A 51-year-old woman presented with severe dizziness. The brain magnetic resonance image revealed a 5.5 cm multiloculated mass with a thick rim in the left temporal lobe. Cytological examination of frozen diagnosis of the mass showed hypercellular sheets of round and rhabdoid cells in a hemorrhagic background, and two mitotic figures were observed. Histologically, the excised dura-based mass consisted of predominantly round cells with small foci of rhabdoid tumor cells in a pseudoalveolar pattern in a hemorrhagic background, and the cells showed nuclear positivity for signal transducer and activator of transcription 6 as well as frequent mitosis. The mass was diagnosed as a grade 3 solitary fibrous tumor (SFT)/hemangiopericytoma (HPC). The cytological diagnosis of SFT/HPC is challenging because of the heterogeneous cytological findings, such as histological heterogeneity, and because there are no standardized cytological criteria for malignant SFT/HPC. Cytological findings, such as singly scattered small cells, hypercellularity, rare ropy collagen, and round and rhabdoid cells with pseudoalveolar pattern, may assist in the diagnosis of malignant SFT/HPC.
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Background The term solitary fibrous tumor (SFT) is preferred over meningeal hemangiopericytoma (HPC), because NAB2-STAT6 gene fusion has been observed in both intracranial and extracranial HPCs. HPCs are now considered cellular variants of SFTs. Methods: This study analyzes 19 patients with STAT6-confirmed SFTs, who were followed for over 11 years in a single institution. Ten patients (10/19, 56.2%) had extracranial metastases (metastatic group), while the remainder (9/19) did not (non-metastatic group). These two groups were compared clinicopathologically. Results: In the metastatic group, the primary metastatic sites were the lungs (n = 6), bone (n = 4), and liver (n = 3). There was a mean lag time of 14.2 years between the diagnosis of the initial meningeal tumor to that of systemic metastasis. The median age at initial tumor onset was 37.1 years in the metastatic group and 52.5 in the non-metastatic group. The 10-year survival rates of the metastatic- and non-metastatic groups were 100% and 33%, respectively. The significant prognostic factors for poor outcomes on univariate analysis included advanced age (≥45 years) and large initial tumor size (≥5 cm). In contrast, the patients with higher tumor grade, high mitotic rate (≥5/10 high-power fields), high Ki-67 index (≥5%), and the presence of necrosis or CD34 positivity showed tendency of poor prognosis but these parameters were not statistically significant poor prognostic markers. Conclusions: Among patients with SFTs, younger patients (<45 years) experienced longer survival times and paradoxically had more frequent extracranial metastases after long latent periods than did older patients. Therefore, young patients with SFTs require careful surveillance and follow-up for early detection of systemic metastases.
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The Sertoli-Leydig cell tumor is a gonadal tumor of sex-cord stromal type, similar to that seen in of the various phases of testicular development in the male. This tumor is exceedingly rare, accounting for only 0.1% to 0.5% of all primary ovarian neoplasms. It occurs predominantly in the second and third decades(mean age about 25 years), less than 10% after menopause. We investigated a case of poorly differentiated Sertoli-Leydig cell tumor of right ovary, occured in a 76-year-old woman. Grossly, the tumor measured 2, 100 gm in weight and 25 x 19 x 8 cm in dimensions. The outer surface was smooth and glistening without rupture of the capsule. Cut sections revealed a multilobulated brown solid mass with multiple cystic change. Microscopically, it showed the typical findings o a Sertoli-Leydig cell tumor.
The characteristic feature is hemangiopericytoma paftem of sarcomatoid spindle cells. Therefore, we present it with a brief review of the literature.
Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that usually arises in the pleura and has been recently reported from unusual locations. We present a case of SFT that occurred in the scrotum. The patient was a 38-year-old man who presented with a painless, left, scrotal mass of five years' duration. Scrotal sonography and pelvic CT scan showed a soft-tissue mass of 11 cm in diameter. The resected tumor measured 11 8 7 cm and was well demarcated from the surrounding soft tissue. The cut surface revealed homogenously grayish-white and gelatinous appearance. No hemorrhage or necrosis was found. Microscopically, the tumor showed hypercellular spindle cell areas intermixed with hypocellular areas lying in a myxoid or collagenous stroma.
The spindle cells had no mitosis or low mitotic figures, and little or no nuclear atypia. They exhibited a variety of growth patterns, including "patternless" pattern, and a prominent vasculature with hemangiopericytic pattern.
Vimentin, CD34, and bcl-2 protein immunoreactivity were observed. Characteristic histologic and immunohistochemical features of this lesion were consistent with SFT. To arrive at a correct diagnosis of this lesion, especially when it occurs in unusual sites, immunohistochemical study including CD34 & bcl-2 protein is required in addition to characteristic histologic features.
Several, but not many, cases of congenital meningeal hemangiopericytoma have been described to date. Herein, a case of meningeal hemangiopericytoma in a newborn is described. The patient was a 2-week-old boy with expanded fontanelles and frequent vomiting. A 5 cm sized mass was found in the left temporo-parietal lobe, which was well circumscribed and lobulated. Histologically the tumor was compatible with anaplastic (malignant) hemangiopericytoma, which showed pleomorphic nuclei, frequent mitotic figures and extensive hemorrhagic necrosis. The tumor cells were immunoreactive for vimentin, but negative for CD34, desmin and S-100 protein. Differential diagnosis for meningeal hemangiopericytoma was discussed, and the fact that meningeal hemangiopericytoma might have occurred congenitally was emphasized.
Primary pulmonary hemangiopericytoma is a rare, potentially malignant tumor and assumes more malignant course than that originated in the soft tissue. It can be asymtomatic until attaining a large size within the lung. We report a case of malignant hemangiopericytoma of the lung in a 29 year old man. It represented as a sharply demarcated, huge homogeneous opaque mass in the right upper lobe. The cytologic and histologic features were that of malignant hemangiopericytoma.
Hemangiopericycic maningioma is clinically and pathologically similar to peripheral Hemangiopericytoma and now tends to be terned as hemangiopericytoma of central nervous system. The authors studied 3 cases of hemangiopericytic meningioma obtained from 3 patients, 1 case of meningotheliomatous meningioma, angioblastic meningioma and transitional meningioma, and 2 cases of peripheral hemangiopericytoma, which had operated from November 1988 to May 1989 at the department of neurosurgery, Pusan Inje University Hospital. The authors analysed and compared the immunohistochemical finding and light microscopic apearance. The results obtained were summarized as follows; 1) Classic maningioma (meningotheliomatous meningioma, transitional meningioma and angioblastic meningioma) shows reactivity to both EMA and vimentin. 2) Hemangiopercicytic meningioma and peripheral hemangiopericytoma are reactive only to vimentin, so the two tumors are suggested as same type of tumor. 3) One of 3 cases of hemangiopericytic meningioma shows whorling and interlacing bundles of spindle cells, the peculiar light microscopic features of transitional meningioma, suggesting transitional or mixed form of hemangiopericytic meningioma and transitional meningioma.