Fine needle aspiration diagnosis of lymphangiomyomatosis. A case report

BACKGROUND: Lymphangiomyomatosis is a rare disease of females, usually of reproductive age. There is a proliferation of lymphatic smooth muscle in mediastinal, retroperitoneal and often pulmonary lymphatics and lymph nodes. CASE: A 45-year-old female presented with a right pleural effusion and increasing retroperitoneal adenopathy with palpable left inguinal adenopathy. Three months previously she had undergone a right salpingo-oophrectomy for an ovarian fibroma with concomitant left ovarian wedge biopsy, myomectomy for leiomyomas and partial omentectomy. Three years previously, at age 42, she had experienced two transient episodes of chylous pleural effusion with no sequelae. She underwent computed tomography-guided fine needle aspiration of a 4-cm inguinal lymph node to rule out lymphoma. CONCLUSION: Fine needle aspiration of lymphangiomyomatosis yields distinctive cytologic morphology. This characteristic morphology, in combination with the appropriate history, permits a minimally invasive, timely and in this particular case, entirely unexpected diagnosis.     

Lymphangioleiomyomatosis. Is fine needle aspiration cytodiagnosis possible?

Lymphangioleiomyomatosis is a rare condition characterized by a proliferation of the smooth muscle cells, mainly in the lymphatic system, giving rise to blockage of the thoracic duct with chylothorax and chyloascites. This condition was seen in a 34-year-old woman with an abdominal tumor that was subjected to fine needle aspiration. The cytologic diagnosis of this neoplasm is difficult since the cellularity and the cytologic picture indicate malignancy. The organoid configuration together with the chylus formation gave an important clue to the correct diagnosis, which, however, was not made until after a biopsy was performed and the cytologic and histologic findings were combined. The cytologic, histologic and electron microscopic findings are presented.     

Survival of patients with malignancy-associated effusions

For a better understanding of the prognosis after the onset of a malignancy-associated effusion in patients known or subsequently shown to have cancer, survival time was compared with the findings and the date of the first cytologic diagnosis of an effusion. The number of patients studied was 254; 171 had a pleural and 83 a peritoneal effusion. The average survival time was 25.5 weeks, which was about equal for both sites of effusions. After two years, only 6% of all patients were alive. When the cytologic diagnosis of the effusion was "malignant," only 4% survived after two years; when the cytologic diagnosis was "suspicious for malignancy" and "nonmalignant," these figures were 5% and 7%, respectively. This indicates that a cytologic diagnosis of benign or nonmalignant is not a good indicator of a better prognosis in cancer patients for whom benign causes of the effusion have been excluded. There appeared to be a prognostic relationship between the length of the interval from the initial diagnosis of cancer to the time of examination of the first sample of the effusion: a longer interval was correlated with a better survival. When survival time was viewed in relation to therapy, patients whose pleural effusions were only treated by aspiration were found to have a particularly short average survival (13.9 weeks).     

Cytologic diagnosis of a metastatic oligodendroglioma in a pleural effusion. A case report

BACKGROUND: Extraneural metastasis of oligodendroglioma is extremely rare and is diagnosed primarily by biopsy or autopsy and very occasionally by fine needle cytologic examination. We report a case of metastatic oligodendroglioma diagnosed by cytologic examination of a pleural effusion. Such a diagnosis has not been reported before. CASE: A 64-year-old woman developed anemia and bilateral pleural effusion 7 years after an operation for an oligodendroglioma over the left frontal lobe. Cytologic examination of the pleural effusion showed aggregates of atypical polygonal cells containing round, hyperchromatic nuclei and scanty, granular cytoplasm in Liu's and Papanicolaou stain and cell blocks. Immunohistochemical staining of the tumor cells revealed a positive reaction for antibodies to glial fibrillary acidic protein, S-100 and Olig2. Pleural biopsy confirmed the cytologic diagnosis of pleural effusion. A pathologic fracture of the right humeral and femoral bones was noted 1 month later, and the specimen also showed infiltrating oligodendroglioma cells in bone tissue. CONCLUSION: To the best of our knowledge, this is the first metastatic oligodendroglioma diagnosed by pleural cytology. Fine needle cytology can provide a reliable and rapid way to detect an extracranial metastatic oligodendroglioma in different organs.     

