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SOFT TISSUE
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Primary Extracranial Meningiomas: An Analysis of 146 Cases
Rushing, EJ, Bouffard JP, McCall S, Olsen C, Mena H, Sandberg
GD, Thompson LDR.
Head and Neck Pathol (2009) 3:116-130.
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.
PMID: 19644540
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Splenic inflammatory myofibroblastic
tumor (inflammatory pseudotumor): a clinicopathologic and
immunophenotypic study of 12 cases.
Neuhauser TS, Derringer GA, Thompson LDR, Fanburg-Smith JC, Aguilera
NS, Andriko J, Chu WS, Abbondanzo SL
Arch Pathol Lab Med 2001;125:379-85
CONTEXT: Inflammatory pseudotumor is an uncommon and enigmatic
lesion. The spindle cells found in this tumor have features of
myofibroblasts. Because of the indefinite relationship of these
lesions with inflammatory fibrosarcoma and their indefinite biologic
behavior, inflammatory pseudotumor is currently classified as
inflammatory myofibroblastic tumor (IMT). To date, only case reports
or small series have been published on these tumors, which are
primary in the spleen.
DESIGN: In this study, we describe the clinical, morphologic, and
immunophenotypic findings of 12 cases of splenic IMT and examine
their relationship to Epstein-Barr virus (EBV).
RESULTS: The patients included 8 women and 3 men, ranging from 19 to
77 years of age (mean, 53 years; median, 60 years). Demographic data
were unavailable for 1 patient. Patients generally presented with
abdominal pain (n = 5) and fever (n = 4). Associated lesions
included renal cell carcinoma (n = 2), colonic adenocarcinoma (n =
1), and cholecystitis (n = 1). All tumors were composed of a bland
spindle cell proliferation in association with a variable mixed
inflammatory component. There were 2 growth patterns, namely, a
cellular spindle cell pattern and a hypocellular fibrous pattern. An
immunohistochemical panel confirmed the myofibroblastic nature of
the spindle cells. The spindle cells of 2 cases were immunoreactive
for EBV latent membrane protein 1, whereas 6 of 10 cases were
positive for EBV-encoded RNA using in situ hybridization. Follow-up
was available for 8 patients; 6 were alive with no evidence of
recurrence and 2 were dead of other causes.
CONCLUSION: Splenic IMTs are uncommon lesions that can be
distinguished from other conditions using a combination of clinical,
histologic, and immunophenotypic findings. Epstein-Barr virus may
play a role in the pathogenesis of splenic IMT, and there may be an
association of splenic IMT with concomitant disease or malignancy.
Most splenic IMTs have an excellent long-term prognosis.
PMID: 11231487
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Spindle-cell lipoma.
Thompson LDR.
Ear Nose Throat J. 2009 Jul;88(7):992-3.
FIRST PARAGRAPH: Histologically, spindle-cell lipoma is a
distinctive type of lipoma on a continuum with pleomorphic lipoma.
It accounts for approximately 1.5% of all adipose tissue neoplasms.
Men are affected significantly more commonly than women (9:1) at a
mean age in the sixth decade. The vast majority of tumors are
located in the subcutaneous tissue of the posterior neck, upper
back, and shoulders. Patients present with a painless, mobile,
subcutaneous mass. In rare cases, these tumors develop in other head
and neck mucosal sites, such as the buccal fat pad. Spindle-cell
lipomas grow as large as 13 cm (mean: 3.5). Grossly, they resemble
ordinary lipomas, although they may be somewhat firmer, especially
if the spindle-cell component predominates.
PMID: 19623524
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