Urethroplasty for balanitis xerotica obliterans

PURPOSE: To report a 20-month-old child with a rapidly growing dome-shaped red nodule on the left lower eyelid. The lesion was diagnosed clinically as an hemangioma, but microscopy disclosed an epithelioid Spitz nevus. METHOD: Case report. RESULT: Histopathologic examination of the excised lesion disclosed a Spitz nevus (benign juvenile melanoma) of a chiefly epithelioid cell type. CONCLUSIONS: The differential diagnosis of eyelid skin nodules in children should include Spitz nevus. This uncommon nevus has many cytologic features in common with nodular malignant melanoma. Histologically, it may be difficult to distinguish between nodular malignant melanoma and Spitz nevus.     

"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series

Eighteen examples of an unusual malignant soft-tissue neoplasm, the morphology of which ranged from that of "atypical" epithelioid sarcoma to that of a rhabdoid tumor or undifferentiated carcinoma (with transitional forms) are described. Patients included 11 males and seven females; their median age was 35.5 years with most patients aged 20 to 40 years. Development of a mass was the main presenting symptom. Six tumors developed in the pelvis and perineal region, four in the pubic region and vulva, three in the buttocks, one in the deep soft tissues of the left hip, one on the penis, one in left forearm, one in left axilla, and one on the occiput. Tumor size ranged from 1 to 20 cm (median, 4 cm). On microscopic examination, the tumor cells invaded the subcutaneous or deep soft tissues, had prominent epithelioid or rhabdoid features, had marked cytologic atypia, and grew in a multinodular pattern in half of the cases. Areas of necrosis were often seen. A granuloma-like pattern reminiscent of that observed in classic epithelioid sarcoma was observed in only two cases. Immunohistochemically, positivity for cytokeratin, epithelial membrane antigen, and vimentin was seen in all but one of the cases. Of 16 cases, 10 and eight tumors reacted with desmin and CD34, respectively; five of 15 reacted at least focally with smooth-muscle actin, whereas three of 13 and one of 10 reacted for HMB-45 and carcinoembryonic antigen, respectively. S-100 protein and CD31 yielded negative results. Seven tumors were investigated at the ultrastructural level, four of which showed prominent intracytoplasmic intermediate filament aggregates, often accumulating into paranuclear whorls, which is in keeping with the rhabdoid phenotype. Five tumors showed features of epithelial differentiation (i.e., tonofilament-like structures or desmosomes or both), whereas one tumor displayed features of myofibroblastic differentiation. Differential diagnoses include mainly conventional epithelioid sarcoma, extrarenal malignant rhabdoid tumor, epithelioid malignant peripheral nerve sheath tumor, melanoma, rhabdomyosarcoma, and undifferentiated carcinoma. Follow-up information on 14 patients (range, 4 months to 8 years; median, 19 months) revealed local recurrence in one case and metastatic dissemination in six patients, leading to death in five. In our opinion, the above-described neoplasms represent a usually "proximal-type" of epithelioid sarcoma. In contrast to the conventional, "distal-type" epithelioid sarcoma, the proximal variant is characterized by a predominantly large-cell, epithelioid cytomorphology, marked cytologic atypia, frequent occurrence of rhabdoid features, and lack of a granuloma-like pattern in most cases. It appears to be somewhat more aggressive (or at least metastasizes earlier) than usual epithelioid sarcoma.     

Gastrointestinal autonomic nerve tumor (plexosarcoma): report of a case with fine needle aspiration biopsy and histologic, immunocytochemical and ultrastructural study

BACKGROUND: Gastrointestinal stromal tumors (GISTs) encompass a large group of mesenchymal neoplasms that display common cytologic spindle-shaped morphology on light microscopy. Immunocytochemical and ultrastructural studies can demonstrate several patterns of differentiation. CASE: A 70-year-old male presented with two intraabdominal small bowel masses. The cytopathologic features of a fine needle aspiration biopsy (FNAB) included plump spindle cells in densely populated aggregates or in a fasciculated pattern, without significant pleomorphism. An epithelioid component in a lobular arrangement with abundant, eosinophilic cytoplasm was also noted. The nuclei were vesicular, with a very evident, eosinophilic nucleolus and finely distributed chromatin. Groups of loosely cohesive cells with slender, dendritic-like cytoplasm were evident. Immunocytochemical study of the embedded, fine needle aspirated fragments of the neoplasm demonstrated immunoreactivity for vimentin and neuron-specific enolase. Cytokeratin immunoreactivity or muscular, vascular, neuroendocrine or nerve sheath differentiation failed to be demonstrated. The cytologic and immunocytochemical findings correlated well with the histologic features of the neoplasm. The morphologic diagnosis was confirmed by ultrastructural study. CONCLUSION: FNAB and immunocytochemistry can be valuable in making the correct diagnosis between gastrointestinal stromal tumors.     

