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Mesenchymal Neoplasms (mesenchymal + neoplasm)
Selected AbstractsMelanoma with cartilaginous differentiation: Diagnostic challenge on fine-needle aspiration with emphasis on differential diagnosisDIAGNOSTIC CYTOPATHOLOGY, Issue 1 2009Krisztina Z. Hanley M.D. Abstract Fine-needle aspiration (FNA) is a minimally invasive, fast, and accurate diagnostic method for the evaluation of patients with locally recurrent or distant metastases of malignant melanoma. In the vast majority of cases, the diagnosis is straightforward with the characteristic cytologic features well documented in the literature. Divergent differentiation (chondroid, neural, myofibroblastic, and osteocartilagenous) in a melanoma is rare and can potentially create diagnostic challenges if the evaluator is unaware of the same. We report a case of a 46-year-old female with a history of primary anal melanoma who presented with a groin mass. The FNA of the groin mass showed a neoplasm rich in chondroid matrix and raised the possibility of a second primary mesenchymal neoplasm rather than metastasis from the patient's known primary anal melanoma. A review of the histologic features of the anal melanoma showed divergent chondroid differentiation in the anal melanoma with the metastatic deposit in the groin exhibiting extensive chondroid differentiation. The differential diagnostic considerations are discussed. Diagn. Cytopathol. 2009. © 2008 Wiley-Liss, Inc. [source] Myopericytoma of the oral cavityHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 6 2007Vivekanand Datta MD Abstract Background. Myopericytoma is a rare mesenchymal neoplasm of pericytic cells demonstrating myoid differentiation. The lesion typically arises within the subcutaneous tissue of the extremities. We report a case that, to the best of our knowledge, is the first case of myopericytoma involving the soft tissue of the oral cavity. Methods. A 36-year-old woman had a 5-mm sessile, whitish-pink, firm tongue nodule. The patient underwent excisional biopsy, and histopathologic examination as well as immunohistochemical analysis were performed. Results. The differential diagnosis by histologic analysis included solitary fibrous tumor, myofibroma, glomus tumor, and myopericytoma. The results of immunohistochemical analysis, when combined with the histologic features, led to a diagnosis of myopericytoma. Conclusions. Applying strict morphologic criteria and appropriately selective immunohistochemical markers will help to distinguish myopericytoma in the oral cavity. © 2007 Wiley Periodicals, Inc. Head Neck, 2007 [source] Metastatic acinic cell carcinoma in a neurofibroma mistaken for carcinosarcomaHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 1 2005Michael L. Cohn MD Abstract Background. Tumor-to-tumor metastasis is a rare, but well-recognized, entity most commonly involving metastatic carcinoma to a mesenchymal neoplasm. We report a case of acinic cell carcinoma of the parotid gland metastatic to a neurofibroma. Methods and Results. A 55-year-old man with a history of a high-grade acinic cell carcinoma of the parotid was seen with a mass at the surgical site and metastatic foci in the scalp 10 months postoperatively. The resection specimen revealed a spindle cell lesion with metastatic foci of high-grade adenocarcinoma, initially diagnosed as a carcinosarcoma. The bland morphology and S-100,positive expression of the spindle cell lesion confirmed the diagnosis of neurofibroma. The high-grade features of the carcinomatous foci and their similarity to the primary tumor confirmed the presence of a tumor-to-tumor metastasis. Conclusion. To our knowledge, this is the first reported case of acinic cell carcinoma metastatic to a neurofibroma, an important entity in the differential diagnosis of biphasic tumors of the head and neck. © 2004 Wiley Periodicals, Inc. Head Neck27: 76,80, 2005 [source] Two cases of dermatomyofibroma (plaque-like dermal fibromatosis)INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 8 2010Elisabeth Gomez-Moyano MD Background, Dermatomyofibroma is a rare but distinct benign cutaneous mesenchymal neoplasm of fibroblastic/myofibroblastic differentiation. It is more common in adolescents and young adults, with a female preponderance. In most cases, the lesions are asymptomatic and small, measuring from 10 to 20 mm. Early and active lesions tend to be actin positive. Case report, We present a) a new case of dermatomyofibroma in an 11-month-old male infant, the youngest case reported to date, and b) the second reported case of a giant annular dermatomyofibroma, measuring 10 cm × 6 cm, in a 52-year-old woman. In both cases, histological examination showed a spindle-cell proliferation embedded among the collagen fibers of the dermis, arranged