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Rare Neoplasm (rare + neoplasm)
Selected AbstractsSkin Angiosarcoma Arising in an Irradiated Breast: Case-Report and Literature ReviewDERMATOLOGIC SURGERY, Issue 3 2006FAUSTO CATENA MD BACKGROUND Angiosarcoma (AS) is a rare, invasive malignancy originating from endothelial cells caused by many different clinical situations. AS following radiotherapy for breast cancer after conservative surgery is a rare but well-known association. OBJECTIVE The aim of this article is to describe a case of AS after breast conserving surgery and to review the literature to date. RESULTS We report the case of an 84-year-old woman who developed AS four years after she was subjected to quadrantectomy for invasive ductal cancer, followed by 30 tangent field radiotherapy sessions. She presented with a one-month history or red papular skin eruptions on the operated breast. Skin lesions were submitted for biopsy, and they were positive for AS. The patients was subjected to surgical excision of the remaining breast including all AS lesions. She is alive with no evidence of disease after 10 months follow-up. CONCLUSION Post-radiotherapy AS is rare neoplasm, but it should be considered in the case of patients with red lesions after breast conserving surgery and adjuvant radiotherapy. [source] Malignant Glomus Tumor: A Case Report and Review of the LiteratureDERMATOLOGIC SURGERY, Issue 9 2001John D. Kayal MD Glomangiosarcoma, or malignant glomus tumor, is a very rare neoplasm that when seen typically arises from a benign glomus tumor. Despite having histologic features of malignancy, these tumors usually do not metastasize. However, when metastasis occurs this disease is often fatal. We report a case of a malignant glomus tumor arising de novo on the nose of an 89-year-old white woman, and we review the literature concerning glomangiosarcomas. [source] Intracytoplasmic lumina in invasive micropapillary carcinoma of the lungDIAGNOSTIC CYTOPATHOLOGY, Issue 3 2006Naoto Kuroda M.D. Abstract Micropapillary carcinoma of the lung is a rare neoplasm, and several reports on micropapillary carcinoma of the lung have been presented to date. We present a case of micropapillary carcinoma of the lung here. A 75-yr-old Japanese man received the medical checkup and his chest X-ray disclosed the abnormal shadow of the lower lobe of the left lung. The histological examination of resected lung and extirpated lymph node showed the finding of micropapillary carcinoma. Some neoplastic cells of primary site contained intracytoplasmic lumina positive for Alcian blue and PAS stains. Pleural effusion appeared 9-mo after the operation. The cytology of pleural effusion showed cohesive clusters of neoplastic cells consisting of 3,20 cells without fibrovascular core. Additionally, intracytoplasmic lumina were observed in some neoplastic cells. Finally, carcinoma cells with micropapillary morphology may possess the intracytoplasmic lumina in the cytoplasm of metastatic site as well as primary site. Diagn. Cytopathol. 2006;34:224,226. © 2006 Wiley-Liss, Inc. [source] Cervicovaginal (Papanicolaou) smear findings in patients with malignant mixed Müllerian tumorsDIAGNOSTIC CYTOPATHOLOGY, Issue 5 2003Mary B. Casey M.D. Abstract Malignant mixed Müllerian tumor is a rare neoplasm that occurs most frequently in elderly patients. It is characterized by a mixture of malignant epithelial and sarcomatous components. Little has been published about Papanicolaou smear findings pertaining to malignant mixed Müllerian tumors. We present our experience, with an emphasis on cytologic detail. Nine patients (median age, 65 yr) met our study criteria. All available smears and surgical specimens were reviewed. Four smears were positive for malignancy, with a sensitivity of 44% (3 adenocarcinoma, and 1 squamous-cell carcinoma, small-cell type). The results of our study showed that Papanicolaou smear findings pertaining to malignant mixed Müllerian tumors are seen in patients with advanced-stage disease with involvement of the lower uterine segment or cervix. The usual finding is large numbers of high-grade epithelial malignant cells in a necrotic background. The mesenchymal component rarely sheds cells visible on Papanicolaou smear. Diagn. Cytopathol. 