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PubMed1999

Endovascular papillary angioendothelioma (Dabska tumor) of bone.

Endovascular papillary angioendothelioma, also known as Dabska tumor, is a rare vascular neoplasm that usually involves the skin or subcutaneous tissue of children. There have been no reported cases of this lesion occurring in bone. We report a Dabska tumor in the distal femur of a 45-year-old woman who, clinically and radiologically, was felt to have an osteoid osteoma. Histologic study of the lesion showed a hemangioma with budding fronds of endothelial cells, a feature characteristic of the Dabska tumor. We feel that the lesion arose in a pre-existing hemangioma, a hypothesis suggested in a few other case reports. Endovascular papillary angioendothelioma is a low-grade malignant neoplasm, although only one patient has died due to this lesion. Our patient is asymptomatic without evidence of recurrence 1 year post curettage.

Bone Neoplasms↗

PubMed2004

Fine-needle aspiration of primary and recurrent dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) is a nodular cutaneous mesenchymal tumor of intermediate malignancy. Studies on fine-needle aspiration of DFSP are extremely rare; to our knowledge, only 33 cases have been reported. We have reviewed 14 examples of DFSP in 13 patients. Ten primary tumors were aspirated before surgical biopsy and four recurrent lesions (all from superficial lesions) were also investigated by fine-needle aspiration. All smears were surprisingly homogeneous and composed of isolated spindle cells in all cases (one unsatisfactory smear is excluded). Tissue fragments with a stroriform pattern were seen in 11 cases, fibrillary stromal fragments in 10 cases, naked nuclei in 8 cases, slight to moderate cytonuclear atypia in 5 cases. Mitotic figures, myxoid background, mast cells, and dispersed adipocytes were rare. Giant cells, necrosis, or marked cytonuclear atypia were not seen. DFSP shares morphological characteristics of some low-grade spindle-cell neoplasms. It should be differentiated from other benign low- and intermediate-grade spindle neoplasm such as low-grade fibrosarcoma, fibromyxosarcoma, low-grade malignant peripheral nerve sheath tumor, benign peripheral nerve sheath tumor, nodular fasciitis, and fibrous histiocytoma.

Adolescent↗

PubMed2026

Spatial analysis reveals the evolving organization of IDH-mutant glioma.

Adult diffuse gliomas are composed of malignant cell states interwoven with the non-malignant brain microenvironment. Here, we combine spatial transcriptomics and spatial proteomics of isocitrate dehydrogenase (IDH)-mutant gliomas to define organizational principles across histological grades. In low-grade tumors, spatial organization is shaped by underlying brain anatomy. We identify a functional white-gray matter junction that restricts cortical invasion and is associated with marked changes in tumor composition and cellular phenotypes. This junction is preferentially traversed by oligodendrocyte progenitor (OPC)-like malignant cells, suggesting a role in tumor expansion. In contrast, tumors with intermediate histological features are largely disorganized, with few recurring interactions between cancer cell states and microenvironmental cell types. In high-grade tumors, hypoxia-associated structure emerges, resembling IDH-wild-type glioblastoma. Together, these findings reveal two independent axes of spatial organization-from anatomy-driven structure in low-grade tumors to hypoxia-driven organization in high-grade tumors-and establish a framework linking tumor grade to recurrent spatial interactions.

Isocitrate Dehydrogenase↗

PubMed2006

Solid and papillary epithelial neoplasm of the pancreas--a case report.

Solid and papillary epithelial neoplasm (SPENP) of the pancreas is a rare pancreatic tumour of low malignant potential, that is seen mostly in young females. The aetiology and pathogenesis is unclear but it is considered to be arising from primordial pancreatic cells. We report two cases of SPENP who had palpable abdominal lumps and were diagnosed on histopathology. In the first case, the tumour was unresectable and patient died within one year. In the second case, at laprotomy the patient had perineurial as well as capsular infiltration but after wide resection of the growth, patient has been doing well for the past 6 months. Since SPENP is a low grade malignant neoplasm, it should be treated aggressively with complete resection and metastatectomy. Prognosis after adequate surgery is good. A clinicopathological study and brief review of literature is presented.

