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1/6. Nuchal fibrocartilaginous pseudotumor: a clinicopathologic study of five cases and review of the literature.

    The clinicopathologic features of five cases of a fibrocartilaginous mass developing in the nuchal ligament, the nuchal fibrocartilaginous pseudotumor, are described. Only six examples of this lesion were previously reported in the English-language medical literature. The lesions clinically manifested in five adults (3 women, 2 men) ranging in age from 22 to 46 years (mean, 37 yr). The process presented as a nodular mass that was asymptomatic in three patients and accompanied by vague neck pain or stiffness in the remaining two. Three patients related a history of head and neck trauma that preceded the discovery of the tumor. All of the tumors were situated in the deep soft tissue overlying the posterior aspect of the lower cervical vertebrae. The five patients were managed by complete local excision. The tumors measured 1.3 to 3.0 cm. in greatest dimension (mean, 2.5 cm.). Microscopically, the lesion consisted of a poorly delineated, nodular proliferation of moderately cellular fibrocartilaginous tissue arising within the substance of the nuchal ligament and extending into the surrounding soft tissues. No cytologic atypia or mitotic activity was identified. Follow-up data from four of the cases in this study (range, 10-324 mo) and four previously reported examples with follow-up (range, 3-12 mo) show no evidence of recurrent or persistent disease after simple excision. The nuchal fibrocartilaginous pseudotumor is a benign lesion caused by fibrocartilaginous metaplasia of the lower portion of the nuchal ligament, probably as a result of localized trauma or chronic mechanical stress. ( info)

2/6. Surgical strategies in the removal of malignant tumors and benign lesions of the anterior skull base.

    The choice of surgical approaches to the tumors of the anterior skull base is determined by the location, dimensions of such lesions and their relations to the surrounding structures. Furthermore, the need for the reconstruction of the dura and skull base structures has an important influence on the decision about the surgical procedure. Transfacial approaches provide limited exposure, especially when tumors damage the floor of the anterior cranial fossa and involve the frontobasal dura and brain. Transcranial, craniofacial and subcranial approaches in particular may aid a surgeon in the removal of such lesions, and often these surgical procedures are the only beneficial methods. Our study comprised 15 patients. Transcranial approaches were used in ten cases. In five further cases, we adopted craniofacial or subcranial approaches. Total removal of these lesions was possible in 13 cases. Neither important complications nor death after surgery was observed except for two cases (craniofacial/subcranial approach) where the CSF leak and CNS infection were reported. We deem that the transcranial approach creates a good possibility for total removal of anterior skull base tumors, particularly of the benign lesions, and permits reconstruction of the skull base damaged by the tumor. However, in patients with large malignant tumors, the en bloc resection via the combined craniofacial/subcranial approach achieved better outcome. ( info)

3/6. Glomangiomatosis: magnetic resonance imaging findings in three cases.

    The term glomangiomatosis refers to multiple glomus tumors, a rare condition. Unlike solitary glomus tumors, multiple glomus tumors are not always located in the subungual region, making diagnosis difficult. We report on three cases of glomangiomatosis in the foot, and describe their MR findings and review previous case reports on this subject. ( info)

4/6. perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.

    PEComas, occasionally associated with the tuberous sclerosis complex, are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanocytic markers. This family of tumors includes angiomyolipoma (AML), clear cell sugar tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), and very rare tumors in other locations. Because non-AML/non-LAM PEComas are extremely rare and their natural history and prognostic features undefined, we present our experience with 26 PEComas of soft tissue and the gynecologic tract, the largest series to date. We also performed a detailed review of the literature, with special attention to features predictive of clinical behavior. All PEComas exclusive of AML and LAM were retrieved from our consultation files. immunohistochemistry for pan-cytokeratin (CK), S-100 protein, smooth muscle actins (SMA), desmin, vimentin, HMB45, Melan-A, microphthalmia transcription factor (MiTF), TFE3, CD117, and CD34 was performed. Clinical follow-up information was obtained. Fisher's exact test was performed. The median patient age was 46 years (range, 15-97 years); there was a marked female predominance (22 females, 4 males). Sites of involvement included the omentum or mesentery (6 cases), uterus (4 cases), pelvic soft tissues (3 cases), abdominal wall (2 cases), uterine cervix (2 cases), and vagina, retroperitoneum, thigh, falciform ligament, scalp, broad ligament, forearm, shoulder, and neck (1 case each). The tumors ranged from 1.6 to 29 cm in size (median, 7.8 cm). Tumors were epithelioid (N = 9), spindled (N = 7), or mixed (N = 10). Multinucleated giant cells were present in 18 cases. High nuclear grade was noted in 10 cases, high cellularity in 7 cases, necrosis in 8 cases, and vascular invasion in 3 cases. Mitotic activity was 0 to 50 mitotic figures (MF)/50 high power fields (HPF) (median, 0 MF/50 HPF) with atypical MF in 6 cases. IHC results were: SMA (20/25), desmin (8/22), HMB45 (22/24), Melan-A (13/18), MITF (9/18), S-100 protein (8/24), CK (3/23), vimentin (12/14), TFE3 (5/17), c-kit (1/20), and CD34 (0/7). Clinical follow-up (24 of 26 patients, 92%; median, 30 months; range, 10-84 months) showed 3 local recurrences and 5 distant metastases. At last available clinical follow-up, 2 patients (8%) were dead of disease, 4 patients (17%) were alive with metastatic or unresectable local disease, and 18 patients (75%) were alive with no evidence of disease. No patient in our series had a history of tuberous sclerosis complex. recurrence and/or metastasis was strongly associated tumor size > median size (8 cm), mitotic activity greater than 1/50 HPF, and necrosis. We conclude that PEComas of soft tissue and gynecologic origin may be classified as "benign," "of uncertain malignant potential," or "malignant." Small PEComas without any worrisome histologic features are most likely benign. PEComas with nuclear pleomorphism alone ("symplastic") and large PEComas without other worrisome features have uncertain malignant potential. PEComas with two or more worrisome histologic features should be considered malignant. Occasional PEComas express unusual markers, such as S-100 protein, desmin, and rarely CK. The role of TFE3 in PEComas should be further studied. ( info)

5/6. Multiple mucinous and lipomatous variant of eccrine angiomatous hamartoma associated with spindle cell hemangioma: a novel collision tumor?

    Eccrine angiomatous hamartoma (EAH) is a rare, benign condition characterized histologically by increased numbers of eccrine elements, as well as numerous capillary channels. In most cases, EAH arises as a single lesion; however, multiple variants have been reported. We report a 35-year-old female patient with multiple, sudoriparous, subcutaneous nodules on the right foot, which showed typical histopathological findings of EAH, and vascular components of the tumor consisted of thin-walled dilated vascular spaces intermixed with spindle cells and some histiocytoid endothelial cells representing spindle cell hemangioma (SCH). To our knowledge, the co-existence of EAH with SCH is a novel finding and not yet described. ( info)

6/6. Oncogenic osteomalacia associated with an occult phosphaturic mesenchymal tumour: clinico-radiologico-pathological correlation and ultrastructural studies.

    A middle-aged man presented with bone pain at multiple sites due to tumour-induced osteomalacia. The underlying occult phosphaturic mesenchymal tumour was identified by octreotide scan 5 years after presentation and confirmed by computed tomography. Tumour resection resulted in normalisation of blood chemistry and bone densitometry. Clinico-radiologico-pathological correlation and ultrastructural studies of the tumour threw light on the pathogenesis and pathophysiology of this rare disease. ( info)



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