2003;28:245,249. © 2003 Wiley-Liss, Inc. [source] A case of small-cell gastric carcinoma with an adenocarcinoma component and hepatic metastases: treatment with systemic and intra-hepatic chemotherapyEUROPEAN JOURNAL OF CANCER CARE, Issue 5 2007T. CIOPPA md Primary small-cell carcinoma (SmCC) of the stomach is a rare neoplasm with a poor prognosis and unclear histogenesis: to date, only 50 cases, including ours, have been reported in the literature. In the World Health Organization gastrointestinal tumours' classification, SmCC of the stomach has been recognized as an ,independent entity affecting the stomach'. In this paper, the authors present a clinical case and the surgical treatment of an adult with a SmCC of the stomach associated with gastric adenocarcinoma. After laparotomy, a large neoplasm with locoregional extension and multiple liver metastases were found. A palliative resection, subtotal gastrectomy, was performed, followed by systemic and intra-hepatic chemotherapy: computed tomography scan demonstrated a marked response, but the patient died 15 months after the operation. A review of the literature showed that the diagnosis of gastric SmCC is based on immunohistochemical findings. Our experience confirmed the high aggressiveness of this neoplasm, which is generally diagnosed in advanced stage and is unresponsive to chemotherapy, but the combined use of systemic and intra-hepatic chemotherapy shows an acceptable result in a palliative care perspective. [source] Mucoepidermoid carcinoma of Stensen's duct: A case report and review of the literatureHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 9 2005Roland Giger MD Abstract Background. Mucoepidermoid carcinoma of Stensen's duct is a rare neoplasm, with only five cases reported in the literature. Methods. We report another case of mucoepidermoid carcinoma of Stensen's duct and review the literature. Results. Stensen's duct neoplasms tend to be symptomatic at an early stage by causing an obstruction of the parotid duct. New imaging techniques such as MR sialography and sialoendoscopy are very helpful in diagnosis and patient management. Conclusions. Although the rarity of this condition prevents definitive conclusions about the optimal treatment, we propose that Stensen's duct neoplasms should be treated like similar neoplasms occurring in the parotid gland tissue, taking into consideration clinical stage, tumor grade, and surgical margins. © 2005 Wiley Periodicals, Inc. Head Neck27: XXX,XXX, 2005 [source] The sarcomatous guise of cervical ectopic hamartomatous thymoma,HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 8 2002Jeff Marschall MD Abstract Background Ectopic hamartomatous thymoma is a rare benign neoplasm occurring in the deep soft tissues adjacent to the sternoclavicular joint. Although clinical presentation and diagnostic imaging can be consistent with a malignant lesion such as a sarcoma, recognition of pathologic features can readily exclude such a diagnosis. However, this remains a challenge caused by their unusual histologic features and diverse composition. Recognition of this tumor is, however, important, because it follows a benign clinical course, and conservative surgical excision is the therapy of choice. Methods A literature review of all reports of ectopic hamartomatous thymoma was undertaken and compared with our indexed case. Results From this review, we identified 26 previous reports. Most patients were men, with a 4.5:1 predominance. There was a wide range of ages at presentation, from 26 to 79 years. All tumors were located in the lower neck, with the exception of one arising presternally. Treatment predominantly consisted of tumor resection, with no reported recurrences on follow-up. Conclusions We conclude that ectopic hamartomatous thymoma is an extremely rare neoplasm usually presenting in the lower neck. Correct identification of this tumor is important, because it follows a benign course, and surgical excision is adequate therapy. © 2002 Wiley Periodicals, Inc. Head Neck 24: 800,804, 2002 [source] Combined endoscopic and laparoscopic en bloc resection of the urachus and the bladder dome in a rare case of urachal carcinomaINTERNATIONAL JOURNAL OF UROLOGY, Issue 4 2007Francesco Porpiglia Abstract: Urachal carcinoma is a rare neoplasm treated with surgical resection. We report a case of adenocarcinoma of the urachus treated with a new surgical technique. In a 44-year-old man affected by urachal carcinoma we performed a combined endoscopic,laparoscopic surgical en bloc resection of the urachus and bladder dome. The procedure lasted 240 min, and no postoperative complications were recorded. The patient was discharged on fourth day and the catheter was removed on eighth day. Bladder capability resulted normal with no evident physical change. Multiple bladder biopsy and computed tomography scans at 6, 12 and 18 months proved negative. [source] Prostate mucinous adenocarcinoma with signet ring cellINTERNATIONAL JOURNAL OF UROLOGY, Issue 4 2003EYUP GUMUS Abstract Prostate mucinous adenocarcinoma with signet ring cell is a rare neoplasm with only 11 cases reported to date. We present the 12th case of prostate mucinous adenocarcinoma with signet ring cell. The case was detected