Fine needle aspiration cytology diagnosis of renal medullary carcinoma: a case report.

BACKGROUND: Renal medullary carcinoma is a recently described, highly aggressive neoplasm that affects predominantly young African American males with a history of sickle cell trait. To the best of our knowledge, this is the first report of fine needle aspirate cytology (FNAC) findings of renal medullary carcinoma. CASE: A 14-year-old, African American male with a history of sickle cell trait presented with the sudden onset of third cranial nerve palsy. Radiographic examination demonstrated possible tumor masses in the brain, thorax and left kidney. Ultrasound-guided fine needle aspiration was performed on the left kidney, and a cytologic diagnosis of "suspect renal medullary carcinoma" was rendered. The cytologic diagnosis was confirmed by tissue examination. CONCLUSION: The cytologic features of renal medullary carcinoma include loosely cohesive clusters and single epithelioid cells with cytologic atypia, including high nuclear/cytoplasmic ratios, hyperchromasia, prominent nucleoli and cytoplasmic vacuolation. These cytologic findings, coupled with clinical findings (young black male with sickle cell trait), allow recognition of this rare renal neoplasm.

Laboratory, morphologic, and immunophenotypic correlates of surface immunoglobulin heavy chain isotype expression in B-cell chronic lymphocytic leukemia.

We compared surface immunoglobulin heavy chain isotype expression with a number of laboratory, morphologic, and immunophenotypic features in a series of 76 cases of B-cell chronic lymphocytic leukemia (B-CLL). Fifty-five cases were IgM+/IgD+, a phenotype associated with antigenically naïve B cells; 16 cases expressed IgD without IgM, a phenotype seen in a subset of normal B cells with extensive somatic immunoglobulin variable region (IgV) gene mutations; and 5 cases were IgD-, a phenotype associated with memory B cells. WBC count, atypical morphologic features, atypical immunophenotypic characteristics, and CD38 expression were nonrandomly distributed among the 3 categories of heavy chain isotype expression. Moreover, a WBC count more than 30,000/microL (30 x 10(9)/L), atypical morphologic features, and CD38 expression in more than 30% of neoplastic cells (all adverse prognostic factors in B-CLL) were less common among IgD-only cases than among IgM+/IgD+ and IgD- cases. These data demonstrate that surface immunoglobulin heavy chain isotype expression is associated with several laboratory, morphologic, and immunophenotypic features in B-CLL. The subset of B-CLL with the IgD-only phenotype is associated with several favorable prognostic factors, suggesting the possibility that IgD-only B-CLL may be associated with a favorable prognosis.

Cyclin D1 expression in high-grade endometrial carcinomas--association with histologic subtype.

Endometrial endometrioid adenocarcinoma (EC) and serous carcinoma (ESC) are associated with different epidemiologic risk factors, precursor lesions, morphology, and survival outcomes. They also possess distinct molecular profiles. We investigated the expression of cyclin D1, a member of the G1 cyclin family that regulates the G1/S transition in the cell cycle, and estrogen and progesterone receptors (ERs and PRs, respectively) in a group of ECs and ESCs matched for histological grade. We also sought to correlate the expression of cyclin D1 with ER and PR because cyclin D1 has been reported to stimulate transcription of ER- and PR-regulated genes (1,2). We hypothesize that cyclin D1 expression covaries with histologic subtype and is related to the expression of ER and PR. Twenty ESCs and 21 ECs were examined histologically and evaluated immunohistochemically for cyclin D1, ER, and PR using commercially available monoclonal antibodies in archival, formalin-fixed, and paraffin-embedded tissue. Three ESCs (15%) and 10 ECs (48%) expressed cyclin D1 (p = 0.02). Twelve ESCs (60%) and 16 ECs (76%) expressed ER, which is not significantly different. ER-positive ECs were significantly more likely to express cyclin D1 compared with ER-positive ESCs (p = 0.03), but a relationship between cyclin D1 and ER expression in EC was not found. We also did not find a significant relationship between cyclin D1 and PR expression. Therefore, cyclin D1 expression in poorly differentiated endometrial carcinomas is associated with endometrioid histology. This is consistent with pathobiologic divergence in poorly differentiated endometrial carcinomas.

Kaposi's sarcoma following allogeneic hematopoietic stem cell transplantation for chronic myelogenous leukemia.

Unlike solid organ transplantation, Kaposi's sarcoma (KS) occurs rarely following hematopoietic stem cell transplantation (HSCT). In fact, only 5 cases of KS have been reported after allogeneic or autologous HSCT. The usual treatment combines a substantial decrease in, or elimination of, immunosuppressive therapy along with local measures such as surgical excision, cryotherapy or radiation therapy. A 46-year-old woman with chronic myelogenous leukemia who had received an allogeneic HSCT previously from an HLA-identical sibling, presented on day +814 with human herpes virus-8-associated KS involving her left lower extremity. She had been on continuous immunosuppressive therapy since her transplant because of chronic graft-versus-host disease. The intensity of immunosuppressive therapy was decreased once a diagnosis of KS had been established. However, the nodular lesions continued to progress in size and number. Therefore, a course of irradiation was administered to sites of bulk disease on her legs. Furthermore, thalidomide was initiated along with a topical retinoid, alitretinoin 0.1% gel applied twice daily to the nonirradiated lesions. This approach yielded a partial response in both irradiated and nonirradiated lesions over the course of the following 7 months. Both thalidomide and alitretinoin 0.1% gel appear to be beneficial in HSCT-associated KS and exhibit tolerable side effects.