Role of cytopathology in the diagnosis of ocular malignancies

OBJECTIVE: To determine the positive predictive value of ocular cytologic specimens and to describe the cytopathologic findings encountered in ocular samples from patients with intraocular neoplasms. STUDY DESIGN: Intraocular fluids and ocular fine needle aspirates (FNAs) cytologically diagnosed as either suspicious or positive for malignancy during a 15-year period were reviewed, and follow-up was obtained. RESULTS: Seventeen patients with intraocular samples diagnosed as suspicious or positive for malignancy (9 vitreous, 6 anterior chamber, 3 FNAs) were identified. The mean patient age was 58 years (range, 3-91). Cytologic diagnoses included: lymphoma (5), suspicious for lymphoma (2), melanoma (6), suspicious for melanoma (2), carcinoma (2) and retinoblastoma (1). Clinical and/or surgical follow-up was available in 12 cases and was consistent with the presence of malignancy in all but one case, which proved to be fungal endophthalmitis. One of two patients with a cytologic diagnosis of carcinoma had melanoma on follow-up. Cytologic samples suspicious or positive for lymphoma showed single, large cells with scant cytoplasm and prominent nucleoli. Cytologic samples suspicious or positive for the epithelioid type of melanoma showed loosely cohesive groups or single cells, marked cellular pleomorphism, large nucleoli, scant to moderately abundant cytoplasm and variable amounts of melanin. Cytologic samples from spindle cell melanomas showed spindle cells without nuclear or cellular pleomorphism, without hyperchromasia, and with inconspicuous nucleoli and occasional nuclear grooves. Loose aggregates of small cells with hyperchromatic nuclei and scant cytoplasm characterized the retinoblastoma samples. CONCLUSION: The positive predictive value of intraocular fluid cytology was 92%. Reactive lymphoid processes may be difficult to differentiate from lymphoma and epithelioid melanoma from carcinoma in intraocular cytologic specimens.     

Prospective, randomized, double-blind study of high-dose aprotinin in pediatric cardiac operations

OBJECTIVE: To describe the characteristic cytologic features of fine needle aspirates (FNAs) of primary extragonadal germ cell tumors (PEGCTs). STUDY DESIGN: Thirteen patients with PEGCTs, including 2 seminomas, 2 mixed germ cell tumors, 3 immature teratomas, 1 choriocarcinoma and 5 yolk sac tumors (YSTs) were studied. The final diagnosis of PEGCT in all cases was established by histologic examination of the tumor tissues. Fine needle aspiration was done on either the primary tumor or metastatic foci. The aspirates were stained with one of the Romanovsky stains and Papanicolaou stain. RESULTS: Each type of PEGCT has its own morphologic characteristics. In seminoma, the tumor cells are large and noncohesive, with one to several distinct nucleoli; some lymphocytes are also present. YSTs show many pleomorphic cells with vacuoles in the cytoplasm and nuclei; tumor cells frequently aggregate in a microglandular or papillary pattern. Choriocarcinoma consists of syncytiotrophoblasts and cytotrophoblasts. The former are very large cells with eosinophilic cytoplasm, one to several nuclei and distinct nucleoli; the latter are medium-sized cells with vacuolated, basophilic cytoplasm and eccentric nuclei. Immature teratomas are composed of a mixture of cell types, including elongated epithelioid cells, mesenchymal cells and many large, naked, amorphous nuclei with a homogeneous chromatin pattern. Diagnosis of mixed germ cell tumor is difficult but can be made if two or more subtypes of tumor cells are observed in the FNA. CONCLUSION: Cytologic examination of FNAs of primary or metastatic lesions of PEGCTs, stained either with Romanovsky or Papanicolaou stain, is of diagnostic value for such diseases. The use of immunochemistry can help to confirm the cytologic impression.     