incidentally as a result of a biopsy taken from a lesion in the prostatic urethra during a urethrocystoscopy of a 47-year-old male patient who underwent an internal urethrotomy operation due to urethrostenosis. Endoscopic examination showed a loose, spongy, gray,white structure covering the prostatic urethra, especially the right lobe of the prostate. The diagnosis resulting from the pathological examination of the biopsy was prostate mucinous adenocarcinoma with signet ring cell. A total of 50 Gy radiotherapy was applied to the patient. In the 27th month of follow-up after treatment, thoracoabdominal computed tomography, bone scintigraphy, and tumor markers (PSA, CEA and CA19-9) were found to be normal. [source] Leiomyosarcoma of the Penis Presenting as a Cutaneous LesionJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2008Thomas L. Cibull We report a case of a 68-year-old man with cutaneous leiomyosarcoma of the penis. Leiomyosarcoma of the penis is an extremely rare neoplasm that usually presents in middle to old age, and to our knowledge only approximately 30 cases have been reported in the literature to date. This is an important diagnosis in the differential diagnosis of cutaneous spindle cell neoplasms of the male genital tract. [source] A case of cutaneous myoepithelial carcinomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 8 2007Jin Tanahashi Background:, Cutaneous myoepithelioma, both benign and malignant, is a rare neoplasm composed of neoplastic myoepithelial cells showing diverse histopathological features, and criteria for discriminating benign or malignant have not been fully clarified. Patient:, We present a case of cutaneous myoepithelial carcinoma in a 62-year-old woman presenting a solid mass in the right back. Results:, Resected tumor was located in the whole dermis and subcutis. Histopathologically, two different growth patterns were noted: a small-nested or trabecular pattern in the superficial part and a large nodular pattern with extensive central necrosis in the deep part. Tumor cells were all epithelioid, although plasmacytoid and glycogen-rich clear cells were also observed within the large nodules of the deep part. Immunohistochemically, the cells were positive for both epithelial and myogenic markers, suggesting myoepithelial origin. Lymphatic invasion and lymph node metastasis were evident despite inconspicuous atypia and low mitotic rate. Conclusion:, The final diagnosis was cutaneous myoepithelial carcinoma. At present, it seems to be difficult to predict the behavior of myoepithelioma of the skin and soft tissue, although atypia and high mitotic rate are reported to be associated with local recurrence and metastasis. [source] Epithelioid angiosarcoma of the breast involving the skin: a highly aggressive neoplasm readily mistaken for mammary carcinomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 2 2003M. C. Fariña Background: Angiosarcomas are malignant neoplasms of endothelial cells. Angiosarcoma of the breast is a rare neoplasm that behaves in a highly malignant fashion. It must be differentiated from benign vascular proliferations and from mammary carcinoma. Methods: We report on a 49-year-old-woman who presented with a large mass involving the left breast. Results: The lesion had an erythematoviolaceous hue and livedoid pattern at the periphery. Histopathologic study showed an epithelioid malignant neoplasm, and immunohistochemical studies demonstrated that neoplastic cells expressed immunoreactivity for endothelial cell markers. Conclusions: A diagnosis of epithelioid angiosarcoma of the breast was established. The patient was treated with radical mastectomy, but she refused any other additional therapy. [source] Clear Cell Hidradenocarcinoma Developing in Pacemaker PocketPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 11 2008CESAR V. REYES M.D. An octagenerian woman developed clear cell hidradenocarcinoma, a rare neoplasm of eccrine sweat gland origin, 4 years following pacemaker implantation in her right lateral chest. The tumor immunohistochemically mimicked a metastatic lobular breast carcinoma, for example, strongly positive estrogen, weakly positive progesterone, and weakly reactive mammoglobin. A complete surgical excision of the tumor was complemented with ipsilateral dissection of involved adjacent axillary lymph nodes. Recommended irradiation was refused by the patient. Retrospective 3-year mammogram review, 2-year postsurgery follow-up, and complete postmortem evaluation failed to prove a primary breast malignancy or other metastatic lesion elsewhere. [source] Adenoid basal carcinoma of the uterine cervix: Report of two cases with reference to adenosquamous carcinomaPATHOLOGY INTERNATIONAL, Issue 7 2005Norihiro Teramoto Adenoid