Fine-needle aspiration biopsy diagnosis of extrarenal malignant rhabdoid tumor

Two cases of extrarenal malignant rhabdoid tumors are presented in which diagnosis was suggested by fine-needle aspiration biopsy and confirmed by histologic and electron microscopic examination. Fine-needle aspiration smears in both cases revealed round to polygonal cells with vesicular nuclei and prominent nucleoli. Several tumor cells contained cytoplasmic inclusions composed of intermediate filaments. A majority of the tumor cells stained strongly for vimentin and cytokeratin. Electron microscopic examination revealed many cells with large aggregates of intermediate filaments corresponding to the cytoplasmic inclusions. Fine-needle aspiration biopsy may be used for diagnosing malignant rhabdoid tumor. The diagnosis may be further confirmed by immunohistochemistry and electron microscopy. Diagn Cytopathol 1994;11:271–276. © 1994 Wiley-Liss, Inc.     

膀胱软组织恶性肿瘤

原发于膀胱的肿瘤大多为上皮性肿瘤,其中恶性者多数为尿路上皮癌,而软组织来源的肿瘤较少见.之前我们已经对膀胱良性软组织肿瘤的临床病理学、免疫组织化学特点和鉴别诊断进行过详细阐述~[1],本文拟就膀胱的恶性软组织肿瘤的相应特点作一介绍.     

Fine-needle aspiration biopsy diagnosis of malignant rhabdoid tumor of the kidney

A series of six fine-needle aspiration biopsies from three patients with malignant rhabdoid tumor of the kidney is reviewed. The smears revealed round, polygonal, and irregularly shaped cells with large nuclei and prominent nucleoli. Some of the tumor cells contained light pink to purple cytoplasmic inclusions that correlated with the eosinophilic inclusions seen in histopathologic sections and filamentous cytoplasmic aggregates noted on ultrastructural examination. Diagnosis of malignant rhabdoid tumor of the kidney may be suggested from aspiration biopsy smears; however, further confirmation of the diagnosis by histologic or ultrastructural examination is desirable.     

Ultrastructure of malignant rhabdoid tumor of the kidney: A distinctive renal tumor of children

An unusual and highly malignant childhood renal tumor has been noted among the specimens of the National Wilms' Tumor Study. Prominent nucleoli, PAS positive cytoplasmic inclusions, and light microscopic features suggestive of rhabdomyoblastic differentiation are hallmarks of this tumor. Ultrastructural examination of 11 specimens revealed the filamentous nature of the cytoplasmic inclusions, some features of epithelial cells, but no diagnostic rhabdomyoblastic elements. The similarity of the filamentous cytoplasmic inclusions to those of some APUD tumors may suggest a neural crest origin of the renal tumor. Alternatively the filamentous masses may have no histogenetic significance but may serve as a useful marker for this prognostically unfavorable tumor. Because of its striking light microscopic resemblance to rhabdomyosarcoma but lack of acceptable rhabdomyoblastic ultrastructural features, we refer to this tumor as malignant rhabdoid tumor of the kidney.     

直肠原发性恶性横纹肌样瘤一例

患者女,23岁.因大便性状改变及便血1个月余入院.1个月前无明显诱因出现便秘,呈羊粪状,外附血迹,色红,量少,偶阵发性腹痛,并逐渐加重,出现水样便.体检肛诊:距肛门5 cm左右直肠前壁可扪及一肿块,质硬,表面凹凸不平.     

Malignant rhabdoid tumor: a highly malignant childhood tumor with minimal karyotypic changes

Malignant rhabdoid tumors (MRT) are rare; thus very few cytogenetic studies of this type of tumor have been performed. We report the results of cytogenetic studies of 10 MRTs from various anatomic primary sites. Six cases had normal diploid karyotypes with no detectable rearrangements or aneuploidy except for occasional tetraploid cells. In 4 of these cases the tumor phenotype was verified by electron microscopic studies. In a seventh case only normal cells were identified in short-term culture, but a del(13)(q14) appeared after 4 months in culture. A soft tissue MRT contained a translocation, t(8;15)(q12;p11), and a liver MRT contained a del(3)(q21) or t(3;?)(q21;?). The single case of a primary brain MRT had monosomy 22 with deletion of part of the remaining chromosome 22. Our findings indicate that visible chromosomal rearrangements occur in fewer than half of MRTs. When combined with other reported series, our study indicates that monosomy 22 is a non-random chromosomal abnormality in primary MRT of the brain.     

Epithelioid variant of malignant peripheral nerve sheath tumor (malignant schwannoma) of the urinary bladder.

