1/169. Sialoblastoma: clinicopathological/immunohistochemical study.Sialoblastoma is an extremely rare salivary tumor diagnosed at birth or shortly thereafter with significant variability in histologic range and clinical course, so that for an individual case it may be difficult to predict the most appropriate therapy. We detail the case of a toddler noted to have a firm 1-2-cm mass in the left cheek at 21 months. Parotidectomy was performed at 26 months, revealing a sialoblastoma; the resection margins were positive. During the next 10 months, the mass recurred or persisted, necessitating numerous procedures. The tumor was composed of basaloid cells with fine chromatin and other more mature cuboidal epithelial cells. Ductules and solid organoid nests with some tendency toward peripheral pallisading were also noted. There was no perineural invasion; necrosis initially was sparse but increased over time. The mitotic rate also increased from 6 to 7/10 high-power fields in the first resection to 20/10 high-power fields in the last resection. Nuclear pleomorphism increased with time. The MiB1 proliferative index revealed a dramatic increase in the number of labeled nuclei: from 3 cells/10 high-power fields in the first specimen to 94 cells/10 high-power fields for the last specimen. Cytokeratin accentuated the ductal structures. S-100 showed a diffuse staining pattern, with darker staining of the spindled myoepithelial cells. The Her-2-neu protein showed moderate cytoplasmic staining, whereas the p53 showed only occasional labeling of nuclei. This is the first case of sialoblastoma with evidence of increasing anaplasia based on increasing proliferative capacity. Therefore, the distinction between benign and malignant sialoblastomas may not be as well defined as previously thought. The patient's prognosis is likely to be determined by the tumor grade as well as the stage at presentation and the extent of resection.- - - - - - - - - - ranking = 1keywords = spindle (Clic here for more details about this article) |
2/169. Angiosarcoma of the testis.A primary angiosarcoma of the testis in a 74-year-old patient was a highly anaplastic epthelioid angiosarcoma, which was positive for endothelial markers immunohistochemically. The tumour was unrelated to testicular germ cell neoplasm; the patient had received no previous radiation or chemotherapy.- - - - - - - - - - ranking = 3145.5179443354keywords = sarcoma (Clic here for more details about this article) |
3/169. Embryonal "Botryoid" rhabdomyosarcoma of the larynx: a clinicopathologic and immunohistochemical study of two cases.Two cases of embryonal rhabdomyosarcoma of the larynx are reported. The tumors occurred in a 16-year-old boy and in a 66-year-old man. They manifested clinically with nonspecific symptoms, including voice hoarseness and sense of throat fullness. Treatment consisted of total and partial laryngectomy, respectively. Grossly, both lesions had an exophytic growth pattern and microscopically featured a proliferation of small round to oval cells. Cell cytoplasms were occasionally stainable and fibrillary. Quite often, tumor cellularity was denser beneath the covering mucosa, recalling a "cambium layer" pattern. Tumor cells immunoreacted for desmin, actins, myoglobin, and sarcomeric actin; no immunostaining was noted for epithelial markers. No further antitumoral treatment was administered after surgery. There has been no recurrence of tumor at 2 and 10 years, respectively. Based on our series and the available literature, it seems that rhabdomyosarcoma of the larynx pursues a less-aggressive course than that seen in the homonimic juvenile or adult soft tissue lesion. Surgery alone appears to be a valid treatment option, especially when a polypoid, or "botryoid" gross pattern, coupled with the embryonal small cell histotype is encountered. In light of these findings, it is suggested that botryoid rhabdomyosarcoma of the larynx may deserve a specific consideration among the various laryngeal mesenchymal malignancies.- - - - - - - - - - ranking = 3669.8259321317keywords = sarcoma, soft (Clic here for more details about this article) |
4/169. Fine needle aspiration cytology of undifferentiated embryonal sarcoma of the liver: a case report.The cytological features of a rare case of undifferentiated (embryonal) sarcoma of the liver are presented. The cytology smears showed singly dispersed polygonal and spindle cells as well as loose clusters of cells held together in myxoid material. Neoplastic cells were generally large with round, oval or lobulated nuclei. The cytoplasm was variable in amount with ill-defined borders. Occasional multinucleated cells were also present. Hyaline globules were present on sections of the cell block. Immunohistochemical studies performed showed positivity for vimentin, cytokeratin and alpha-1-antitrypsin (AAT) in the tumour cells.- - - - - - - - - - ranking = 2624.3943164589keywords = sarcoma, spindle cell, spindle (Clic here for more details about this article) |
5/169. Embryonal sarcoma of adult and pediatric kidneys: report of a case with localized submucosal invasion of the renal pelvis and long-term survival.A case of cystic embryonal sarcoma of the kidney (CESK) with a rapidly fatal outcome was recently reported.1 Here, we report another case of a 12-year-old boy with a localized but ill-defined submucosal lesion of CESK in the right renal pelvis. The tumor consisted principally of small mesenchymal cells with oval to spindle nuclei and scanty cytoplasm, infiltrating in dense arrangements. Two growth patterns were distinguished in the tumor cells: (i) a diffuse infiltrating pattern without an epithelial component; and (ii) a foliated pattern with an epithelial lining over the surface. Foci of the diffuse pattern predominated over those that were lobular, infiltrating superficial layers of renal sinuses and along pyramids, in both of which remarkable intravenous invasion was evident. Foci of the foliated pattern invaded deeper portions of a few sinuses and frequently penetrated into their veins, producing together with their epithelial lining a characteristic foliated structure. Lining epithelial cells around lobular foci often appeared hob-nailed or eosinophilic in the cytoplasm. Despite the remarkable intravenous encroachment, the patient has remained well without a recurrence for more than 26 years after a simple nephrectomy. The present case report expands our understanding of the biological nature of CESK.- - - - - - - - - - ranking = 2622.2649536129keywords = sarcoma, spindle (Clic here for more details about this article) |
