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7 May 2014 - Case of the Week #311

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Thanks to Dr. Raul Gonzalez, Vanderbilt University Medical Center, Tennessee (USA), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.




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Website news:

(1) We have updated numerous topics in the Ovary-nontumor chapter to add images with captions from Scully, Young & Clement: Atlas of Tumor Pathology (AFIP 3rd series, Volume 23, 1999).

(2) We posted a detailed and important article, Part A and Medical Directorship Contracts, by Mick Raich, Vachette Pathology.

(3) We posted updated reviews of these topics:
Breast-embryology by Belinda Lategan, M.D., Lymph nodes-not Lymphoma - gold deposits by Jaya Balakrishna, M.D. and Abdelsalam Sharabi, M.D., Mandible-Maxilla, Residual cyst by Annie Morrison, M.D. and Kelly R. Magliocca, M.D., Molecular - Melanoma Mutations - GNAQ by Joshua Bradish, M.D. and Liang Cheng, M.D., Oral Cavity - SCC of oropharynx by Anshu Jain, M.D. and Ovary tumor - Leiomyosarcoma by Nalini Gupta, M.D.


Case of the Week #311

Clinical History:
A 38 year old woman presented with a 4 cm ankle mass, which was biopsied.

Micro images:



What is your diagnosis?































Diagnosis:
Angiofibroma of soft tissue of the ankle

Discussion:
Angiofibroma of soft tissue was first described in 2012 by Mariño-Enríquez and Fletcher as a benign fibrovascular tumor that resembles a low grade sarcoma (Am J Surg Pathol 2012;36:500). These tumors typically present as a slow growing painless mass of the extremities, and occur at all ages (median age 49 years), more commonly in females.

Grossly, the tumors are well circumscribed, median 3.5 cm. Histologically, there are 2 distinct components: (a) uniform proliferation of bland, spindled cells with scant cytoplasm and ovoid / tapered nuclei, and (b) myxoid or collagenous stroma with prominent vasculature, usually small, branching, thin walled vessels. Occasional findings are mild mitotic activity, mild degenerative atypia, short fascicles, vaguely storiform or swirling arrangements (Int J Clin Exp Pathol 2013;6:2208).

Immunohistochemistry is not diagnostic. Tumor cells often express EMA (44%), and occasionally CD34, SMA or desmin, but are consistently S100-. The t(5;8)(p15;q13) translocation is common, which results in a fusion of AHRR and NCOA2 (Genes Chromosomes Cancer 2012;51:510).

The differential diagnosis includes:
Cellular angiofibroma: usually in genital region, more cellular, usually ER+, PR+
Low grade fibromyxoid sarcoma: often > 6 cm and infiltrative, 45% have epithelioid areas, 40% contain poorly formed but large collagen rosettes
Low grade myxofibrosarcoma: infiltrative borders, often sends out long tentacles, more solid areas are often seen similar to typical undifferentiated pleomorphic sarcom; has curvilinear vessels (thick walled with broad arc) with condensation of cells around vessels
Myxoid liposarcoma: prominent chicken wire vasculature, numerous signet ring lipoblasts particularly at periphery of lobules, mucoid matrix rich in hyaluronidase sensitive acid mucopolysaccharides, may have large mucoid pools, S100+
Solitary fibrous tumor: fibroblast like cells with patternless pattern, thick bands of collagen and prominent branching, hyalinized vessels; CD34+, CD99+.

Excision is typically adequate, even with involved surgical margins, although local recurrence is possible. No metastases have been reported to date.



Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
PathologyOutlines.com, Inc.
30100 Telegraph Road, Suite 408
Bingham Farms, Michigan (USA) 48025
Telephone: 248/646-0325
Email: NatPernick@Hotmail.com
Alternate email: NatPernick@gmail.com