Article
Infratentorial angioleiomyoma of the cavernous subtype: a rare location of a soft tissue tumor
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Authors
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Alexander Younsi
- Neurochirurgische Klinik, Universitätsklinikum Heidelberg
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Leonille Schweizer
- Pathologisches Institut, Abteilung für Neuropathologie, Universitätsklinikum Heidelberg
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Oliver W. Sakowitz
- Neurochirurgische Klinik, Universitätsklinikum Heidelberg
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Andreas Unterberg
- Neurochirurgische Klinik, Universitätsklinikum Heidelberg
| Published: | June 2, 2015 |
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Outline
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Objective: Angioleiomyomas (ALMs) are highly vascularized, benign tumors, mostly located within the dermis or the subcutanoeus tissue of the lower extremities in predominantly middle-aged persons. An intracranial location of ALMs is exceedingly rare. To our knowledge, only seven cases have been reported so far. We present the second known case of an infratentorial ALM of the cavernous subtype.
Method: A 76-year-old male patient was transferred to our neurosurgical department after he had experienced unilateral brachio-facial numbness, gait disturbances and vertigo for several weeks. Physical examination on admission showed no neurological deficits. MR-imaging revealed a 4,2 x 4 x 3,8 cm diameter space-occupying lesion located in the right cerebellar hemisphere with dural attachment, little perifocal odema and inhomogeneous contrast-enhancement. Thus, the mass was regarded as a meningioma pending histological confirmation.
Results: The patient underwent a right suboccipital osteoplastic craniotomy and a moderately vascularized mass was excised completely with its dural attachment. Duraplasty was performed. The postoperative course was uneventful. Histological examination identified a solid, mesenchymal tumor composed of dilated vascular spaces intermingled with stomal cells. Atypia, mitoses or necrosis were absent, immunhistochemistry evidenced a positive reaction to smooth muscle actin (SMA), CD-31 and CD-34 whereas reaction to epithelial membrane antigen (EMA) stayed negative. The tumor was classified as a benigne ALM of the cavernous subtype. The patient could be discharged shortly after his operation. No recurrence has been noted during a 12 month follow-up.
Conclusions: The prognosis of intracanranial ALM is good, as suggested in all of the reported cases as well as in our current case. Although ALMs are a rare occurrence, it is unknown why intracranial sites have not been reported more frequently. The reason could be misinterpretation of diagnostic findings, as ALMs can be mistaken for meningiomas or hemangiomas on preoperative imaging.