basal carcinoma (ABC) of the uterine cervix is a rare neoplasm with excellent prognosis. Differential diagnosis between ABC and an ABC-like lesion of adenosquamous cell carcinoma (ASC) of the cervix is important due to their contrasting prognosis. Reported herein are two cases of ABC that have been compared with seven ASC exhibiting ABC-like lesions from approximately 2600 resected uterine cervical malignancies diagnosed at Shikoku Cancer Center. The two ABC were incidentally found in the uterine cervix of 69-year-old and 59-year-old Japanese women due to cervical intraepithelial neoplasia grade 3 and to squamous cell carcinoma, respectively. The ABC consisted of infiltrating nests of basaloid cells with low nuclear atypia. The patients remained alive without recurrence for 9 years and 18 months, respectively. An ABC-like lesion was defined as basaloid cell nests simulating ABC, but with some features indicating malignant potential. However, the differential diagnosis was sometimes difficult because two of seven ABC-like lesions were originally diagnosed as ABC. Immunohistochemically, cytokeratin 7 was negative for the basaloid cells of two ABC, but positive for six of six ABC-like lesions of ASC, while cytokeratin 8 was positive for both ABC and ASC. This cytokeratin pattern might provide a good tool for distinguishing between ABC and an ABC-like lesion of ASC when the histological findings are equivocal. [source] Small cell carcinoma of the extrahepatic bile duct: Case report and immunohistochemical analysisPATHOLOGY INTERNATIONAL, Issue 12 2003Kazuya Kuraoka A small cell carcinoma of the extrahepatic bile duct in a 75-year-old Japanese man is reported. The patient suffered from obstructive jaundice, and percutaneous transhepatic cholangiography-drainage (PTCD) revealed a massive lesion in the lower common bile duct. Because it was diagnosed as a malignant tumor, pancreaticoduodenectomy was performed. A nodular infiltrating tumor measuring 4.5 × 3.0 × 2.0 cm was located in the intrapancreatic portion of the extrahepatic bile duct. Histologically, the tumor was composed of a dense proliferation of small atypical cells with a little region of high-grade dysplasia in the adjacent epithelium of the common bile duct. Tumor cells were immunoreactive to neuroendocrine markers such as chromogranin A, synaptophysin, CD56, and Leu7. Although carcinoma cells invaded into pancreas and duodenum, there were no histological findings that indicated the carcinoma arose from the mucosa of either the pancreatic duct or duodenum. These results indicated that the tumor was a small cell carcinoma derived from the epithelium of the extrahepatic bile duct; a rare neoplasm with only a few cases reported. A few neuroendocrine cells were recognized in the adjacent epithelium of the extrahepatic bile duct, suggesting that the tumor cells might be derived from them. Using immunohistochemical examination, no p53 abnormality was found. Tumor cells showed positive nuclear staining for p16, while negative for cyclin D1, suggesting that functional retinoblastoma protein (pRB) might be lost in the p16/pRB pathway, as in small cell lung cancer. [source] Malignant glomus tumor in the branchial muscle of a 16-year-old girlPATHOLOGY INTERNATIONAL, Issue 9 2001Kazuhito Matsumoto Malignant glomus tumor is an extremely rare neoplasm and its histological features are not well characterized. We report a 16-year-old female patient with a malignant glomus tumor. The patient was admitted to our hospital presenting with a mass in the right upper arm that she had noticed for the previous 6 months. Computed tomography and magnetic resonance imaging revealed an expanded mass involving the surrounding tissues. At surgery, an ill-defined and expanded mass was found, 5 × 4 × 3 cm in size, in the right branchial muscle. The tumor was extirpated, along with neighboring muscle tissues. Histologically, tumor cells were round to short-spindle shaped, forming solid sheets admixed with vessels of varying size. Their nuclei were uniformly oval to round, and their cytoplasms were slightly eosinophilic. The growth pattern of the tumor cells resembled that of glomus tumor, but mitotic figures were frequent (as high as 10 per 10 high-power fields). Immunohistochemically, the tumor cells were positive for vimentin and muscle actin, but negative for desmin. There were no areas typical of benign glomus tumor or sarcomatous change. These findings led us to a diagnosis of primary malignant glomus tumor arising de novo. There has been no recurrence or metastasis for 21 months after wide excision. [source] Adult-onset giant juxtaglomerular cell tumor of the kidneyPATHOLOGY INTERNATIONAL, Issue 3 2000Naoto Kuroda Abstract The juxtaglomerular cell tumor (JGCT) of the