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PubMed2002

Microarray-based copy number and expression profiling in dedifferentiated and pleomorphic liposarcoma.

Sixteen dedifferentiated and pleomorphic liposarcomas were analyzed by comparative genomic hybridization (CGH) to genomic microarrays (matrix-CGH), cDNA-derived microarrays for expression profiling, and by quantitative PCR. Matrix-CGH revealed copy number gains of numerous oncogenes, i.e., CCND1, MDM2, GLI, CDK4, MYB, ESR1, and AIB1, several of which correlate with a high level of transcripts from the respective gene. In addition, a number of genes were found differentially expressed in dedifferentiated and pleomorphic liposarcomas. Application of dedicated clustering algorithms revealed that both tumor subtypes are clearly separated by the genomic profiles but only with a lesser power by the expression profiles. Using a support vector machine, a subset of five clones was identified as "class discriminators." Thus, for the distinction of these types of liposarcomas, genomic profiling appears to be more advantageous than RNA expression analysis.

Algorithms↗
PubMed1975

[A heterologous mesodermal tumor of the uterus of the pleomorphic rhabdomyosarcoma type].

In the available literature mesodermal tumours of the uterus are described under various names (botryoid sarcoma, carcinocarcoma, chondrosarcoma, rabdomyosarcoma, etc.). For the sake of unification of terminology of these tumours, Kehrer suggested the term "mesodermal mixed tumours", thereby reflecting their origin from cellular elements of the mesoderma. Histologically, benign and malignant variants of tumours are identified; these tumours are characterized by multi-component composition of tissues forming integral parts thereof, the latter may be homologous or heterologous. Clinically, the said tumours are diagnosed in rare cases. Diagnosis is made on the basis of a microscopical investigation of a scrape from the uterine cavity, or investigation of the operation material. Heterologic tumours of the uterus of the type of rabdomyosarcoma have been described by a number of authors. As to their microscopical structure, they may be divided into embryonal, alveolar and pleomorphous. A case of heterologic mesodermal tumour, verified as a pleomorphous rabdomyosarcoma, which could develop in a long existed mesodermal tumour of the uterus in a woman 67 years of age, is described.

Aged↗
PubMedDate not supplied

Pleomorphic xanthoastrocytoma: some new observations.

Fourteen cases of pleomorphic xanthoastrocytoma (PXA) occurring in Japan were investigated to evaluate its various pathological features. The patients were generally young (average: 19 years), the tumor was superficially located and CT revealed well circumscribed contrast enhancing mass which was often accompanied by a cyst. Histopathological study revealed that the PXA is a pleomorphic and frequently desmoplastic astrocytic tumor characterized by chronic degenerative changes including lipidization of tumor cells. The desmoplasia was assumed to secondary proliferation of meningeal fibroblasts. Although the general consensus is that pial astrocytes are the most likely origin of PXA, "ordinary" astrocytoma may well develop into PXA by invading overlying meninges and inducing desmoplasia.

Adolescent↗
PubMed1992

Cutaneous pleomorphic large cell lymphoma.

The case of a primary cutaneous pleomorphic large cell lymphoma occurring in a twenty-one-year-old woman who presented with a blue-reddish nodule on her left cheek of three months' duration is presented. The tumor consisted of pleomorphic blast cells showing high mitotic activity. On immunohistochemical examination, the majority of the tumor cells expressed CD 3 (Leu-4), CD 4 (Leu-3), HLA-DR CD 30 (Ki-1/Ber-H 2), and CD 25 (IL2 receptor). Twenty-two months after excision of the tumor there is no detectable systemic spread of the lymphoma. This case provides further evidence for recent observations that primary cutaneous Ki-1-positive large cell lymphomas without lymph node involvement may have a favorable prognosis after local treatment despite showing histologic pattern of malignancy.

Adult↗
PubMed2003

[Development of salivary gland tumors in pleomorphic adenoma gene 1 transgenic mice].

