Case of the Month
A young man with a history of gastric bypass surgery presented with dyspnea, fever, and a history of recurrent pneumonia. On imaging, a solitary nodule was found in the right lower lobe, and a few tree-in-bud opacities were seen bilaterally. A surgical lung biopsy was performed.
What is your diagnosis?
a. Talc granulomatosis (intravenous drug abuse)
b. Particulate matter aspiration
d. Necrotizing sarcoid granulomatosis
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Answer to last issue’s Case of the Month…
D. Granular cell angiosarcoma
There are only three prior cases of granular cell angiosarcoma in the literature. Hitchcock, Hurt, and Santa Cruz were the first pathologists to describe this tumor in 1994 (1). The patients were a 76-year-old female with a tumor in the parotid and temporal regions, whose death likely resulted from a spontaneous pneuomothorax caused by metastatic lung lesions, and a 72-year-old male with an ulcerated lesion in the nasal bridge that had spread to the cheek. A second paper described a 78-year-old male with a scalp tumor, misinterpreted as a follicular cyst, who experienced no recurrence (2).
Angiosarcoma is common in the head and neck of elderly patients; nearly 25 percent of cases involve the trunk. The prognosis is worse in elderly patients, but better for trunk disease. Microscopy shows abundant epithelioid cells mixed with some spindle cells, infiltrating the dermis and subcutaneous layer with moderately pleomorphic nuclei and distinct membrane. The granular cytoplasmic alteration occurs because of an overabundance of lysosomes (evidenced by CD68 and NKI/C3 positivity). Immunostaining is also positive for ERG, CD31, and CD34 – focal in some cases, but diffuse in ours.
To make this diagnosis, look for vessels in the deeper portions of the subcutaneous tissue or in the periphery of the lesion. In our case, vessels were found in the subcutaneous layer, with cells showing granular changes – a rare phenomenon.
Submitted by César Augusto Alvarenga, Pathologist at the Instituto de Patologia de Campinas in São Paulo, Brazil.
- MG Hitchcock et al., “Cutaneous granular cell angiosarcoma”, J Cutan Pathol, 21, 256–262 (1994). PMID: 7962828.
- A Jarell, TH McCalmont, “Granular cell angiosarcoma”, J Cutan Pathol, 39, 476–478 (2012). PMID: 22515218.
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