bromochloroacetic-acid and Retroperitoneal-Neoplasms

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are rare. This is the first reported case in the literature in English of PRMC in a man. The 64-year-old man presented with a large retroperitoneal cystic tumour measuring 24 x 20 x 16 cm3, which was removed intact. Areas ranging from a benign mucinous cyst to borderline mucinous tumour to mucinous cystadenocarcinoma were observed on microscopy. Strong patchy staining for cytokeratins 7 and 20 and strong diffuse staining for MUC2 and MUC5AC core peptides, similar to staining patterns in ovarian mucinous tumours, were shown in the benign and atypical epithelium. Staining for CA19.9 and carcinoembryonic antigen was also shown by both components. The theory of its origin from the mucinous metaplasia of peritoneal (mesothelial) inclusion cysts, rather than from ectopic ovarian tissue or ovarian teratomas, is supported by the occurrence of such a tumour in a male patient.

Topics: Cystadenocarcinoma, Mucinous; Humans; Keratins; Male; Middle Aged; Mucins; Neoplasm Proteins; Retroperitoneal Neoplasms

Since the concept of malignant fibrous histiocytoma (MFH) was introduced and subsequently popularized in the 1960's and 1970's, it has become widely regarded as the commonest soft-tissue sarcoma of adulthood. Although the initial notion that MFH was a true histiocytic tumor showing faculative fibroblastic differentiation has been disproved, and despite the lack of definable, reproducible diagnostic criteria and considerable immunophenotypic, ultrastructural and karyotypic heterogeneity, MFH is still accepted widely as a discrete clinicopathologic entity. On the other hand several recent studies have expressed considerable doubts about MFH, or at least pleomorphic MFH, as an "entity" and have suggested that it represents a common morphologic manifestation of a host of poorly differentiated sarcomas and, more rarely, other neoplasms. This article reviews the clinicopathologic features of MFH and its established variants in the context of this debate and considers the evidence for and against their continued acceptance as distinct entities or as a cohesive group. We conclude that the pleomorphic, giant cell and inflammatory variants each represent heterogeneous diagnostic groups which are hard to defend as cohesive entities, while the myxoid ("myxofibrosarcoma") and angiomatoid types are distinct, reproducible tumor types.

Topics: Desmin; Fibrosarcoma; Giant Cell Tumors; Histiocytoma, Benign Fibrous; Humans; Keratins; Leiomyosarcoma; Retroperitoneal Neoplasms; Soft Tissue Neoplasms

A primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is an extremely rare lesion. To date, only 49 cases have been reported. The presence of mural nodules in a PRMC may indicate a worse prognosis. We report the case of a 40-year-old Japanese woman with a PRMC with mural nodules. Microscopic examination revealed that the stromal cells of the nodules were spindle-shaped and varied in size. The nodules were immunoreactive for vimentin but negative for cytokeratin and EMA, and the nuclei of the stromal cells were pleomorphic and strongly Ki-67 immunoreactive. The nodules were diagnosed as true sarcoma. To the best of our knowledge, this is 11th published case report of a PRMC with mural nodules.

Topics: Adult; Cystadenocarcinoma, Mucinous; Female; Humans; Keratins; Prognosis; Retroperitoneal Neoplasms; Sarcoma; Vimentin

Primary retroperitoneal mucinous tumors (PRMTs) are uncommon neoplasms occurring almost exclusively in women. PRMTs are divided into mucinous cystadenoma (MC), mucinous borderline tumors or tumors of low malignant potential (MLMP), and mucinous carcinomas (MCas). In this retrospective study, we present the clinicopathologic features of 18 such cases, the largest series to date. All patients were women, ranging in age from 20 to 63 years (mean 38.6 y). All except 2 patients presented with an enlarged mass during a routine examination or by self-palpation. All tumors were located exclusively in the retroperitoneum, with histologic or clinical confirmation of the lack of ovarian involvement. The tumors ranged from 7 to 26 cm (mean 13.2 cm). The gross appearance was variable: unilocular cyst with a thin wall (4 cases), predominantly cystic with papillary areas or nodule(s) (8 cases), multiloculated cyst with or without nodules (1 case each), and predominantly solid with cystic areas (4 cases). Histologically, there were 2 cases of MC, 7 of MLMP (7 cases; 3 of them with intraepithelial carcinoma and 1 with microinvasion), and 9 of MCas (9 cases, 5 of them associated with MLMP and 1 associated with MC). Three of the MCas had areas of anaplastic or sarcomatoid carcinoma whereas 1 had an associated sarcoma. Immunohistochemical studies were performed in 6 cases. Cytokeratin 7 was diffusely positive in all cases studied, whereas cytokeratin 20 and cytokeratin 17 were focally positive in 4 and 2 cases, respectively. All patients underwent surgical resection of the entire tumor. Two patients with MCa and sarcoma or sarcomatoid carcinoma received chemotherapy. Follow-up was available in 16 cases, ranging from 1 to 148 months (mean 40 mo, median 22 mo). Two patients died of disease at 5 and 9 months; both had MCa with anaplastic carcinoma or sarcoma. Three patients with MCa were alive with disease at 14, 26, and 58 months. The remaining patients were alive with no evidence of disease. In this study, MLMP and MCa were more common than MC. PRMTs seem to be nonaggressive neoplasms, except in cases containing anaplastic carcinoma or sarcoma.