OBJECTIVE: Activation and overexpression of pleomorphic adenoma (PLAG1) gene due to t(3;8)(p21;q12) translocation are associated with the development of human pleomorphic adenomas of the salivary glands. This study was conducted to generate ubiquitously-expressed or tissue-specific expressed PLAG1 transgenic mice and to elucidate the role of PLAG1 gene in tumorigenesis in vivo. METHODS: Human PLAG1 cDNA was cloned from salivary gland tumor or placenta tissues by RT-PCR. Ubiquitous expression vector pCMV-EGFP/PLAG1 driven by CMV promoter and tissue-specific expression vector pMMTV-PLAG1 driven by MMTV LTR were constructed. NIH3T3 cells transiently transfected with pCMV-EGFP/PLAG1 showed high expression of PLAG1 in nucleus. Transgenes were microinjected into pronucleus of zygotes to generate transgenic mice. RESULTS: It was found that the human PLAG1 cDNA cloned from several salivary gland tumor and normal placenta tissues consistently showed a variation of a single nucleotide at the same position when compared with the human PLAG1 cDNA sequence in Genbank (Accession No. U65002), which led to T458P at protein level. It might be a single nucleotide polymorphism (SNP)locus. Fused EGFP/PLAG1 protein was found to be localized in the nucleus of NIH3T3 cells transiently transfected with pCMV-EGFP/ PLAG1. Several pCMV-EGFP/PLAG1 and pMMTV-PLAG1 transgenic mouse lines were obtained respectively. As might be expected, pMMTV-PLAG1 transgenic mice spontaneously developed salivary gland tumors in three independent lines, among which, line 42 showed tumorigenic phenotype in 100% of transgenic mice within three months after birth. CONCLUSION: Overexpression of PLAG1 gene plays a crucial role in tumorigenesis of salivary gland tumors.

Animals↗
PubMedDate not supplied

Pleomorphic lipoma: a definite histopathological entity.

Pleomorphic lipomas are rare benign tumours that can resemble a variety of malignant tissue tumour on histological examination. We describe a case of pleomorphic lipoma arising on the posterior aspect of the neck of a 70-year-old man, successfully treated by surgical excision. A review of the literature is presented, summarizing the principal clinical and morphological characteristics of this rare tumor.

Aged↗
PubMed2005

The relative endogenous expression levels of the IFNAR2 isoforms influence the cytostatic and pro-apoptotic effect of IFNalpha on pleomorphic sarcoma cells.

Based on our previous studies where we found that IFNAR2-1, the short IFNalpha/beta receptor variant, was expressed in pleomorphic sarcoma cells, we decided to determine the relative levels of expression of IFNAR2.1 versus the longer form, named IFNAR2.2, in different pleomorphic sarcoma cells in relation to their response to interferon alpha treatment. When examining a panel of PS cells isolated from surgical specimens, we found that IFNAR2.1 prevailed in 6 out 7 lines analysed and that these generally showed cell cycle arrest and low levels of apoptosis upon IFNalpha treatment. The reverse ratio, i.e. higher constitutive levels of IFNAR2.2 than IFNAR2.1, was associated with an irreversible inhibition of cell growth and pronounced apoptosis. Impairment of tumour growth by low- and high-dose IFNalpha treatment of nude mice inoculated with PS cells expressing predominantly IFNAR2.1 further asserted the effect of the cytokine also in vivo. A proteomic analysis of 120 signalling components in growth arrested, apoptotic PS cells harbouring higher levels of IFNAR2.2 revealed engagement of the canonical Jak/Stat/ISGF3-pathway, the activation of the mitochodrial apoptotic pathway and a potentially novel mechanism of cell cycle blockade unrelated to down-regulation of cyclin A/B and their interacting/regulating kinases. Our results confirm the dominant negative role of IFNAR2.1, but also suggest that the relative endogenous levels of the two IFNalpha/beta receptor isoforms may dictate the signalling pathways triggered by the ligand, such as to cause exclusively cell cycle arrest or induce programmed cell death. This parameter may be of importance for the clinical outcome of IFNalpha treatment of PS.

Animals↗
PubMed2005

[Pulmonary pleomorphic carcinoma; report of a case].

We report a case of a 64-year-old man with pleomorphic carcinoma of the lung and thymic cyst. He was admitted to our hospital because of an abnormal shadow observed on chest X-ray. Computed tomography (CT) showed a mass lesion located in the right upper lobe and a non-invasive anterior mediastinal tumor adjacent to the left brachiocepharic vein. On enhanced CT, the lung mass showed central low-attenuation areas with a substantial enhancement in the periphery. Preoperative transbronchial blushing cytology of the mass revealed adenocarcinoma. With a diagnosis of primary lung cancer (cT3N0M0) and mediastinal tumor, an operation was performed through a median sternotomy. The mediastinal tumor was excised and a right upper lobectomy and were also accomplished, because the lung tumor did not show adhesion or pleural invasion. Histopathologic examination of the resected specimen revealed that the lung tumor composed of a mixture of spindle and giant cell features and contained a component of adenocarcinoma and squamous cell carcinoma. This finding yielded a pathological diagnosis of pleomorphic carcinoma (pT2N0M0). The mediastinal tumor was diagnosed as thymic cyst. The postoperative course was uneventful, and he is currently well 6 months after surgery.

Adenocarcinoma↗
PubMed1991

[Pleomorphic xanthoastrocytoma. Apropos of 3 new cases. Review of the literature].

