DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.

Tuesday, December 29, 2009

Answer of Dermatopathology Case 26


Visit: Pathology of Glomus Tumour
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Sclerotic Glomus Tumor. Am J Dermatopathol. 2009 Oct 30.
We report an unusual histopathological variant of a glomus tumor that arose in a peculiar topographic site, a sclerotic glomus tumor. Unlike conventional glomus tumors or glomangiomas that have a loose fibrous stroma with variable hyaline and myxoid changes, the case reported herein had a diffuse, hyalinized, sclerotic stroma. A further difference was that the majority of glomus tumors and glomangiomas occur in the subungual area, trunk, or extremities, whereas the present tumor occurred on the ear. Due to the peculiar histological features and location, other tumors were considered in the differential diagnosis to include Merkel cell carcinoma, primitive neuroectodermal tumor, and small cell melanoma. This article illustrates a unique variant of a glomus tumor, which to our knowledge has not been previously described.

Localized multiple glomangiomas on the foot. J Dermatol. 2009 Nov;36(11):604-7.
We report a 15-year-old Japanese male with multiple, soft, blue and painlessnodules on the left foot. The lesions had developed when the boy was 3 years old, and had enlarged gradually thereafter. None of his family members had any similar eruptions. All the lesions were resected under local anesthesia. Histological examination revealed cystically dilated spaces lined by endothelial cells and a few outer layers of glomus cells in the dermis and fat tissue. Immunohistochemistry demonstrated that the tumor cells were positive for alpha-smooth muscle actin. We diagnosed this case as localized multiple glomangioma. Multiple glomus tumors are much less common than solitary ones, and localized multiple glomus tumors are extremely rare. Because the clinical differential diagnosis of multiple glomangiomas includes common venous malformation, particularly blue rubber bleb nevus syndrome, histopathological studies should be performed.

Unusual skin tumors: Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans. Surg Clin North Am. 2009 Jun;89(3):727-38.
This article discusses the epidemiology, etiology, presentation, pathology, evaluation and staging, and treatment of unusual skin tumors, such as Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans.

Symplastic glomus tumor - a rare but distinct benign histological variant withanalogy to other 'ancient' benign skin neoplasms. J Cutan Pathol. 2009 Oct;36(10):1099-102. Epub 2009 Jul 7.
A 78-year-old woman presented with a nail deformity of the index finger of theleft hand associated with paroxysmal pain upon cold exposure. Histologically, a well-circumscribed tumor of 3 mm diameter was found in the dermis. The neoplastic cells in some areas were of pronouncedly variable size and cytomorphology, mostly epithelioid in shape, with eosinophilic cytoplasm and indistinctly defined cell borders. Pronounced nuclear pleomorphism and atypia were striking features, but no mitotic figures were noted. Multinuclear cells were present as were numerous small-to-medium vessels throughout the tumor. The tumor stroma showed myxoidareas. Immunohistochemistry showed cytoplasmic and membranous expression ofsmooth muscle actin and vimentin. The histological features and immunoprofile were consistent with the diagnosis of symplastic glomus tumor, a rare histological variant, which has been defined as a glomus tumor exhibiting marked nuclear atypia, in the absence of any other criteria for malignancy. The biological behavior of the tumor is benign. It is essential to differentiate this entity from malignant glomus tumor, which has metastatic potential. Even prominent cellular atypia and nuclear pleomorphism in a glomus tumor as in our case is not a marker of malignancy in the absence of additional criteria.