Adult↗

PubMed2000

Mucoepidermoid bronchial tumors: a review of 34 operated cases.

OBJECTIVE: Pulmonary mucoepidermoid tumors are commonly included with adenoid cystic carcinoma and carcinoid tumors under the misleading rubric 'bronchial adenomas'. These neoplasms are extremely rare and little is known about their oncologic behaviour. They are considered to be of high, or low malignancy. METHODS: During a 16-year-period 34 consecutive patients (24 male and 10 female with an average age of 53 years) underwent surgery for pulmonary mucoepidermoids in our clinic (0.5% of all resected lung tumors). Fourteen patients were complaint free, in the others obstructive symptoms dominated. In 23 patients the tumors were located in the upper lobes. In 24 cases lobectomy, in four instances limited resection and in six cases pneumonectomy were performed without hospital mortality. RESULTS: Twenty-nine tumors proved to be high grade and five low grade malignancy by histology. In the latest group the 5-year-survival amounted to 80% (all of these tumors were observed in stage T1-2 N0), on the other hand, however, that rate accounted only 31% at high grade malignant mucoepidermoids. There was no 5-year-survivor among patients having N2-disease (n=5). CONCLUSION: Mucoepidermoid tumors have to be treated by radical surgery with lymph node sampling and dissection. Patients with low grade tumors can be expected to be cured following complete resection, on the other hand, however, in cases of high grade malignant neoplasms surgery results in significantly worse prognosis. Careful histological typing plays a key role in prediction of late results.

Adult↗

PubMed1997

The spectrum of mucosa-associated lymphoid tissue lesions in pediatric patients infected with HIV: A clinicopathologic study of six cases.

Mucosa-associated lymphoid tissue (MALT) lesions in nonimmunocompromised individuals include reactive lymphoid proliferations and both low- and high-grade lymphoid neoplasms. These lesions occur at extranodal mucosal sites, such as the gastrointestinal tract, bronchus, salivary gland, and other locations. The spectrum of MALT lesions in children with HIV infection had not been previously described. In this study, six cases that demonstrated the spectrum of MALT lesions in pediatric patients, aged 28 months to 23 years, who had HIV infection were described. Half the patients acquired the infection perinatally, and half acquired it by transfusion. Mucosal sites of involvement included the salivary gland (4 patients), bronchiolar mucosa (2 patients), and oropharyngeal mucosa (1 patient). One patient had lesions in lung and oropharynx sequentially; all others had involvement of solitary sites. The histologic diagnoses included myoepithelial sialadenitis (MESA), MESA with low-grade MALT lymphoma, typical low-grade MALT lymphoma, diffuse large cell lymphoma (DLCL), and atypical pulmonary lymphoid hyperplasia and lymphoid interstitial pneumonitis complex. The two cases of high-grade DLCL were confined to mucosal sites (tonsil and parotid); in one of these patients, a previous biopsy specimen showed a MALT lesion with low-grade features. In two cases, quantitation of the Epstein-Barr virus (EBV) genome by the polymerase chain reaction showed a very high copy number in peripheral blood mononuclear cells but a low copy number in the MALT lesion, which suggested that MALT lesions may not be directly associated with EBV infection. Two patients who had high-grade tumors (DLCL) were successfully treated with chemotherapy and radiation therapy. The remaining patients, all of whom had low-grade MALT lesions, received either corticosteroids or alpha-interferon or no specific therapy; in all patients, the lesions followed an indolent clinical course. Clinicians and pathologists should be alert to the possibility that MALT lesions, including MALT lymphomas, may be present in children who have AIDS.

Adolescent↗

PubMed2026

Intratumoral collagen correlates with histological grade and patient prognosis in breast cancer.