Fine needle aspiration of basal cell adenocarcinoma of the parotid gland. Report of a case with assessment of DNA ploidy in aspirates and tissue sections by image analysis

BACKGROUND: Basal cell adenocarcinoma of the parotid gland is a low grade malignant neoplasm. It has cytologic features of basal cell adenoma and a histologically infiltrative growth pattern of malignant tumors with perineural and vascular invasion. CASE: Fine needle aspiration biopsy findings of basal cell adenocarcinoma of the parotid gland in a 77-year-old male were supplemented by DNA ploidy analysis. CONCLUSION: No single cytologic feature was found to unequivocally distinguish this lesion from basal cell adenoma and/or solid variant of adenoid cystic carcinoma. Therefore, for diagnostic purposes, we grouped all three lesions under the term basal cell tumor. Evaluation of DNA content of tumor cells revealed diploid histograms in both cytologic material and paraffin-embedded tissue. Infiltrative tumor nests, the histologic basis for differentiating basal cell adenocarcinoma from adenoma, showed the same diploid pattern. Though DNA quantitation may not discriminate basal cell adenoma from basal cell adenocarcinoma, it may prove useful in separating them from adenoid cystic carcinoma, which is considered to be a tumor with high malignant potential.     

Prognostic features of cervical dysplasia associated with specific types of HPV DNA and cytologic features characteristic of HPV infection in dysplasia

OBJECTIVE: To study a possible etiologic relationship between the prognosis of uterine cervical dysplasia, association of type-specific human papillomavirus (HPV) DNA and cytologic features characteristic of HPV infection. STUDY DESIGN: Two hundred thirteen cases of uterine cervical dysplasia were selected in which follow-up survey for more than two years was possible. Frequency of the presence of HPV DNA in the DNA samples was determined by polymerase chain reaction. The cervical scrapings were also examined microscopically for the frequencies of cells with cytologic features characteristic of HPV infection. RESULTS: HPV was positive in 98 cases (46.0%). The high-risk type of HPV was detected at almost the same frequencies in both progressive and regressive states of dysplasia. Cytologic features were more evident in cells infected with the low-risk type of HPV. CONCLUSION: Involvement of an as-yet-unknown factor or factors coupled with infection with the high-risk type of HPV is implicated in the progression of uterine cervical dysplasia. Cytologic features characteristic of HPV infection may serve as a diagnostic marker for a favorable prognosis in dysplasia.     

Eruptive epithelioid hemangioendothelioma with spindle cells

A 39-year-old white man presented with four discrete dermal nodules in his right upper arm. Biopsy revealed superficial dermal well-circumscribed nodules composed of solid areas and vascular spaces lined by epithelioid endothelial cells and a similar nodule composed of spindle and epithelioid cells. A moderate mitotic count of 3-4 mitoses/10 hpf was present. Multiple lesions erupted 1 month later distally and proximally to the original lesions. Magnetic resonance imaging of the right arm demonstrated a lesion in the humerus. Biopsy of the humerus showed a vascular tumor with similar histologic features to the overlying skin lesions. The differential diagnosis included epithelioid vascular tumors, bacillary angiomatosis, pyogenic granuloma, and Kaposi sarcoma. Vascular lesions containing epithelioid and spindle cells span a spectrum from benign to malignant. We believe these tumors belong in the category of hemangioendothelioma and propose the name eruptive epithelioid hemangioendothelioma with spindle cells. Our case emphasizes that eruptive cutaneous vascular lesions do not always suggest immunosuppression or malignancy. Additionally, it highlights the association between epithelioid vascular lesions of the skin and bone.     

Kala-azar: liver fine needle aspiration findings in 23 cases presenting with a fever of unknown origin

OBJECTIVE: To study the role of liver fine needle aspiration (FNA) in the diagnosis of kala-azar with an atypical presentation. STUDY DESIGN: The study group consisted of 23 patients (aged 18-37). All were admitted to Shiraz University Hospitals for the investigation of fever of unknown origin. The immunofluorescent antibody titer for kala-azar was positive (> 1:256 dilution). However, routine abdominal sonography revealed multiple small, hypoechoic lesions in the liver, more suggestive of metastatic tumor or miliary tuberculosis. The lesions were aspirated for cytologic diagnosis. RESULTS: The smears revealed many atypical hepatocytes, groups of epithelioid histiocytes and, in 15 patients, macrophages containing few to many Leishman bodies, allowing a diagnosis of kala-azar. The liver needle biopsy specimens confirmed the cytologic diagnosis of kala-azar and liver cell atypia. The patients were given glucantime therapy, and 21 recovered; 1 patient was lost to follow-up, and 1 died during treatment. CONCLUSION: Liver FNA is a useful procedure for the diagnosis of kala-azar, particularly in cases with atypical clinical presentations.     

Therapy with blood and blood products]

Rare musculoskeletal tumors can be difficult to diagnose by light microscopy or immunohistochemistry. Electron microscopy can be of diagnostic assistance especially if histotype specific ultrastructural features exist. In particular, electron microscopy for uncommon sarcomas such as alveolar soft part sarcoma, parachordoma, atypical Ewing's sarcoma and epithelioid sarcoma may be the diagnostic modality of choice.     

