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21/289. hypercalcemia due to parathyroid hormone-related protein produced by primary ovarian clear cell adenocarcinoma: case report.

    OBJECTIVE: Amongovarian carcinomas, clear cell adenocarcinoma is one of the most common histologic subtypes associated with hypercalcemia. However, the mechanisms of hypercalcemia in clear cell adenocarcinoma are still unclear. In the following case report, we tried to determine the etiology of hypercalcemia and also to demonstrate the management of hypercalcemia diagnosed preoperatively. CASE: A 49-year-old woman was diagnosed as having a malignant ovarian tumor with hypercalcemia caused by elevated serum parathyroid hormone-related protein (PTHrP) prior to her primary surgery. Treatment with disodium incadronate promptly normalized the serum calcium level. An immunohistochemical study demonstrated PTHrP expression in the primary ovarian lesion, but not in the metastatic lesion. A Northern blot analysis of the cancer cells from the ovarian tumor confirmed the presence of PTHrP mRNA. CONCLUSION: Humoral hypercalcemia of malignancy in this case has been conclusively shown to be due to the production of PTHrP at the primary ovarian tumor, based on both immunohistochemical and molecular analyses. ( info)

22/289. Mesonephric adenocarcinoma of the urinary bladder: a case report.

    We report a very rare case of mesonephric adenocarcinoma of the urinary bladder, the origin of which is still uncertain. A non-papillary and broad-based tumor was located in the trigone and bladder neck on cystoscopic examination. Pelvic magnetic resonance imaging in T2-weighted images revealed a mass invading into the muscular layer of the bladder wall. Histologic examination of bladder cup-biopsy specimens showed adenocarcinoma. She underwent total cystectomy and pelvic lymph node dissection. Histologically, the tumor was chiefly composed of cells with eosinophilic cytoplasm and partly of cells with clear cytoplasm or hobnail-shaped cells, arranged in tubular or papillary structures, and infiltrated peri-vesical fat tissues. She died of metastatic disease 22 months after surgery. To the best of our knowledge, the present case is the 19th reported in the literature. ( info)

23/289. basement membrane material and tigroid background in a fine needle aspirate of clear cell adenocarcinoma of the cervix. A case report.

    BACKGROUND: Although cytologic findings of clear cell adenocarcinoma of the female genital tract have been reported sporadically, the background on the smear has received little attention. CASE: A 16-year-old female had a large, necrotic mass in the cervix. As cervical brushing cytology and a punch biopsy of the mass could not make a definitive diagnosis, fine needle aspiration cytology was performed from the mass. The smears revealed loose, three-dimensional clusters and sheet arrangements of atypical cells. Dispersed atypical cells were also seen. The atypical cells were large and had abundant, weakly stained cytoplasm and round or oval nuclei with large nucleoli. There were a few tumor cells with clear cytoplasm and distinct cell borders in Papanicolaou-stained smears. The background in Diff-Quik-stained smears revealed a tigroid background and basement membrane material. CONCLUSION: The malignant tumor, revealing both a tigroid background and basement membrane material, seems to have been clear cell adenocarcinoma. Both features are diagnostic clues to clear cell adenocarcinoma in the female genital tract. Our case indicates that aspiration cytology is also an effective method of diagnosing a cervical tumor when the tumor is polypoid and the surface is extensively necrotic. ( info)

24/289. Clear cell carcinoma of the fimbria of the fallopian tube in a BRCA1 carrier undergoing prophylactic surgery.

    We report the case history of a patient with a family history of breast and ovarian cancer who was subsequently found to be a carrier of the BRCA1 gene, in whom a tiny focus of clear cell carcinoma was found at the fimbrial end of one fallopian tube when she underwent prophylactic hysterectomy and bilateral salpingoophorectomy. The implications of this finding are discussed. ( info)

25/289. Follicular carcinoma of the thyroid with extensive clear-cell differentiation: a potential diagnostic pitfall.

    Clear-cell features have been recognized in several different thyroid neoplasms. A case of thyroid follicular carcinoma with extensive clear- and Hurthle-cell features is described in a 37-yr-old white female, with cytochemical and immunohistochemical analysis. The tumor of the thyroid gland, with anterior neck soft-tissue extension, displayed clear cells on fine-needle aspiration, which were negative for thyroglobulin. The surgical specimen displayed predominately clear cells (80%), and only the nonclear-cell areas stained for thyroblobulin. Proper categorization of clear-cell lesions of the thyroid and soft tissues requires a multimodality approach, involving clinical/pathological correlation, morphological analysis, and ancillary tissue studies. Immunohistochemical stains for thyroglobulin are quite definitive in making the distinction between primary clear-cell thyroid tumors vs. metastatic clear-cell tumors. Cytologists should be aware, however, that the clear-cell areas of thyroid tumors might not stain for thyroglobulin. ( info)

26/289. Invasive adenocarcinoma of mixed endocervical and clear cell type, associated with invasive squamous cell carcinoma of the cervix uteri. A case report.

