Apoptosis resistance in pigmented villonodular synovitis

OBJECTIVE: Pigmented villonodular synovitis (PVNS) is a proliferative lesion originating from synovial tissue with a locally aggressive behaviour. We analysed the pathogenetic role of apoptosis resistance for sustained cell proliferation in PVNS. METHODS: The expression of bcl-2, p53 and Ki-67 was examined in 80 cases of PVNS using immunohistochemistry. In 43 of these cases, DNA content and distribution of cell-cycle phases were investigated by flow cytometry. Additionally, 10 cases of PVNS were analysed by multi-parametric flow cytometry for expression of p53, caspase3, and bcl-2 and by TUNEL to detect DNA fragmentation. RESULTS: No apoptotic cell fractions were detected in any investigated cases. Expression of bcl-2 was found in 84% of cases (up to 6.5% of cells) and was significantly associated with DNA-fragmentation observed by TUNEL (p=0.037). Orthologous p53 expression was observed in 37% of cases. The level of p53 expression correlated with the proliferative activity and the expression of both caspase3 (p=0.017) and bcl-2 (p=0.0013). (No statistically significant correlations between expression of bcl-2, p53, caspase3, DNA fragmentation or proliferative index and age, sex of patients, disease recurrence, growth pattern or size of lesion were found). CONCLUSION: Apoptosis resistance is a critical event in the progression of PVNS and may contribute to the survival of the proliferating synovial cells in PVNS and to the permanent slow progression of these lesions.     

Arthroscopic treatment of localized and diffuse pigmented villonodular synovitis of the knee

Pigmented villonodular synovitis (PVNS) is a rare proliferative synovial disorder of uncertain etiology. Two forms of this disorder, a localized (LPVNS) and diffuse (DPVNS) form, are well differentiated. The therapy of choice for LPVNS is arthroscopic partial synovectomy with excision of the lesion. Total synovectomy, whether done arthroscopically or through an open arthrotomy, is the recommended treatment for DPVNS. During an eight-year period 13 patients, six male and seven female, average age 28 years (range, 16 to 60 years) were treated for PVNS of the knee with arthroscopic synovectomy. Average follow-up was 84 months (range, 28 to 127 months). Four patients were affected by localized PVNS and were subjected to partial arthroscopic synovectomy (two to three portals) with a complete lesion excision. The remaining nine patients presented with the diffuse form of PVNS and all of them underwent total arthroscopic synovectomy (five portals). The diagnosis was confirmed by synovial biopsy. Each patient was evaluated before treatment and at final follow-up. Results were assessed clinically, radiographically and subjectively and were rated as excellent, good, fair, or poor. No complications or recurrences were noted in the LPVNS group, and all four patients were rated as excellent. In the DPVNS group, eight patients were rated as excellent and one patient was rated as fair and it was the patient who suffered the only recurrence in our case series. No relevant complications were encountered. No cases of infection, joint stiffness or neurovascular lesions were seen. Arthroscopy has become the golden standard in treatment of LPVNS, and can undoubtedly give results that are as good as with open synovectomy when treating DPVNS, if performed by an experienced arthroscopic surgeon.     

Cytologic appearance of carcinosarcoma (malignant ameloblastoma and fibrosarcoma) of the maxilla. A case report.

The cytologic appearance of a carcinosarcoma (malignant ameloblastoma and fibrosarcoma) of the maxilla in a 63-year-old man is described. On his first admission the diagnosis of malignant ameloblastoma was made on biopsy. After five surgical excisions, radiotherapy and chemotherapy, the diagnosis was changed to carcinosarcoma because the stromal cells of the tumor had become malignant. Aspiration biopsy cytology of the tumor, with a cystic lesion found at the left suborbital area, revealed malignant epithelial cells, indicating malignant ameloblastoma. Imprint smears of both surgically resected and autopsy material showed two types of malignant neoplastic cells, of epithelial and mesenchymal origin.     

Cytologic features of chordoid meningioma. A case report

BACKGROUND: Chordoid meningioma is a rare subtype of meningioma characterized by myxoid matrices deposited among epithelioid or vacuolated tumor cells and infiltrates of inflammatory cells, and its cytologic features have rarely been reported. CASE: A 57-year-old man with a history of headache and visual disturbance presented with a tumor in the suprasellar region. Intraoperative touch smear cytology of the tumor disclosed a cord-like arrangement of polygonal tumor cells occasionally containing intranuclear inclusions. Furthermore, periodic acid-Schiff-positive, mucinous matrices were deposited among the tumor cells. Also, infiltrates of lymphocytes and plasma cells were noted. Histologic, immunohistochemical and ultrastructural examination confirmed the diagnosis of chordoid meningioma. CONCLUSION: Intraoperative smear cytology in a case of chordoid meningioma showed distinctive cytologic features suggestive of the histologic patterns. The cytologic features, together with a histologic examination, are useful for its diagnosis.     