Fine needle aspiration cytology of acinar cell carcinoma of the pancreas: a report of two cases

BACKGROUND: Fine needle aspiration in lieu of needle biopsy is widely used for the diagnosis of pancreatic neoplasms. The cytologic features of ductal carcinomas are well characterized, but the appearances of less common pancreatic neoplasms, such as acinar cell carcinoma (ACC), are not well described. CASES: We present the cytologic, histologic, immunocytochemical and ultrastructural features of two cases of ACC. The tumors occurred in a 36-year-old woman and 43-year-old man. The aspirate from one case contained neoplastic cells with smooth-contoured nuclei containing one or two prominent nucleoli. The aspirated material from the second case was necrotic, with numerous neutrophils and scattered nests of tumor cells similar to those present in the first case. Histologically, both tumors manifested solid and acinar patterns, and each contained some cells with periodic acid-Schiff-positive granules that were resistant to diastase. The neoplasms were immunochemically positive for trypsin and negative for neuroendocrine markers. Ultrastructurally, the aspirate from one case demonstrated apical microvilli, zymogenlike granules and abundant rough endoplasmic reticulum. CONCLUSION: Uncommon pancreatic neoplasms may be difficult to diagnose due to their cytologic and histologic subtleties. Supplemental studies including immunocytochemistry, cytochemistry and electron microscopy are important in facilitating their identification.     

Fine needle aspiration cytology of the kidney, renal pelvis, and adrenal

Fine needle aspiration biopsy for cytologic evaluation of mass lesions of the kidney, renal pelvis and adrenal is a safe economic and accurate diagnostic procedure. Tumors and inflammatory or degenerative lesions arising from these organs have characteristic cytologic features permitting their correct identification in the majority of cases.     

Simplified technique to measure mandibular range of motion

Bile peritonitis (BP) is a rare but acute and serious condition that may be associated with high mortality. BP results from generalized or localized leakage of bile into the peritoneal cavity. At best, radiologic studies may be suggestive of BP. We observed a spectrum of cytologic findings in aspirated peritoneal fluids (PF) from 3 patients with BP. Occasional bile pigment-laden macrophages, extracellular lakes of green stringy material admixed with variable numbers of histiocytes, mesothelial cells, and acute and chronic inflammatory cells, were seen. Numerous candida were present in one case. To the best of our knowledge, this is the first report describing the cytologic features of BP. Cytology is a simple, rapid, and cost-effective means of examining PF, and can therefore play a significant role in establishing the diagnosis of BP. Early recognition of BP can result in rapid, therapeutic intervention that may prevent significant morbidity and mortality.     

Fine-needle aspiration of histiocytic necrotizing lymphadenitis (Kikuchi's disease)

Fine-needle aspiration (FNA) of the lymph node was done in five patients with histiocytic necrotizing lymphadenitis (Kikuchi's disease). In four patients, the aspirates were found to have many small and large atypical lymphocytes, some reactive, phagocytic histiocytes, and intense extracellular debris. Neutrophils, plasma cells, or multinucleated giant cells were not seen. These cytologic findings were considered diagnostic for Kikuchi's disease. In one patient, the aspirate did not show significant histiocytosis or tissue necrosis and was considered nondiagnostic. In patients with both typical clinical features and characteristic cytologic findings in the lymph node aspirates, FNA of the lymph node alone will suffice for diagnosis. In those patients with typical clinical features but nondiagnostic findings in the FNA aspirates, the diagnosis of Kikuchi's disease may have to be established either on repeated nodal FNA or on lymph node biopsy.     

Fine needle aspiration biopsy of metastatic malignant melanoma with "rhabdoid" features. Frequency, cytologic features, pitfalls and ancillary studies

OBJECTIVE: To characterize the cytopathology of metastatic malignant melanoma (MM) with "rhabdoid" features, a recently described, rare morphologic variant of MM that can be incorrectly diagnosed in fine needle aspiration (FNA) biopsy. STUDY DESIGN: A retrospective review of all FNA biopsy material with the diagnosis of metastatic MM was performed at two institutions. Only cases with a predominant composition of cells that met criteria defined as "rhabdoid" morphology were selected for study. The cytomorphologic features, immunocytochemistry and clinical features of these cases were reviewed. RESULTS: Of 88 FNA cases previously diagnosed as metastatic MM, 4 (4.6%) had a predominance of cells with rhabdoid features. These cases consisted of scattered atypical cells having enlarged, eccentrically placed nuclei; prominent nucleoli; and a moderate amount of cytoplasm possessing round, globular inclusions in Papanicolaou- and Diff-Quik-stained smears. Immunochemistry showed strong S-100, HMB-45 and vimentin staining in two of four cases. CONCLUSIONS: Metastatic MM may present in FNA biopsy as a poorly differentiated malignancy with rhabdoid features, potentially leading to an incorrect cytologic diagnosis. MM must be considered when evaluating neoplasms with a rhabdoid phenotype. Correlation of the cytologic finding with the clinical history and immunohistochemical studies can help in diagnosing this morphologic variant.     

