Primary Extracranial Meningiomas: An Analysis of 146 Cases

Abstract

Primary extracranial meningiomas are rare neoplasms, frequently misdiagnosed, resulting in inappropriate clinical management. To date, a large clinicopathologic study has not been reported. One hundred and forty-six cases diagnosed between 1970 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemistry analysis was performed (n = 85), and patient follow-up was obtained (n = 110). The patients included 74 (50.7%) females and 72 (49.3%) males. Tumors of the skin were much more common in males than females (1.7:1). There was an overall mean age at presentation of 42.4 years, with a range of 0.3–88 years. The overall mean age at presentation was significantly younger for skin primaries (36.2 years) than for ear (50.1 years) and nasal cavity (47.1 years) primaries. Symptoms were in general non-specific and reflected the anatomic site of involvement, affecting the following areas in order of frequency: scalp skin (40.4%), ear and temporal bone (26%), and sinonasal tract (24%). The tumors ranged in size from 0.5 up to 8 cm, with a mean size of 2.3 cm. Histologically, the majority of tumors were meningothelial (77.4%), followed by atypical (7.5%), psammomatous (4.1%) and anaplastic (2.7%). Psammoma bodies were present in 45 tumors (30.8%), and bone invasion in 31 (21.2%) of tumors. The vast majority were WHO Grade I tumors (87.7%), followed by Grade II (9.6%) and Grade III (2.7%) tumors. Immunohistochemically, the tumor cells labeled for EMA (76%; 61/80), S-100 protein (19%; 15/78), CK 7 (22%; 12/55), and while there was ki-67 labeling in 27% (21/78), <3% of cells were positive. The differential diagnosis included a number of mesenchymal and epithelial tumors (paraganglioma, schwannoma, carcinoma, melanoma, neuroendocrine adenoma of the middle ear), depending on the anatomic site of involvement. Treatment and follow-up was available in 110 patients: Biopsy, local excision, or wide excision was employed. Follow-up time ranged from 1 month to 32 years, with an average of 14.5 years. Recurrences were noted in 26 (23.6%) patients, who were further managed by additional surgery. At last follow-up, recurrent disease was persistent in 15 patients (mean, 7.7 years): 13 patients were dead (died with disease) and two were alive; the remaining patients were disease free (alive 60, mean 19.0 years, dead 35, mean 9.6 years). There is no statistically significant difference in 5-year survival rates by site: ear and temporal bone: 83.3%; nasal cavity: 81.8%; scalp skin: 78.5%; other sites: 65.5% (P = 0.155). Meningiomas can present in a wide variety of sites, especially within the head and neck region. They behave as slow-growing neoplasms with a good prognosis, with longest survival associated with younger age, and complete resection. Awareness of this diagnosis in an unexpected location will help to avoid potential difficulties associated with the diagnosis and management of these tumors.

Ectopic Sphenoid Sinus Pituitary Adenoma (ESSPA) with Normal Anterior Pituitary Gland: A Clinicopathologic and Immunophenotypic Study of 32 Cases with a Comprehensive Review of the English Literature

“…A meningothelial pattern is not seen in ESSPA, but the presence of intranuclear cytoplasmic inclusions, a sheetlike to packeted growth, and isolated psammoma calcifications can raise the differential diagnosis of meningioma. However, meningioma is usually positive with EMA and CK7 (in a pre-psammoma body like pattern), while negative with neuroendocrine and pituitary hormone markers [68,69].…”

Primary Extracranial Meningioma: The Royal Pearl Experience

“…An analysis of 146 cases of primary extracranial meningiomas showed that majority originated from the skin and scalp (n = 59) followed by middle ear (n = 26) and sinonasal tract (n = 25). Other rare locations in the head and neck included the temporal bone, mandible, nasopharynx, parotid gland, orbit and neck [5]. Different mechanisms have been proposed to explain the etiopathogenesis of these lesions.…”

“…Arachnoid islets displaced by trauma or cerebral hypertension 4. Undifferentiated mesenchymal cells [5].…”

“…Both lesions have abundant psammoma bodies but, meningiomas lack associated osteoblasts and osteoclasts rimming the psammoma bodies and the distribution of psammoma bodies in meningioma is haphazard. The back ground stroma in ossifying fibroma is storiform and more compact [5].…”

“…No intracranial component was demonstrable in either of the 2 cases. Surgery is reported to be the only curative modality for these tumours [5,6]. Complete extirpation of theses masses may be accomplished by endoscpic, open or a combination of both approaches [5,10].…”