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Temporal lobe tumor demonstrating ganglioglioma and pleomorphic xanthoastrocytoma components. Case report.

The case is reported of a 16-year-old boy with a left temporal lobe tumor composed of a ganglioglioma and a pleomorphic xanthoastrocytoma. Histologically, the tumor had two different components. One component involved the cortex of the left posterior temporal lobe and showed an aggregation of neuronal cells with an astroglial stroma. Ultrastructurally, numerous dense-cored vesicles, diagnosed as ganglioglioma, were found in the neuronal cells. The other component involved the adjacent cortex and white matter of the left anterior temporal lobe and the surrounding subarachnoid space. This was composed of pleomorphic cells with many multinucleated giant cells and occasional foamy cells. Most of the tumor cells were positive for glial fibrillary acidic protein. These features correspond well to earlier descriptions of pleomorphic xanthoastrocytoma. At 24 months following total tumor extirpation, the patient is alive and has had no evidence of tumor recurrence.

Adolescent↗

Pleomorphic xanthoastrocytoma in the spinal cord. Case report.

The authors present the clinical, radiological, and pathological features of a patient with a recurrent pleomorphic xanthoastrocytoma located in the spinal cord. This is the first report of a pleomorphic xanthoastrocytoma at this location; until now, only cerebral supratentorial pleomorphic xanthoastrocytomas have been described. The treatment of this case is discussed.

Aged↗

A new human pleomorphic rhabdomyosarcoma cell-line, HS-RMS-1, exhibiting MyoD1 and myogenin.

A number of human cell lines derived from alveolar or embryonal rhabdomyosarcoma (RMS) have been described. To our knowledge, however, no cell line established from pleomorphic RMS has been reported. We describe here the establishment and characterization of a new human cell line, HS-RMS-1, which originated from a typical pleomorphic RMS arising in the gluteal muscle of a 26-year-old man. HS-RMS-1 cells had pseudotetraploid complex karyotypes with no specific abnormalities. Both in vitro and in vivo the cells on light microscopic examination exhibited pleomorphic features with immunopositive reaction for myogenic antigens including MyoD1 and myogenin, although no Z band-like structures were detected electron-microscopically. RT-PCR demonstrated the expression of MyoD1 and myogenin in HS-RMS-1 cells at the mRNA level, and direct sequencing analysis revealed cDNAs of MyoD1 and myogenin identical to those previously reported. This cell line, HS-RMS-1, established from pleomophic RMS will be useful for further studies including the molecular aspects of human RMS.

Adult↗

Pleomorphic giant cell carcinoma of the esophagus with coexpression of cytokeratin and vimentin and neuroendocrine differentiation.

A pleomorphic (giant cell) carcinoma of the esophagus is reported in a 52-year-old man who had dysphagia and weakness. The 8-cm-high vegetating tumor consisted of solid sheets of poorly cohesive epithelioid cells broken into clusters by strands of stroma. Numerous giant cells showing phagocytic phenomenon were present. Immunochemical analyses demonstrated the epithelial origin of the neoplasm, although most of the tumor cells strongly expressed vimentin. Numerous tumor cells expressed synaptophysin. Neurosecretory granules were detected in some tumor cells on electron microscopic examination. The patient died 4 months after he became symptomatic. As far as we can ascertain, this is the first case report describing a pleomorphic carcinoma arising in the esophagus. This poorly differentiated carcinoma might be of neuroendocrine differentiation. In the esophagus, pleomorphic carcinoma must be distinguished from polypoid tumors such as carcinosarcoma and malignant melanoma.

Biomarkers, Tumor↗

Combined pleomorphic xanthoastrocytoma-ganglioglioma of the cerebellum.

Combined pleomorphic xanthoastrocytoma-ganglioma is a rare neoplasm, occurring in patients younger than 30 years. The clinical course of these tumors is difficult to predict because of their rarity. We report a case of combined pleomorphic xanthoastrocytoma-ganglioma that, in addition to the patient's age, is unusual in several respects. The lesion was located in the cerebellar vermis of a 60-year-old man and was radiographically solid. Histologically, there was an admixture of markedly pleomorphic astrocytic cells and neoplastic ganglion cells, with permeation of the overlying leptomeninges and surrounding cerebellum. In addition, there was focal capillary endothelial proliferation. There was no necrosis, and mitotic activity was rare at 1 mitotic figure per 40 high-power fields. The patient underwent a near gross total resection and postoperative radiotherapy and remains well through 16 months of follow-up.

Astrocytoma↗

Pleomorphic giant cell carcinoma of the prostate.