Fine needle aspiration cytology of malignant mesothelioma

The results of fine needle aspiration (FNA) cytology in 19 cases of malignant mesothelioma are presented. Adequate material for a diagnosis of malignancy was obtained in 17 cases, and in 8 cases a specific diagnosis of mesothelioma could be made. In four other cases, the findings were either consistent with or suggestive of mesothelioma; in four, accurate distinction from other neoplasms was not possible, and in two cases, adenocarcinoma was suggested. The spectrum of cytologic findings ranged from neoplasms of purely epithelial appearance through more pleomorphic biphasic neoplasms to anaplastic tumors. A combination of epithelial-like cell clusters, pavement-like sheets of epithelial cells with well-defined cell borders and prominent cell separation, dispersed angular cells with dense cytoplasm and some spindle-cell forms was the most specific cytologic pattern for mesothelioma. In four neoplasms, ultrastructural examination of aspirated material provided the additional evidence for a definitive diagnosis. The identification of hyaluronic acid within intracytoplasmic vacuoles, either in smears or in cell blocks, confirmed the diagnosis in three tumors. Only in one case, with a strong clinical background suggesting mesothelioma, was the cytologic preparation sufficient for diagnosis without ancillary diagnostic methods. FNA is of particular value in the diagnosis of pleural mesothelioma in patients who do not present with a pleural effusion. Obtaining material for cell block preparations, cytochemistry or ultrastructural study is generally necessary for definitive tumor typing.     

Cytologic findings of lymphangioleiomyomatosis in pleural effusion: a case report

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare disease occurring almost exclusively in females of reproductive age. Patients usually present with insidious and progressive dyspnea. Episodes of hemoptysis, pneumothorax, and chylothorax may occur, and the patient progresses to eventual respiratory failure and death. The characteristic pathologic finding is proliferation of immature smooth muscle cells (LAM cells) in the lungs, lymphatics and lymph nodes of thorax and abdomen/ retroperitoneum. CASE: A 47-year-old woman with a 1-year history of LAM diagnosed on iliac lymph node biopsy presented with progressive dyspnea and pleural effusion. A chest tube was placed. The collected pleural fluid, which represented chylothorax, yielded cohesive clusters of cells consisting of 2 cell populations: an outer, discontinuous layer of flattened cells and an inner portion of ovoid spindle cells. By immunohistochemistry the inner cells stained with smooth muscle actin and were negative for keratin. CONCLUSION: The characteristic constellation of clinical findings and distinctive cytology in conjunction with immunohistochemistry staining can render the diagnosis of LAM in effusions.     

Anaplastic large cell lymphoma presenting as a pleural effusion and mimicking primary effusion lymphoma. A report of 2 cases

BACKGROUND: Systemic anaplastic large cell lymphoma (ALCL) is predominantly a nodal disease, but extranodal involvement can occur during the disease course or as the primary presentation. We report two rare cases of ALCL presenting with a pleural effusion, mimicking primary effusion lymphoma (PEL). CASES: Two patients, a 47-year-old woman and an 81-year-old man, presented with a pleural effusion for investigation. The pleural fluid contained abundant, large, lymphoid cells with marked nuclear atypia. These neoplastic cells strongly expressed CD30 and EMA and showed a T-cell phenotype (CD3+CD45RO+ for case 1 and CD4+ for case 2). Case 1, in addition, showed ALK1 expression. The tumor cells in both cases were negative for human herpes virus type 8 (HHV8) and Epstein-Barr virus (EBV). ALCL shows overlapping cytologic features with PEL, but the T-cell phenotype, ALK1 expression in case 1, lack of association with HHV8 and EBV, HIV seronegativity and subsequent discovery of nodal disease in case 2 were all in favor of ALCL over PEL. CONCLUSION: In rare cases a pleural effusion is the presenting feature of ALCL, and distinction from PEL depends on correlation with clinical findings, detailed immunophenotyping and study of the status of HHV8 and EBV.     

Primary rhabdomyosarcoma of the prostate. Diagnosis by needle biopsy and immunocytochemistry

Needle aspiration biopsy of the prostate in a patient who initially presented with multiple metastatic lesions in the lungs and a pleural effusion showed the presence of many malignant cells. These cells appeared either as noncohesive round and oval cells or as clusters. The cytologic impression that these were sarcoma cells was confirmed by subsequent histologic and immunochemical studies. By correlating the cytologic, histologic and immunochemical findings with the clinical findings, it was possible to determine that the primary site of the tumor in this patient was the prostate. Our experience in this case suggests that air-dried smears stained with Romanowsky stains are suitable and useful for cytodiagnosis.     

Cytologic features of pleural effusion in apocrine sweat gland carcinoma. A case report

BACKGROUND: Carcinoma arising in the apocrine sweat glands is very rare, and there are few reports of the cytologic features. We encountered a case of metastatic apocrine carcinoma in a pleural effusion. CASE: A 46-year-old male had a dark reddish nodule in the right axillary region that was diagnosed as apocrine carcinoma of skin appendage origin. Three years after wide resection and chemotherapy, widespread metastases developed with a massive pleural effusion. Needle aspiration fluid cytology contained clusters of adenocarcinoma. Some tumor cells had abundant cytoplasm or periodic acid-Schiff-positive, coarse granules. Decapitation secretion was occasionally found on the cell surface. Immunohistochemically, the tumor cells were often positive for BRST-2 and BRST-3. CONCLUSION: Cytologic features of metastatic apocrine sweat gland carcinoma show some characteristics of adenocarcinoma. Moreover, its definitive diagnosis in a pleural effusion can be made because of retaining the characteristics of apocrine sweat gland.     