Histological grading, using the Nottingham Grading System (NGS), is a major prognostic indicator for breast cancer. NGS involves the scoring of cancer cell-related morphological features, yet it overlooks tumor microenvironment (TME) components such as collagen. Collagen proteins, integral to the extracellular matrix (ECM), influence tumor architecture and progression but their relationship with histological grade is not fully characterized. Here, we assessed intratumoral collagen deposition using Masson Trichrome staining of whole slides (n = 166), proteomic profiling (n = 2) and transcriptomic analyses of the METABRIC (n = 1827) and TCGA-BRCA (n = 753) cohorts. We showed that low-grade tumors display significantly higher intratumoral collagen deposition compared to high-grade tumors. Moreover, we demonstrated that collagen expression at the transcript and protein levels (Masson Trichrome) could discriminate Grade II carcinomas into distinct prognostic groups, in which patients with Grade II carcinomas with elevated levels of collagen expression were associated with lower pTNM stage and better survival outcomes. Our results support the inclusion of TME features, such as collagen deposition, to enhance prognostic accuracy in breast cancer.

Humans↗

PubMed2026

Diagnostic performance of intraoperative in vivo hyperspectral imaging for meningioma grading and molecular alterations: results from a prospective feasibility study.

OBJECTIVE: Hyperspectral imaging (HSI) is an emerging intraoperative, noninvasive, contrast agent-free imaging modality that enables quantitative assessment of tissue composition. The present study aimed to investigate whether HSI-derived tissue parameters correlate with WHO grade and molecular markers of aggressiveness in cranial meningiomas. METHODS: In this prospective study, intraoperative in vivo HSI was performed using the TIVITA tissue system, capturing spectral signatures between 500 and 1000 nm. Quantitative tissue parameters included tissue oxygen saturation (StO2), near-infrared perfusion index, organ hemoglobin index (OHI), and tissue water index (TWI). HSI parameters were correlated with histopathological WHO grade and molecular alterations, including CDKN2A/B deletion, TERT promoter mutation, and 1p/22q loss. Group differences were analyzed using one-way ANOVA, and diagnostic performance was assessed using receiver operating characteristic (ROC) analysis. RESULTS: Forty-six meningiomas were included, comprising WHO grade 1 (n = 35) and WHO grade 2-3 (n = 11) tumors. TWI was significantly higher in WHO grade 2-3 meningiomas compared with WHO grade 1 tumors (mean 0.49 [SD 0.12] vs 0.38 [SD 0.17], p = 0.048). ROC analysis demonstrated an area under the ROC curve (AUC) of 0.71 (95% CI 0.56-0.86, p = 0.036) for TWI in discriminating higher-grade disease. A TWI cutoff ≥ 0.367 identified all WHO grade 2-3 meningiomas with 100% sensitivity and 100% negative predictive value. In a molecular subgroup (n = 15), OHI appeared higher in tumors with homozygous CDKN2A/B deletion than in nondeleted tumors (mean 0.77 [SD 0.04] vs 0.62 [SD 0.10]). However, only 3 CDKN2A/B-deleted cases were available, and these findings should be considered descriptive. ROC analysis yielded an AUC of 0.89 (95% CI 0.71-1.00). An OHI cutoff ≥ 0.712 identified all three CDKN2A/B-deleted tumors (100% sensitivity), with 83.3% specificity and 86.7% accuracy. CONCLUSIONS: The present investigation demonstrated that HSI-derived tissue water and hemoglobin metrics provide biologically meaningful information in meningiomas. Low tissue water content appeared to rule out higher-grade diseases in this first subset cohort, while elevated hemoglobin showed a potential association with CDKN2A/B deletion in a small exploratory subgroup. These findings support the potential of HSI as a real-time noninvasive tool for intraoperative risk stratification and should be evaluated in large-scale studies. German Clinical Trials Register no. DRKS00036771 (www.drks.de).

Humans↗

PubMed2004

"Atypical" blue nevus, "malignant" blue nevus, and "metastasizing" blue nevus: a critique in historical perspective of three concepts flawed fatally.