We report the clinical and pathologic features of 2 cases of pleomorphic giant cell carcinoma of the prostate. One case was found at autopsy in a 77-year-old man and was composed of high-grade prostatic adenocarcinoma with prominent anaplastic giant cells. The patient presented with metastases to multiple retroperitoneal lymph nodes, liver, and lumbar vertebrae. The second case occurred in a 45-year-old man who underwent transurethral resection of the prostate and was found to have high-grade prostatic adenocarcinoma with an extensive anaplastic giant cell component. The patient presented with distant metastases and died within 9 months. Both regular adenocarcinoma and anaplastic giant tumor cells displayed cytoplasmic immunoreactivity for prostate-specific antigen, prostatic acid phosphatase, and keratin AE1/AE3; in one case, scattered cells were also positive for chromogranin and epithelial membrane antigen. Pleomorphic giant cell carcinoma is a rare variant of prostatic adenocarcinoma with a poor prognosis that should be considered in the differential diagnosis of prostatic pleomorphic tumors.

Adenocarcinoma↗

Primary pleomorphic rhabdomyosarcoma of the liver: a case report.

Primary intrahepatic rhabdomyosarcoma is extremely rare in children. We describe a case of pleomorphic rhabdomyosarcoma originating from the liver in an eight-year-old boy presenting with abdominal pain, spiking fever and a rapidly growing abdominal mass for one week. Preoperative imaging studies revealed a large solid tumor in the right lobe of the liver without any tumor elsewhere in the body. Serological study was negative for HBsAg and positive for anti-HBs. Biochemical tests including serum glutamic-oxalacetic and glutamic-pyruvic transaminase, alkaline phosphatase, bilirubin and alpha-fetoprotein were all within normal limits. The tumor was removed by an extended right hepatectomy. Histological and immunohistochemical examination confirmed a pleomorphic rhabdomyosarcoma. The patient eventually succumbed to tumor recurrence with massive internal hemorrhage two months after resection of the tumor. This is the first report of primary pleomorphic rhabdomyosarcoma of the liver in children. Abdominal ultrasonography, computed tomography scan and angiography are valuable for preoperative planning but the imaging findings are non-specific. Pathological examination with immunohistochemical stains remains the most important method in arriving at the exact diagnosis. The poor prognosis and early death of most previously reported cases imply the need for investigation of a more effective treatment method of this uncommon tumor.

Child↗

Expression of macrophage/histiocytic antigens in pleomorphic xanthoastrocytomas.

Pleomorphic xanthoastrocytoma (PXA) is a rare variant of a superficial cerebral astrocytoma characterised by distinct clinical and histological features. Its derivation from subpial astrocytes has been proposed, although the capacity of neoplastic cells for expression of different immunohistochemical markers is still under debate. These immunohistochemical studies were performed on eight cases of PXA in order to evaluate the expression and co-expression of glial and macrophage/histiocytic markers in various tumour cell populations. The expression of antigens was examined with the use of single- and double-immunolabelling methods for GFAP, vimentin, LCA, CD68, HLA-class II and MAC 387. All the cases of PXA showed variable immunoreactivity to GFAP, both in spindle-shaped and pleomorphic lipidised tumour cells. A subset of neoplastic cells was stained strongly with HLA-class II monoclonal antibody and with antibody to CD68. The reactivity to LCA and MAC 387 was absent in neoplastic cells, while it was easily evidenced in the non-neoplastic infiltrative component. The immunohistochemical double staining demonstrated the co-expression of GFAP and HLA-class II or CD68 antigens in the cytoplasm of individual neoplastic cells, including large pleomorphic, lipid-laden ones. It seems that tumour cells in PXAs derived from subpial astrocytes reveal monocyte/macrophage immunophenotype and demonstrate the capability of functional behaviour as mesenchymal cells with phagocytic activities. The variability in expression of antigens related to glial and monocytic/macrophage differentiation stressed the immunophenotypic heterogeneity of tumour cells in PXAs.

Adolescent↗

[Malignant pleomorphic adenoma of salivary glands:A clinicopathologic study of 83 cases]

This paper presented the clinicopathologic features of 83 cases of malignant pleomorphic adenomas(MPA) of the salivary glands.The average age of the patients was 53 years old.The ratio from male to female was 1.24 to 1.Thirty nine tumors located in parotid glands,twenty in submandibular glands and seventeen in palate.Histopathologically,the malignant component of 76 tumors was identified as carcinoma and 7 tumours as carcinosarcoma both in various types.The statistic analysis showed the factors which influenced the prognosis(P>0.05) included 1. location,2. histologic subtype of malignant component,3.differentiation grade,4.infiltration status,and 5.with or without lymph node metastasis in the initial operation.The prognosis of MPA was more ominous than that of/the conventional types of carcinoma,with only 52.7% of year survival rate.The authors concluded that the pleomorphic adenoma was the pre-cancerous lesion of MPA.Therefore,it is necessary to resect the pleomorphic adenoma as early as possible in order to prevent the occurence of the MPA.

