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1/110. syncope two years after hysterectomy.

    A 61-year-old woman presented to the emergency department after experiencing palpitations, shortness of breath, and syncope while taking a shower. Her husband revived her with mouth-to-mouth resuscitation. She had had a similar episode three days earlier while making her bed and had lost consciousness for about 10 sec. She did not appear to have had a seizure. Five months earlier, while taking a walk, she had experienced dizziness, dyspnea, and chest pressure lasting about an hour. A workup at that time included cardiac catheterization, lung scanning, and esophagogastroduodenoscopy, but no abnormality was found. lower extremity edema was noted. ( info)

2/110. Multiple endometrial stromal nodules with sparse cysts and glands in the lung--a nodular variation of endometriosis that may mimic metastases of sarcoma.

    We report an unusual case of a nodular variation of pulmonary endometriosis. To our knowledge, there is no previous report on a morphological investigation of this entity. The etiology of this rare condition is still a matter of discussion. The well-circumscribed nodular mass is composed of cells identical to, or closely resembling, those of endometrial stroma containing sparse cysts and glands. Immunohistochemically, the cells showed an extensive co-expression of cytokeratin AE1/AE3 and vimentin and were highly positive for progesterone receptor (PRICA) and estrogen receptor (ERICA). Cells lining the cysts and glands as a monolayer were reactive for Ber-Ep4, cytokeratin Pan and cytokeratin AE1/AE3 and negative to all other markers used including PRICA and ERICA. The differential diagnosis of this entity included fibrous tumor of the pleura and metastatic low-grade-endometrial-stromal-sarcoma. The morphological findings are correlated with immunohistochemical studies and results of cell image analysis. This study details the clinicopathological features of the nodular variation of pulmonary endometriosis. ( info)

3/110. Stromal sarcoma arising on endometriosis. A clinicopathological and immunohistochemical study of 4 cases.

    The development of stromal sarcomas on the foci of endometriosis is extremely rare and the differential diagnosis from other tumors of myogenic, vascular, hemopoietic or epithelial origin may present great diagnostic difficulties. We investigated the clinicopathological and immunohistochemical characteristics of 4 cases of endometrial stromal sarcoma that developed on endometriotic foci of the uterus, vagina and omentum. Thye were classed as high grade (1/4) or low grade (3/4) malignant potential tumors, according to their mitotic activity. Immunohistochemically these tumors gave a positive reaction to vimentin, but were negative to desmin, smooth muscle actin, factor viii, EMA and LCA. These characteristics permit their identification and a proper therapeutic approach. ( info)

4/110. Magnetic resonance appearance of endometrial sarcoma: report of a case with unusual findings.

    We report the MR findings of an endometrial stromal sarcoma. The uterus was enlarged and completely replaced by neoplastic tissue, with full-thickness myometrial infiltration. It had isointense signal on T1- and irregularly hyperintense signal on T2-weighted images and the dynamic study with intravenous gadoteridol showed centripetal enhancement. This sarcomatous-like pattern correlates well with the pathologic and CT findings reported in the literature. ( info)

5/110. Endometrial stromal sarcomas: immunohistochemical, electron microscopical and cytogenetic findings in two cases.

    Uterine sarcomas are approximately 3% of all malignant uterine corpus tumours. Of these, the tumours that originate solely in the stromal elements of the uterine wall are infrequent and have not been well characterized cytogenetically. We report two cases of endometrial stromal sarcomas (ESS), one low grade and one high grade, diagnosed by conventional histology, immunocytochemistry, electron microscopy and cytogenetics. Morphologically clear-cut differential structures were seen at optical, immunohistochemical, and electron microscopic levels, permitting a clear differential diagnosis. The low-grade ESS expressed hormonal receptors and vimentin, whereas the high-grade ESS showed no hormone receptors, high Ki-67 activity, and occasional cytokeratin-positive cells. Ultrastructurally, no malignant epithelial differentiation was seen in the tumour cells, but cilia were found in both cases. Cytogenetic study of the low-grade ESS showed pseudodiploid karyotype with chromosomes 6 and 20 rearranged. The high-grade ESS showed a complex karyotype with clonal numerical and structural anomalies. The chromosomes involved in the structural rearrangements were 1, 3, 6, 7, 13, 14, 15, 17, 19, and 21. ( info)

6/110. Metastatic endometrial stromal sarcoma masquerading as pulmonary lymphangioleiomyomatosis.