Sarcoma represents less than 2% of all neoplasms diagnosed or recognized in effusions. Epithelioid peripheral nerve sheath tumor is a rare tumor that is difficult to differentiate from other epithelioid tumors without the use of ancillary studies. A 39-year-old paraplegic man presented with hematuria and a bladder mass that extended to involve the pelvic peritoneum. Light microscopy using hematoxylin-eosin, Papanicolaou, and immunohistochemical stains as well as transmission electron microscopy showed features of epithelioid malignant peripheral nerve sheath tumor with rhabdoid features and an accompanying eosinophilic infiltrate. Cytologic smears confirmed the similarities between the primary tumor in the bladder and the cells in the pelvic fluid and excluded the possibility of reactive changes related to postsurgical radiation. Ancillary studies were critical in narrowing the differential diagnoses and reaching the final conclusion.     

Urothelial tumor of the urinary bladder: immunohistochemical features

Morphological and clinical characteristics of the urinary bladder cancers (UBC) are important diagnostics and prognostic criteria, however the possibilities of biopsy using/for prognosis of recidivation or efficiency of UBC treatment are limited. The most popular diagnostic and prognostic immunohistochemical markers are the regulators of cell cycle (P53, P21, Ki-67) and cytokeratins. In order to revealed immunohistochemical criteria, objectively reflected the malignancy of UBC, we studied the expression level of P53, proliferative index of Ki-67 and the malignant of UBC according to CK20 in the biopsy of 32 patients with superficial UBC. The immunohistochemical reaction with antibody to P53, CK20 and Ki-67 was carried out at paraffin slices. We detected differences of P53 expression levels at carcinomas with high and low malignancy rate. The expression levels of CK20 and the Ki-67 proliferative index were different between Ta and T1-T2 cancers (p = 0.006). Intensity of nuclear staining with P53 antibodies could be use for estimation of the UBC malignancy rate. Pathological type of CD20 expression and high percentage of Ki-67-positive staining nucleus are evidence of the invasive urothelial tumors. The using of P53, Ki-67 and CD20 could be recommended for pathohistological investigation of biopsy and surgical material of UBC to diagnostic objectification and prognosis of its clinical course.     

Epithelioid sarcoma or malignant rhabdoid tumor of soft tissue? Epithelioid immunophenotype and rhabdoid karyotype

Two children and one young adult with extremity sarcomas demonstrating an aggressive clinical behavior are described. Histologically, all three tumors displayed features compatible with a deep-seated epithelioid sarcoma or with a malignant rhabdoid tumor of soft tissue. Immunohistologically, both vimentin and epithelial antigens were demonstrated; however, no desmin was detectable. In all three cases, the DNA profile was diploid. In one case, a trisomy of chromosome 2 was found in the tumor cells, a phenomenon also observed in embryonal rhabdomyosarcomas. It is concluded that, although the immunohistologic findings support the epithelioid character of the tumors, this chromosomal finding suggests a relationship with rhabdomyosarcomas and justifies the designation of rhabdoid.     

Pseudosarcomatous fibromyxoid tumor of the urinary bladder

The light-microscopic, immunohistochemical, and ultrastructural features of two examples of a pseudosarcomatous fibromyxoid tumor of the urinary bladder are reported. Both patients were women, 56 and 52 years old. Gross hematuria was the chief complaint and occurred for two days and two weeks, respectively, before presentation. Cystoscopy revealed a 2 X 1 X 1 cm mass located at the dome in case 1 and a 4 X 3 X 3 cm mass at the left posterior lateral wall in case 2. Microscopically, the lesions were composed of spindle, plump, or stellated fibroblast-like cells embedded in myxoid stroma with little collagen; mitotic figures were about 2 per 10 high-power fields, and both cases showed encroachment of the superficial muscle bundles. Because of bizarre spindle cell proliferation, occasional mitoses, and invasion to the underlying muscle, these lesions were initially diagnosed as embryonal rhabdomyosarcoma. However, follow-up examination disclosed the benign nature of these lesions. There was no previous instrumentation or surgery on the genitourinary tract. Immunohistochemical and ultrastructural studies revealed the fibroblastic-myofibroblastic nature of these lesions. These cases illustrate that clinicopathologic correlation is essential to define certain pseudosarcomatous lesions.     

Malignant glomus tumor of the urinary bladder

We present a case of a malignant glomus tumor arising in the urinary bladder of a 57-year-old woman with metastatic pulmonary nodules who died 2 months later. Pathologically and clinically confirmed malignant glomus tumors are exceedingly rare, especially those that arise in the visceral organs. The present case retained its architectural similarity to a benign glomus tumor and consisted of sheets of highly malignant round cells showing cytoplasmic positivity for smooth muscle actin. On reticulin histochemical staining, we found that reticulum fibrils surrounded individual tumor cells, suggesting cellular investment by basement membrane. We discuss the concept of malignant glomus tumors and emphasize the criteria that distinguish them from other malignant tumors.     

