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| Year : 2010 | Volume
: 53
| Issue : 4 | Page : 837-839 |
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| Intracranial leiomyoma in a male patient |
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Pranav Dorwal1, Sumaid Kaul1, Deepshikha Arora1, Rajendra Prasad2
1 Department of Histopathology, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India
2 Department of Neuropathology, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India
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| Date of Web Publication | 27-Oct-2010 |
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How to cite this article:
Dorwal P, Kaul S, Arora D, Prasad R. Intracranial leiomyoma in a male patient. Indian J Pathol Microbiol 2010;53:837-9 |
A 17-year-old male presented to the ophthalmology department, with the chief complaints of protrusion of left eye ball, of 1 year duration. It was associated with progressive loss of vision of the same eye and mild vague headache for 3 months. There was no associated sensory or motor deficit. Any stigma of neurofibromatosis (NF) was not seen. The patient was negative for Human Immunodeficiency Virus (HIV). Patient was negative for Epstein Barr Virus (EBV) as tested by Polymerase Chain Reaction (PCR). Magnetic Resonance Scanning (MRI) revealed a large extra-axial lesion, measuring 5.4 × 5 × 6 cm, hypointense on T1, hypo to isointense on T2 weighted image, and showed significant heterogenous enhancement, involving the left infratemporal fossa, orbit, cavernous sinus, ethmoid and sphenoid sinus [Figure 1]a, b. It was eroding the lateral wall of the body of sphenoid, greater and lesser wing of sphenoid anteroinferiorly and medial wall of orbit. Multiple intralesional hypointense foci suggestive of calcification were seen. The tumor was attached to the meninges and its medial portion was attached to the cavernous sinus, hence it was very vascular. Radiologically, it was suspected to be a meningioma. The patient underwent tumor embolization of the feeders followed by left frontotemporal flap, craniotomy and microdecompression [Figure 2]. | Figure 1: (a,b) MRI: A large extra-axial lesion, measuring 5.4 × 5 × 6 cm; hypointense on T1, hypo to isointense on T2 weighted image, with contrast enhancement, involving the left infratemporal fossa, orbit, cavernous sinus, ethmoid and sphenoid sinus
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 | Figure 2: Intraoperative picture: Left fronto-temporal flap, craniotomy and microscopic decompression
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Microscopically, the sections showed a tumor composed of whorls, sheets and rare interlacing fascicles. The individual cells were spindly, with poorly defined cell borders, ample eosinophilic cytoplasm and an elongated blunt ended vesicular nucleus [Figure 3]. Any nuclear atypia was not observed. Rare mitotic Figure was seen (<1/10 hpf). Rare focus of osteoid metaplasia was seen. There was no evidence of tumor necrosis. Any parent tissue was not identified. Differential diagnosis of meningioma, cellular schwannoma and leiomyoma were considered. A panel of immunohisthochemical markers comprising vimentin, epithelial membrane antigen (EMA), S-100, CD34, smooth muscle actin (SMA) and caldesmon was put up. The neoplastic cells were immunoreactive to vimentin, SMA [Figure 4] and caldesmon [Figure 5]. They were negative for S-100, EMA [Figure 6] and [Figure 7] and CD34. The microscopic and immunohistochemical profile were characteristic of a leiomyoma. Thoracic and abdominal computed tomography (CT) scans were performed and they ruled out metastasis from another primary tumor. | Figure 3: Cells arranged as whorls, sheets and rare interlacing fascicles. The individual cells are spindly, with poorly defined cell borders, ample eosinophilic cytoplasm and an elongated blunt ended vesicular nucleus, (H and E ×600)
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Primary intracranial neoplasms of mesenchymal origin are exceedingly rare. Most of these tumors represent metastasis from other primary sites. [1] Benign metastasizing leiomyomas have been observed intracranially in female patients. [2] The smooth muscle origin of leiomyomas is confirmed on histology, immunohistochemistry and electron microscopy, although in our case, we used only histology and immunohistochemistry to confirm the same. Various suggestions regarding the cell of origin of intracranial leiomyomas include pleuripotent mesenchymal cell, blood vessel smooth muscle cells, embryonic rests, leptomeninges and perivascular connective tissue. [3],[4] In one of the study involving four cases, all the patients were females, with age ranging from 14 to 57 years. The tumors were located at third ventricle, suprasellar region, cavernous sinus and left dural transverse sinus. [5] These tumors are common in immunocompromised host and associated with HIV and EBV infection; [1],[5] although our patient was negative for both. Till date, only seven cases of primary intracranial leiomyomas have been observed and all the reported cases have been females. [1] This, to the best of our knowledge, is the first case of intracranial leiomyoma in a male patient till date.
To conclude, leiomyomas, although rare, should be kept in mind, if an intracranial neoplasm has spindle cell morphology on routine histopathologic examination. Although not a diagnostic challenge, this case is being reported for its rarity.
 Acknowledgment | |  |
The authors are thankful to the technical staff at the Department of Histopathology, Indraprastha Apollo Hospitals, New Delhi.
| References | | |
| 1. | Ali AE, Fazi M, Bilbao JM. Primary intracranial leiomyoma: A case report and literature review. Virchows Arch 2006;449:382-4. 
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| 2. | Lai PH, Yang CF, Huang CH. Primary intracranial leiomyoma. Neuroradiology 1998;40:238-41.
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| 3. | Kroe DJ, Hudgins WR, Simmons JC. Primary intrasellar leiomyoma. J Neurosurg 1968;29:189-91.
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| 4. | Thierauf P, Weiland H. Intracranial leiomyoma. Med Wlt 1978;29:1212-80.
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| 5. | Kleinschmidt-DeMasters BK, Mierau GW, Sze CI, Breeze RE, Greffe B, Lillehei KO, et al. Unusual dural and skull based mesenchymal neoplasms: a report of four cases. Hum Pathol 1998;29:240-5.
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Correspondence Address:
Pranav Dorwal
s/o Dr. S K Sharma, Usha Clinic, P.O. Ateli Mandi 123 021, Teh: Narnaul (Haryana)
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.72074
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7] |
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