Cases reported "Neoplasm Invasiveness"

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1/83. Primary hepatic carcinoid tumor.

    A primary hepatic carcinoid tumor arising in a 49-year-old woman is reported. The patient was admitted with multiple hepatic tumors and treated by a left lobectomy and cholecystectomy. Cut sections of the specimen revealed a solid and necrotic mass, measuring 10 x 12 x 13 cm, with multiple small satellite nodules. Histologically, the tumor cells had small oval-shaped nuclei and presented with a trabecular arrangement and rosette-like formation. Both Grimelius and Fontana-Mason stainings were positive. The tumor cells were positive for chromogranin a and negative for other antigens. Ultrastructural studies of the tumor cells revealed duct-like formation with microvilli and a cluster of dense small immature neurosecretory granules in the cytoplasm. These findings were consistent with those of carcinoid tumors. Postoperatively, the patient was treated with repeated transcatheter arterial chemoembolization for any remnant tumors. However, she died of the disease 5 years after the initial surgery. The autopsy findings suggested the primary site to be the liver.
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2/83. Intraosseous epithelioid hemangioendothelioma of the mandible: a case report with an immunohistochemical study.

    Epithelioid hemangioma is the prototype of a group of vascular tumors characterized by epithelioid endothelial cells. hemangioendothelioma of bone is a rare lesion that constitutes less than 0.5% of primary malignant skeletal tumors. We report and discuss a case of epithelioid hemangioendothelioma arising intraosseously in the anterior portion of the mandible in a 76-year-old woman. The case was treated successfully by wide resection. Radiographically, the tumor mass showed osteolysis and expansion. Histologically, the tumor showed invasive and destructive growth, although it lacked frequent mitotic figures and severe atypia. On immunohistochemical study, tumor cells exhibited characteristics of mesenchymal and endothelial origin, i.e., strong to moderate immune reactivity against vimentin, factor viii-related antigen (F8RA), ulex europaeus agglutinin type 1 lectin (UEA-1), and CD 34, but not against keratin, epithelial membrane antigen (EMA) or S-100 protein (S100). The proliferating cell nuclear antigen (PCNA)-positive cell index was 27.5%. These pathological findings suggested a borderline malignant potential for this tumor. Thus, clinically, wide resection with or without dissection of regional lymph nodes is recommended.
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3/83. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood.

    Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma (ALCL) is an unusual tumor in the pediatric population. However, the nodal-based form of the disease compared with other histologic subsets of childhood non-Hodgkin's lymphomas (NHL) more frequently involves skin, soft tissue, and bone. The objective of this article is to determine the histologic and immunologic characteristics of childhood primary cutaneous Ki-1(CD30) positive ALCL and its prognosis. The clinical data, histologic features and immunohistochemical profiles of skin biopsy specimens from 3 children with cutaneous Ki-1(CD30) positive lymphoma were reviewed. A literature search was performed and disclosed information on 5 childhood cases. The 3 patients with primary cutaneous Ki-1(CD30) positive ALCL all presented similarly as rapidly growing masses initially and clinically believed to be infectious/reactive processes. The diagnosis was established on the basis of histopathologic examination and immunohistochemical studies. Histologic sections revealed an extensive infiltrate of tumor cells extending throughout the entire dermis into the subcutaneous fat with frank ulceration in 1 patient. No significant epidermotropism was noted. Tumor cells exhibited striking cellular pleomorphism and a high mitotic rate with numerous atypical mitoses. Inflammatory cells were present in all patients. The tumor cells stained positively for Ki-1 antigen (CD30), epithelial membrane antigen, and for T-cell markers (UCHL-1, CD3). One of 3 cases, however, failed to stain for leukocyte common antigen (LCA). No clinically apparent adenopathy was observed in any of the patients. In all instances the patients developed recurrent disease in the skin at sites separate from the primary location. None of the patients demonstrated any involvement of lymph nodes, bone marrow, or other organ systems. All patients were treated with chemotherapy with good response. Primary cutaneous Ki-1(CD30) positive lymphoma is rare in children and is characterized by recurrences. The prognosis seems to be favorable.
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4/83. Salivary duct carcinoma: an unusual case of long-term evolution.