The same errors that spawned, sustained, and continue to spur the notions of "atypical" Spitz's nevus, "malignant" Spitz's nevus, and "metastasizing" Spitz's nevus are animating of 3 other concepts flawed equally, namely, those of "atypical blue nevus," "malignant blue nevus," and "metastasizing blue nevus." Our intention here is to compel to the conclusion, by way of critique in historical perspective, that all neoplasms claimed to be "malignant blue nevus" and "metastasizing blue nevus;" in fact, are melanomas, that all "atypical blue nevi" are either a nevus or a melanoma, and that the trio of curious designations that serve as title of this work are mere evasions transparently from a diagnosis, straightforwardly, of 1 of only 3 possibilities, to wit, "blue nevus," melanoma, or melanoma in association with a "blue nevus." Rather than admit uncertainty forthrightly, those who employ circumlocutions that we deplore, such as those under scrutiny here, resort to linguistic maneuvers that, at first blush, seem to have the cachet of scholarship (the jargon used being in keeping with a slew of other well-accepted, but equally bogus diagnoses in [dermato]pathology, among those being "minimal deviation melanoma," "borderline melanoma," "nevoid melanoma," "potentially low-grade melanocytic neoplasm," and "melanocytic proliferation of uncertain biologic potential"). All those terms and phrases are constructed in a manner designed to make them appear to convey unbridled confidence on the part of a histopathologist, rather than what they are in actuality, that is, a cover abjectly for tentativeness. Scrutiny of the lingo, in very abbreviated form, just catalogued reveals it to be devoid of content utterly. For example, "malignant blue nevus" and "metastasizing blue nevus" not only are contradictions in terms, but they are outrageous violations of principles fundamental to classic Virchowian pathology. We seek here to debunk that claptrap in the same manner we did "atypical Spitz's nevus," "malignant Spitz's nevus," and "metastasizing Spitz's nevus." It is our hope that readers will consider our arguments worthy because they are logical, will find them convincing because they are irrefutable, and will incorporate the lessons communicated through them so that their own professional life, pathology as a discipline, and, ultimately, patients, are the beneficiaries.

Adult↗

PubMed1999

Evaluation of MIB-1 immunoreactivity and nucleolar organizer regions in nonneoplastic and neoplastic thyroid lesions.

Epithelial hyperplastic lesions of the thyroid gland still pose many diagnostic problems, despite considerable progress in the diagnosis of thyroid disorders. Especially borderline lesions are difficult to differentiate. The aims of this study were to evaluate the diagnostic value and compare the number of AgNORs and MIB-1 expression in thyreocytes from various pathological thyroid changes with special emphasis on borderline lesions (hyperplastic nodule-adenoma-follicular carcinoma). Material included 83 sections from 72 thyroid glands (55 women, 17 men, and mean age 45). According to histological examination the sections were divided into 7 groups: parenchymal goiter, nodular goiter, follicular adenoma, follicular carcinoma, papillary carcinoma, oxyphilic carcinoma and anaplastic carcinoma. Statistical analysis of the results revealed a significant correlation between the values of the number of AgNORs or MIB-1 proliferation index and neoplasm malignancy grade as well as a correlation between the number of AgNORs and MIB-1 proliferation index. The results demonstrated that both the number of AgNORs and the proliferative activity marker MIB-1 may be an additional diagnostic criterion in differential diagnosis of borderline thyroid lesions (each feature separately, but better effects are obtained when considering both).

Adenocarcinoma, Follicular↗

PubMed1994

Monocyte/macrophage system and malignancies.

Neoplasias of the monocyte/macrophage system are rare. They include malignant histiocytosis, sarcomas of dendritic cells, and possibly a malignant form of Langerhans cell histiocytosis. Characterization of all these neoplasms can be easily done along the lines which have been developed for the derivation of the normal cellular counterparts. The possibility of a malignant transformation of the cells of the monocyte/macrophages system is suggested by the fact that all members of this system can undergo mitotic division. The most controversial among the different entities is malignant histiocytosis, which has proved to be anaplastic large cell lymphoma (ALCL) with Ki1 expression in most cases. The few cases of true malignant histiocytosis may or may not express the Ki1 antigen. Sarcomas of dendritic cells have been related mainly to interdigitating or follicular dendritic cells. Prognosis of follicular dendritic cell sarcoma is probably more favorable than that of interdigitating cell sarcoma. Besides these more frequent subtypes, a sarcoma of the sinus lining cells can be separated which represents a low-grade malignant neoplasm. All cases encountered so far were associated with a protracted clinical course, albeit multiple recurrences. A distinctly more aggressive behavior is encountered in those cases which have been identified as a malignant form of Langerhans cell histiocytosis. However, the issue of this particular form of histiocytic disorder is still unsettled.