Familial papillary serous carcinoma of the cervix, peritoneum, and ovary: a report of the first case.

OBJECTIVE: We report an occult primary papillary serous carcinoma of the endocervix that was encountered in a woman whose mother and identical twin sister died of papillary serous carcinomas (PSCs) of the peritoneum and ovary, respectively. METHODS: The medical records and the histologic material belonging to the patient, her sister, and her mother were reviewed. RESULTS: The cervical PSC was histologically similar to the peritoneal and ovarian carcinomas. The patient has recurred with peritoneal carcinomatosis 24 months following surgery and postoperative radiotherapy and chemotherapy. CONCLUSIONS: Primary papillary serous carcinoma of the cervix is a very rare adenocarcinoma variant; there have been approximately 30 such cases reported, and, to our knowledge, this is the first documented case of familial peritoneal/ovarian/uterine papillary serous carcinoma. The prophylaxis achieved through bilateral oophorectomy in individuals with a family history of ovarian cancer does not address the risk of PSCs arising in the uterus, cervix, or peritoneum.

Distinctive p53 and mdm2 immunohistochemical expression profiles suggest different pathogenetic pathways in poorly differentiated endometrial carcinoma.

Endometrial serous carcinoma (ESC) and FIGO (International Federation of Gynecology and Obstetrics) grade 3 endometrioid adenocarcinoma (EC) are high-grade endometrial tumors that have different clinical and morphologic attributes. Alteration of p53 tumor suppressor protein function has been implicated in the pathogenesis of both tumors, although the mechanisms may differ. We sought to investigate this difference by comparing immunohistochemical expression of p53 and mdm2. p53 immunoreactivity often correlates with gene mutation, whereas increased mdm2 expression is linked to complex formation with wild-type p53 resulting in its inactivation. Twenty cases of ESC and 21 cases of EC were evaluated and an immunoreactivity score (IRS) was assigned using both the percentage of cells stained and the intensity of staining. The overall IRSs were significantly different in ESCs versus ECs for both p53 and mdm2 (p < 0.001 and p < 0.01, respectively). Strong mean immunoreactivity for p53 was detected in 15 (75%) ESCs as compared to only weak mean immunoreactivity in 17 (81%) ECs. Conversely, for mdm2 expression, 17 (81%) ECs had moderate mean immunoreactivity whereas 9 (45%) ESCs showed only weak mean immunoreactivity. mdm2 expression more closely correlated with p53 expression in ECs than in ESCs. In ECs, mdm2 was detected in 16 of 17 (94%) p53-positive tumors but in only 1 of 3 (33%) p53-negative tumors (p < 0.025). In ESCs, mdm2 was detected in 9 of 15 (60%) p53-positive tumors but in none of the 5 p53-negative tumors (p < 0.10). Overall, our results demonstrate an inverse relationship between the expression of p53 and mdm2 in ESC versus high-grade EC. Specifically, strong p53 immunoreactivity is associated with weak mdm2 expression-in ESC and weak p53 expression is associated with moderate mdm2 expression in EC. These results suggest different pathogenetic pathways resulting in loss of normal p53 function in these two tumors: by p53 gene mutation (strong p53 overexpression) in ESCs, or by mdm2 complex formation and inactivation of p53 in high-grade ECs.

The CD44v6-negative phenotype in high-grade uterine carcinomas correlates with serous histologic subtype.

Uterine serous carcinomas (USCs) and poorly differentiated (International Federation of Gynecology and Obstetrics Grade 3) endometrioid adenocarcinomas (ECs) are two histologic subtypes of high-grade uterine carcinoma that differ in clinical presentation, patterns of dissemination, and biologic aggressiveness. To investigate the mechanisms responsible for this heterogeneity, we studied CD44 and CD44v6 expression in a series of 20 USCs and 21 poorly differentiated ECs. CD44 is a protein involved in cell adhesion and lymphocyte homing, and one of its isoforms, CD44v6, might be related to capillary-lymphatic space invasion and metastasis. Eight (40%) of 20 USCs expressed CD44, compared with 18 (86%) of 21 ECs (P < .005). None of the USCs were reactive with antibodies against CD44v6, whereas 45% of the ECs were immunoreactive (P < .001). CD44v6 expression was observed in the myoinvasive ECs but was not seen in foci of capillary-lymphatic space invasion in either the ECs or the USCs. In summary, the USCs were significantly more likely to demonstrate a CD44- and CD44v6-negative phenotype than were the poorly differentiated ECs.