HIV associated leiomyosarcoma (intracranial)

Case contributed by Dr Trent Orton

Presentation

HIV +ve (CD 4+ count / HAART etc.. unknown).

Patient Data

Age: 50 years
Gender: Male
Modality: CT

Enhancing left middle cranial fossa mass, with wide dural attachment. 

Modality: MRI

Extra-axial mass with low T2 signal and vivid contrast enhancement. 

Case Discussion

The patient went on to have a craniotomy and resection. 

Histology

Microscopic Description:

Sections show a spindle cell neoplasm. It is composed of irregularly arranged fascicles of spindle cells. Areas of microcystic change are noted. Occasional areas of necrosis are present. There are occasional mitotic figures.

Immunohistochemistry shows positive staining for smooth muscle actin. The tumour is negative for CD34, CD31, GFAP and S-100 protein. The tumour is positive for caldesmon.

Electron microscopy shows the tumour is comprised of spindle cells with intermediate filaments. Many of these intermediate filaments undergo focal densifications, characteristic of smooth muscle cells. However, in addition, many of these tumour cells show fibronexus junctions, such as have been described in myofibroblasts.

FINAL DIAGNOSIS

Leiomyosarcoma 

This tumour was reviewed by Dr. [X], who has expertise in soft tissue tumors. He feels that this is a malignant tumour and that it is a leiomyosarcoma. He noted that such lesions have been described in patients infected with HIV and these are related to Epstein-Barr virus infection. He did immunohistochemical stains and these showed strong positive staining for caldesmon, a low MIB-1 index and strong positive staining throughout the lesion for Epstein-Barr virus in situ hybridization. There was negative staining for HHV8. These features would be in keeping with a leiomyosarcoma associated with an Epstein-Barr virus infection in immune-suppressed patient.

 

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Case Information

rID: 4867
Case created: 27th Oct 2008
Last edited: 6th Nov 2016
Inclusion in quiz mode: Included

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