kidney is a rare neoplasm which commonly secretes renin. This tumor often occurs in teenagers. This paper documents the 14th adult-onset (over 30-years-old) case with a giant JGCT which measured 9.0 × 8.0 × 7.5 cm. Histologically, this tumor was composed of both vascular and tubular components. Immunohistochemically, the vascular component reacted with renin, cytokeratin 7, ulex europaeus agglutinin-1, vascular endothelial growth factor (VEGF) and Flk-1 (VEGF-R2), whereas the tubular component was positive for renin, epithelial membrane antigen (EMA), cytokeratin 7, , -1-antitrypsin, VEGF and Flk-1. This finding suggests that both vascular and tubular components of JGCT may promote neoplastic proliferation via an autocrine mechanism through the action of VEGF. [source] Orbital giant cell angiofibroma,APMIS, Issue 9 2006Report of a recurrent case A case of giant cell angiofibroma in a 73-year-old man is reported. The tumour was located in the upper-inner quadrant of the right orbit and recurred 16 months after surgical removal. The clinicopathological features of this rare neoplasm,that is nowadays considered a variant of solitary fibrous tumour,are reviewed and briefly commented on. [source] Cytogenetic analysis of 101 skull base tumorsHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 5 2008Ziv Gil MD Abstract Background. Skull base tumors are rare neoplasms and the cytogenetic data on these tumors are limited. The authors cytogenetically analyzed a large series of tumors and compared the findings with patients' pathologic data. Methods. The karyotypes of pathologically confirmed samples of 101 patients, who were operated for oncological extirpation of tumors, were analyzed using G-banding and spectral-karyotyping techniques. Results. Of the 67 malignant tumors, 32 (48%) had chromosomal aberrations, some with complex numerical and structural chromosomal anomalies. Recurrent chromosomal breakpoints were identified in squamous cell carcinomas, adenoid cystic carcinomas (ACCs), sinonasal undifferentiated carcinomas, chordomas, and sarcomas. Specific breakpoints established the diagnosis of various soft tissue sarcomas. Novel chromosomal aberrations were found in various other malignant and benign tumors. Conclusion. This study highlights the value of cytogenetic analysis for diagnosis of skull base tumors. The data add further information on the biological behavior of these rare neoplasms. © 2007 Wiley Periodicals, Inc. Head Neck, 2008 [source] Myoepithelioma of the Skin with P63 ExpressionJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005M.J. Smith-Zagone A case of cutaneous myoepithelioma is reported. A 37 year-old woman presented with a 2 cm well-circumscribed dermal nodule of the forehead. The tumor was enucleated with the clinical diagnosis of an epidermal inclusion cyst. Histologically, the tumor was located within the dermis and was well circumscribed. It was composed of spindle-shaped and epithelioid cells arranged in organoid nests. Focal areas of extracellular hyalinized stroma were present. Well-defined glandular structures, chondroid matrix, and significant nuclear pleomorphism were absent. The tumor expressed widespread cytoplasmic positivity for cytokeratin (using AE1/AE3) and nuclear positivity for p63. Variable reactivity was noted with EMA and S-100. The tumor was negative for smooth muscle actin, GFAP, chromogranin, synaptophysin, and CEA. These immunohistochemical results supported myoepithelial differentiation. Myoepitheliomas of the skin are rare neoplasms that have only recently been recognized in the skin. A single publication has reported the diagnostic utility of p63 in the diagnosis of myoepithelial tumors of the skin. Myoepitheliomas often display variable expression of myoepithelial markers, with no single marker that is 100% sensitive. The current case highlights the need for a battery of markers, including p63, to detect myoepithelial differentiation. [source] Magnetic resonance imaging in the detection of pancreatic neoplasmsJOURNAL OF DIGESTIVE DISEASES, Issue 3 2007Liang ZHONG Recently, with the rapid scanning time and improved image quality, outstanding advances in magnetic resonance (MR) methods have resulted in an increase in the use of MRI for patients with a variety of pancreatic neoplasms. MR multi-imaging protocol, which includes MR cross-sectional imaging, MR cholangiopancreatography and dynamic contrast-enhanced MR angiography, integrates the advantages of various special imaging techniques. The non-invasive all-in-one MR multi-imaging techniques may provide the comprehensive information needed for the preoperative diagnosis and evaluation of pancreatic neoplasms. Pancreatic neoplasms include primary tumors and pancreatic metastases. Primary tumors