Cell Differentiation↗

PubMed1996

Expression of mucin carbohydrate antigens (T, Tn and sialyl Tn) and MUC-1 gene product in intraductal papillary-mucinous neoplasm of the pancreas.

Aberrant or incomplete glycosylation of mucins results in expression of T, Tn, and sialyl-Tn (STn) antigens in various malignant neoplasms. MUC-1 gene product (a mucin core protein of mammary type) is known to alter or overexpress in several malignant tumors. However, expression of these mucin-related antigens has rarely been examined in intraductal papillary-mucinous neoplasm (IPN) of the pancreas. The authors examined immunohistochemically the expression of these antigens and MUC-1 gene product in nine IPN. In normal pancreas (n = 5), pancreatic ducts did not express T, Tn, or STn antigens, but expressed MUC-1 gene product. Among the nine IPNs, two (22%) expressed T antigen, nine (100%) expressed Tn antigen, and seven (78%) expressed STn antigen. MUC-1 gene product was expressed in nine (100%) IPNs. In invasive ductal adenocarcinoma of the pancreas (n = 6), all cases showed strong expression of Tn and STn antigens and the MUC-1 gene product, but expressed no T antigen. The expression of these antigens and MUC-1 gene product was focal in IPN, whereas it was diffuse in invasive ductal adenocarcinoma of the pancreas. These data suggest that aberrant or incomplete glycosylation occurs in epithelial mucins of IPN, that IPN is a borderline or low grade malignant neoplasm in terms of mucin-related antigen expression, and that mucin core protein (MUC-1 gene product) does not alter during pancreatic ductal carcinogenesis.

Adenocarcinoma, Papillary↗

PubMed1999

Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma.

Conventional chondrosarcoma (CSA) of the skull base is an uncommon neoplasm that can resemble chordoma, and indeed it is misdiagnosed frequently as such. This has important clinical implications, because when treated with similar aggressive treatment strategies, CSA has a much better prognosis than chordoma. In an effort to identify those morphologic and immunohistochemical features that help to identify conventional skull base CSA correctly and to understand its prognosis better, particularly compared with chordoma, when treated with surgery and proton beam irradiation, the authors performed a clinicopathologic analysis of 200 CSAs. The patients ranged in age from 10 to 79 years (mean, 39 years), 87 patients were male and 113 patients were female, and most presented with symptoms related to the central nervous system. Approximately 6% of the tumors arose in the sphenoethmoid complex, 28% originated in the clivus, and 66% developed in the temperooccipital junction. Histologically, 15 tumors (7.5%) were classified as hyaline CSA, 59 (29.5%) as myxoid CSA, and 126 (63%) as mixed hyaline and myxoid CSA. A total of 101 (50.5%) tumors were grade 1, 57 (28.5%) had areas of grades 1 and 2, and 42 (21%) were pure grade 2 neoplasms. The vast majority of patients originated from referring hospitals, and the diagnosis was changed prospectively at our institution to CSA from chordoma in 74 patients (37%). Of the tumors studied immunohistochemically, 96 of 97 (98.9%) stained for S-100 protein, 0 of 97 (0%) stained for keratin, and faint staining for epithelial membrane antigen was seen in 7 of 88 tumors (7.95%). All patients underwent high-dose postoperative fractionated precision conformal radiation therapy with a dose that ranged from 64.2 to 79.6 Cobalt-Gray-equivalents (median, 72.1 Cobalt-Gray-equivalents, given in 38 fractions. The 200 patients had a median follow-up of 63 months (range, 2.1 mos - 18.5 yrs). Tumor control was defined as lack of progression by clinical and radiographic assessment. Based on this definition, there were three local recurrences, and two of these patients died of tumor-related complications. The 5- and 10-year local control rates were 99% and 98% respectively, and the 5- and 10-year disease-specific survival rates were both 99%. In contrast to CSA, the 5- and 10-year survival rates of chordoma have been reported to be approximately 51 % and 35% respectively, and in our institution intensive treatment has resulted in 5- and 10-year progression-free survival rates of 70% and 45% respectively. CSA of the skull base can be distinguished reliably from chordoma, and this distinction is important because skull base CSA has an excellent prognosis when treated with surgery and proton beam irradiation, whereas chordomas have a substantially poorer clinical course despite similar aggressive management.