Cytologic characteristics of pulmonary papillary adenoma. A case report

BACKGROUND: Pulmonary papillary adenoma is a benign pulmonary neoplasm. Previously pulmonary papillary adenoma was described in terms of immunohistochemistry and ultrastructure. However, there are no previous reports describing the cytologic characteristics of pulmonary papillary adenoma. CASE: A 50-year-old male was admitted for evaluation of a coin lesion in the left upper lung field. Radiologic images showed a solid, round tumor approximately 25 mm in diameter in the left upper lung. Transbronchial needle aspiration biopsy (TBNA) was performed, and small numbers of atypical cells were collected. Adenocarcinoma was suggested clinically, and left upper segmentectomy was performed. The histologic diagnosis was pulmonary papillary adenoma. Imprint cytology of the cut surface of the tumor showed tumor cells arranged in sheets that contained scant or vesicular cytoplasm. The nuclei were oval or round, without obvious anisokaryosis, and their chromatin was fine, without hyperchromasia. Cytologically, the nuclei of the tumor cells in the imprint specimen (38.70 +/- 8.69 microns 2) were uniform in size and similar to the atypical cells in the TBNA specimen (38.29 +/- 11.56 microns 2) but significantly larger than the nuclei of the bronchial cells (23.61 +/- 5.98 microns 2) (P < .0001). CONCLUSION: The cytologic appearance of pulmonary papillary adenoma was characterized morphologically and morphometrically. The possibility of cytodiagnosis by TBNA was suggested.     

Cytologic finding of chyloascites in lymphangioleiomyomatosis. A case report

BACKGROUND: Lymphangioleiomyomatosis is a rare disease, histologically characterized by an abnormal proliferation of smooth muscle around the lymphatics. Lung is the most common site of involvement, and patients usually present with dyspnea, chest pain, and cough. Chylous pleural effusion and ascites occasionally appear during the course of the disease. There are only a few reports on the cytologic findings in this disease. To our knowledge, the cytologic findings of chylous pleural effusion and chyloascites have not been reported before. CASE: A 23-year-old female presented with chylothorax, chyloascites and a retroperitoneal mass. Cytologic examination of chylous pleural effusion and chyloascites revealed numerous cohesive and thick clusters of cells with a high nuclear/cytoplasmic ratio, oval nuclei and slightly increased chromatin content. Mitosis and necrosis were not observed. Exploratory laparotomy and transbronchial lung biopsy were performed, and the histologic diagnosis was lymphangioleiomyomatosis involving the retroperitoneal lymph nodes, uterine fundus and lungs. Immunohistochemistry showed that the characteristic clusters in chylous fluids were positive for alpha-smooth muscle actin. CONCLUSION: A diagnosis of lymphangioleiomyomatosis is possible from cytologic findings of effusions with the aid of clinical findings.     

Cytologic diagnosis of florid peritoneal endosalpingiosis. A case report

The cytologic findings in a case of endosalpingiosis presenting in peritoneal washings taken at the time of staging laparotomy for endocervical adenocarcinoma are described. Dense papillary epithelial clusters with distinct ciliated cell borders were found in the cytologic specimens. Cell nuclei were oval, with finely dispersed chromatin and uniform nuclear membranes. These findings, in conjunction with the discovery of tubal-type epithelial inclusions in pelvic and periaortic lymph nodes, established a diagnosis of endosalpingiosis or benign glandular inclusions involving the pelvic peritoneum and lymph nodes.     

Cytologic findings in a fetal intracranial teratoma. A case report

BACKGROUND: Fetal neoplasms are very rare. Recently we had the opportunity to examine the fine needle aspiration (FNA) biopsy of a fetal intracranial teratoma. CASE: The tumor was found in a 30-week-gestation fetus; the mother was 32 years old, gravida 4, para 1. She presented with a rapid increase in abdominal girth over a two-week period. An ultrasound scan showed severe fetal hydrocephalus and a massive intracranial tumor thought to be a teratoma because of variations in echogenicity and spotty calcification. An FNA biopsy was performed under ultrasound guidance. It showed mainly neuroepithelial cells, so a differential diagnosis of malignant neuroepithelial tumor was considered. At autopsy, several other tissue types were found in the tumor, consistent with a teratoma. CONCLUSION: Advances in diagnosis of fetal anomalies by ultrasound have been associated with an increase in the use of fetal interventions performed in utero. This includes the availability of fetal surgery in some centers. FNA biopsy of fetal lesions does not appear to be well described. Increased experience with this technique is necessary if its full potential is to be realized.     

