1/471. Signet ring epithelioid stromal tumor of the small intestine. Cells having a signet ring appearance can occur in mesenchymal, lymphoid, and other nonepithelial neoplasms. We report the case of an intestinal stromal tumor with smooth muscle differentiation and a prominent signet ring cell component. The presence of signet ring forms of smooth muscle cells in sections of paraffin-embedded tissue often contrasts with a lack of cytoplasmic spaces by electron microscopy, and the ultrastructural finding of signet ring-like areas in the present case can be attributed to the fact that the tissue for electron microscopy was retrieved from paraffin blocks where this peculiar artifact already existed. Ultrastructural examination of the signet ring-like areas suggests that they originated as retraction spaces which may have resulted from variations in intracellular tension forces related to the distribution of actin filaments. ( info) |
2/471. Treatment of upper abdominal malignancies with organ cluster procedures. Upper abdominal exenteration for upper abdominal malignancies was carried out in 15 patients with removal of the liver, spleen, pancreas, duodendum, all or part of the stomach, proximal jejunum and ascending and transverse colon. Organ replacement was with the liver, pancreas and duodenum plus, in some cases, a short segment of jejunum. Eleven of the 15 patients survived for more than 4 months; 2 died, after 61/2 and 10 months, of recurrent tumor. Of the 9 patients who are surviving after 61/2 to 14 months, recurrent tumor is suspected in only 1 and proven in none. Four patients with sarcomas and carcinoid tumors (2 each) have had no recurrences. The other 5 survivors had duct cell cancers (3 examples), a cholangiocarcinoma (1 example), and a hepatoma (1 example). The experience so far supports further cautious trials with this drastic cancer operation. ( info) |
3/471. Renal angiomyolipoma resembling gastrointestinal stromal tumor with skenoid fibers. We report an unusual case of renal angiomyolipoma occurring in 68-year-old man. The tumor lacked well-developed vascular and adipose components and was composed almost exclusively of smooth muscle cells. Numerous skenoid-like periodic acid-Schiff-positive globules were interspersed between the tumor cells; the lesion therefore closely resembled a low-grade stromal tumor of the gastrointestinal tract. The HMB45-positive/CD34-negative immunophenotype was essential for the diagnosis of angiomyolipoma. Neither gastrointestinal tumor nor any signs of tuberous sclerosis were found. This lesion should be included in the list of morphologic variations of angiomyolipoma, which may cause diagnostic difficulties. ( info) |
4/471. Data to the clinical manifestation of the Krukenberg tumour. Ovarian cancer is one of the most frequent malignant tumours in the female population. The screening of this tumour type is unsolved. The tumours are usually diagnosed in the advanced stage; thus the results of survival are unfavourable. Their histopathological appearance has a wide variety, with the occurrence of numerous metastatic forms. In these metastatic cases the choice of treatment is more difficult and prognosis is also worse. Among the metastatic tumours, the primary tumours of the gastrointestinal tract occur the most frequently. They are known as the Krukenberg tumour. Authors present two cases of Krukenberg tumours in order to summarize our knowledge on this rare tumour type and to give some practical advice. ( info) |
| BACKGROUND: patients with ataxia telangiectasia (AT), known to have an inherent increased susceptibility to the development of cancer, may present with malignancies that are unusual for the patient's age, are often difficult to diagnose clinically and radiographically and respond poorly to conventional therapy. MATERIALS AND methods: We reviewed the clinical presentation and imaging studies of 12 AT patients who developed malignancies. RESULTS: Eight of the twelve patients developed non-Hodgkin's lymphoma (CNS, thorax, bone), two developed Hodgkin's disease, and two were diagnosed with gastrointestinal mucinous adenocarcinoma. CONCLUSION: The lymphomas were commonly extra nodal, and infiltrative rather than mass-like. The recognition of the tumors was often delayed due to confusion with the known infectious complications in AT patients. ( info) |
6/471. Chromosome instability in lymphocytes from two patients affected by three sequential primary cancers: the role of fragile sites. The chromosomal aberration rate and the expression of fragile sites induced by aphidicolin were evaluated in metaphase chromosomes obtained from peripheral blood lymphocytes of two untreated patients with multiple primary cancers. Spontaneous aberrations of chromosome number and structure and chromosome fragility were compared with controls with the use of the same methods. Chromosomal aberration rates and expression frequencies of fragile sites were significantly higher in the patients than in normal control subjects. In the patients, all but one structural chromosome aberration involved at least one fragile site. Our results suggest that fragile sites may be unstable regions of the human genome, which might play an important role in the genetic instability associated with cancer predisposition. ( info) |