Adolescent↗

PubMed2026

ERP44 Is Associated With Poor Prognosis and Promotes Proliferation and Temozolomide Resistance in Lower-grade Glioma.

BACKGROUND/AIM: Endoplasmic reticulum resident protein 44 (ERP44), a protein disulfide isomerase family member, has been implicated in tumor biology, but its role in lower-grade glioma (LGG) remains unclear. This study investigated the prognostic significance and biological function of ERP44 in LGG, focusing on proliferation and temozolomide (TMZ) resistance. MATERIALS AND METHODS: ERP44 expression, clinicopathological associations, and prognostic value were analyzed using The Cancer Genome Atlas (TCGA), Genotype-Tissue Expression (GTEx), and Chinese Glioma Genome Atlas (CGGA) datasets. Time-dependent receiver operating characteristic (ROC) curves, Cox regression, and a prognostic nomogram were constructed. Differential expression, Gene Set Enrichment Analysis (GSEA), Gene Ontology (GO) enrichment, immune infiltration, and drug sensitivity analyses were performed. Functional validation was conducted in SW1088 and SW1783 cells using shRNA-mediated ERP44 knockdown, followed by RT-qPCR, western blotting, CCK-8, colony formation, and TMZ IC50 assays. Subcutaneous xenograft models with or without TMZ treatment were used for in vivo validation. RESULTS: ERP44 was markedly upregulated in LGG and associated with higher WHO grade, IDH wildtype status, 1p/19q non-codeletion, and poor survival in TCGA and CGGA cohorts. ERP44 showed strong prognostic performance and improved risk stratification in a multivariable nomogram. Enrichment analyses linked high ERP44 expression to immune/inflammatory pathways and reduced neuronal functional signatures. ERP44 positively correlated with immune infiltration, proliferation/stemness markers, and predicted TMZ resistance, while its knockdown inhibited proliferation and colony formation, reduced TMZ IC50, suppressed xenograft growth, enhanced TMZ efficacy, and decreased Ki67 positivity. CONCLUSION: ERP44 is a prognostic biomarker that promotes LGG proliferation and TMZ resistance, suggesting its potential as a therapeutic target.

Humans↗

PubMed2026

Spindle Cell Predominant Anaplastic Pleomorphic Xanthoastrocytoma (WHO Grade 3) With Focal Piloid Features: A Rare Case Study With Comprehensive Molecular Profiling.

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor of the central nervous system. The typical form demonstrates relatively low-grade histologic features, whereas an anaplastic variant shows more aggressive behavior, including increased mitotic activity and necrotic changes. These tumors are often associated with alterations involving key growth signaling pathways and cell cycle regulatory genes, with molecular features that may resemble those seen in other high-grade astrocytic neoplasms. We describe an unusual example of an anaplastic pleomorphic xanthoastrocytoma showing focal piloid differentiation. The patient presented with acute neurologic symptoms, and imaging demonstrated a large, enhancing, well-circumscribed cerebral lesion with limited surrounding edema. Histologic evaluation revealed a highly cellular astrocytic neoplasm composed of spindle-shaped cells with marked pleomorphism, including scattered multinucleated forms, brisk mitotic activity, and necrotic areas. At the periphery, regions with elongated bipolar glial cells and occasional cytoplasmic inclusions suggestive of piloid morphology were identified. Molecular analysis demonstrated an activating alteration in the mitogen-activated protein kinase (MAPK) pathway along with additional genomic abnormalities, while mutations commonly associated with diffuse gliomas were not detected. The presence of piloid features within an otherwise anaplastic tumor is rare and may be relevant to the relatively favorable outcome observed during extended follow-up.

anaplastic↗

PubMed1992

Cytogenetic abnormalities in renal oncocytic neoplasms.

