Paul Zaharopoulos

Malignant Ovarian Sex Cord Tumor with Annular Tubules in a Patient with Peutz-Jeghers Syndrome: A Case Report

The majority of ovarian sex cord tumors with annular tubules (SCTAT) are benign neoplasms that arise sporadically. In patients who have Peutz-Jeghers syndrome (PJS), ovarian SCTAT is often an incidental finding. Malignant behavior in SCTAT has heretofore been reported only in sporadic cases. We report a case of bilateral, malignant SCTAT developing in a 47-year-old woman who had PJS, originally diagnosed as adenocarcinoma on cervicovaginal cytology. Cervicovaginal and peritoneal fluid cytologic preparations were characterized by pseudopapillary clusters and three-dimensional tubes of tumor cells with scanty cytoplasm and high nuclear:cytoplasmic ratio. Examination of surgical resection specimens revealed bilateral, solid ovarian tumors composed of simple and complex annular tubules with hyaline cores, typical of SCTAT. Tumor emboli were present within salpingeal lymphovascular spaces and in both right and left pelvic lymph nodes. Flow cytometry of tumor cells demonstrated a diploid phenotype. This case represents the first documented example of bilateral, malignant SCTAT arising in a patient who had PJS, presenting with an atypical cervicovaginal smear.

Follicular Carcinoma of the Thyroid Metastatic to the Kidney 37 Years After Resection of the Primary Tumor

A case is described of thyroid carcinoma presenting as renal metastases 37 years after initial resection of the primary. Thyroid carcinoma metastatic to the kidney detected during life is rare, only 2 cases having been reported to date. An additional feature of this case is that the patient had donated the contralateral kidney for transplantation before discovery of the metastases. Metastatic thyroid carcinoma is reviewed and the implication of organ donation in patients with thyroid carcinoma is discussed.

Peutz‐Jeghers syndrome with intestinal carcinoma. Report of the association in one family

The Peutz‐Jeghers Syndrome is a rare, genetically inherited disorder. There are insufficient data to establish this syndrome as a precancerous condition, but documented cases of gastrointestinal cancer arising in this syndrome have been reported with an incidence of 2–3%. Since the syndrome is transmitted as an autosomal dominant and is associated with cancer, family members so afflicted may show an increased cancer risk. This is the first reported case of a father and son with both Peutz‐Jeghers Syndrome and colonic carcinoma.

Fine-needle aspiration cytology of hemangiopericytoma: report of two cases.

The fine‐needle aspiration biopsy (FNAB) findings in two cases of hemangiopericytoma (HP), arising in the parotid gland and on the inner chest wall, respectively, are reported. Smear preparations in each case showed cytologic features of an undifferentiated spindle‐cell neoplasm, whereas a core needle biopsy specimen of the chest wall mass showed a spindle‐cell tumor with a “staghorn‐like” arrangement of endothelium‐lined vascular channels. Immunostains performed on this core biopsy, and on the surgical resection specimens in both cases, showed positive staining of tumor cells for vimentin and CD34, with negative staining for a variety of smooth muscle, epithelial, neural, and neuroendocrine markers. Electron microscopy performed in one case further supported the diagnosis of HP. With adequate sampling and appropriate use of ancillary studies, a diagnosis of HP can be reliably suggested on the basis of FNAB and core biopsy of a soft‐tissue mass. Diagn. Cytopathol. 2000;23:187–191.

Cytologic diagnosis of syphilitic pleuritis: a case report.

We report on a case of a 68‐yr‐old man with secondary syphilis diagnosed by biopsy of skin lesions, who concomitantly suffered from left lower lobe pneumonia with associated pleuritis. Cytologic examination of the pleural fluid was diagnostic of syphilis, not only by the characteristic cytomorphology but also by demonstration of spirochetes by the May‐Grünwald‐Giemsa (MGG) and Steiner staining methods. This suggests that the pneumonia was also syphilitic. The patient was seropositive for HIV‐1, but this probably did not contribute to the thoracic manifestations of syphilis, as there was no evidence of immunodeficiency by the CD4/CD8 T lymphocyte count. This is the second reported demonstration of Treponema pallidum in a pleural fluid, and the first diagnosed by cytopathologic examination. Diagn. Cytopathol. 16:35–38, 1997.

Disseminated Histoplasma capsulatum infection presenting as genital ulcerations.