Immunoglobulin crystal-storing histiocytosis in a pleural effusion from a woman with IgA kapa multiple myeloma: a case report

BACKGROUND: Crystal-storing histiocytosis (CSH) is a rare disorder occurring in patients with lymphoproliferative diseases, predominantly multiple myeloma and low grade B-cell lymphoma. This report presents the first case of CSH diagnosed on pleural fluid from a patient with multiple myeloma (MM). CASE: A 79-year-old women with IgA kappa MM underwent thoracocenthesis and thoracic drainage because of a pleural effusion. Cytologic and immunocytochemical examination of pleural fluid revealed abundant histiocytic, CD68-positive cells with prominent intracytoplasmic, needlelike, crystalloid inclusions showing strong immunopositivity for IgA heavy and kappa light chains. Identical crystals were observed on an extracellular background. No myeloma infiltration was detected. Two weeks later, examination of new pleural fluid from the patient showed a similar cytologic picture, but, in addition, isolated plasma cell features were identified. They were too few for a meaningful determination of clonality. The patient died I month after the CSH diagnosis. CONCLUSION: This case illustrates the value of cytologic examination of serous fluids from patients with plasma cell dyscrasias, not only to evaluate possible infectious or neoplastic causes but also to diagnose CSH.     

Endodermal sinus tumor of the mediastinum. Cytologic diagnosis on a pleural effusion

A primary mediastinal endodermal sinus tumor in a young man was diagnosed by cytologic examination of a pleural effusion. Subsequent evaluation revealed a greatly elevated serum alphafetoprotein (AFP); computed tomographic scan of the chest showed a large anterior mediastinal mass. Routine examination of the smears and cell block preparations revealed clusters of tumor cells with a few intracytoplasmic hyaline droplets. Immunohistochemical stains for AFP, alpha-1-antitrypsin and cytokeratin were positive in the tumor cells while stains for carcinoembryonic antigen and the beta subunit of human chorionic gonadotropin were negative. This supported the diagnosis of endodermal sinus tumor, a rare primary tumor within the mediastinum.     

Cytologic presentation of malignant mesothelioma in pleural effusions

1,601 pleural effusions were found to be malignant between 1976 and 1987. Among these were 26 (1.6% of the malignant effusions) mesothelioma. Only 2 cases showed pronounced cytologic features that made a definite diagnosis possible on cytologic criteria alone. In 20 cases diagnosis of mesothelioma was strongly suggested by the patient's history and cytology of the effusion was compatible with mesothelioma. In the other 4 cases special examinations (histo- and immunohistochemistry, electron microscopy) led to the final diagnosis. The cytologic features of mesothelioma and other examination techniques, needed to resolve the differential diagnosis of mesothelioma versus other neoplasm in pleural effusions, are discussed.     

Aspiration biopsy of osseous metastasis of retroperitoneal paraganglioma. Report of a case with cytologic features and differential diagnostic considerations

BACKGROUND: Paragangliomas are uncommon tumors, only 10% of which are malignant, as evidenced by metastatic disease. It is rare for paraganglioma to present with symptomatic osseous metastases. CASE: A retroperitoneal paraganglioma presented in a 52-year-old man as painful metastases in the rib and vertebrae. Fine needle aspiration (FNA) of a lumbar vertebral lesion showed cells arranged singly and in loose clusters with fragile, vacuolated or finely granular cytoplasm, marked anisonucleosis and mitoses. Rare zellballen-type structures and intranuclear inclusions were present. Immunohistochemical studies of a subsequent FNA core biopsy of the retroperitoneal mass showed strong immunoreactivity with chromogranin and negative staining for keratin; that was helpful in differentiating this tumor from others in the differential diagnosis. CONCLUSION: The cytologic diagnosis of paraganglioma is difficult as these tumors exhibit a plethora of features that overlap those of many other neoplasms. The diagnosis can be confirmed with appropriate immunohistochemical studies of corresponding core biopsies.     

Pleural effusion cytology in a case of cytophagic histiocytic panniculitis (subcutaneous panniculitic T-cell lymphoma). A case report

BACKGROUND: Cytophagic histiocytic panniculitis (CHP) presents with subcutaneous panniculitis associated with hemophagocytic syndrome. Many cases of CHP are now being classified as a natural disease progression of subcutaneous panniculitic T-cell lymphoma (SPTL). There have been no cytologic reports dealing with pleural aspirates in cases of CHP or SPTL. CASE: A pleural aspirate obtained from a 19-year-old female revealed lymphoma cells and hemophagocytic histiocytes. A skin biopsy specimen showed the presence of CD8-positive lymphoma cells in fat lobules associated with cytologically benign histiocytes with erythrophagocytosis and lymphophagocytosis. CONCLUSION: Hemophagocytic histiocytes were seen in the pleural effusion from a patient with SPTL.     