6/169. Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature.Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant tumour with a poor prognosis. The absence of specific symptoms, the rapid tumour growth, the normality of the common tumour markers, and the consequential delay in the diagnosis often result in significant enlargement of the tumour. To our knowledge, there have been only 42 reported cases of USL in adults worldwide during the 40 years since the clinicopathological entity of USL was defined. We report here a 27-year-old male with USL who has been treated successfully with surgical resection and adjuvant chemotherapy using ifosfamide, adriamycin and cisplatin. Although the prognosis of the disease remains generally poor, long-term survival has been achieved recently in patients with a combination of surgery and multi-agent chemotherapy.- - - - - - - - - - ranking = 2621.2649536129keywords = sarcoma (Clic here for more details about this article) |
7/169. Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature.Undifferentiated (embryonal) sarcoma of the liver (USL) is a highly malignant tumor of early life. Treatment choices for USL, especially with intraperitoneal rupture, are uncertain. Outcomes have been almost uniformly poor until recently. We describe two 7-year-old girls treated for ruptured USL. In the more recent patient, operative biopsy was followed by three cycles of cisplatin (CDDP), adriamycin (ADR), and cyclophosphamide (CPM). A fluid-filled cavity in the tumor showed enlargement and was drained. Two cycles of CDDP, ADR, vincristine (VCR), and ifosfamide were accompanied by reduction in tumor size, and trisegmentectomy was performed. She has no evidence of disease 3.5 years after surgery. In the other patient, left lobectomy was followed by a less intensive regimen, including CPM, VCR, and fluorouracil. This patient died of dissemination within 5 months. In 170 reported pediatric patients with USL, the 2-year disease-free survival was 17%. For the 96 such patients reported since 1980, 2-year disease-free survival had improved to 27%. More aggressive chemotherapy has been associated with this change. Of 8 patients with tumor rupture whose details have been reported (including the 2 present patients) after resection of the tumor, 4 died, 1 was alive with disease, and 3 were free of disease at 8, 49, and 58 months, respectively, after diagnosis. Ruptured USL should be treated with combination chemotherapy including CDDP and ADR, as well as with curative resection.- - - - - - - - - - ranking = 2621.2649536129keywords = sarcoma (Clic here for more details about this article) |
8/169. Mixed germ cell tumor of the ovary with sarcomatous component.Germ cell tumors constitute a very complicated group of tumors of the ovary and their histogenesis is not yet clarified. Besides their histological heterogeneity, sarcomatous areas have also been described. A right ovarian mass was found in a 23-year-old female, who was being treated in the hospital for miscarriage. Disseminated omental metastases were detected during abdominal laparotomy. Pathological examination of the dissected material revealed the tumor to be a mixed germ cell tumor (immature teratoma and dysgerminoma) with sarcomatous component. Areas resembling granulosa cell tumor were also encountered. This ovarian tumor with many different histopathological features is presented with a review of the literature. The importance of thorough sampling in determining the type and extent of the malignant components is also emphasized due to their direct relation with the prognosis.- - - - - - - - - - ranking = 3145.5179443354keywords = sarcoma (Clic here for more details about this article) |
9/169. Embryonal sarcoma of the liver mimicking a hydatid cyst in an adult.Undifferentiated (embryonal) sarcoma of the liver is a rare malignant mesenchymal tumour. This presents predominantly in late childhood. It carries a dismal prognosis. Surgical resection with or without adjuvant therapy remains the mainstay of treatment. The present report describes a sixteen year old girl who presented with a large hepatic mass mimicking a hydatid cyst: clinically, radiologically and on serology.- - - - - - - - - - ranking = 2621.2649536129keywords = sarcoma (Clic here for more details about this article) |
10/169. Undifferentiated embryonal sarcoma with unusual features arising within mesenchymal hamartoma of the liver: report of a case and review of the literature.Undifferentiated embryonal sarcoma (UES) is a rare and highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. It has replaced malignant mesenchymoma, under which diagnostic term the first three cases were described. A link between embryonal sarcoma and mesenchymal hamartoma of the liver (MHL) has long been proposed, because of clinicopathologic overlaps of these entities; however, until recently, this association remained tenuous. Cases of UES arising in a background of mesenchymal hamartoma of the liver have previously been reported in two teenage girls. Discovery of a similar genetic abnormality in MHL and UES has clinched the supposed link between them. There have also been two reports of UES with prominent cystification, one associated with peripheral eosinophilia, and thereby masquerading as hydatid cyst of the liver. We report a case of UES arising in a young boy with MHL, with unusual histologic features, including large mesothelial-lined cysts and ectopic adrenal cortical tissue under Glisson's capsule.- - - - - - - - - - ranking = 3145.5179443354keywords = sarcoma (Clic here for more details about this article) |
| | Next -> |