Primary malignant melanoma of the urinary bladder.

Primary malignant melanoma is an unusual neoplasm in the urinary bladder that is infrequently found in association with melanosis. We report a case of bladder-invasive malignant melanoma with melanosis in which the melanosis exhibited melanocytic atypia extending through to melanoma in situ and was diagnosed by immunohistochemical techniques using a monoclonal antibody, HMB-45. To our knowledge, such findings have not been reported previously.     

Solitary fibrous tumor of the urinary bladder

Solitary fibrous tumor (SFT) is a distinct spindle cell tumor arising mainly in the pleura, however, SFT of extra-pleural sites had been reported. To our knowledge, five cases of the urinary bladder SFT had been reported. We have recently experienced a case of SFT of the urinary bladder in a 56-year- old man who was admitted to the hospital with a 4-month history of voiding difficulty, frequency, and residual urine sensation. A computerized tomography scan revealed a 12cm sized intravesical mass. The mass was marginally excised under the impression of it being a benign spindle cell tumor by pre-operative needle biopsy. On gross examination, the mass was multinodular, creamy white, and the cut surface was fibrotic with a rubbery consistency. Microscopic examination revealed haphazardly arranged spindle cells with a lace-like deposition of inter- and pericellular collagen. Areas of increased cellularity show nuclear atypia and occasional mitoses (2-3/10HPF). The immunohistochemical staining revealed an intense CD34 and bcl-2 immunoreactivity. Although the exact biologic behavior of these tumors has not been clarified, most of the reported cases have undergone a benign clinical course.     

Cytokeratin subunits of inclusion bodies in rhabdoid cells: immunohistochemical and clinicopathological study of malignant rhabdoid tumor and epithelioid sarcoma

Extrarenal malignant rhabdoid tumor (MRT), which is recognized as being histologically similar to renal MRT, is characterized by the presence of "rhabdoid cell" (RC) and a highly aggressive biological behavior. Recently it has been proposed that "proximal variant" of epithelioid sarcoma (ES), whose morphology is similar to that of MRT, actually has a more aggressive clinical course than classical type ES. Detailed immunohistochemical analysis of cytokeratin (CK) subunits was performed in 3 cases of extrarenal MRT, 3 cases of renal MRT, and 11 cases of ES comprising 2 "proximal variants" and 9 classical types. Renal and extrarenal MRTs showed positive immunoreactivity for both CK8 and CK18. Classical type ESs were diffusely positive, not only for CK8 and CK18, but also for other cytokeratin subunits including CK4, 6, 10, 13, 16, 17, and "high-molecular-weight" CKs (CK1, 5, 10, and 14). On the other hand, proximal ES revealed limited immunohistochemical reactivity for cytokeratins, compared with classical ES. In conclusion, the inclusion bodies of RCs show immunoreactivity confined to CK8, CK18, and vimentin. Furthermore, ES has additional CK expressions, while proximal ES possesses characteristics intermediate between those of classical ES and those of external MRT.     

膀胱恶性颗粒细胞瘤一例

患者女,46岁。2000年3月在外院行“子宫肌瘤”手术,术中发现膀胱与阴道之间有一个巨大肿块,未切除,活检病理诊断：颗料肌母细胞瘤,低度恶性。单纯介入治疗2次出院。     

Primary carcinoid tumor of the urinary bladder.

A 62-year-old woman who presented with urinary frequency and microscopic hematuria was found to have a 1.2 x 1.0 x 0.6-cm polypoid carcinoid tumor of the urinary bladder. The tissue resected from the base after removal of the polypoid lesion disclosed a small focus of residual carcinoid tumor, associated with Brunn's epithelial nests, cystitis cystica, and cystitis glandularis. Tumor cells exhibited strong argyrophilia and weak argentaffinity. Immunohistochemical staining reactions were strongly positive for chromogranin and serotonin, and electron microscopy revealed characteristic dense-core granules. Flow cytometric evaluation revealed an aneuploid cell population with a DNA index of 1.20.     

Osteosarcoma of the urinary bladder with a prostatic cancer as a 2d primary tumor

Osteosarcomas of the urinary bladder are exceedingly rare neoplasms. Post mortem findings of such a tumor are described in a 78 year old man who also had a primary prostate cancer. Diagnostic principles as well as differential diagnosis are discussed and the literature concerning this tumor is reviewed.