    Salivary duct carcinoma is a highly malignant adenocarcinoma of salivary origin. Its pathologic features are distinct from the other salivary gland tumors and bear a remarkable histologic resemblance to ductal breast carcinoma. The clinical course is rapid and the prognosis is dismal. Aggressive therapy is warranted, including primary tumor resection, cervical neck dissection, and radiotherapy. We present a case of salivary duct carcinoma of parotid origin with a very long-term evolution in clear contrast to its supposed aggressiveness. Tumor cells expressed low- and high-molecular-weight cytokeratins, epithelial membrane antigen, carcinoembryonic antigen, and c-erbB-2 but not estrogen and progesterone receptors, actin, and S-100.
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5/83. Primary invasive signet-ring cell melanoma.

    The histopathological variants of malignant melanoma include the common type (lentigo maligna, superficial spreading melanoma, nodular melanoma, acrolentiginous melanoma), spindle cell, desmoplastic, balloon cell, pleomorphic (fibrohistiocytic), myxoid, small cell melanoma and malignant blue nevus. Recently, signet-ring cell melanoma was introduced as an additional cytologic variant. We describe a 72-year-old patient with a primary signet-ring cell melanoma of the skin located on the upper arm. Histopathologic examination disclosed a melanocytic tumor extending from the epidermis to the deep reticular dermis. Numerous pleomorphic tumor cells showed large, intracellular vacuoles and oval to spindle-shaped nuclei at their periphery. Mitotic figures and multinucleated melanocytes were also observed. Some of the signet-ring cells exhibited cytoplasmatic periodic acid-Schiff (PAS)-positivity. immunohistochemistry showed positive reaction of the tumor cells for S-100, HMB-45 protein and vimentin, confirming their melanocytic differentiation. Tumor cells were negative for cytokeratins, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The signet-ring cell melanoma disclosed an invasion to Clark Level IV and tumor thickness of 2.2 mm. Signet-ring cell melanoma is a rare morphologic variant of melanoma. Its recognition is important for differentiation from other tumors featuring signet ring cells.
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6/83. Sebaceous carcinoma of the breast.

    We report on a rare distinctive variant of infiltrating ductal carcinoma characterized by sebaceous differentiation of tumor cells. The neoplasm was identified in a lumpectomy specimen from a 45-year-old woman with extensive metastatic disease. In addition to conventional in situ and invasive ductal components, approximately half of the tumor cells exhibited a phenotype resembling tumors of the sebaceous skin appendage with coarsely vacuolated cytoplasm and peripherally displaced nuclei. The sebaceous moiety was also present in the distant metastatic deposits. There was no evidence of mucin production by tumor cells. Ultrastructurally, empty-appearing non-membrane bound vacuoles attested to the sebaceous cells' lipid content. The immunoprofile of the lesion included positivity for cytokeratin and epithelial membrane antigen. vimentin, S100 protein and carcinoembryonic antigen were not expressed. Most tumor cell nuclei reacted with antibodies to oestrogen and progesterone receptors but failed to show overexpression of the HER2/neu protein. The MIB-1 labeling index averaged 16%. At variance with sebaceous breast carcinomas on record, the present case is notable for its prolonged clinical course.
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7/83. A hepatocellular carcinoma with lymph node metastasis and invasion into the gallbladder: preoperative difficulty ruling out a gallbladder carcinoma.

    We present a case of resected hepatocellular carcinoma (HCC) which invaded the gallbladder with a metastasis to a lymph node. It was extremely difficult to make a differential diagnosis between HCC and gallbladder cancer preoperatively. A 68-year old man was admitted to hospital with complaint of a fever. ultrasonography (US) and CT scan showed a mass, growing invasively from the gallbladder bed of the liver (S4) to the lumen of the gallbladder. A selective arteriography showed the mass stained by the cholecystic artery, internal branch of the left hepatic artery, and frontal branch of the right hepatic artery. Endoscopic retrograde cholangiopancreatography (ERCP) showed the non-visualized gallbladder, a constriction of the common hepatic duct with suspicion of metastatic lymph nodes in the hepatoduodenal ligament. The tumor markers were: alpha-fet-protein 13175 ng/ml, PIVKA-II 26200 mAU/ml and CA19-9 0.0 U/ml. Both HBs antigen and HCV antibody were negative. We performed cholecystectomy with en-block resection of the anterior and middle inferior segment of the liver, the common bile duct and a part of the transverse colon, with dissection of the lymph nodes. The tumor, 8 cm in diameter, was brown colored without a capsule, growing diffusely in the liver, to the inside of the gallbladder and the transverse colon. Histopathological inspection of the specimen revealed moderately differentiated hepatocellular carcinoma with a metastatic lymph node along the common hepatic artery. TNM classification was IVB phase [T3,N0,M1 (LYM,OTH)]. There are only 3 previous cases of HCC reported with invasion into the gallbladder. At most 2.2% of the resected cases of HCC had metastatic lymph nodes at resection, while it was as high as 20-50% of the autopsy cases. Operation on such an invasive HCC case should consider lymph node metastasis.
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8/83. Biologic characteristics of paragangliomas of the nasal cavity and paranasal sinuses.