    A case of invasive adenocarcinoma (predominantly of endocervical type but also with clear cell elements blended in part of the growth) associated with squamous cell carcinoma of the uterine cervix is reported in a young woman aged 18 years. Malignant squamous and atypical glandular cells were detected in Papanicolaou (Pap) smears, which raised the question of a mixed tumor. A diagnosis of cervical cancer was justified by means of a punch biopsy and the patient was treated by modified radical hysterectomy. Histologic and immunohistochemical examination of the hysterectomy specimen revealed two distinct types of an invasive malignant tumor, i.e., of glandular and squamous cell origin, closely related but not integrated. Interestingly, the glandular component comprised both endocervical and clear cell elements. Careful consideration of squamous or glandular atypia in cytological smears may lead to a more precise diagnosis, especially in young women. ( info)

27/289. Clear cell adenocarcinoma in a female urethral diverticulum.

    The incidence of a neoplasm within the female urethral diverticulum is rare. Clear cell adenocarcinoma, which is the most common neoplasm, arises from the metaplasia of surface transitional epithelium and the paraurethral ducts. These tumors are usually diagnosed at a late stage, which worsens their prognosis. The treatment of choice is surgery. ( info)

28/289. Dedifferentiated clear cell chondrosarcoma.

    Dedifferentiation, a change in the histologic character and clinical behavior of a tumor to a more immature and aggressive one, occurs in approximately 11% of all chondrosarcomas. The original lesion is usually a low-grade chondrosarcoma. Clear cell chondrosarcoma is a rare cartilaginous tumor of low-grade malignancy with a preference for the ends of long bones. It is usually curable by resection. recurrence commonly follows inadequate surgery, and metastases to lung, brain, and bones can develop. However, dedifferentiation has not yet been described in association with clear cell chondrosarcoma. Three patients are described who were initially diagnosed as having clear cell chondrosarcoma of the femur. Two were treated with en bloc resection for a clear cell chondrosarcoma. One of these had an undifferentiated sarcoma in a local recurrence after 6 years. In the second, metastasis of the clear cell chondrosarcoma developed 5(1/2) years after surgery; autopsy revealed undifferentiated sarcoma in the lung, heart, and lumbar spine. The third patient had dedifferentiated clear cell chondrosarcoma at the time of resection following the biopsy diagnosis of clear cell chondrosarcoma. All three died with metastatic disease. These three patients represent three different manifestations of dedifferentiation-at initial diagnosis, at recurrence, and at metastasis. To our knowledge, this is the first description of dedifferentiation occurring in clear cell chondrosarcoma. ( info)

29/289. Follicular carcinoma of the thyroid with extensive clear-cell differentiation: a potential diagnostic pitfall.

    Clear-cell features have been recognized in several different thyroid neoplasms. A case of thyroid follicular carcinoma with extensive clear- and Hurthle-cell features is described in a 37-yr-old white female, with cytochemical and immunohistochemical analysis. The tumor of the thyroid gland, with anterior neck soft-tissue extension, displayed clear cells on fine-needle aspiration, which were negative for thyroglobulin. The surgical specimen displayed predominately clear cells (80%), and only the nonclear-cell areas stained for thyroglobulin. Proper categorization of clear-cell lesions of the thyroid and soft tissues requires a multimodality approach, involving clinical/pathological correlation, morphological analysis, and ancillary tissue studies. Immunohistochemical stains for thyroglobulin are quite definitive in making the distinction between primary clear-cell thyroid tumors vs. metastatic clear-cell tumors. Cytologists should be aware, however, that the clear-cell areas of thyroid tumors might not stain for thyroglobulin. ( info)

30/289. HMB-45/melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: additional example of clear cell 'sugar' tumors.

    HMB-45-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis of a 13-year-old Japanese girl is described. The well-defined tumor was completely removed and measured 9 x 7 x 6 cm. Cut sections showed a tan-white, homogeneous appearances with no hemorrhage or necrosis. The tumor was composed of nests or sheets of polygonal or oval-shaped cells rich in clear or finely granular cytoplasm. Capillary network was well developed, and sinusoid vessels were often seen with occasional perivascular hyalinization. There was moderate nuclear atypia but mitotic figures were absent. periodic acid-Schiff stain showed a large amount of glycogen digested by diastase. Immunohistochemical stains for smooth muscle actin, Melan-A, and HMB-45 were positive in most of the tumor cells. Stains for vimentin, muscle actin, and HAM56 were focally positive, whereas stains for desmin, cytokeratin, epithelial membrane antigen, S-100, CD34, CD68, CD99, neurofilament proteins, and estrogen/progesterone receptors were negative. Ultrastructurally, the cytoplasm contained a considerable number of mitochondria, monoparticipate or membrane-bound glycogen, and longitudinally oriented thin filaments with focal condensations and subplasmalemmal densities. The histopathology of the present case, originally interpreted as epithelioid leiomyoma, was consistent with clear cell "sugar" tumors. The present case may indicate ubiquitous distribution of clear cell "sugar tumors" of which histogenesis remains unknown but is presumed to be of perivascular epithelioid cell origin. ( info)
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