Topics: Adult; Biomarkers, Tumor; Cystadenocarcinoma, Mucinous; Cystadenoma, Mucinous; Disease-Free Survival; Female; Humans; Immunohistochemistry; Keratins; Middle Aged; Retroperitoneal Neoplasms; Retrospective Studies; Treatment Outcome; Young Adult

We report the detailed molecular study of angiogenesis-ralated genes and target therapy of the case of a male 46-year-old patient with extrarenal rhabdoid tumor of pelvic retroperitoneum. The patient was found to have a huge pelvic soft tissue sarcoma and underwent pelvic tumorectomy and appendectomy. The microscopically morphological features and molecular profile by immunohistochemical analysis supported the surgical histological diagnosis of extrarenal rhabdoid tumor. The tumor recurred two weeks after surgery and metastasized to the lung, left abdominal wall and mesenteric lymph nodes. Systemic chemotherapy including ifosfamide, liposomal doxorubicin, Taxol and cisplatin, concurrently with pelvic radiotherapy (58 Gy of total dose). However, the patient did not respond to the combination of chemotherapy and radiotherapy. Immunohistochemical staining and fluorescence in situ hybridization of tumor cells indicated negative expression of human epidermal growth factor receptor-2 (HER-2) and epidermal growth factor receptor (EGFR) and positive expression of vascular endothelial growth factor (VEGF) and vascular endothelial growth factor receptor (VEGFR). So anti-VEGF targeted therapy (Bevacizumab) was administered following the fourth course chemotherapy. However, the condition worsened after the administration of the second cycle of Bevacizumab. Multiple organ failure led to the death of the patient. The patient only survived five months and 20 days after the surgery of the primary tumor.

Topics: Diagnosis, Differential; Fatal Outcome; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Neovascularization, Pathologic; Retroperitoneal Neoplasms; Rhabdoid Tumor; S100 Proteins; Salvage Therapy; Vimentin

Topics: Adult; Dendritic Cells; Diagnosis, Differential; Humans; Immunohistochemistry; Keratins; Lymph Nodes; Male; Prognosis; Retroperitoneal Neoplasms

Topics: Adult; Cell Division; Female; Humans; Keratins; Microscopy, Electron; Retroperitoneal Neoplasms; Tropomyosin; Vimentin

Schwannomas have been variably observed to be glial fibrillary acid protein (GFAP) and occasionally keratin positive, with antibodies reacting with multiple keratins (pankeratins, keratin cocktail (CK), but specific keratin polypeptides (K) have not been examined for in schwannoma. Since we observed CK positivity in retroperitoneal schwannomas, we wanted to study a large group of retroperitoneal and peripheral schwannomas with GFAP, CK and Ks to explore the frequency and biologic background of this finding. We immunohistochemically evaluated a large number of retroperitoneal (n=115) and peripheral schwannomas (n=22) for GFAP, 16 individual K and AE1/AE3 keratin cocktail. The great majority (104/115, 90%) of retroperitoneal schwannomas were positive for GFAP, and 72/104 (69%) cases were positive for AE1/AE3, often extensively. Both markers highlighted the cellular Antoni A areas, particularly adjacent to the capsule, myxoid or degenerative areas, and perivascularly. Most cases 87/104 (84%) stained for both AE1/AE3 and GFAP at least focally. No tumors stained for keratins that were GFAP negative. None of the immunostains for individual K showed positivity comparable to that obtained with AE1/AE3 CK. However, 62% were focally positive for high molecular weight K1 and 8/61 (13%) for K7. None of the retroperitoneal schwannomas were positive for other keratins including K2, 4, 5, 8, 9, 10 and K14-20. Peripheral schwannomas showed GFAP-positivity in only three of 22 cases (14%), and all were negative for keratins, both cocktail and individual K. We conclude that crossreactivity of AE1/AE3 with other intermediate filament proteins, such as GFAP, as previously observed in brain and glioma tissue, probably accounts for the extensive keratin-positivity seen in some retroperitoneal schwannomas. However, focal expression of K1 and K7 cannot be ruled out. Keratin-positive schwannomas should not be confused with other keratin-positive tumors, such as sarcomatoid carcinoma, mesothelioma, and synovial sarcoma.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Glial Fibrillary Acidic Protein; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Neurilemmoma; Peripheral Nervous System Neoplasms; Retroperitoneal Neoplasms