predominantly parallel to the skin surface. In both cases the spindle cells stained positive for smooth muscle actin and the elastic fibers were increased and fragmented. Conclusion, Dermatologists and pediatricians should be aware of this benign entity in order to avoid unnecessary treatment. [source] Proximal-type epithelioid sarcoma: case report and result of comparative genomic hybridizationJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2004Mi-Woo Lee Background:, Epithelioid sarcoma is a rare mesenchymal neoplasm. Recently, a more aggressive, so-called ,proximal type' epithelioid sarcoma has been described. Clinical case:, A 40-year-old-woman presented with 5 × 4 cm, erythematous, indurated, non-movable, painful mass on the pubic area. Histopathology demonstrated diffuse tumor-cell infiltration into the subcutaneous and fascia, which was consisted of prominent epithelioid cells and scattered rhabdoid cells. A multinodular growth pattern or granulomatous appearance with central necrosis was not observed. The tumor cells showed positive reactions for vimentin, cytokeratin (AE1/AE3), and CD34. Despite the surgery, left inguinal mass with lymphadenopathy occurred one month later. We also carried out comparative genomic hybridization (CGH) with tumor cells. CGH revealed chromosomal gain of 5q32-qter, 12q24-qter, and 22q. Conclusion:, We report a case of proximal-type of epithelioid sarcoma, which showed the chromosomal gains of 5q32-qter, 12q24-qter, and 22q by CGH. [source] Extragenital subcutaneous cellular angiofibroma,APMIS, Issue 3 2007Case report Cellular angiofibroma (CAF) is a rare distinctive mesenchymal neoplasm that occurs almost exclusively in the genital area. We report the case of a 38-year-old woman who presented with an asymptomatic subcutaneous mass, 3.5 cm in diameter, located in the left hypochondrium, which had progressively enlarged during the previous 6 months. The lesion was completely excised. No recurrence was observed 3 months after the excision. A review of the literature,and including the present report,revealed five cases (three men and two women) with location outside the urogenital tract. Mean age was 57 (range 38,78) years; mean 41 years for women and 68 years for men. Average tumor size was 9 (range 3.5,25) cm; mean 5 cm for women and 12 cm for men. Tumors were located in the superficial soft tissue of the trunk, except for one case in the retroperitoneum. Mean follow-up was 29 (range 3,102) months, and no patient developed recurrence or metastasis. Extragenital CAF, except for location, shows similar clinicopathological features to genital CAF. Simple tumorectomy appears to be adequate treatment. Morphologically, CAF is closely related to spindle cell lipoma. [source] Trends in incidence and survival of mesenchymal neoplasm of the digestive tract within a defined population of Northern Norway,APMIS, Issue 3 2006SONJA ERIKSSON STEIGEN Population-based incidence and survival data for gastrointestinal stromal tumor (GIST) are sparse due to the fact that GIST is a rather novel entity both clinically and pathologically, and has not been registered as a separate entity in population-based cancer registries. The aim of the present study was to reclassify all mesenchymal tumors within a defined population of northern Norway over a time-span of 30 years with the purpose of estimating trends of incidence and survival. One hundred and forty-one patients with mesenchymal neoplasms of the digestive tract were identified: 102 as GISTs, 32 as leiomyomatous tumors, 4 as schwannomas, and 3 as fibromas. Incidence rates of GIST showed a significant increase over the whole period, which was not observed for the non-GIST cases. Analysis of GIST cases showed that cases with more than 5 mitoses per 50 high power fields had an increased expected mortality 4 times that of those with fewer mitoses, and the combination of mitotic count and size of tumor can be recommended for categorizing the tumors into different risk levels. The study confirms that GIST is by far the most frequent mesenchymal neoplasm of the digestive tract and that the incidence has increased over the last 30 years. [source] Diagnosis of metastatic pancreatic mesenchymal tumors by endoscopic ultrasound-guided fine-needle aspiration,DIAGNOSTIC CYTOPATHOLOGY, Issue 11 2009Linda Varghese M.D. Abstract Involvement of the pancreas by metastatic sarcoma is rare, and can prove challenging to differentiate from sarcomatoid carcinomas which occur more commonly. The endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) technique has been successfully used for the diagnosis of pancreatic carcinomas whether primary or metastatic, and is now considered the most effective noninvasive method for the identification of pancreatic metastases. However, to date very few reports detail the diagnosis of mesenchymal neoplasms by EUS-FNA. Herein, we report a series of four patients who underwent EUS-FNA of the pancreas, where the diagnosis of metastatic sarcoma was made based on morphology and ancillary studies. The cases include metastases of leiomyosarcoma, liposarcoma, alveolar rhabdomyosarcoma, and solitary fibrous tumor. The history of a primary sarcoma of the chest wall, mediastinum, and respectively lower extremity was known for the first three of these patients while in the case of the solitary fibrous tumor a remote history of a paraspinal "hemangiopericytoma" was only elicited after the EUS-FNA diagnosis was made. We conclude that EUS-FNA is efficient and accurate in providing a diagnosis of sarcoma, even in patients without a known primary sarcoma, thus allowing institution of therapy without additional biopsies. Diagn. Cytopathol. 2009. © 2009 Wiley-Liss, Inc. [source] Cytohistologic correlations in schwannomas (neurilemmomas), including "ancient," cellular, and epithelioid variantsDIAGNOSTIC CYTOPATHOLOGY, Issue 8 2006Jerzy Klijanienko M.D. Abstract Schwannoma accounts for one of the most common benign mesenchymal neoplasms of soft tissues. Although it is well defined in the cytology literature, particular histologic subtypes such as "ancient," cellular and epitheliod variants could be a source of diagnostic difficulties. We have reviewed cytology aspirates and corresponding histologic sections from 34 schwannomas diagnosed at Institut Curie. Histologically, 24 cases were classic, 5 were "ancient," 4 were cellular, and 1 was epithelioid schwannomas. No example of melanotic schwannoma was recorded. Original cytologic diagnosis was schwannoma in 13 (38.2%) cases, benign soft tissue tumor in 11 (32.4%), pleomorphic adenoma in 2 (6%) cases, angioma in 1 (2.9%) case, nodular fasciitis in 1 (2.9%) case, suspicious in 3 (8.8%) cases, and not satisfactory in 3 (8.8%) cases. There were no major differences between classical, "ancient," cellular, and epithelioid variants on cytology smears. Myxoid stroma, mast cells, and intranuclear inclusions were limited to classical subtype. Similarly, cyto-nuclear atypia was more frequent in classical subtype than in other subtypes. Schwannoma should be differentiated from well-differentiated malignant peripheral nerve sheath tumor, neurofibroma, and pleomorphic adenoma, in the last instance particularly for head and neck lesions. Diagn. Cytopathol. 2006;34:517,522. © 2006 Wiley-Liss, Inc. [source] Clinical significance of oncogenic KIT and PDGFRA mutations in gastrointestinal stromal tumoursHISTOPATHOLOGY, Issue 3 2008J Lasota Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. Despite clinicopathological differences, GISTs share oncogenic KIT or platelet-derived growth factor-alpha (PDGFRA) mutations. Imatinib, KIT and PDGFRA inhibitor, has been successfully used in the treatment of metastatic GISTs. There are primary KIT or PDGFRA mutations diagnosed before imatinib treatment, linked to GIST pathogenesis, and secondary mutations detected during treatment, causing drug resistance. KIT exon 11 mutations are the most common. Gastric GISTs with exon 11 deletions are more aggressive than those with substitutions. KIT exon 11 mutants respond well to imatinib. Less common KIT exon 9 Ala502_Tyr503dup mutants occur predominantly in intestinal GISTs and are less sensitive to imatinib. An Asp842Val substitution in exon 18 is the most common PDGFRA mutation. GISTs with such mutation are resistant to imatinib. PDGFRA mutations are associated with gastric GISTs, epithelioid morphology and a less malignant course of disease. GISTs in neurofibromatosis 1, Carney triad and paediatric tumours generally lack KIT and PDGFRA mutations. Secondary KIT mutations affect exons 13,17. GISTs with secondary mutations in exon 13 and 14 are sensitive to sunitinib, another tyrosine kinase inhibitor. KIT and PDGFRA genotyping is important for GIST diagnosis and assessment of sensitivity to tyrosine kinase inhibitors. [source] Perivascular epithelioid cell tumor (PEComa) with perirenal manifestationINTERNATIONAL JOURNAL OF UROLOGY, Issue 5 2005SVEN GUNIA Abstract We present the first case report documenting an 8-month post-surgical follow-up of an exceedingly rare perivascular epithelioid cell tumor (PEComa) with a perirenal location. Furthermore, prognostic parameters discussed in the literature are summarized and concise information regarding imaging characteristics on magnetic resonance imaging is provided. In conclusion, our report points toward PEComa as a family of very rare mesenchymal neoplasms that should be included into deliberations concerning the differential diagnosis of perirenal mass lesions. [source] Anaplastic lymphoma kinase proteins in growth control and cancerJOURNAL OF CELLULAR PHYSIOLOGY, Issue 3 2004K. Pulford The normal functions of full-length anaplastic lymphoma kinase (ALK) remain to be completely elucidated. Although considered to be important in neural development, recent studies in Drosophila also highlight a role for ALK in gut muscle differentiation. Indeed, the Drosophila model offers a future arena for the study of ALK, its ligands and signalling cascades. The discovery of activated fusion forms of the ALK tyrosine kinase in anaplastic large cell lymphoma (ALCL) has dramatically improved our understanding of the pathogenesis of these lymphomas and enhanced the pathological diagnosis of this subtype of non-Hodgkin's lymphoma (NHL). Likewise, the realisation that a high percentage of inflammatory myofibroblastic tumours express activated-ALK fusion proteins has clarified the causation of these mesenchymal neoplasms and provided for their easier discrimination from other mesenchymal-derived inflammatory myofibroblastic tumour (IMT) mimics. Recent reports of ALK expression in a range of carcinoma-derived cell lines together with its apparent role as a receptor for PTN and MK, both of which have been implicated in tumourigenesis, raise the possibility that ALK-mediated signalling could play a role in the development and/or progression of a number of common solid tumours. The therapeutic targeting of ALK may prove to have efficacy in the treatment of many of these neoplasms. © 2004 Wiley-Liss, Inc. [source] Gastrointestinal stromal tumours: A clinico-radiologic review from a single centre in South IndiaJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 6 2009A Singh Summary Gastrointestinal stromal tumours (GISTs) are rare tumours but are the commonest mesenchymal neoplasms in the gastrointestinal tract. To our knowledge, there is no large case series in Asian countries in which a clinico-radiological descriptive analysis of these tumours has been carried out. In this retrospective study, we analysed our experience of 70 patients with histopathologically proven GISTs, who were presurgically investigated by using CT, and describe the demography, anatomical distribution, imaging features and clinical course of the GIST. We found an unusually large predominance of males in our study, stomach and small bowel appeared to have been involved similarly and small bowel tumours had a higher rate of metastases. We also highlight some unusual CT features of these tumours that we encountered during the study, such as the presence of metastatic lymphadenopathy and satellite nodules, relapse in appendices epiploicae of the bowel, metachronous liposarcoma, adrenal and lung metastases, multiplicity of lesions and aneurysmal dilatation of the bowel. Two of our patients also had multiple neurofibromas, whose association with GIST has been seen in earlier reports. To the best of our knowledge, this article presents one of the largest series of articles on GISTs, to date, in Asian countries. We conclude with a differential diagnosis of GIST, with salient features distinguishing each entity. [source] Neoadjuvant treatment of soft-tissue sarcoma: A multimodality approachJOURNAL OF SURGICAL ONCOLOGY, Issue 4 2010David Reynoso BS Abstract Unlike epithelial cancers that are both more homogeneous and easily categorized by their respective tissues of origin (e.g., breast or lung cancer), sarcomas represent a diverse class of molecularly distinct bone and soft-tissue mesenchymal neoplasms of more than 50 subtypes. This diversity, as well as the relative rarity of sarcomas as a whole, has presented challenges in conducting prospective randomized clinical trials to assess the value of neoadjuvant chemotherapy for any given subtype. Most clinical trials and meta-analyses have neglected the phenotypic and molecular heterogeneity differentiating one sarcoma subtype from another in favor of a simplified grouping that ensures timely trial completion. As the success of treating gastrointestinal stromal tumors (GISTs) with imatinib demonstrates, a decision to provide neoadjuvant chemotherapy must take into consideration both the subtype being treated and the effect such treatment would be expected to exert upon that subtype. J. Surg. Oncol. 2010; 101:327,333. © 2010 Wiley-Liss, Inc. [source] Identification of CXCL13 as a new marker for follicular dendritic cell sarcoma,THE JOURNAL OF PATHOLOGY, Issue 3 2008W Vermi Abstract The homeostatic chemokine CXCL13 is preferentially produced in B-follicles and is crucial in the lymphoid organ development by attracting B-lymphocytes that express its selective receptor CXCR5. Follicular dendritic cells (FDCs) have been identified as the main cellular source of this chemokine in lymphoid organs. Recently, genome-wide approaches have suggested follicular CD4 T-helper cells (THF) as additional CXCL13 producers in the germinal centre and the neoplastic counterpart of THF (CD4+ tumour T-cells in angioimmunoblastic T-cell lymphoma) retains the capability of producing this chemokine. In contrast, no data are available on CXCL13 expression on FDC sarcoma (FDC-S) cells. By using multiple approaches, we investigated the expression of CXCL13 at mRNA and protein level in reactive and neoplastic FDCs. In reactive lymph nodes and tonsils, CXCL13 protein is mainly expressed by a subset of FDCs in B-cell follicles. CXCL13 is maintained during FDC transformation, since both dysplastic FDCs from 13 cases of Castleman's disease and neoplastic FDCs from ten cases of FDC-S strongly and diffusely express this chemokine. This observation was confirmed at mRNA level by using RT-PCR and in situ hybridization. Of note, no CXCL13 reactivity was observed in a cohort of epithelial and mesenchymal neoplasms potentially mimicking FDC-S. FDC-S are commonly associated with a dense intratumoural inflammatory infiltrate and immunohistochemistry showed that these lymphocytes express the CXCL13 receptor CXCR5 and are mainly of mantle zone B-cell derivation (IgD+ and TCL1+). In conclusion, this study demonstrates that CXCL13 is produced by dysplastic and neoplastic FDCs and can be instrumental in recruiting intratumoural CXCR5+ lymphocytes. In addition to the potential biological relevance of this expression, the use of reagents directed against CXCL13 can be useful to properly identify the origin of spindle cell and epithelioid neoplasms. Copyright © 2008 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. [source] Gastrointestinal stromal tumors (GISTs): a reviewAPMIS, Issue 2 2009SONJA E. STEIGEN Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms in the gastrointestinal tract, which, over the last 10 years, have emerged from a poorly understood neoplasm to a well-defined tumor entity exhibiting particular molecular abnormalities and for which promising novel treatment modalities have been developed. GISTs probably arise from the precursor cell of the interstitial cell of Cajal, express KIT tyrosine kinase in most of the cases and harbor mutations of importance for individualized treatment. The molecular targets for therapeutic interventions are not only of importance for the treatment of GIST patients but also useful for in the development of novel drug modalities and new strategies in basic cancer therapy. [source] Trends in incidence and survival of mesenchymal neoplasm of the digestive tract within a defined population of Northern Norway,APMIS, Issue 3 2006SONJA ERIKSSON STEIGEN Population-based incidence and survival data for gastrointestinal stromal tumor (GIST) are sparse due to the fact that GIST is a rather novel entity both clinically and pathologically, and has not been registered as a separate entity in population-based cancer registries. The aim of the present study was to reclassify all mesenchymal tumors within a defined population of northern Norway over a time-span of 30 years with the purpose of estimating trends of incidence and survival. One hundred and forty-one patients with mesenchymal neoplasms of the digestive tract were identified: 102 as GISTs, 32 as leiomyomatous tumors, 4 as schwannomas, and 3 as fibromas. Incidence rates of GIST showed a significant increase over the whole period, which was not observed for the non-GIST cases. Analysis of GIST cases showed that cases with more than 5 mitoses per 50 high power fields had an increased expected mortality 4 times that of those with fewer mitoses, and the combination of mitotic count and size of tumor can be recommended for categorizing the tumors into different risk levels. The study confirms that GIST is by far the most frequent mesenchymal neoplasm of the digestive tract and that the incidence has increased over the last 30 years. [source] The 2007 WHO Classification of Tumors of the Nervous System: Controversies in Surgical NeuropathologyBRAIN PATHOLOGY, Issue 3 2008Bernd W. Scheithauer MD Abstract Controversy surrounds the recent 2007 WHO Classification of Tumours of the Nervous System. A number of nosologic issues remain to be resolved, some a reflection of conceptual disagreement, others the result of inadequate data to permit their definitive resolution. Among these and discussed herein are (i) the nosologic place of highly anaplastic oligoastrocytic tumors, (ii) the forms and significance of microvascular changes in high-grade gliomas, (iii) the makeup of the glioneuronal tumors category, (iv) the subclassification of pineal parenchymal tumors of intermediate type, and (v) the classification of principle forms of mesenchymal neoplasms, specifically hemangiopericytoma and solitary fibrous tumor. These issues and others are the substance of this and an upcoming companion article. [source] | ||||||||||||||||||||||