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Rare Tumor (rare + tumor)
Selected AbstractsTumoral Calcinosis of the Scalp: An Unusual Site for a Rare TumorTHE LARYNGOSCOPE, Issue 1 2007Jubbin Jagan Jacob MD Abstract Tumoral calcinosis is a rare calcifying disorder that is associated with deposition of calcium crystals in the periarticular tissues. The mass is most often around the hips, elbows, shoulders, and feet but may be occasionally found elsewhere. We report a case of multiple sporadic tumoral calcinoses in an adult male over the scalp. The scalp as a site of tumoral calcinosis has not been previously reported in adults. Previous surgical excisions done on two occasions had resulted in recurrence of the tumors. This report highlights the need to include tumoral calcinosis in the differential diagnosis of tumors of the scalp. [source] Fine-needle aspiration biopsy of benign adenomyoepithelioma of the breast: Radiologic and pathologic correlation in four casesDIAGNOSTIC CYTOPATHOLOGY, Issue 11 2007Cecilia L. Mercado M.D. Abstract Benign adenomyoepithelioma of the breast is a rare tumor in which the cytologic findings have been described in only a few cases. While benign, the imaging and pathologic features may be mistaken for malignancy. We report the aspiration biopsy findings in four cases of adenomyoepithelioma with radiologic and histologic correlation. Cytopathologists should familiarize themselves with this entity to avoid a misdiagnosis of carcinoma. Diagn. Cytopathol. 2007;35:690,694. © 2007 Wiley-Liss, Inc. [source] Fine needle aspiration biopsy of epithelioid hemangioendothelioma of the oral cavity: Report of one case and review of literatureDIAGNOSTIC CYTOPATHOLOGY, Issue 3 2006Guo-Xia Tong M.D., Ph.D. Abstract Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor with biological behavior intermediate between hemangioma and angiosarcoma. It rarely occurs in the oral cavity. We report a case of an 81-yr-old woman with a 2-mo history of a 2 × 2 cm2 submucosal buccal mass. Fine needle aspiration (FNA) smears were paucicellular and showed mainly single atypical large epithelioid cells in a bloody background. The atypical cells had abundant dense cytoplasm, some with fine vacuoles. Occasionally, cells with large cytoplasmic lumina were seen. Cytology preparations from fresh tissue received for frozen section revealed numerous neoplastic cells with large intracytoplasmic lumina, some of which contained red blood cells. In addition, cells with distinct intranuclear inclusions were present. Histologic sections and immunohistochemical stains confirmed the diagnosis of EHE. Although the histologic features of EHE are well recognized, reports of FNA cytology findings are sparsely existent in the literature as several case reports. The characteristic cytological features of EHE are reviewed in this report. We believe that the diagnosis of this rare tumor can be suggested when an adequate FNA specimen is obtained. Diagn. Cytopathol. 2006;34:218,223. © 2006 Wiley-Liss, Inc. [source] Biological indicators of prognosis in Ewing's sarcoma: An emerging role for lectin galactoside-binding soluble 3 binding protein (LGALS3BP)INTERNATIONAL JOURNAL OF CANCER, Issue 1 2010Diana Zambelli Abstract Starting from an experimental model that accounts for the 2 most important adverse processes to successful therapy of Ewing's sarcoma (EWS), chemoresistance and the presence of metastasis at the time of diagnosis, we defined a molecular signature of potential prognostic value. Functional annotation of differentially regulated genes revealed 3 major networks related to cell cycle, cell-to-cell interactions and cellular development. The prognostic impact of 8 genes, representative of these 3 networks, was validated in 56 EWS patients. High mRNA expression levels of HINT1, IFITM2, LGALS3BP, STOML2 and c-MYC were associated with reduced risk to death and lower risk to develop metastasis. At multivariate analysis, LGALS3BP, a matricellular protein with a role in tumor progression and metastasis, was the most important predictor of event-free survival and overall survival. The association between LGALS3BP and prognosis was confirmed at protein level, when expression of the molecule was determined in tumor tissues but not in serum, indicating a role for the protein at local tumor microenvironment. Engineered enhancement of LGALS3BP expression in EWS cells resulted in inhibition of anchorage independent cell growth and reduction of cell migration and metastasis. Silencing of LGALS3BP expression reverted cell