Three cases of pleomorphic xanthoastrocytomas (P.X.A.), a low grade leptomeningeal glioma are reported. Prominent histological features used for diagnosis were a cellular pleomorphism of G.F.A.P. positive cells, with intracytoplasmic lipidic vacuols. A reticulinic network and mononuclear cells infiltrates have been observed. A weak mitotic activity and lack of necrosis and of endothelial cells proliferation were significant additional features necessary for diagnosis. Our cases were observed during the surgical management of young patients with resistant epilepsy. Neuroradiological examinations showed a tumor superficially located within the temporal or the parietal lobe. This tumor could be calcified and/or cystic. Operative aspects showed a firm and non-encapsulated leptomeningeal tumor with possible various colors. Our patients were seizures-free after surgery even during the follow-up. From the currently reported cases clinical follow-up ranging for 1.5 to 3 years is not sufficient to predict a favorable carcinologic prognosis. P.X.A. is an uncommon tumor and less than 50 cases are reported throughout the literature. This tumor affecting young subjects mainly during the second decade is revealed in the majority of cases (3/4) by epileptic seizures, less frequently by a deficit or by an intracranial hypertension. The great majority of clinical events are observed before 20 years. The functional prognosis is rather good after surgery with a disappearance of epileptic fits in about 50% of the cases. Throughout the literature the prognosis of this tumor seems to be comparable to low-grade astrocytomas. Optimal management of P.X.A. seems to be primary surgical resection with later surgery for residual or recurrent tumor. The role of radiotherapy in the management of P.X.A. is at this time uncertain.

Adult↗
PubMed2006

Pleomorphic lipoma of the oral cavity. Report of a case.

Pleomorphic lipoma (PL) is a rare benign tumor mainly located in the upper back, upper shoulders, and back of the neck in elderly men. More rarely it is located in the head and neck region and in the oral cavity. The differential diagnosis should be made with sclerosing liposarcoma and well-differentiated liposarcoma. A 59-year-old male patient was referred for the presence of a lesion involving the marginal and adherent gingiva of teeth # 5; this lesion extended into the vestibular mucosa. The lesion had a 2 cm diameter, showed no tenderness, had a hard-parenchymatous consistency, was mobile on the underlying tissues and was covered by normal appearing mucosa. Under local anesthesia, the lesion was completely removed. A free gingival graft from the palate was used to cover the defect. Microscopically, it was possible to observe mature adipocytes, spindle cells and rare ''floret-like'' cells. Lipoblasts and mitoses were absent. The definitive pathologic diagnosis was pleomorphic lipoma. No recurrences were present after a 5 years follow-up. Local excision is adequate for PL and the tumor does not recur.

Adipocytes↗
PubMed2006

[Pleomorphic carcinoma of the lung rapidly developed multiple metastases after surgery].

Pleomorphic carcinoma of the lung is a type of carcinoma with spindle and/or giant cells with a poor diagnosis. A 73-year-old male was referred to our hospital because of the pulmonary tumor. Lung biopsy revealed that the tumor was poorly differentiated adenocarcinoma. No distant metastasis were observed by systemic examination. A right middle lobectomy with partial resection of the right upper lobe and lymph node dissection were performed, because the tumor (5.3 x 4.0 x 4.0 cm) was located in peripheral S' and invaded S3 via the interlobular space. Histological findings showed adenocarcinoma comprised of spindle cell components that reacted positively to epithelial membrane antigen (EMA) and no lymph node metastasis. Therefore, he was diagnosed with pleomorphic carcinoma of the lung, pT2N0M0, stage IB. But metastatic lesions newly appeared in the thoracic skin, the liver, the diaphragm, the bilateral adrenal glands, and the retroperitoneal space on the 30th postoperative day. He died of peritonitis and pleuritis on only 60 days after the operation.

Adenocarcinoma↗
PubMed2006

[Pulmonary pleomorphic carcinoma; report of 2 cases].

The 1st case was a 74-year-old male diagnosed as femoral neck fracture. Biopsy of the bone revealed metastatic adenocarcinoma. Chest computed tomography (CT) showed a mass lesion located in the right lower lobe. With a diagnosis of primary lung cancer (cT2N1M1), two-staged operation was performed. Pathological diagnosis was pleomorphic carcinoma [pT2N1M1 (OSS), stage IV]. He died 8 months after surgery due to metastasis to the thoracic spine. The 2nd case was a 80-year-old female who complained of lateral chest pain. Chest CT revealed a tumor in the right hilar region, which was diagnosed as adenocarcinoma by transbronchial lung biopsy. Only thoracic drainage was performed since metastases to the brain and the rib were demonstrated. She died 2 months after admission. Autopsy revealed pleomorphic carcinoma of the lung with metastasis to the brain, costa and mediastinal lymph nodes.