The efficacy of re-TUR after 3 weeks of initial TUR treatment for locally advanced bladder cancer

Epithelioid sarcomas are soft tissue tumors with an indolent, but potentially aggressive, clinical behavior. Distinction from other benign and malignant entities may be a diagnostic dilemma. In this study, we evaluate the presence of loss of heterozygosity (LOH) of chromosome 22q in tumor DNA from 13 epithelioid sarcomas, four epithelioid angiosarcomas, and two epithelioid hemangioendotheliomas, and investigate its possible role in diagnosis. LOH was detected in 6 of 10 (60%) of the informative epithelioid sarcomas. No allele loss was detected in the informative vascular tumors, three angiosarcomas, and two hemangioendotheliomas. Chromosome 22q carries the locus of a tumor suppressor gene, the neurofibromatosis 2 (NF2) gene, which has been shown to be lost or mutated in some NF2-related tumors, sporadic meningiomas, and vestibular schwannomas, as well as a few other tumors. Our data suggest that a region of chromosome 22q may be the locus of a tumor suppressor gene involved in the tumorigenesis of these neoplasms. Genetic alterations of yet-unknown tumor suppressor genes in this region, or even the NF2 tumor suppressor gene, may play a role in epithelioid sarcomas tumorigenesis. The fact that LOH was only detected in epithelioid sarcomas and not in the vascular tumors studied suggests a possible role for this marker in diagnosis.     

In situ hybridization: a possible diagnostic aid in leptomeningeal metastasis

In this review we discuss the clinical features and pathophysiology of leptomeningeal metastasis (LM), and elaborate on diagnostic tools for the detection of this serious complication of cancer. Because of the low sensitivity of the cytologic examination, new diagnostic approaches have been developed. The in situ hybridization technique may prove to be a reliable and early test for the detection of LM.     

Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma)

A series of 127 surgical specimens of epithelioid leiomyomatous tumors (leiomyoblastomas) of the gastric wall from the files of the Armed Forces Institute of Pathology (AFIP) were studied as to biologic behavior, morphogenesis, and histologic features of value in distinguishing benign and malignant variants. These tumors affect middle-aged men primarily and usually present with upper gastrointestinal bleeding or peptic ulcer-like symptoms. They are composed of a mixture of round epithelioid and spindle cells, many of which have clear cytoplasm. The cells are ensheathed by delicate reticular fibers. The presence of a perithelial or glomoid pattern in some tumors suggests a possible relationship to angiomyoma, glomus tumors, and "pericytoma." The epithelioid leiomyoma, the benign form, often arises in the mid- and distal stomach, especially on the anterior wall. Microscopically, it is recognized by the presence of large epithelioid cells and infrequent mitotic figures. Of 103 epithelioid leiomyomas, only one metastasized and thus was biologically malignant. The epithelioid leiomyosarcoma often arises in the proximal stomach and also distally, especially on the posterior wall. Two histologic types of epithelioid leiomyosarcoma are distinguished from the benign epithelioid leiomyoma by the small size of the cells and occasional higher mitotic counts. One sarcoma variant is a small cell caricature of the leiomyoma. The other is more anaplastic, assoicated with a loss of reticular fibers surrounding the cells and an alveolar arrangement. Epithelioid leiomyosarcomas are the most common type of gastric sarcoma. They are aggressive neoplasms; 63% metastasized, usually within 2 years after diagnosis.     

Epithelioise sarcoma. A myofibroblastoma tumor

A case of epithelioid sarcoma in a 48 year old man is described by the authors. The ultrastructural study showed the presence of abundant cellular elements showing some features closely similar to those of the so-called myofibroblasts. These were: folds in the nuclear membrane, abundant rough endoplasmic reticulum, thick bundles of microfilaments resembling those of the smooth muscle, junctional complexes and degenerative changes (lipid droplets and autophagic vacuoles). These findings suggest the possibility that the epithelioid sarcoma could be a neoplasia originating from the myofibroblasts.