    Paragangliomas of the nasal cavity and paranasal sinuses are extremely rare. This study was conducted to investigate the biologic characteristics of these lesions on different molecular levels and to estimate their biologic behavior. Operative specimens of three patients who underwent surgery for a paraganglioma of the nasal cavity (one case) or paranasal sinuses (two cases) were investigated by routine histology, quantitative dna analysis, and immunohistochemical assessment of proliferation markers (i.e., proliferating cell nuclear antigen, PCNA; Ki67-MIB-1), the expression of cell-surface antigens, which reflect the tumor-stroma interaction (i.e., CD 44 v0.4/5 and 6, CD 54, CD 106), oncogene products (nm-23; p53), and bcl-2 as a marker of apoptosis. Histologically, two tumors were paragangliomas of the adenomatous subtype, one lesion was classified as angiomatous. According to dna analysis, aneuploid cells were detected in all tumors. Two of three paragangliomas were classified as a dna type III pattern, implying a high percentage of aneuploid cells and an aggressive behavior. Immunohistochemically, paragangliomas of the nasal cavity showed increased scores for both proliferation markers tested, indicating a rapid growth pattern. According to the expression of cell-surface markers and oncogene products, these tumors displayed an aggressive behavior and an infiltrating growth pattern. The highest value for the parameters of quantitative dna analysis and highest proliferation scores were found in a tumor of a patient who developed multiple tumor recurrences after radical excision of the lesion with clear margins, and finally died of disease. In conclusion, paragangliomas of the nasal cavity and paranasal sinuses examined in this study should be regarded as suspicious concerning their biologic and clinical behavior. Radical excision, and in cases with highly aggressive biology, postoperative radiation therapy are recommended. Tumor biologic examinations can help to recognize high-risk patients for developing recurrences and possibly lesions with a malignant behavior and to enhance our understanding of the biology of these extremely rare tumors of the nasal cavity and paranasal sinuses.
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9/83. Ectopic chordoma with orbital invasion.

    PURPOSE: To report a rare ectopic chordoma within the orbital wall. methods: Case report. RESULTS: A 63-year-old woman developed swelling of the eyelid, tearing, blurred vision, and progressive proptosis RE of 1 month's duration. neuroimaging studies revealed an osteolytic mass with epicenter at the sphenozygomatic suture that eroded intracranially, invaded into the orbit, and compressed orbital soft tissues. Surgical debulking was done followed by radiation treatment. The pathologic findings of physaliphorous epithelial cells with multiple vacuoles containing mucin, prominent nuclei, and positive immunohistochemical staining for S-100, vimentin, epithelial membrane antigen, and pancytokeratin were diagnostic for chordoma. CONCLUSION: Orbital wall ectopic localization of a chordoma distant from the clivus is a rare occurrence.
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10/83. A squamous cell carcinoma of the gastric cardia showing submucosal progression.

    We report a case of squamous cell carcinoma (SCC) of the gastric cardia showing submucosal progression with direct invasion of the liver. A 71-year-old man was admitted with dysphagia. Esophagogastroscopy showed a protruding tumor covered with normal gastric mucosa in the anterior wall of the gastric cardia, although no abnormal findings were detected in the esophagus, including the esophagogastric junction. serum SCC-related antigen level was elevated (6.6 ng/ml; normal level, less than 2.5 ng/ml). Endoscopic biopsy specimens taken from this tumor did not show malignant cells. Based on these findings, the preoperative diagnosis was a submucosal tumor of the stomach. laparotomy was done; however, the tumor was not resected because it had direct invasion to the left lateral segment of the liver and adjacent tissues. As the tumor showed continuous bleeding from the stomach after surgery, total gastrectomy, combined with transhiatal lower esophagectomy, left lateral segmentectomy of the liver, splenectomy, and distal pancreatectomy was performed. Because histologic findings showed poorly or moderately differentiated SCC with direct invasion of the liver, the final diagnosis was SCC of the gastric cardia showing submucosal progression with hepatic invasion. Such a case of SCC of the gastric cardia showing submucosal progression is rare, and accurate preoperative diagnosis was very difficult. However, it may be important to consider SCC of the gastric cardia in such a situation.
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