behavior with respect to in vitro parameters, thus providing further functional validation of genetic data obtained in clinical samples. Thus, we propose LGALS3BP as a novel reliable indicator of prognosis, and we offer genetic signatures to the scientific communities for cross-validation and meta-analysis, which are indispensable tools for a rare tumor such as EWS. [source] Primary mucinous eccrine carcinoma of the skin , a rare clinical tumor with many differential diagnosesJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 6 2010Ina M. Scholz Summary Primary mucinous eccrine carcinoma of the skin is an extremely rare tumor most frequently occurring in the face or capillitium. It is felt to be an eccrine carcinoma, characterized by slow progress and unpredictable locally infiltrative growth. Therefore micrographic surgery is important to avoid local recurrence. It can be difficult to differentiate this carcinoma histologically from cutaneous metastases of breast or gastrointestinal tumors. Because of this, a primary staging in affected patients is essential. [source] Myxoinflammatory fibroblastic sarcoma of the neckJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2005Rob McFarlane Background:, Myxoinflammatory fibroblastic sarcoma (MIFS), also named inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg cells, is a rare tumor typically presenting as a painless mass in the extremities. Patients:, We present an unusual case of MIFS presenting as a subcutaneous neck mass. This is the first reported case of MIFS presenting in the neck. Results:, Therefore, this lesion must be considered in the differential diagnosis for painless subcutaneous masses presenting not only in the distal extremities, but also in the neck. Conclusion:, MIFS has only recently been recognized. The differential diagnosis for MIFS is broad, and it can often be mistaken for several different inflammatory and neoplastic processes, which may require different treatment. [source] Lymphoepithelioma-Like Carcinoma of the Skin: A Case ReportJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005T. Mitsuhashi Lymphoepithelioma-like carcinoma of the skin is a rare tumor with a microscopic resemblance to lymphoepitheliomatous carcinoma of the nasopharynx. We present a 93-year-old man with papules on the left auricle to the cheek that were gradually enlarged. By the time of a biopsy, it grew to a 5.0 × 3.0 × 2.8 cm dark red mass, and necrotic debris was attached to the surface. Histologically, a relatively well-demarcated, dermal-hypodermic multiple lobules were composed of irregular islands of atypical epithelial cells. The uniform tumor cells had moderate amounts of lightly eosinophilic cytoplasm and vesicular nuclei with one or two prominent nucleoli. A dense lymphocytic infiltrate was present within the neoplastic islands, obscuring the epithelial component. The neoplastic cells were unconnected to the overlying epidermis. Neither squamous nor glandular differentiation was present. Immunohistochemically, the neoplastic cells were positive for epithelial membrane antigen and cytokeratin, and negative for latent membrane protein 1. No Epstein-Barr virus-encoded RNA (EBER) was detected by in situ hybridization. The negativity for Epstein-Barr virus may be a help in the differential diagnosis from metastatic undifferentiated nasopharyngeal carcinoma, which is uniformly positive for EBER. [source] Intradermal spindle cell/pleomorphic lipoma of the vulva: case report and review of the literatureJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2002Jorge S. Reis-Filho Background: Spindle cell/pleomorphic lipoma (SC/PL) is a benign adipose tissue tumor that usually affects the subcutaneous tissues of shoulders, backs, and neck region of middle-aged male patients. Histologically, it is characterized by the presence of primitive CD34-positive spindle cells arranged in short fascicles, bizarre floret-like multinucleated giant cells, mature adipocytes, and a small number of lipoblasts. Recently, an intradermal subset has been described, which mainly affects female patients and presents a wider antomical distribution when compared to the classical variant of SC/PL. Methods: We report a case of intradermal SC/PL affecting the labium majus of a 56-year-old female patient. Results: The histological examination disclosed the typical histological features, however the lesion showed poorly demarcated and infiltrative borders, as well as involvement of dermal nerves. The immunohistochemical analysis according to streptovidin-biotin-peroxidase