Cytologic diagnosis of primary adenocarcinoma of Bartholin's gland. A case report.

The cytologic and histologic findings in an extremely rare case of adenocarcinoma of Bartholin's gland are described. The tumor cells in scraping and fine needle aspiration smears were in clusters. The nuclei were oval to oblong, and some cells had a peripherally displaced nucleus. The chromatinic material was slightly increased, and some nuclei had prominent nucleoli. The cytoplasm was basophilic and abundant. Microcalcifications and psammoma bodies were numerous. The tentative diagnosis was primary adenocarcinoma of Bartholin's gland, based on the cytologic findings and location of the tumor. Similar findings were noted in the biopsy and surgical specimens.     

Cytologic findings of epithelioid angiosarcoma of the thyroid. A case report

BACKGROUND: Angiosarcoma of the thyroid is a rare and aggressive tumor and occurs mainly in patients from central Europe, especially the alpine region. The fine needle aspiration findings of a keratin-positive epithelioid angiosarcoma of the thyroid occurring in a nonmountainous area in South America is described. CASE: A 65-year-old male from S?o Paulo, Brazil, presented with a mass in the anterior part of the neck with progressive enlargement for three months. The cytologic findings on the fine needle aspirate were a cellular smear composed of single cells and small clusters of neoplastic cells, oval and round. Cell borders were indistinct, and the cytoplasm was vacuolated. The nuclei were eccentrically located, with irregular nuclear membranes; single, prominent nucleoli; and a coarse chromatin pattern. Features suggestive of intracytoplasmic lumens were identified. Open surgical biopsy demonstrated a tumor infiltrating the thyroid gland and composed of large, round, atypical epithelioid cells lining vascular spaces. These neoplastic cells were immunoreactive for AE1:AE3, CK7, vimentin, CD31 and factor VIII. CONCLUSION: Epithelioid angiosarcoma should be considered in the differential diagnosis of epithelioid neoplasms of the thyroid. An immunohistochemical panel should include vascular markers even in the presence of immunoreactivity for epithelial markers.     

Cytologic findings of atypical adenosis of the breast. A case report

BACKGROUND: Atypical apocrine adenosis, a well-described histopathologic entity, can sometimes be misdiagnosed as carcinoma. Apocrine cells can also appear atypical in cytopathology and be mistaken for carcinoma. Occasional case reports describe false positive cases due to the presence of apocrine cells in a few cases of radial scars and atypical apocrine metaplasia and in a degenerated cyst. CASE: A 37-year-old female underwent ultrasound-guided fine needle aspiration of an ill-defined breast nodule. The aspirate showed clusters and single cells containing abundant granular to focally vacuolated cytoplasm; enlarged, pleomorphic nuclei with irregular nuclear membranes; granular chromatin; and prominent nucleoli. These cells were distinct from and larger than the surrounding ductal and myoepithelial cells. Excision showed a nodular area of atypical apocrine adenosis adjacent to previous biopsy changes, correlating with the cytologic findings. CONCLUSION: Atypical apocrine adenosis can mimic carcinoma in histopathology and cytopathology. One should be cautious when reviewing apocrine cells in cytology, given their atypical features, especially their single, dispersed nature. However, the presence of accompanying benign cellular elements supports a benign diagnosis. Surgical biopsy should be recommended based on the cytologic findings.     

Cytologic diagnosis of vaginal papillary squamotransitional cell carcinoma. A case report

BACKGROUND: Papillary squamous and squamotransitional cell carcinomas of the cervix and vagina are infrequent morphologic variants of squamous cell carcinoma that may be underdiagnosed due to a bland histologic appearance. To our knowledge, this entity has not been previously detected by Pap smear evaluation. CASE: Vaginal wall pap smears were collected from a patient with a previous hysterectomy for microinvasive cervicovaginal squamous cell carcinoma and extensive carcinoma in situ. The smears were characterized by: (1) large, darkly staining, three-dimensional, branching, papillary epithelial fragments with prominent fibrovascular cores and lined with loosely cohesive epithelial cells; (2) a highly cellular background population of dissociated single epithelial cells with features of severe dysplasia, including hyperchromatic, coarse chromatin; scant, delicate, frayed cytoplasm and karyorrhectic debris; (3) syncytial aggregates of severely dysplastic epithelial cells morphologically similar to the single cells; and (4) lack of a recognizable, morphologically distinct "transitional cell" population. CONCLUSION: Papillary squamotransitional cell carcinoma of the vagina is a rare morphologic variant of squamous cell carcinoma that should be distinguished from benign vaginal squamous papillomas, condylomatous lesions and verrucous carcinoma. However, this lesion is also related to human papillomavirus infection, particularly the high-risk types. Papillary squamotransitional cell carcinoma can be suspected on Pap smear when high grade squamous intraepithelial lesion features are found in combination with three-dimensional papillary tissue fragments with prominent fibrovascular cores.     