of the pancreas may be mainly classified as ductal adenocarcinomas, cystic tumors and islet cell tumors (ICT). Pancreatic adenocarcinomas can be diagnosed in a MRI study depending on direct evidence or both direct and indirect evidence. The combined MRI features of a focal pancreatic mass, pancreatic duct dilatation and parenchymal atrophy are highly suggestive of a ductal adenocarcinoma. Most cystic neoplasms of the pancreas are either microcystic adenomas or mucinous cystic neoplasms. Intraductal papillary mucinous tumors are the uncommon low-grade malignancy of the pancreatic duct. ICT are rare neoplasms arising from neuroendocrine cells in the pancreas or the periampullary region. ICT are classified as functioning and non-functioning. The most frequent tumors to metastasize to the pancreas are cancers of the breast, lung, kidney and melanoma. The majority of metastases present as large solitary masses with well-defined margins. [source] Review of non-positron emission tomography functional imaging of primary musculoskeletal tumours: Beyond the humble bone scanJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 6 2005YY Ho Summary Bone and soft tissue tumours are rare neoplasms. There are five major roles of imaging in the management of primary musculoskeletal tumours, that is, to differentiate between benignity and malignancy, to evaluate for local tumour extension, to screen for metastases, to judge the effect of chemotherapy, and to monitor for recurrence. To accomplish this, multiple modalities are required because no single examination is able to complete all these tasks. These modalities include plain radiography, CT, MRI, conventional nuclear medicine as well as positron emission tomography (PET) imaging. Elsewhere, PET imaging has been discussed at length, because it is likely to be superior in the assessment of bone and soft tissue tumours over conventional nuclear medicine procedures. However, conventional nuclear medicine may be of value when PET is unavailable. In this review, an overview of anatomical imaging will be given and the role of non-PET functional imaging will be discussed in detail. A variety of illustrative cases will be presented. [source] Bladder pheochromocytoma encountered on sonographyJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 3 2004M Çelikta Summary Pheochromocytomas of the bladder are rare neoplasms, constituting <0.06% of all vesical tumours. Common presenting features of this tumour include episodes of sweating, hypertension, haematuria and postmicturition syncope. We describe a case of bladder pheochromocytoma in a 66-year-old man whose only symptom of macroscopic haematuria was initially assessed with ultrasonography. Clinical presentation highlights the need for a high index of suspicion during sonographic evaluation of bladder neoplasms because such tumours might present without symptoms of adrenergic excess. [source] Primary mucinous adenocarcinoma of the vagina: Possibility of differentiating from metastatic adenocarcinomasPATHOLOGY INTERNATIONAL, Issue 6 2005Maki Saitoh Primary vaginal adenocarcinomas are rare neoplasms. Herein is reported a case of primary vaginal mucinous adenocarcinoma with an interesting mucin profile, presumably arising from a lesion of adenosis in a patient without in utero exposure to diethylstilbesterol (DES). The patient, a 44-year-old woman, had undergone vaginal total hysterectomy 10 years previously for myoma uteri corporis. The histological features of the vaginal intramural tumor found in this patient resembled those of mucinous adenocarcinoma of the endocervical type. Therefore, it was necessary to determine whether or not the tumor was metastatic from an occult cervical adenocarcinoma. However, the adenocarcinoma cells of the present case did not contain sulfomucin at all, being different from most mucinous adenocarcinoma cells of the endocervical type. Moreover, there were foci of adenosis adjacent to the adenocarcinoma foci, which also did not contain sulfomucin. These findings indicate that the mucinous adenocarcinoma arose from vaginal adenosis. Further studies are necessary to investigate whether lack of sulfomucin expression is a characteristic feature of vaginal adenosis. [source] Adult primary extragonadal germ cell tumors: Treatment results and long-term follow-upPEDIATRIC BLOOD & CANCER, Issue 1 2003Argon Andac MD Abstract Background Primary extragonadal germ cell tumors (PEGCT) are rare neoplasms. They have a poor prognosis, different behavior, and natural course compared to their gonadal counterparts. Both primary and salvage treatment of these tumors constitute a challenge. We retrospectively evaluated the clinicopathologic status, therapeutic implications, and outcome of our patients with