technique showed immunoreactivity for CD34 and vimentin in the spindle cells, as well as S100 protein and vimentin in the adipocytic cells. Conclusions: To the best of our knowledge, this is the first case of intradermal SC/PL affecting the vulvar region. Care must be taken not to misdiagnosis this rare tumor as well-differentiated liposarcoma, cellular angiofibroma, solitary fibrous tumor, and cutaneous neurofibroma. [source] Primary mucinous cystadenocarcinoma of the appendix: An unusual presentation of a rare tumorJOURNAL OF DIGESTIVE DISEASES, Issue 3 2008Nikolaos S SALEMIS [source] Ectomesenchymal chondromyxoid tumor of the hard palate , a case reportJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 2 2006Sonu Nigam Ectomesenchymal chondromyxoid tumor (ECMT) is a rare tumor. Only 26 cases of ECMT have been reported, all occurred in the anterior tongue. We present a case of a 30-year-old male with a nodule in the hard palate, which was reported as ECMT on histopathology. The differential diagnosis considered included are extraskeletal myxoid chondroma, ECMT, pleomorphic adenoma, oral focal mucinosis, chondroid choristoma, and ossifying fibromyxoid tumor. After serial sections no other component was observed and a diagnosis of ECMT was made by exclusion. The patient is asymptomatic on follow up. A review of existing literature is also presented here. [source] Liver transplantation for non,hepatocellular carcinoma malignancy: Indications, limitations, and analysis of the current literatureLIVER TRANSPLANTATION, Issue 8 2010Eric J. Grossman Orthotopic liver transplantation (OLT) is currently incorporated into the treatment regimens for specific nonhepatocellular malignancies. For patients suffering from early-stage, unresectable hilar cholangiocarcinoma (CCA), OLT preceded by neoadjuvant radiotherapy has the potential to readily achieve a tumor-free margin, accomplish a radical resection, and treat underlying primary sclerosing cholangitis when present. In highly selected stage I and II patients with CCA, the 5-year survival rate is 80%. As additional data are accrued, OLT with neoadjuvant chemoradiation may become a viable alternative to resection for patients with localized, node-negative hilar CCA. Hepatic involvement from neuroendocrine tumors can be treated with OLT when metastases are unresectable or for palliation of medically uncontrollable symptoms. Five-year survival rates as high as 90% have been reported, and the Ki67 labeling index can be used to predict outcomes after OLT. Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin. The data from single-institution series are limited, but compiled reviews have reported 1- and 10-year survival rates of 96% and 72%, respectively. Hepatoblastoma is the most common primary hepatic malignancy in children. There exist subtle differences in the timing of chemotherapy between US and European centers; however, the long-term survival rate after transplantation ranges from 66% to 77%. Fibrolamellar hepatocellular carcinoma is a distinct liver malignancy best treated by surgical resection. However, there is an increasing amount of data supporting OLT when resection is contraindicated. In the treatment of either primary or metastatic hepatic sarcomas, unacceptable survival and recurrence rates currently prohibit the use of OLT. Liver Transpl 16:930-942, 2010. © 2010 AASLD. [source] Intrinsic haemangioma of the median nerve: Report of a case and review of the literatureMICROSURGERY, Issue 2 2008Marios D. Vekris M.D. Intrinsic haemangioma of the median nerve is an extremely rare tumor that represents a challenge to diagnose and treat. Only a few cases have been reported in the literature. We present a 10-year-old girl who was diagnosed having an intrinsic haemangioma of the median nerve and treated with total surgical resection of the tumor, under high magnification, using microneurolysis and without the need to resect and graft the median nerve. Three years later, the patient is free of symptoms and no recurrence of the mass was noticed. © 2008 Wiley-Liss, Inc. Microsurgery, 2008. [source] Mucoepidermoid carcinoma of the thyroid gland showing marked ciliation suggestive of its pathogenesisPATHOLOGY INTERNATIONAL, Issue 11 2008Mizuo Ando Mucoepidermoid carcinoma of the thyroid gland is a rare tumor first described by Rhatigan et al. in 1977. Its pathogenesis is still controversial. With regard to its most likely origin, some authors have suggested that it arises directly from follicular epithelium whereas others have proposed that it