7/471. Correlation between clinical course and CEA levels during chemotherapy. In order to evaluate the correlation between clinical progress and CEA levels in gastroenteric tumours, particularly during chemotherapeutic treatment, CEA assay was performed on 330 patients (126 with gastric neoplasms, 204 with large bowel carcinoma). 175 out of these had a pre-operative assay. Moreover CEA test positivity (CEA larger than or equal to 5 ng/ml according to the technique employed by us) is higher in colon neoplasms compared with gastric neoplasms and in adenocarcinomas in comparison with undifferentiated forms; besides it depends on the pathologic stage. In colon tumours CEA test showed a higher positivity for left than for right forms (66.6% versus 38%). 240 patients were followed up with repeated CEA assays: the following observations were made: a - After radical surgery 72% of the patients shows normalized CEA values. b - In 198 patients who underwent radical surgery, not requiring chemotherapy, there was a close correlation between CEA levels and clinical evolution in 98% of the cases. c - 60 out of 68 patients (88%) submitted to chemotherapy for advanced neoplasms show a close correlation between CEA response to the chemotherapy and clinical response (p less than 0.001). These investigations stress, above all, the importance of CEA test to monitor the treatments performed (surgical and chemotherapeutic) and to adopt, eventually, more effective chemotherapeutic modalities. ( info) |
8/471. Multiple lymphomatous polyposis of the gastrointestinal tract is a heterogenous group that includes mantle cell lymphoma and follicular lymphoma: analysis of somatic mutation of immunoglobulin heavy chain gene variable region. Multiple lymphomatous polyposis (MLP) is characterized by multiple polyps involving long segments of the gastrointestinal (GI) tract. MLP is thought to represent mantle cell lymphoma (MCL) of the GI tract; however, some cases of follicular lymphoma (FL) of the GI tract are found with a multiple polypoid appearance. In the present study, to clarify the cellular origin of MLP, clonal immunoglobulin heavy chain (IgH) gene rearrangement of four cases with MLP was amplified by polymerase chain reaction (PCR) and analyzed for the presence of somatic mutation. The IgH variable (VH) region sequences of three cases (CD5 CD10- cyclin d1 ) showed a little somatic mutation compared with the closest published germline. The other case (CD10 CD5- cyclin d1-) was highly mutated and showed intraclonal heterogeneity (ongoing somatic hypermutation). These data indicate that three of the cases with MLP are derived from pregerminal center B cells (mantle zone B cells) and one case with MLP from germinal center B cells. Our study suggests that MLP is a heterogenous group that includes MCL and FL. ( info) |
9/471. Imaging of a metastatic gastrointestinal carcinoid by F-18-DOPA positron emission tomography. The localization of carcinoids in the gastrointestinal tract is frequently difficult if not impossible with the imaging procedures used to date. It is reported on a patient with metastasizing carcinoid in whom various imaging procedures were not successful in detecting the primary tumor. Due to the importance of primary tumor proof for potential curative surgical therapy, a whole-body positron emission tomography with F-18-DOPA was performed. PET enabled localization of a potential primary tumor in the ileum. Moreover, in addition to the known abdominal lymph node and liver metastases, it detected a mediastinal lymph node metastasis and a pulmonary metastasis. F-18-DOPA whole-body PET may be a very promising imaging approach to the localization and staging of gastrointestinal carcinoids. ( info) |
10/471. Use of the sentinel lymph node to determine metastases of gastrointestinal malignancies: a word of caution. Identifying the sentinel lymph node has been shown to carry prognostic and therapeutic implications in the surgical treatment of solid tumors. Recently, sentinel lymphadenectomy has been described for gastrointestinal malignancies, but its clinical value remains uncertain. We describe the case of a patient with appendiceal carcinoid who underwent a right hemicolectomy 4 months after appendectomy, out of concern over residual local or regional disease. One sentinel lymph node was identified in the colonic mesentery using the blue dye technique. This sentinel node and 35 others were negative for metastases, but one lymph node not identified through blue dye carried evidence for micrometastatic disease on hematoxylin and eosin (H&E) and immunohistochemical chromogranin stains. The case raises some issues about the value and limitations of sentinel lymph node biopsies in gastrointestinal cancer. Aspects related to technique, learning curve, gastrointestinal lymphatic drainage patterns, the impact of prior operations, and the limited therapeutic implications compared to cutaneous or subcutaneous solid malignancies are discussed. We conclude that at this point in time, the information obtained from biopsies of sentinel lymph nodes during the surgical treatment of gastrointestinal cancer should be utilized with due caution. ( info) |