We have performed cytogenetic studies on five renal oncocytic neoplasms (three grade 2 tumors and two grade 1 tumors) identified histologically by light microscopy. One grade 1 tumor failed to produce mitotic cells. The other four tumors exhibited both normal and abnormal cell lines. Numerical abnormalities were found in both the single grade 1 and two of the grade 2 tumors whereas structural abnormalities were limited to grade 2 tumors. Aneuploidy of chromosome 12 was observed in both grade 1 and 2 tumors. Grade 2 tumors showed more extensive numerical change than the grade 1 tumors. Abnormalities of chromosome 3 characteristic of renal cell carcinoma were not found in any tumor in this series. A combination of C-banding and HaeIII endonuclease banding was used to identify an ambiguous marker. In our four cases and in the cases previously reported, loss of a sex chromosome, abnormalities of chromosomes 1 and 22, and trisomy 12 are findings most often observed in renal oncocytoma.

Adult↗

PubMed1993

Biologic and clinical significance of cytogenetic and molecular cytogenetic abnormalities in benign and malignant cartilaginous lesions.

Cartilaginous neoplasms are often histologically and therapeutically challenging. Predicting biologic behavior can be difficult. In this study, 120 nonneoplastic, benign, and malignant cartilaginous lesions from 103 patients were cytogenetically analyzed in a 6-year period after short-term culture. For selected cases, fluorescent in situ hybridization (FISH) techniques using chromosome-specific probes were performed on metaphase/interphase preparations and on paraffin-embedded tissue sections. Clonal abnormalities of chromosomes 2, 3, 5, 7, 8, and 12 were most frequently observed. Involvement of chromosomes 5, 8, and 12 may be etiologically significant because of the gene localizations for the human cartilage link protein, Langer-Giedion syndrome (a rare syndrome characterized by multiple exostoses), and type II collagen (a major component of normal cartilage) respectively, to these three chromosomes. That chromosome 7 abnormalities were observed only in malignant tumors is of diagnostic value. The identity of three marker chromosomes and the significance of trisomy 7 (a finding of controversial meaning), were determined with FISH. That the presence of chromosome aberrations and increasing histologic grade strongly correlated (p = 0.001) is of prognostic importance. Moreover, complex aberrations were observed nearly exclusively in high-grade tumors (p = 0.001). The data show that nonrandom chromosome loci are aberrantly affected in cartilaginous lesions and that these abnormalities may be of significant histopathogenetic consequence. In addition, these chromosome abnormalities appear to be diagnostically and prognostically valuable in classifying and grading chondromatous neoplasms.

Adult↗

PubMedDate not supplied

Osteosarcoma of the jaw.

BACKGROUND: Osteogenic sarcoma of the jaw has clinical and prognostic differences from that of long bones. METHODS: We reviewed 23 confirmed cases of osteosarcoma of the jaw seen at the Instituto Nacional de Cancerologia of Mexico between 1972 and 1990. RESULTS: Fifty-seven percent of the patients were women. Median age for all patients was 28 years. In 52% of cases, the neoplasm involved the maxilla. Tumor size ranged from 5 to 24 cm (median 10 x 7 cm) and 87% of the neoplasms were grade III or IV (Broder's). Twenty one (91%) patients were treated with surgery (s); radiotherapy (Rt) was administered to 14 (61%) and chemotherapy (Ct) to 11 (48%) patients. Clear (negative) surgical margins were obtained in 43% of surgically treated patients. Median follow-up for 20 patients eligible for evaluation was 16.5 months. Survival at 5 years was 10%. CONCLUSIONS: We found that tumor size and surgical margins were significant prognostic factors. Early diagnosis of these tumors is mandatory to improve survival; the role of Ct and Rt is still unknown.

Adolescent↗