arises from ultimobranchial body (diverticulum from the fourth pharyngeal pouch) remnants, also known as solid cell nests (SCN). Herein is reported a unique case of thyroid mucoepidermoid carcinoma. The patient, a 67-year-old man, presented with a non-tender thyroid mass and vocal cord fixation. The tumor was poorly defined, necessitating subtotal thyroidectomy with composite resection of the adjacent structures. Pathologically, the tumor cells had characteristics of mucoepidermoid carcinoma, along with layers of columnar cells showing marked ciliation resembling respiratory-type epithelium, suggesting that this rare tumor had originated from SCN. p63 immunopositivity in the tumor provided additional evidence for the pathogenesis. [source] Fibrocartilaginous mesenchymoma of the distal femur: Case report and literature reviewPATHOLOGY INTERNATIONAL, Issue 8 2001Gokhan Gedikoglu A case of a 9-year-old boy with fibrocartilaginous mesenchymoma located in the distal femur is presented. Fibrocartilaginous mesenchymoma is a rare tumor. There have been 17 cases reported since 1984. The presenting complaint was pain and local discomfort. Radiological examination showed lytic and sclerotic lesions located in the distal femur. Magnetic resonance imaging showed that there was no soft tissue expansion. Differential diagnosis included fibrocartilaginous dyplasia, dedifferentiated chondrosarcoma, desmoplastic fibroma, fibrosarcoma and low-grade osteosarcoma. The diagnosis of the tumor in a state hospital would not have been possible. Wide resection of the tumor and resection arthrodesis with an intramedullary nail has yielded good results. The patient has been disease free for 4 years. Pathological diagnosis of this very rare tumor may be challenging; the treatment should be wide resection of the tumor. [source] Signet-ring cell carcinoma of the breastPATHOLOGY INTERNATIONAL, Issue 1 2000Shian-Min Liu Primary signet-ring cell carcinoma of the breast is a very rare tumor and is not recognized as an independent entity of the World Health Organization classification of breast tumor. Primary signet-ring cell carcinoma of the breast is usually considered as a variant of mucinous carcinoma or lobular carcinoma and usually originates from the lobular epithelium. A case of primary signet-ring cell carcinoma of the breast in a 68-year-old woman is presented. Histologically, the majority of neoplastic cells had an intracytoplasmic mucin collection. The histological presence of ductal carcinoma in situ, absence of lobular lesion and immunoreactivity for estrogen and progesterone receptors implicated the tumor cells arising from ductal epithelium. The papillary or organoid growth pattern is characteristic in this case. The patient underwent a modified radical mastectomy and was subsequently followed up for 6 months. [source] Regression of a congenital mesoblastic nephroma,PEDIATRIC BLOOD & CANCER, Issue 2 2010Sarah Whittle BA Abstract Histologically, the cellular variant of congenital mesoblastic nephroma (CMN) is very similar to another rare tumor of infancy, infantile fibrosarcoma (IFS). In addition to the histologic similarities, these tumor types share cytogenetic abnormalities including translocation t(12;15)(p13;q25). We describe herein the case of a child who did not have immediate surgical resection of a CMN and whose tumor was untreated for 8 months. During that time, the tumor demonstrated a significant degree of regression. The shared translocation with IFS, a tumor with well-documented potential for spontaneous regression, suggests that this genetic abnormality may have contributed to the favorable clinical course. Pediatr Blood Cancer. 2010;55:364,368. © 2010 Wiley,Liss, Inc. [source] Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma,,PEDIATRIC BLOOD & CANCER, Issue 3 2007John R. Priest MD Abstract Background Pleuropulmonary blastoma (PPB) is a rare tumor of pleura and lung in young children. Central nervous system (CNS) complications, particularly cerebral parenchymal metastases, occur in aggressive forms of PPB: Types II and III PPB. This article evaluates cerebral and meningeal metastases, cerebrovascular events (CVA) caused by tumor emboli, spinal cord complications, and intracranial second malignancies in PPB. Procedure International PPB Registry and literature cases were evaluated for CNS events. Cerebral metastasis patients were evaluated for gender, side of origin of PPB, PPB Type, interval from diagnosis to metastasis, status of chest disease, treatment, and outcome. Standard statistical methods