology ...