Cases reported "Neoplasm Invasiveness"

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1/33. p53 and p16INK4A mutations during the progression of glomus tumor.

    Glomus tumors are significantly rare tumors of carotid body. The great majority of these tumors are benign in character. Here we present two brothers with hereditary glomus jugulare tumor who had consanguineous parents. radiotherapy was applied approximately 8 and 10 years ago for treatment in both cases. Eight years later, one of these cases came to our notice due to relapse. The mutation pattern of p53, p57KIP2, p16INK4A and p15NK4B genes which have roles in the cell cycle, was analyzed in tumor samples obtained from the two affected cases in the initial phase and from one of these cases at relapse. The DNA sample obtained from the case in initial diagnosis phase revealed no p53, p57KIP2, p16INK4A or p15INK4B mutation. He is still in remission phase. Despite the lack of p53, p57KIP2, p16INK4A and p15INK4B mutation at initial diagnosis the tumor DNA of the other case in relapse revealed p53 codon 243 (ATG-->ATC; met-->ile) and p16 codon 97 (GAC-->AAC; asp-->asn) missense point mutations. No loss of heterozygosity in p53 and p16INK4A was observed by microsatellite analysis of tumoral tissues in these cases. P53 and p16INK4A mutations observed in relapse phase were in conserved regions of both genes. No previous reports have been published with these mutations in glomus tumor during progression. The mutation observed in this case may due to radiotherapy. In spite of this possibility, the missense point mutations in conserved region of p53 and p16INK4A genes may indicate the role of p53 and p16INK4A in tumor progression of glomus tumors.
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2/33. Identifying differentially expressed genes associated with metastasis of follicular thyroid cancer by cDNA expression array.

    patients with follicular thyroid carcinoma have a higher incidence of metastasis than papillary thyroid carcinoma when thyroid cancer is diagnosed. The cDNA expression array technology is utilized herein to profile differentially expressed genes from metastatic human follicular thyroid carcinoma and reveal new tumor markers as well as target genes for therapeutic intervention. Tissue samples were obtained during surgical resection of the thyroid follicular carcinoma and metastatic tissue in the brain of the same patient. Two identical Atlas human cDNA expression arrays were hybridized with 32P-labeled cDNA probes derived from rna of either primary thyroid cancer or metastatic tissue. Parallel analysis of the hybridized signals allowed us to identify the alteration of gene expression in the metastasis process. Eighteen genes significantly overexpressed and 40 genes significantly underexpressed were identified in the metastatic thyroid cancer. genes that displayed an altered expression were associated with the processes of cell cycle regulation, apoptosis, dna damage response, angiogenesis, cell adhesion and mobility, invasion, and immune response. An expression profile of genes that are associated with metastasis process of follicular thyroid cancer was also discussed. Further investigation is required to understand the precise relationship between the altered expression of these genes and the metastasis process of follicular thyroid cancer.
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3/33. pancreaticoduodenectomy for perforated gastric malignant lymphoma: report of a case.

    We herein report the case of a 65-year old man with gastroduodenal malignant lymphoma (diffuse large B-cell type) accompanied with a bulbar perforation. A pancreaticoduodenectomy was performed because the duodenal second portion and pancreatic head were involved. However, extensive lymphadenectomy was not carried out because many lymph nodes were involved. The postoperative course was uneventful and CHOP therapy (pirarubicin 30 mg/m2, cyclophosphamide 500 mg/m2, vincristine 1 mg/m2, prednisolone 30 mg/m2) was started 1 month after surgery. Seven cycles of CHOP therapy markedly reduced the residual lesions including lymph nodes, and a computed tomography scan after the final chemotherapy revealed no obvious lesions. Complete remission was obtained and the patient was discharged 9 months after surgery.
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4/33. Large B-cell lymphoma manifesting as an invasive cardiac mass: sustained local remission after combination of methotrexate and rituximab.

    Cardiac tumor is an infrequent but serious manifestation of lymphoma and optimal management has not been well defined in these rare cases. Here we describe a patient who after autologous transplant for large B-cell lymphoma, relapsed with an invasive cardiac tumor, infiltrating the full thickness of the right myocardial wall. During therapy, she developed hemodynamic collapse due to complex atrial arrhythmias and had to be treated and stabilized in the intensive cardiac care unit. Despite initial significant regression of the cardiac tumor, new extranodal sites of lymphoma soon appeared in the skin, stomach and nervous system. Because of poor compliance, she was given salvage therapy with weekly four i.v. infusions of methotrexate and rituximab followed by monthly four maintenance cycles of rituximab alone. This produced an unexpected prolonged complete remission lasting 12 months, but she then developed a fatal leukemic phase. The relapse spared the heart and no cardiac abnormality was evident clinically or by any imaging technique. To the best of our knowledge this case, unique in terms of tumor size and extent of invasion, represents the first report of the treatment of invasive cardiac lymphoma using a combination of modest chemotherapy and rituximab.
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5/33. Primary ALK positive anaplastic large cell lymphoma of the pancreas.

    Here we present an unusual case of anaplastic lymphoma kinase positive anaplastic large cell lymphoma (ALCL), appearing as a primary tumor of the pancreas which invaded into the adjacent duodenal wall, causing upper gastrointestinal bleeding. After complete resection of the tumor (Whipple's operation), the patient received 4 cycles of CHOP chemotherapy. Currently, 2 years after diagnosis the patient still remains lymphoma free. Primary ALCL of the pancreas is very rare and has only been reported on one previous occasion. Nevertheless, lymphoma should be considered in the differential diagnosis of pancreatic tumors and an attempt to obtain tissue diagnosis is always necessary before continuing with radical surgery, especially in young patients.
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6/33. Successful treatment with erwinia L-asparaginase for recurrent natural killer/T cell lymphoma.

    We describe a patient with natural killer (NK)/T cell lymphoma who relapsed after autologous peripheral blood stem cell transplantation (auto-PBSCT) and was successfully treated with escherichia coli (E. coli) and erwinia L-asparaginase. A 38-year-old male patient with ulcerated tumor at the left thigh was diagnosed as having nasal type NK/T cell lymphoma on the basis of histopathological and flowcytometric findings of tumor, revealing diffuse infiltration of atypical lymphoid cells into blood vessels and expression of CD7 and CD56 antigens, but not CD3. He had tumor infiltration in the bone marrow and at the right lower lung field. After five cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy, the patient achieved complete remission and received high-dose chemotherapy with auto-PBSCT, although the tumor recurred in the right leg 10 months later. Despite salvage chemotherapy, followed by local irradiation and surgical amputation, a tumor recurred at the left upper gingiva 10 days after. Using E. coli L-asparaginase (6000 U/m2/day), the tumor regressed, fever was alleviated and the serum lactate dehydrogenase decreased to normal range after several days. The asparagine synthetase expression in tumor cells was immunohistochemically negative on paraffin-embedded tissues. Because of the anaphylactoid reaction developing after E. coli L-asparaginase, alternative erwinia L-asparaginase (6000 U/m2/day) was administered, resulting in regression of tumor and fever lysis. L-asparaginase is a promising agent for the treatment of NK/T cell lymphoma.
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7/33. scalp tumour as a sign of systemic B-cell lymphoma.

    An 86-year-old man presented with a painful reddish tumour on the scalp with a 3-month history, mental confusion with recent onset and lymphadenopathies. Histological examination of the lymph node and cutaneous lesion revealed a dense infiltrate of atypical and large B cells. There was no evidence of bone marrow invasion. According to REAL (Revised European-American classification of Lymphoid neoplasms), this lymphoma was considered as a diffuse large B-cell lymphoma with concurrent cutaneous and nodal involvement. Cerebral computerized tomography (CT) scan showed bone and dura mater invasion in the right parieto-occipital region with collapse of lateral ventricle. The patient was submitted to systemic chemotherapy with cyclophosphamide, vincristine and prednisolone (CVP). There was a good response with regression of the cutaneous lesion, but the patient died after the third cycle. We point out the unusual clinical presentation and aggressive behaviour of this lymphoma.
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8/33. Evolutive peritoneal disease after conservative management and the use of infertility drugs in a patient with stage IIIC borderline micro-papillary serous carcinoma (MPSC) of the ovary: case report.

    A young nulliparous woman with stage IIIC bilateral borderline micro-papillary serous carcinoma (MPSC) of the ovary underwent conservative surgery with optimal preservation of future fertility. The left ovary and a substantial portion of the right ovary were removed. The patient became pregnant at the first IVF cycle attempted after conservative management. A cesarean section was performed in the 37th week of pregnancy and combined with very precise exploration; there were multiple non-invasive implants on the peritoneal surface and liver, and contra-lateral ovarian tissue was of normal appearance. Abdominal hysterectomy and right oophorectomy were done as a definitive treatment 3 months after the Caesarean section. The patient showed a rapid progression to invasive ovarian carcinoma in this period of time. MPSC has the greatest risk of malignant transformation among the advanced stage serous borderline tumours. Fertility-sparing surgery is an option for young, childless women who would like to preserve their fertility. However, the treatment must be taken very cautiously and requires rigorous surveillance.
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9/33. Epstein-Barr virus-associated extranodal NK/T-cell lymphoma following mosquito bites in an elderly patient without prior hypersensitivity.

    We describe a 73-year-old woman who developed fever and inflammation with ulceration at the site of mosquito bites in the lower thigh. Soon she developed disseminated skin lesions characterized by redness, induration, and local heat. Some lesions showed necrosis and ulceration, including those located in the nasal cavity. She had no history of hypersensitivity to mosquito bites, and the serum IgE concentration was within the reference range. A skin biopsy specimen from the lower thigh adjoining the mosquito bites was diagnosed pathologically as showing extranodal NK/T cell lymphoma, nasal type. Southern analysis of the biopsy specimen showed an oligoclonal band representing Epstein-Barr virus (EBV) DNA. bone marrow examination revealed infiltration by lymphoma cells and marked hemophagocytosis. The patient underwent three cycles of chemotherapy with carboplatin, etoposide, ifosfamide, and dexamethasone (DeVIC), but died of lymphoma progression during treatment. We speculate that, rather than an allergic reaction, this late-life occurrence of hypersensitivity to mosquito bites might represent lymphoproliferative disease induced by a direct action of mosquito salivary gland secretions on EBV- infected NK cells.
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10/33. The Ilizarov method in the treatment of malignant neoplasms of the tibia.

    A total of 3 malignant neoplasms of the tibia are presented: 1 is a mesenchymal chondrosarcoma of the tibial pylon (male aged 14 years), and 2 are cases of squamous skin carcinoma of the leg with tibial infiltration (1 male and 1 female aged 32 and 64 years, respectively). The resections carried out (16.5 cm on the average) were treated by bone transport. Simple transport was used in the first patient, double in the other two. At the end of transport a second surgical stage involving astragalotibial arthrodesis was performed in the first case, and revision of the junction site of bone segments transported with application of autoplastic bone grafts was performed in the other two.The regenerate obtained was slowly corticalized in the first patient, submitted to various cycles of chemotherapy during the course of distraction. In the other two cases, which were not treated by chemotherapy during distraction, corticalization occurred over a shorter amount of time. Follow-up was obtained after 10 years in the first case, and after 2 years in the other 2 patients. There was no long-term recurrence.
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