were used to calculate the cumulative probability of cerebral metastasis and survival following metastasis. Results Thirty-nine cases of cerebral metastasis were identified in 5/53 Registry Type II cases, 15/44 Registry Type III cases, and 19/143 literature Type II/III cases. Metastases occurred 1,60, median 11.5 months after diagnosis. Chest disease was controlled in 50% of children at time of metastasis. The cumulative probability of cerebral metastasis by 5 years from diagnosis was 11% for Type II patients (95%CI (confidence interval): 2,20%) and 54% for Type III patients (95%CI: 31,76%). Seven children survive cerebral metastasis. Other CNS complications were post-operative CVA (five cases), spinal cord invasion or compression (six), leptomeningeal disease (three), and second intracranial malignancies (two). Conclusions Cerebral metastasis is more frequent in PPB than in other childhood sarcomas. Clinicians should screen for this complication. Diverse other CNS complications are less common and require careful diagnosis. Pediatr Blood Cancer 2007;49:266,273. © 2006 Wiley-Liss, Inc. [source] Salivary gland neoplasms in children: The experience of the Istituto Nazionale Tumori of MilanPEDIATRIC BLOOD & CANCER, Issue 6 2006Marco Guzzo MD Abstract Background Epithelial salivary gland tumors are very uncommon in pediatric age. We report a series of 52 cases treated at the Istituto Nazionale Tumori of Milan, Italy, over a 30-year period. These results are presented in conjunction with a literature review of salivary tumors with a view to providing an up-to-date overview of the clinical course, prognosis, and treatment options for this rare tumor. Procedure Fifty-two cases of epithelial salivary tumors were reviewed and the clinical-pathological information concerning tumor characteristics, therapy, and follow-up were collected. Patients' age ranged between 4 and 18 years. Results The major salivary glands were the main site of tumor occurrence (79% of cases arose in parotid glands); 37 patients had benign tumors (pleomorphic adenoma), 15 had malignant tumors (12 mucoepidermoid carcinoma, 9 low grade). All the patients were treated by surgery; local relapses after parotidectomy were 4% and 25%, in benign and malignant tumors, respectively. When tumor enucleation was performed, recurrences occurred in 50% of benign neoplasms. At the time of the report, all patients with benign tumors were alive, 35(95%) without evidence of disease; only one patient with malignant tumor died of disease. Conclusions Epithelial salivary glands tumor in children had different characteristics compared with their adult counterpart with respect to the frequency of histotypes and site of occurrence, but their prognosis seems to be similar. Parotidectomy (total or superficial) is the best choice for achieving good cure rates in both benign and malignant tumors. Pediatric Blood Cancer 2006;47:806,810. © 2006 Wiley-Liss, Inc. [source] Isolated Plexiform Neurofibroma: Treatment with Three-Dimensional Conformal Radiotherapy,THE LARYNGOSCOPE, Issue 7 2004Thomas C. Robertson MD Abstract Objectives: To present a case of an unusual benign tumor of the tongue treated successfully with radiotherapy. Study Design: Case report. Methods: Retrospective chart review. Results: A 60-year-old man presented with a painful submucosal lesion of the tongue base. Computed tomography showed an infiltrative soft-tissue mass involving the left base of the tongue. Operative biopsy revealed plexiform neurofibroma. Because of the patient's operative risk and the potential morbidity of surgical resection, he was treated with three-dimensional conformal radiotherapy (3DCRT). His treatment was accomplished using a five-field arrangement treating exclusively the mass lesion to a total tumor dose of 60 Gy. After treatment, the patient's tongue pain resolved, and he noted minimal transient xerostomia. Serial follow-up radiographic examinations showed the base of tongue mass to be slightly smaller 4 months after treatment. The most recent follow-up magnetic resonance image reveals a further decrease in size of the mass. The patient is now over 3 years out from treatment. Conclusions: Solitary plexiform neurofibroma of the tongue base is a rare tumor. These benign neoplasms are usually treated with either observation or surgical excision. This case demonstrates that, when significant symptoms necessitate active management, these lesions may be successfully treated with minimal morbidity using 3DCRT. The ability of this technique to deliver a conformal radiation