Retroperitoneal lymphangiomyomatosis diagnosed by fine needle aspiration: a case report

BACKGROUND: Lymphangiomyomatosis is a rare condition affecting women of childbearing age. It is characterized by an abnormal proliferation of smooth muscle cells around lymphatics, giving rise to blockage of the large lymphatics, including the thoracic duct, and resulting in chylothorax and/or chyloascitis. The lung is the most common site of involvement. Retroperitoneum and lymph nodes can be also involved. CASE: A 40-year-old woman presented with lower urinary tract symptoms after a history of trauma and was found to have a retroperitoneal mass. Fine needle aspiration cytologic examination of the milky fluid aspirated from the mass revealed a few cohesive, 3-dimensional clusters of medium-sized cells with scanty cytoplasm, and ovoid and hyperchromatic nuclei. The background contained numerous mature lymphocytes. Laparoscopy revealed a multicystic mass filled with milky fluid. Histologic examination confirmed the cytologic diagnosis of lymphangiomyomatosis. CONCLUSION: Fine needle aspiration of lymphangiomyomatosis can be performed if cohesive clusters and a lymphoid background are present in chylous-type fluid and provided that adequate clinical information is available.     

The positive pleural effusion. A retrospective study of cytopathologic diagnoses with autopsy confirmation.

We performed an investigation focusing on the distribution of tumor types responsible for positive pleural effusions in 143 patients who died of malignancy and underwent autopsy. The principal malignant tumors were lung carcinoma (41 cases, 51.2%) and pleural mesothelioma (23 cases, 28.7%) in males and breast carcinoma (24 cases, 38.2%) and lung carcinoma (13 cases, 20.6%) in females. Histologically, most of the cases belonged to the adenocarcinoma category. The first morphologic diagnosis was a cytologic one in 86 cases (60.1%), especially regarding lung cancer. In breast cancer a positive pleural effusion always preceded recurrent disease with a rapidly progressive course, even a long time after the initial surgery. The results of this study, based on both cytomorphologic features and postmortem data on the tumor sites, may be a useful working framework for the cytologist dealing with a positive pleural effusion.     

Immunocytochemical diagnosis of oat-cell carcinoma in pleural effusion

Immunocytochemical studies were performed in order to accurately recognize the origin of tumor cells in a pleural effusion from a patient with a mediastinal mass. The immunoalkaline phosphatase technique was employed to examine several cell-specific surface antigens via their specific monoclonal antibodies. The tumor cells in this case did not have any demonstrable antigens of the human blood cells but did have abundance of an antigen specific for the tumor cells of oat-cell carcinoma. This diagnosis obtained by the immunochemical studies was confirmed by both cytologic and clinical findings. Immunocytochemical studies may become a mainstay for specific cytodiagnosis in the future.     

Pneumocystis carinii in pleural fluid. The cytologic appearance

We describe the cytologic appearance of Pneumocystis carinii in pleural fluid of a patient with acquired immunodeficiency syndrome and a rapidly accumulating pleural effusion. The diagnosis of P carinii infection was made by examination of air-dried, Diff-Quik-stained Cytospin preparations of the pleural fluid. The diagnostic appearances of P carinii organisms stained by this method and by the Papanicolaou stain are reviewed. The unusual predominance of the trophozoite forms of the organism in this case made Diff-Quik an ideal special stain for identifying the organisms. Furthermore, this case illustrates a novel presentation of P carinii infection and suggests that P carinii should be considered an etiologic agent in the differential diagnosis of pleural effusion in an immunocompromised host.     

Intraabdominal desmoplastic small cell tumor mimicking adenocarcinoma. A case report

BACKGROUND: Fine needle aspirates and washings from intraabdominal desmoplastic small cell tumors (IADSCTs) are rarely encountered by pathologists. Immunocytochemical examination of histologic material is usually necessary for a definitive diagnosis. CASE: A 23-year-old man presented with abdominal pain, ascites and bilateral pleural effusion. Examination of ascitic fluid suggested adenocarcinoma, but histologic and immunocytochemical examination of surgically resected tissue showed features of an IADSCT. CONCLUSION: This case is a reminder that IADSCT should be included in the differential diagnosis of intraabdominal neoplasms and that its diagnosis in cytologic preparations requires a high index of suspicion. In particular, this case appears to be the first reported example of IADSCT mimicking adenocarcinoma.