Journal Article↗

Combined osteoclastic giant cell and pleomorphic giant cell tumor of the pancreas: a rarity. An immunohistochemical analysis and review of the literature.

CONTEXT: The combination of an osteoclastic giant cell tumor and a pleomorphic giant cell carcinoma of the pancreas is distinctly unusual and is associated with an adverse outcome. The origin of these two components within a tumor has long been debated based on the immunohistochemical and ultra-structural analysis. CASE REPORT: Herein we describe a tumor with amalgamation of these two distinct histomorphologies along with a minute focus of well-differentiated ductal adenocarcinoma (on multiple sections) in a 50-year male. On immunohistochemical analysis, osteoclastic giant cells were reactive for CD68 and vimentin confirming histiocytic/mesenchymal derivation whereas pleomorphic giant cells and mononuclear cells were reactive for cytokeratin which proved their epithelial nature. CONCLUSIONS: Although the present case had an equal proportion of both components, it is very important to correctly assess the predominant histology since osteoclastic giant cell tumor has a better prognosis as compared to the more aggressive pleomorphic giant cell carcinoma component.

Adult↗

Structural alterations of the c-mos locus in benign pleomorphic adenomas with chromosome abnormalities of 8q12.

Recent mapping studies have assigned the human c-mos proto-oncogene to chromosome 8, bands q11-12. This region is frequently affected by chromosomal translocations in benign pleomorphic adenomas of the salivary glands. Using Southern blot analysis we report here that the c-mos gene and its flanking sequences are structurally altered in pleomorphic adenomas with chromosomal rearrangements of 8q12. Rearrangements were detected in two out of 23 tumors. Restriction fragment analysis indicated that the rearrangements were due to multiple, subtle mutations involving the c-mos open reading frame and its flanking sequences. There was no direct evidence of translocation of mos in any of the tumors. Control DNAs from the two patients showed a normal restriction pattern for all enzymes tested, indicating that the rearrangements are tumor specific. Collectively, our cytogenetic and molecular data suggest involvement of the c-mos gene in the pathogenesis of pleomorphic adenomas.

Adenoma↗

[Immunohistochemical study of monomorphic adenoma (clear cell and basal cell) and pleomorphic adenoma of the parotid gland].

The present paper reports the results of an immunohistochemical study of 6 cases of monomorphic (2 clear cell, 4 basal cell) and 12 cases of pleomorphic adenomas in the parotid gland. The surgical specimens, embedded in paraffin, were tested by antisera vs. epithelial cell markers with epithelial membrane antigen (EMA) and cytokeratins (CK). They were also tested vs. myoepithelial and non-epithelial markers with S-100 protein and Glial Fibrillary Acidic Protein (GFAP). In pleomorphic adenomas, positive cells were found for all the tested antigens. Positive cell distribution showed a marked variability from case to case and even within each area, depending on cell morphology. In particular, areas showing tubular structures were constantly EMA-positive and CK-positive in more than 50% of the cases. In epithelioid areas EMA was negative while many cells were variably positive for CK, S-100 and GFAP. At times, the same cell proved positive to two different antigens. In those fuso-stellate (myxoid) cell areas considered to be of myoepithelial origin, cells were slightly CK-positive and widely GFAP and S-100 positive. In basal cell adenomas, widespread CK positivity was uniformly present while rare, focal positivity was evidenced for EMA and widespread non reactivity was found for S-100 and GFAP. In clear cell adenomas focal CK expression was found in the tubular lumina while clear cells, of myoepithelial origin, were steadily S-100 positive and GFAP-negative. It was noted that a sort of S-100/GFAP positive myoepithelial cell with fuso-stellate morphology was present in pleomorphic adenomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenoma↗

[Pleomorphic xanthoastrocytoma--a case report].

Pleomorphic xanthoastrocytoma (PXA) is a very rare and special kind of astrocytoma. Most of the patients have a relatively favorable prognosis despite its bizarre and pleomorphic cells. A 13 year old girl with PXA in the right occipitoparietal region is reported. Under light microscope, the tumor was markedly pleomorphic; mitosis was occasionally encountered and necrosis was absent. Immunohistochemically, cytoplasm of the tumor cells was positive for GFAP stain. Under electron microscope, many tumor cells contained glial filaments and large lipid vacuoles. Pericellular basal laminae could be found about some tumor cells, indicating a possible origin from subpial astrocyte. The clinicopathologic features, histogenesis, differential diagnosis and prognosis of PXA are discussed with review of literature.

Adolescent↗

Correlation of c-myc expression with nuclear pleomorphism in human renal cell carcinoma.