dose to the tumor volume while sparing the surrounding normal tissues may expand the application of radiotherapy in the treatment of these benign lesions of the head and neck. [source] First Evidence of Genetic Imbalances in AngiofibromasTHE LARYNGOSCOPE, Issue 2 2002Bernhard Schick MD Abstract Objective/Hypothesis Angiofibromas are clinically well characterized by their origin at the posterior lateral nasal wall close to the sphenopalatine foramen, their occurrence in male adolescent patients, and the histological findings of a benign fibrovascular neoplasm with irregular, endothelium-lined vascular spaces in a fibrous stroma. However, their etiology and genetic causes remain unknown. The present study addresses genetic imbalances in angiofibromas. Study Design The present pilot study compared genomic hybridization in three angiofibromas to search for chromosomal abnormalities in this rare tumor. Methods Fluorescence-marked normal DNA and angiofibroma DNA were compared using genomic hybridization screening to detect chromosomal abnormalities. Their binding ratio to metaphase chromosomes were analyzed by special digital image analysis. Results Chromosomal gains and losses showing a high level of agreement were detected in all three angiofibromas. Specifically, DNA gains were observed on chromosomes 3q, 4q, 5q, 6q, 7q, 8q, 12p, 12q, 13q, 14q, 18q, 21q, and X, and DNA losses were screened on chromosomes 17, 19p, 22q, and Y. Finding chromosomal abnormalities at the sex chromosomes X and Y of this rare tumor is remarkable. Concurrent chromosomal gain on 8q12q22 was noted in all three tumor specimens. Conclusions Comparative genomic hybridization is suitable for screening angiofibromas on a genetic level. The results on these screens indicate that further genetic investigations of this rare benign tumor may provide more details about the tumor's genetic abnormalities and perhaps clarify the etiology of angiofibromas. [source] Laryngeal Angiosarcoma: A Clinicopathologic Study of Five Cases With a Review of the Literature,THE LARYNGOSCOPE, Issue 7 2001Benjamin M. Loos MD Abstract Objective Primary laryngeal angiosarcoma (LA) is rare without a reported series evaluating these tumors. Study Design/Methods Five patients with LA were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology. Results Three men and 2 women, aged 29 to 71 years, presented with hoarseness (n = 4) and hemoptysis (n = 1). Two patients had previous neck radiation. The tumors involved the supraglottis (n = 4) with a mean size of 3.1 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, frequent atypical mitotic figures, and hemorrhage. All cases tested (n = 4) demonstrated immunoreactivity with antibodies to Factor VIII-RA and CD34. All patients had surgery followed by postoperative radiation (n = 3 patients). Three patients died with disease (mean, 17 mo), whereas one patient is alive with no evidence of disease at 18 years. Conclusions LA is a rare tumor, frequently associated with previous radiation, usually involving the supraglottis with characteristic histomorphologic and immunophenotypic features. LA has a poor prognosis, making appropriate separation from other conditions important. [source] Alveolar soft part sarcoma of the endometrium with expression of CD10 and hormone receptors,APMIS, Issue 7 2007Case Report Alveolar soft part sarcoma (ASPS) is a rare tumor of uncertain histogenesis, mainly localized in the extremities. ASPS originating in the uterine corpus is quite rare; only eight such cases have been reported in the literature. We here present another case of ASPS found in the endometrium in a 50-year-old woman. Metastatic malignant tumor, including ASPS from other organs, was excluded by physical examination and imaging modalities. Thallium 201 was only localized in the uterus. The tumor showed characteristic histological features of ASPS: alveolar architecture with fibrovascular septa and abundant eosinophilic granular cytoplasm with periodic acid-Schiff-positive crystalline material. Diffuse nuclear immunoreactivity for TFE3, a marker recently reported to be specific for ASPS, further supported the diagnosis of ASPS. Interestingly, this tumor was negative for myogenic markers, but positive for CD10, progesterone receptor, and estrogen receptor. These immunohistochemical results and the tumor location suggest a possible link between endometrial stromal cells and the development of this tumor. [source] A Common Tumor, An Uncommon Location: Basal Cell Carcinoma of the Nipple and Areola in a 49-Year-Old WomanDERMATOLOGIC SURGERY, Issue 4 2005Nathan Rosen MD Background. Basal cell carcinoma (BCC) occurring on sun-protected regions is an uncommon phenomenon. BCC of the nipple is an exceedingly rare event. Method. We review the literature on BCC of the female nipple and herein describe the eighth reported case in the English literature. Our patient was treated with Mohs micrographic surgery and sentinel lymph node biopsy. Conclusion. BCC of the nipple are extremely rare tumors with unclear etiology. They can be aggressive and are capable of causing significant morbidity and mortality if they are neglected or improperly treated. With continued reporting of the diagnosis, treatment, and follow-up of these patients, we may gain an understanding of the pathogenesis, as well as the best method of control for these unusual tumors. [source] Epithelioid angiosarcoma: A neoplasm with potential diagnostic challengesDIAGNOSTIC CYTOPATHOLOGY, Issue 2 2010Christine F. Lin B.S. (Student) Abstract Epithelioid angiosarcomas are extremely rare tumors associated with poor prognosis and early metastases. Its epithelioid cytomorphology and limited vasoformation make it difficult to distinguish from more common malignancies, such as, carcinoma. This can be a potential diagnostic pitfall for the cytopathologist. In this report, the patient is a 24-year-old man presenting with testicular pain, a pelvic mass, and innumerable liver nodules. Immediate interpretation of the needle core biopsies of the pelvic mass and liver lesions initially favored a poorly differentiated adenocarcinoma. Unusual positive immunohistochemical stains for CD30 and CK7 ultimately led the investigation toward a tumor of mesenchymal origin. Further, immunohistochemical evaluation demonstrated positive CD31 and Factor VIII staining and established the final diagnosis of epithelioid angiosarcoma. The tumor cells were negative for CD34, CK20, alpha-fetoprotein, placental-like alkaline phosphatase, hepatocyte paraffin 1, polyclonal carcinoembryonic antigen, CD10, CA-125, prostate-specific antigen, and prostatic acid phosphatase. This case is reported to illustrate the importance of considering the diagnosis of epithelioid angiosarcoma when encountering an "epithelioid" neoplasm particularly with unusual immunoreactivity for CK7 and CD30. Diagn. Cytopathol. 2010. © 2009 Wiley-Liss, Inc. [source] Genome-wide analysis of genetic changes in intestinal-type sinonasal adenocarcinomaHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 3 2009Mario A. Hermsen PhD Abstract Background Intestinal-type sinonasal adenocarcinomas are rare tumors related to professional exposure to wood dust. Little is known about the genetic changes in these tumors. Methods Twenty-two tumors were analyzed by microarray comparative genomic hybridization (CGH). In addition, DNA ploidy was measured by flow cytometry and microsatellite instability (MSI) by multiplex PCR. Results The most frequent gains were, in descending order, as follows: 5p15, 20q13, and 8q24. Losses occurred most frequently at 4q31-qter, 18q12-22, 8p12-pter, and 5q11-qter. MSI was not detected. Seven cases that harbored very few changes were mostly DNA diploid and had more favorable clinicopathological features, such as lack of intracranial invasion, less metastases, and longer overall survival. Conclusion The microarray CGH results enabled to better define hotspots of chromosomal gains and losses for further investigation of genes involved in the tumorigenesis of sinonasal adenocarcinoma. In addition, the data allowed classification of a group of patients with better clinical outcome. © 2008 Wiley Periodicals, Inc. Head Neck, 2009 [source] Villous adenoma of the urinary bladderINTERNATIONAL JOURNAL OF UROLOGY, Issue 6 2008Wooseuk Sung Abstract: Villous adenomas arising in the urinary tract are an uncommon occurrence. They have been identified in the urachus, urethra, prostate, and throughout the bladder. Villous adenomas arising in the bladder are rare tumors that have been described as isolated cases and a few case series. We report a new case of a large villous adenoma arising in the bladder that was treated by transurethral resection. [source] Primary synovial sarcoma of the kidneyINTERNATIONAL JOURNAL OF UROLOGY, Issue 8 2005ADAM E PERLMUTTER Abstract Primary renal synovial sarcomas (SS) are rare tumors of the kidney. Faria first described primary renal synovial sarcoma in 1999. Twenty-one cases of primary renal synovial sarcoma have been reported to date. Primary renal synovial sarcomas can exist in either a monophasic