Background Histoplasma capsulatum infection in adults is most often subclinical but can result in disseminated disease with weight loss, fever, hepatosplenomegaly, and oropharyngeal ulcerations. Genital ulceration as the presenting sign of the disease has been reported rarely. Case A 63-year-old woman presented with multiple vaginal ulcerations due to chronic disseminated H capsulatum infection. Initial diagnosis was made by Papanicolaou and Giemsa-stained vulvar smears. Ketoconazole therapy resulted in clearing of the lesions in 5 weeks. Conclusion Chronic disseminated histoplasmosis is an insidious and potentially fatal disease that can present rarely as genital mucocutaneous ulcerations in women. Prompt presumptive diagnosis can be accomplished by examination of smears obtained by ulcer abrasion, permitting institution of appropriate therapy.

Membranous lamellar cytoplasmic inclusions in histiocytes and mesothelial cells of serous fluids. Their relationship to phagocytosis of red blood cells.

OBJECTIVE To define the composition of cytoplasmic inclusions forming stacks and concentric whorls in histiocytes and mesothelial cells of serous fluids, imparting to them a resemblance to Gaucher cells, and to draw conclusions on the mechanism of their formation. STUDY DESIGN Three serous fluids (one pleural and two pericardial) containing a fair number of the cells referred to were progressively subjected to the following studies: (1) cytochemistry for mucopolysaccharides, proteins, phospholipids and hemoglobin; (2) immunocytochemistry for immunoglobulins IgA, IgG, IgM and lysozyme; (3) transmission electron microscopy (TEM), and (4) scanning electron microscopy-based energy dispersive X-ray microanalysis (SEM-EDAX). RESULTS All three specimens were blood stained and contained large numbers of histiocytes and mesothelial cells, arranged singly and in groups, with abundant cytoplasmic inclusions. The inclusions stained strongly positive for phospholipids, weakly positive for hemoglobin and negative for all other substances examined by cytochemistry and immunocytochemistry. By TEM the inclusions had a concentric lamellar membranous structure, reminiscent of myelinosomes or lamellar bodies of lipid-forming or -storing cells. There was also phagocytosis by histiocytes and mesothelial cells of red blood cells, which were mostly in a degenerated state. SEM-EDAX of inclusion-bearing cells showed a modest peak for phosphorus and a variable but small peak for iron, which corroborated the cytochemical and TEM findings. CONCLUSION Since there was not metabolic or other systemic disease in the patients to account for these cells, we posit that phospholipids derived from cell membranes of phagocytized cells, especially red blood cells, provide the building blocks for the formation of such inclusions as they enter the metabolic pathway of phagocytic cells (mesothelial cells and histiocytes) and appear in their lysosomal structures. It is advantageous for cytologists to be familiar with significance of such changes and not to mistake them for metabolic or other systemic disease.

Nuclear protrusions in cells from cytologic specimens. Mechanisms of formation.

OBJECTIVE To explore the mechanisms of formation of nuclear protrusions (NPs) encountered in cytologic specimens and specifically the possibility of their being the result of an aberrant division of the cell and to determine how widespread the NP phenomenon is in cells from various tissues. STUDY DESIGN Six hundred fifty-four cervical smears out of 5,000 with abundant cervical columnar epithelium examined were found to have many cells with NPs (group A). These cells were studied: (A) by light microscopy to define the structure and stages of formation of NPs, (B) by transmission electron microscopy (TEM), (C) by tubulin immunostaining for detection of mitotic spindle-associated microtubular structures, (D) by fluorescence in situ hybridization (FISH) utilizing X chromosome probes to monitor chromosomal movement into NPs, and (E) by direct fluorescence microscopy to examine autofluorescence patterns in cells with NPs. Also, tissue sections of 240 cervical cone biopsies, many including intraepithelial neoplasia (group B), were examined for NPs, and sections containing NPs were subjected to TEM. Last, 390 nongynecologic cytologic specimens from various lesions and organs obtained by fine needle aspiration or brushing methods were examined for the presence of NPs. RESULTS NPs were found in a variety of tissues, epithelial and nonepithelial. Their formation in the cases examined appeared to be related to cell division, as indicated by: the light microscopic findings; the TEM findings (centriole at their tip, indication of spindle formation, nucleolar movement into the NP and suggestion of chromosomal movement as well); positive tubulin immunostaining of centrosome-centriole in NPs and also of the underlying region of the nuclear pole, indicating the presence of microtubules consistent with mitotic spindle; and movement of one X chromosome into NPs, as shown by FISH. CONCLUSION NPs are formed in cells from a variety of tissues, epithelial and nonepithelial. In many cases they appear to result from aberrant cell division--namely, unipolar mitosis--occurring before prophase events are completed. Another possible mechanism of NP formation not involving cell division is through alteration or remodeling of the cytoskeleton of the cell; that was shown experimentally to produce nuclear volume and shape changes, including formation of protrusions.