Pigmented villonodular synovitis of the temporomandibular joint. Case Report.

Pigmented villonodular synovitis is a lesion containing giant cells and, because of its tendency to recur, it is easily confused with a malignant process. However, careful review of the histological material will reveal that there is no nuclear atypism or mitotic figures. Also, there have been no reports attributing metastases or death to thid disease. The clinical course of our case was definitely benign.     

Cytologic presentation of alveolar soft-part sarcoma of the vagina. A case report

Alveolar soft-part sarcoma primary in the vagina is an exceptionally rare neoplasm. The cytologic features in a case of this rare sarcoma are described, with a discussion of the differential diagnosis. Structures suggestive of the characteristic PAS-positive, diastase-resistant cytoplasmic crystals were seen in the cytologic specimen; the crystals were well-demonstrated in the histologic material and at the electron microscopic level.     

Cytologic features of atypical polypoid adenomyoma of the endometrium. A case report

BACKGROUND: In 1981, Mazur reported the histologic characteristics of atypical polypoid adenomyoma (APA) of the endometrium. Although most APAs of the endometrium are considered to show benign behavior, there is a small associated risk of the development of adenocarcinoma. The histology of APA of the endometrium is well defined, but the cytologic features of the lesion have not yet been clarified. CASE: A 28-year-old nulligravida with hypermenorrhea had an exophytic, polypoid mass arising from the posterior uterine wall on ultrasonography and magnetic resonance imaging. The results of endometrial smear and biopsy were normal. Transcervical total resection of the tumor was performed with a resectoscope. Frozen sections of the sample suggested APA of the endometrium, and the permanent sections confirmed the diagnosis. The tumor stump/resection plane smears revealed overlapping, highly atypical glandular cells with enlarged, hyperchromatic nuclei; squamous metaplastic cells; and abundant, spindled smooth muscle cells on a clear background, effectively reflecting the epithelial and mesenchymal cell components of the lesion. CONCLUSION: Endometrial smear and biopsy are inaccurate methods for the diagnosis of APA of the endometrium because of limited sampling. Tumor stump/resection plane cytology appears to be useful for detecting APA of the endometrium.     

Cytologic diagnosis of vaginal metastasis from renal cell carcinoma. A case report

BACKGROUND: Metastasis of renal cell carcinoma to the vagina is rare, although it may be the first evidence of the existence of the primary tumor. CASE: A metastatic deposit of renal cell carcinoma in the vagina was diagnosed by cytology as clear cell adenocarcinoma, which was confirmed by biopsy. Radiographic and ultrasound examinations confirmed the renal site of origin, which was corroborated by immunohistochemistry of the biopsy specimen. CONCLUSION: When a cytologic diagnosis of vaginal clear cell adenocarcinoma is made, metastasis of renal cell carcinoma should be considered in the differential diagnosis.     

Cytologic diagnosis of peripheral T-cell lymphoma manifesting as ascites. A case report

BACKGROUND: Patients with malignant lymphoma seldom present with effusions without a known history of malignancy. Because of this, initial diagnosis of malignant lymphoma by effusion cytology is uncommon, with few reported cases. CASE: A 75-year-old male presented with fatigue, decreased appetite and progressively increasing abdominal girth over five weeks. Cytologic examination of ascitic fluid obtained by paracentesis revealed non-Hodgkin's lymphoma with a T-cell phenotype, confirmed by immunophenotypic and molecular studies. Approximately one week later, histologic examination of liver and bone marrow revealed involvement by lymphoma, demonstrating immunophenotypic and molecular profiles identical to those obtained from neoplastic lymphocytes recovered from the ascites fluid. CONCLUSION: This case demonstrates a rare presentation of peripheral T-cell lymphoma, clinically manifesting as ascites. In cases such as ours, where the effusion consists predominantly of small to intermediate-sized lymphocytes, distinguishing lymphoma from reactive lymphocytosis may be difficult. This case not only demonstrates the value of effusion cytology for lymphoma diagnosis but also illustrates how the use of various immunophenotypic and molecular techniques may assist the pathologist in properly diagnosing these difficult cases.