or a biphasic pattern. The monophasic variant of primary renal synovial sarcoma is more common and tends to have a better prognosis than the biphasic variant. We present the case of a 61 year-old woman with a monophasic variant of primary renal synovial sarcoma. [source] An infiltrative variant of non-neural granular cell tumor: a case reportJOURNAL OF CUTANEOUS PATHOLOGY, Issue 2009Iwei Yeh Dermal non-neural granular cell tumors are rare tumors of indeterminate lineage that typically present as well-circumscribed tumors with nuclear pleomorphism and mitotic activity. We describe a dermal non-neural granular cell tumor with a distinctive growth pattern with granular cells interspersed between collagen bundles. This asymptomatic papule arose on the scapula of a 46-year-old woman and consisted of a mixture of epithelioid and spindled granular cells. The immunohistochemical characteristics were similar to those of previously reported dermal non-neural granular cell tumors. Despite mild nuclear pleomorphism and dispersion of lesional cells among collagen bundles, mitoses were not present and Ki-67 staining indicated a low proliferative rate. In addition to being S-100 protein negative and NKI/C3 positive, our case was positive for PGP9.5 and weakly positive for neuron-specific enolase, a staining pattern similar to what has been observed for cellular neurothekeomas. Our case could represent a dermal non-neural granular cell tumor with unique architecture, a granular cellular neurothekeoma or a granular cell dermatofibroma. As both dermal non-neural granular cell tumor and cellular neurothekeoma are of indeterminate lineage, our case with features characteristic of both entities may suggest a common precursor or lineage for dermal non-neural granular cell tumor and cellular neurothekeoma. [source] Retroperitoneal sarcomas: Combined-modality treatment approachesJOURNAL OF SURGICAL ONCOLOGY, Issue 1 2006Chandrajit P. Raut MD Abstract Retroperitoneal sarcomas (RPS) are rare tumors, accounting for approximately 15% of soft tissue sarcomas. Surgical resection of localized tumors with gross and microscopically negative margins remains the standard of care. However, because RPS are frequently large and locally advanced, resections are often incomplete, resulting in local recurrence. Investigators are evaluating combined-modality therapies to improve local control and disease-specific survival. This review outlines current concepts and evolving treatment strategies in the diagnosis, staging, and management of RPS. J. Surg. Oncol. 2006;94:81,87. © 2006 Wiley-Liss, Inc. [source] Thymic carcinoma in children: A report from the Polish pediatric rare tumors study,PEDIATRIC BLOOD & CANCER, Issue 7 2010Teresa Stachowicz-Stencel MD Abstract Background Invasive thymomas and thymic carcinomas are rare tumors jointly accounting between 0.2% and 1.5% of malignancies in adults. They are usually at an advanced stage when diagnosed and have both high recurrence and poor survival rates. In this report, the aim is to explore our experience in the treatment of thymic carcinomas in Polish children. Procedure The clinical data of nine children with thymic carcinomas, treated between 1992 and 2008 in the Polish oncological and surgical centers was retrospectively analyzed. Results In five cases, presenting symptoms resulted from the compression of the respiratory ways by the mediastinal tumor. In two children paraneoplastic autoimmune syndromes were associated with thymic carcinoma. In accordance with the Masaoka classification, two patients had stage II, five had stage III, and two had stage IV of the disease. Diagnostic biopsy of mediastinal tumor was performed on eight patients and one underwent complete primary resection and subsequently received radiotherapy; he has passed 11 years since the conclusion of therapy. Six patients received multi-drug chemotherapy with or without steroids. Delayed surgery was performed in four children (R0-2, R1-1, and R2-1). After complete resection, one child received chemotherapy. In three patients, chemotherapy and radiotherapy was administered. Seven patients died, including six due to progression of the disease with the other as a result of complications following chemotherapy; only two patients classed at stage II remain alive. Conclusions Most thymic tumors in pediatric patients are inoperable at diagnosis, which results in poor prognosis. Improved chemotherapy approaches are needed. Pediatr Blood Cancer 2010;54:916,920 © 2010 Wiley-Liss, Inc. [source] | ||||||||||||||||||||||||||||