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Angiosarcomas developing in unusual sites such as the small bowel are rare, and fewer than 65 cases have been reported in the literature. They are not uncommonly associated with a known eliciting factor. Thus, among hitherto described cases of angiosarcoma of the small bowel, 16 were radiation-induced. One additional example of ileal post-irradiation angiosarcoma (PRA) in a 72-year-old female patient with a past history of uterine leiomyosarcoma is herein reported as a reminder of this causal association. The morphologic and immunohistochemical clues leading to the correct diagnosis of PRA of the small bowel and the differential diagnostic problems are discussed; a comprehensive review of the literature has also been performed with a focus on survival., (Copyright © 2020 Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.)

Ossifying fibromyxoid tumor of soft tissues (OFMT) is considered a rare mesenchymal neoplasm. Its main histological features are sheets and ill-defined lobules of rounded bland cells within a fibromyxoid background and a thick collagenous capsule with an incomplete rim of lamellar bone. This lesion occurs mostly in the soft tissues of the lower extremities and limb girdles. In this paper, we describe a mesenchymal tumor removed from the right thigh of a 41 year-old-woman. The neoplasm differed histologically from typical forms of OFMT for areas of moderate cellularity and atypia, nuclear enlargement and small nucleoli. Focally, stromal tongues of osteoid were centrally and irregularly located within the lesion with evident spindling of tumor cells around them. The mitotic activity was low (up to 19 per 50 HPF) and atypical figures were rarely seen. The tumor was positive to S-100 protein, vimentin, CD10, CD56, CD99, ASMA, calponin and collagen IV. Rare elements were positive for cytokeratin AE1/AE3. To the best of our knowledge, this is the first case of atypical OFMT reported to be positive for calponin. The patient is currently alive and well with no evidence of disease at 96 months following surgery. In spite of low-grade histology, OFMT has high local recurrence rate and low metastatic potential, primarily in the lungs, even several years after surgical removal. The recognition of this entity is important. In this report the authors address differential diagnosis and enigmatic histogenesis of this neoplasm.

The Authors describe a rare case of cutaneous angiolipoleiomyoma in an acral location together with a brief literature review. A 62-year-old male presented with a slow-growing asymptomatic nodule, 2.2 cm in diameter, located in the subcutaneous tissue of the left calf. Ten months after surgical excision, the patient is alive and free of disease. Histologically, the lesion was well-circumscribed and contained three components: areas of mature fat tissue were intermingled with cellular areas of spindle eosinophilic cells, reminiscent of smooth muscle cells, and a complex mixture of vessels of different types and sizes. Immunohistochemically, the cellular spindle component was positive for vimentin and smooth muscle actin, and negative for S-100, HMB-45, MART-1 and oestrogen and progesterone receptors. The Authors discuss differential diagnosis with other benign lesions such as angioleiomyoma and subcutaneous angiomyolipoma.

We describe a rare case of myopericytoma-type perivascular myoma (MTPM) which arose in acral location and the literature on this field is briefly reviewed. The patient, a 68-year-old man, presented with an enlarging painful nodule, 1.4 cm across, located in the subcutaneous tissue of the right foot. The patient is alive and well 17 months after surgical excision of the nodule. Microscopically, the lesion was well-circumscribed and characterized by a biphasic pattern with a concentric perivascular spindle and ovoid cell proliferation and an extensive hemangiopericytomatous growth component. Immunohistochemically, the neoplastic cells were positive for vimentin, smooth muscle actin, desmin and calponin, negative for S-100 protein, CD34, CD31 and cytokeratins (AE1/AE3, Cam 5.2). Electron microscopy showed electrondense cell bodies suggestive for myopericytic differentiation. The clinico-pathological features of the present case are similar to those previously reported in the literature and we also discuss herein the main histological criteria for the differential diagnosis with other spindle cell and vascular lesions of soft tissues.

A case of ganglioneuroma of parapharyngeal space is described and the literature on this field is briefly reviewed. The patient, a 7-year-old female, presented with a left parapharyngeal space mass causing medial displacement of the left lateral pharyngeal wall. The lesion sized 4.4 cm in the largest diameter. Histologically, the tumour showed the coexistence of two distinct cells types--ganglion cells and Schwann cells--within a loose stroma. Immunohistochemically, both cell types were immunoreactive for S-100 protein and negative for cytokeratins.