PEGCT. Procedure Between 1991 and 2000, 18 patients with PEGCT (median age 31 years; range 17,63), diagnosed with tru-cut biopsy and treated with cisplatin-based chemotherapy, were evaluated in respect to treatment response and outcome. Results Cisplatin-based chemotherapy achieved a complete response rate of 27.8% and a partial response rate of 55.5%. Overall response rate was 83.3%. Only three patients were unresponsive to chemotherapy; an additional six patients with residual mass underwent surgical resection and were rendered disease-free by surgery. The 5-year actuarial event-free and overall survival were 63.4 and 71.3%, respectively. Conclusions The outcomes of our patients with extragonadal primaries including mediastinal localization appear to be slightly better than those previously reported. Multimodality therapy is essential for these patients and given the relatively poor prognosis, prospective trials with large sample sizes, and new treatment approaches to improve outcome are required. Med Pediatr Oncol 2003;41:49,53. © 2003 Wiley-Liss, Inc. [source] Disseminated extramedullary myeloid tumor of the gallbladder without involvement of the bone marrowAMERICAN JOURNAL OF HEMATOLOGY, Issue 1 2007Angela N. Bartley Abstract Extramedullary myeloid tumors (myeloid sarcomas) are rare neoplasms that are composed of myeloid precursors. They usually arise concurrently with a diagnosis of acute myeloid leukemia, chronic myeloid leukemia, or other myeloproliferative disorders. They may also indicate relapsing disease in a patient with a prior history of leukemia or myeloproliferative disorder. We present our findings of a 63-year-old female diagnosed with extramedullary myeloid tumor first presenting in the gallbladder. She subsequently developed respiratory failure; pre- and postmortem bone marrow studies were negative for leukemia by morphology, flow cytometry, and karyotypic analysis. However, the myeloid neoplasm was disseminated throughout most of her remaining organs. Immunohistochemical stains of the cells indicated a neoplasm of myelomonocytic derivation (CD4, CD43, CD45, CD68, myeloperoxidase, and lysozyme positive). To our knowledge, this is the first report of an extramedullary myeloid neoplasm of the gallbladder with disseminated disease without involvement of the bone marrow. Am. J. Hematol., 2006. © Wiley-Liss, Inc. [source] Use of octreotide and lanreotide in the treatment of symptomatic non-resectable carcinoid tumoursANZ JOURNAL OF SURGERY, Issue 9 2002Muhammad Rohaizak Background: Carcinoid tumours are rare neoplasms that secrete hormones and biogenic amines, most commonly serotonin. Octreotide and long acting lanreotide are found to be useful in the management of carcinoid syndrome by its interaction with somatostatin receptor, found on the carcinoid tumour. The aim of this study is to look at the efficacy of octreotide and long acting lanreotide in the treatment of symptomatic non-resectable carcinoid tumours. Method: The effects of octreotide and long-acting lanreotide were studied in 10 patients with symptomatic non-resectable carcinoid tumours. Results: Symptom improvement occurred in nine of 10 patients. Three patients responded only to octreotide, three patients responded to both octreotide and long-acting lanreotide and three patients only responded to long-acting lanreotide. Slight reductions in 24-h urine 5-hydroxyindoleacetic acid levels occurred in three of six patients but no patients were found to have objective tumour regression on computed tomography scan. Conclusions: Octreotide and long-acting lanreotide are useful palliative treatments for the control of symptoms in patients with non-resectable carcinoid tumours but there is no evidence of tumour stasis. [source] Surgery for carcinoid tumours of the lower gastrointestinal tractCOLORECTAL DISEASE, Issue 2 2003A. C. Goede Abstract Introduction, Carcinoid tumours are rare neoplasms that originate from neuroendocrine cells of the primitive gastrointestinal tract. Mid- and hind-gut tumours comprise the majority of these rare tumours. With many recent advances in medical treatment the role and importance of surgery and the surgeon needs to be assessed. Method, A Medline, Pubmed and Embase databases search was undertaken. All relevant articles were cross-referenced. Results and conclusions, Incidental findings of carcinoid tumours should be treated at initial surgery whilst elective surgery and further management should be undertaken in specialist centres by a multidisciplinary team. Asymptomatic patients have a better prognosis than those with symptoms. In advanced cases surgery combined with chemotherapy and liver resection is appropriate. The outlook for the majority of cases is good. [source] | ||||||||||||||||||||||||||||