The expression of the c-myc gene product in renal cell carcinomas was examined by immunostaining with monoclonal antibody (mAb) MYC-1. The effects of preservation and fixation of tissues on staining were first examined. In cryostat sections fixed with 4% buffered formalin for 15 min, staining was observed in the nucleus. On the other hand, in paraffin sections after fixation with 10% formalin, staining was observed in the cytoplasm, but not in the nucleus. Because c-myc protein has been shown to be a nuclear protein, the finding that c-myc protein was not detectable in the nucleus appeared to be due to the preservation or fixation procedures used. Therefore, cryostat sections fixed with 4% formalin were used to investigate the correlation between the reaction of MYC-1 mAb and nuclear pleomorphism in primary and metastatic renal cell carcinomas. Among 41 primary tumors, positive staining was observed in 2 of 17 tumors (12%) of grade 1, 17 of 21 (81%) of grade 2, and all 3 (100%) of grade 3. Among 17 metastatic tumors, positive staining was not observed in any of the 5 (0%) of grade 1 but was observed in 2 of 4 (50%) of grade 2 and all 8 (100%) of grade 3. Thus, the frequency of the positive reaction with MYC-1 mAb was correlated with nuclear pleomorphism in primary and metastatic renal cell carcinomas. The reaction of Ki-67 mAb, which recognized a nuclear antigen present in proliferating cells, was also correlated with nuclear pleomorphism. These findings suggest that the c-myc gene product plays a role in cell proliferation in renal cell carcinomas.

Antibodies, Monoclonal↗

Pleomorphic fibroma of the skin: a benign neoplasm with cytologic atypia. A clinicopathologic study of eight cases.

A clinicopathologic study of eight examples of polypoid and dome-shaped cutaneous fibrous lesions with sparse cellularity but striking nuclear atypia and rare mitotic figures is presented. Positive immunohistochemical staining for vimentin and actin supported the fibroblastic nature of these lesions. All eight cases were adults whose ages ranged from 33 to 67 years (mean 52 years). Five were women and three were men. Five lesions were located on extremities, two on the trunk, and one on the face and they measured from 4 to 16 mm in greatest dimension. The lesions were clinically followed from 4 months to 5 years. They all showed benign clinical behavior, with only one local recurrence in a lesion that had been incompletely removed. The nuclear atypia seen in these fibrous lesions may be similar to that which occurs in other benign mesenchymal neoplasms, such as pleomorphic lipoma, pleomorphic leiomyoma, ancient schwannoma, and variants of dermatofibroma with atypical cells. We suggest that "pleomorphic fibroma" is an appropriate term for this lesion based on its histologic differentiation, cytologic atypia, and benign clinical course.

Adult↗

Postradiation pleomorphic malignant fibrous histiocytoma of the breast.

A case of primary malignant fibrous histiocytoma (MFH) of the breast occurring two years after surgical excision and radiation therapy for a carcinoma of the left breast is reported. Fine needle aspiration was positive for malignant cells, consistent with a pleomorphic sarcoma. Cytologic examination revealed giant cells with marked pleomorphism. Some cells showed single large nuclei with cytoplasmic vacuoles while others revealed multinucleation with foamy cytoplasm, phagocytosed erythrocytes and cellular debris. These findings are considered useful in the cytologic diagnosis of the pleomorphic variant of MFH.

Adult↗

Histogenesis of benign pleomorphic mesenchymal tumours.

Benign pleomorphic mesenchymal tumours (lipoma, fibroblastoma) are defined by large cells with multiple peripheral nuclei reminding of florets. Similar cells were previously detected by Mirejovský as a reactive phenomenon in polypous processes. We assume that these cells may occur not only in pseudotumours but also in tumours with stromal conditions similar to those in polyps. Therefore pleomorphic giant cell are of no value for assessment of the histogenesis of tumours. Nevertheless, the pleomorphic giant cell reaction is important in practice because it is not to be mistaken for tumorous atypia.

Fibroma↗

[Pleomorphic carcinoma of the pancreas--report of 3 cases].

The incidence of pleomorphic carcinoma of the pancreas is low. In 34 cases of non-endocrinic cancer of the pancreas detected pathologically by operation and autopsy in our hospital from 1961 to 1984, 3 were pleomorphic carcinoma, comprising 8.8%. In histology, the tumor was clearly characterized by the bizarre uninuclear and multinuclear giant cells with abundant eosinophilic cytoplasm and malignant spindle cells. In these 3 cases, the tumor was originated from the acinic epithelium in 2 and from the ductal epithelium in 1. This tumor should be differentiated from pleomorphic rhabdomyosarcoma, liposarcoma, fibrosarcoma and lymphosarcoma, etc.

Adult↗