Primary intracranial leiomyosarcoma among patients with AIDS in the era of new chemotherapeutic and biological agents

Abstract

Primary intracranial leiomyosarcoma (PIL) is a rare non-infectious aetiology of focal mass lesions among HIV-infected individuals. With only 16 published cases worldwide, information on its pathophysiology, risk factors, clinical course and management options is limited. We report two cases of PIL in HIV-infected Filipino men who presented with 1-3 months history of persistent headache, progressing in severity. Both had cranial MRI revealing intracranial mass diagnosed as leiomyosarcoma by excision biopsy and immunohistochemical staining. Both patients underwent adjuvant cranial radiotherapy and chemotherapy. Biologics were initiated in one patient. Both patients were alive with evidence of the disease.

Keywords: Hiv / Aids; neurooncology.

Figure 1

A contrast-enhanced cranial CT scan showing an enhancement of the hyperdense mass in the left frontoparietal region measuring 3.36×2.64×2.0 cm with areas of central necrosis (as pointed by an arrow).

Figure 2

Cranial MRI with gadolinium showing a well-defined, extra-axial, heterogeneously enhancing solid mass in the left parietal area with central necrosis measuring 3.4×2.6×3.7 cm (as pointed by an arrow).

Figure 3

H&E stain of the left parietal mass showing a well-defined, eosinophilic staining tissue with interspersed blood vessels. Green arrow pointing to an area of a mitotically active nucleus.

Figure 4

Immunohistochemistry staining of the left parietal mass revealed the following: (A) smooth muscle antigen—positive tumour cells (B) epithelial membrane antigen—negative tumour cells and (C) CD34 stain—negative tumour cells. Green arrow pointing to an area of tumor’s vascular supply.

Figure 5

A contrast-enhanced cranial CT scan showing a 2.0×1.7×1.6 cm hyperdense focus on the left occipital lobe (as pointed by an arrow).

Figure 6

H&E stain demonstrating interlacing fascicles of spindle cells with mild to moderate pleiomorphism. Arrow pointing to a mitotically active cell nucleus.

Figure 7

Immunohistochemical stains showing: (A) vimentin, (B) caldesmon, (C) smooth muscle antigen—positive in majority of the tumour cells, (D) desmin—focal, strong positive.

Figure 8

Bone scintigraphy revealed a mildly increased osteoblastic activity in the right lambdoid suture and occipital bone.

Figure 9

Cranial MRI showing increase in the size of the masses in the left occipital and middle aspect of the right middle cranial fossa. A new lesion was seen in the middle aspect of the left middle cranial fossa in the left parasellar region measuring 0.7×0.5×0.6 cm (as pointed by an arrow).