Topics: Adult; Biomarkers, Tumor; Female; Hormones, Ectopic; Humans; Inhibins; Keratins; Ovarian Neoplasms; Ovary; Paraneoplastic Endocrine Syndromes; Retroperitoneal Neoplasms; Vimentin; Virilism

Topics: Aged; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Cystadenocarcinoma, Mucinous; Female; Humans; Immunohistochemistry; Keratin-7; Keratins; Osteosarcoma; Retroperitoneal Neoplasms

Topics: Adenocarcinoma; Adult; Carcinoembryonic Antigen; Cysts; Female; Humans; Immunohistochemistry; Keratin-7; Keratins; Laparoscopy; Retroperitoneal Neoplasms

A large primary retroperitoneal sublumbar neoplasm was identified in an 11-year-old Holstein cow, with metastases to the lungs, kidneys and lymph nodes. The tumour cells proliferated in a characteristic endocrine pattern, were argyrophilic and positive for neuron-specific enolase, and had membrane-bounded intracytoplasmic granules. In addition, the cells were occasionally positive for cytokeratin and had desmosome-like intercellular junctions. The primary tumour mass was diagnosed as a malignant paraganglial tumour of the aortico-sympathetic ganglion (organ of Zuckerkandl), and was considered to contain primitive cells with epithelial differentiation.

Topics: Animals; Biomarkers, Tumor; Cattle; Cattle Diseases; Cytoplasmic Granules; Desmosomes; Female; Ganglia, Sympathetic; Immunoenzyme Techniques; Keratins; Kidney Neoplasms; Lung Neoplasms; Lymph Nodes; Paraganglioma; Phosphopyruvate Hydratase; Retroperitoneal Neoplasms; Silver Staining

A müllerian carcinosarcoma or malignant mesodermal mixed tumor (MMMT) originated from the retroperitoneum of a 51-year-old woman; only two previous similar cases have been reported. The 1,040 gm tumor was found in the left retroperitoneal space; the center of the tumor was extensively liquified. The uterus, fallopian tubes, and ovaries were unremarkable. The histopathological features of the tumor were typical of MMMT, homologous type. The adenocarcinomatous component of the tumor was immunohistochemically positive for cytokeratin 7 and negative for cytokeratin 20. MMMTs arising in extragenital sites are rare, and most of them arise from the peritoneum. The histogenesis of extragenital MMMTs remains speculative, but the origin from the "secondary mullerian system" is most likely.

Topics: Carcinosarcoma; Female; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratin-20; Keratin-7; Keratins; Middle Aged; Mullerian Ducts; Retroperitoneal Neoplasms

A 21 cm retroperitoneal cystic mass was excised from a 71 year old woman. The cyst was filled with a hemorrhagic fluid and contained a 5 cm parietal hemorrhagic nodule. On histology, the nodule was composed of a uniform population of round cells arranged in trabeculae and nests. The neoplastic cells were immunoreactive to cytokeratin, EMA, NSE, chromogranin A, pancreatic polypeptide (PP) and Gastrin (G). Ultrastructural observation of neurosecretory granules confirmed the neuroendocrine nature of the tumor. No other lesions were detected and a diagnosis of primary epithelial neuroendocrine tumor was rendered. The histogenesis of the tumor including the possibility of a paraganglionic origin is discussed.

Topics: Aged; Biomarkers, Tumor; Calcitonin; Carcinoma, Neuroendocrine; Diagnosis, Differential; Epithelium; Female; Gastrins; Humans; Keratins; Neoplasm Proteins; Pancreatic Polypeptide; Paraganglioma; Retroperitoneal Neoplasms; S100 Proteins; Teratoma

Three cases (two ovarian and one retroperitoneal) of mucinous tumors with solid nodules are reported. The predominant picture in the three cases was that of a mucinous cystic tumor, but small nodules of solid anaplastic carcinoma were found in all three cases. In addition, one case showed microscopic foci of microcyst rupture with histiocytic response reminiscent of sarcoma-like mural nodules, one case showed several sarcoma-like nodules, and one case showed apparent transition from anaplastic carcinoma to spindle cell sarcoma. Histologic and immunohistochemical characteristics of the lesions are given, as differentiation of these nodules is important.

Topics: Adult; Aged; alpha 1-Antichymotrypsin; Cystadenocarcinoma; Female; Humans; Immunoenzyme Techniques; Keratins; Membrane Glycoproteins; Mucin-1; Mucins; Ovarian Neoplasms; Retroperitoneal Neoplasms; Sarcoma; Staining and Labeling; Vimentin