    A 39 year old female presented with bilateral pneumothoraces and interstitial shadowing on chest x ray. A diagnosis of lymphangioleiomyomatosis was made following an open lung biopsy. Over the next eight years she developed respiratory failure leading to single lung transplantation but she died in the immediate postoperative period. Necropsy examination and review of the previous open lung biopsy revealed multiple pulmonary metastases from a low grade endometrial stromal sarcoma of the uterus. This case high-lights the importance of an accurate diagnosis before transplantation. ( info)

7/110. Endometrial stromal sarcoma with clonal complex chromosome abnormalities. Report of a case and review of the literature.

    Only eleven endometrial stromal sarcomas (ESS) with clonal chromosomal abnormalities have been reported in the literature. Of these, four have been reported to harbor the t(7;17) translocation. We report here an additional ESS that exhibited clonal complex chromosome abnormalities not described earlier: 38,XX,-1,del(1)(q11),-2,add(2)(p13),-3,der(4)add(4)(p12)psu dic(4;14)(q35;q11.2), add(6)(p21.3),add(7)(q22),del(7)(p11.2p13),-8,-9,add(9)(q34),- 10,add(10)(q24),-11,-11,ins(12;?) (q13;?),-14,-14,-15,ins(15;?)(q22;?),add(16)(q22),add(17)(q11.2),- 18,der(18)t(7;18)(q11.2;p11.2),-19, add(20)(p13),add(21)(p11.2),-22,add(22)(p11.2), 6mar in metaphase cells from primary short-term culture. ( info)

8/110. Unilateral hydronephrosis and recurrent endometrial stromal sarcoma with review of the literature.

    In this case we present a woman with arterial hypertension. Further examination showed an unilateral hydronephrosis caused by extrinsic compression. A tumoral mass, invading the caval inferior vein and the renal vein, is the very origin of the compression. This mass is a recidive of an endometrial stromal sarcoma for which she had a hysterectomy in 1984. ( info)

9/110. Low-grade endometrial stromal sarcoma with intracardiac extension. Evolution of extensive smooth muscle differentiation and usefulness of immunohistochemistry for its recognition and distinction from intravenous leiomyomatosis.

    This case, a rare example of low-grade endometrial stroma sarcoma with extensive smooth muscle differentiation which extended to the inferior vena cava and cardiac chambers closely resembling intravenous leiomyomatosis grossly and microscopically, illustrates the importance of extensive sectioning and the usefulness of immunohistochemistry. Although spindle cell components arranged in interlacing bundles consistent with smooth muscle differentiation were recognizable in the primary tumor (on retrospective review), extensive smooth muscle differentiation in the recurrent tumors masked prototypical morphologic features of stromal sarcoma and only small neoplastic stromal components were preserved in limited areas, leading to initial failure to distinguish the lesion from intravenous leiomyomatosis. The immunophenotyping disclosed two distinct cell populations in the tumor: i.e. vimentin-positive and smooth muscle marker negative stromal cells, and vimentin-negative spindle-shaped desmin-positive smooth muscle cells. Our observation suggests that the predominance of a smooth muscle component in such a tumor can be misleading and does not always warrant a diagnosis of intravenous leiomyomatosis, nor does it predict a benign clinical course. This case also provides an insight into the relationship of the endometrial stroma and myometrium, and their cell of origin and the histogenesis of endometrial stromal sarcoma. ( info)

10/110. Symptomatic intracavitary (noninvasive) cardiac metastasis from low grade endometrial stromal sarcoma of the uterus.

    We report a 49-year-old woman who was operated upon 33 years ago for uterine endometrial stromal sarcoma of low-grade malignancy. The patient showed obstruction of the right ventricular outflow tract. An echocardiogram and MRI showed that a mass emanating from the inferior vena cava filled the right atrium, the right ventricle, and part of the pulmonary artery. The entire intracavitary neoplasm was successfully removed and diagnosed as low-grade endometrial stromal sarcoma. To our knowledge, this is the second report of a low-grade endometrial stromal sarcoma reaching the heart via the inferior vena cava. In this case an intracavitary metastasis may be a feature indicating the progression of the endometrial stromal sarcoma. Therefore, this neoplasm should be included in the differential diagnosis of cardiac intracavitary neoplasms. patients affected by this tumor may benefit from early identification and resection of the obstructing neoplasm. ( info)
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