Adenocarcinoma↗
PubMed2006

[Pleomorphic carcinoma of the lung with high serum granulocyte colony stimulating factor, suggested of pulmonary abscess by preoperative radiology; report of a case].

A 52-year-old man with a history of heavy smoking was hospitalized for evaluation of fever. Pulmonary abscess was initially suspected by computed tomography (CT) showing an ovoid, well-demarcated nodule of 61 mm in diameter with coarse calcification in S2a of the right lung. The patient was treated with antibiotics, but no improvement was seen in inflammatory reactions or lesion size. Marked leukocytosis and high level of granulocyte colony stimulating factor (G-CSF) was shown by laboratory examination. To improve patient condition and ensure correct diagnosis, right upper lobectomy of the lung was performed. Pleomorphic carcinoma of the lung was subsequently diagnosed. G-CSF producing tumor was suspected, since the normalization of serum G-CSF level followed by the improvement of both fever and inflammatory reaction was observed postoperatively. We also present herein a review of 22 Japanese cases of pleomorphic carcinoma producing G-CSF of the lung, characterized by leukocytosis.

Carcinoma↗
PubMed2006

[Pleomorphic rhabdomyosarcoma of the larynx--a case report and literature review].

Rhabdomyosarcoma is a very rare malignant tumor of the larynx which can present in a number of histologic variants. The pleomorphic variant of the tumor has been described in 16 patients to date. Being a rarity, rhabdomyosarcoma is as a rule managed according to the principle of treatment for malignant mesenchymal tumors of the head and neck, and includes radical surgery with postoperative radiotherapy and/or chemotherapy. A case is presented of a pleomorphic rhabdomyosarcoma of the vocal cord. Successfully treated by endoscopic CO2 laser cordectomy with adjuvant chemotherapy and 6-year uneventful follow up.

Humans↗
PubMed2006

[Cavitating pleomorphic carcinoma of the lung; report of a case].

We report a case of a previously healthy 76-year-old male with cavitating pleomorphic carcinoma of the lung. He was admitted because of an abnormal lung shadow on chest X-ray. Computed tomography (CT) showed a well-demarcated nodular shadow within thin-walled cavity in the right upper lobe. Because the lesion was revealed as adenocarcinoma by transbronchial lung biopsy, right upper lobectomy was performed. By histopathologic examination of the resected specimen, the nodule contained a component of spindle cell features and the cavity wall was composed of adenocarcinoma. The final diagnosis was pleomorphic carcinoma. Postoperative course has been uneventful for 12 months after surgery.

Adenocarcinoma↗
PubMed2006

Plasma cell leukemia with pleomorphic plasma cells--a case report.

A case of plasma cell leukemia showing mostly pleomorphic plasma cells in the form of convoluted and multilobated nuclei with some having bilobed nuclei and internuclear bridges is being reported for its rarity of occurrence. Patient presented with congestive cardiac failure and features of nephropathy. There were no lytic lesions in the bone. Serum electrophoresis did not show any M-band while urine electrophoresis demonstrated Bence Jones protein confirming a light chain only type of myeloma. Patient is in remission 5 months after diagnosis. The significance of recognising such pleomorphic plasma cells is discussed.

Bence Jones Protein↗
PubMed1991

[A case of retroperitoneal pleomorphic lipoma].

A 71-year-old male patient was referred to our department for further examination for right retroperitoneal tumor. Exploration was done through a flank approach and the tumor with right adrenal gland was removed. A pleomorphic lipoma was diagnosed histopathologically. There have been 8 reported cases of pleomorphic lipoma including our present case in Japan and we discuss the pathogenesis and treatment of this rare disease.

Aged↗
PubMedDate not supplied

Pleomorphic xanthoastrocytoma with desmoplastic reaction: angiomatous variant. Report of two cases.

Two cases of cerebral pleomorphic xanthoastrocytomas (PXA-s) with prominent vascularity and desmoplastic changes occurring in young subjects are presented. The tumors displayed the marked pleomorphism characteristic of PXA-s and had variable cellularity. The cytoplasm of many tumor cells contained an abundance of lipid droplets. Most tumor cells were positive for glial fibrillary acidic protein. The unusual feature about these tumors was the presence of very large numbers of tiny blood vessels with variable thickness of their walls. In many areas the small vessels and the neoplastic astrocytes were in close proximity to each other, with capillaries adjacent to or protruding into tumor cell cytoplasm, reminiscent of the pattern seen in highly vascularized or "angiomatous" meningiomas. In other areas extensive fibrosis was seen. We feel that the latter, as in the cases of comparably vascular meningiomas, had its origin in congelation and secondary organization of plasma proteins that have exuded through leaky walls of newly formed blood vessels. These are the first reported cases of PXA with an angiogliomatous pattern.

Adult↗