[Ileum and brain, unusual metastatic sites for a thymoma]. / Iléon et encéphale, des sites métastatiques inhabituels pour un thymome.

INTRODUCTION: Thymomas are epithelial neoplasms of thymic origin, preferentially localized in the anterior mediastinum. Recurrences after surgery are uncommon and usually occur in the intrathoracic area. The occurrence of extra-thoracic metastases is an unusual phenomenon. CASE REPORT: Here we report the case of a 61-year-old man with no special medical history. He smoked about 40 pack years but stopped in 1999. Initially he presented with a mediastinal thymoma and underwent surgical resection. One year later the development of abdominal pain and bowel disorders lead to the discovery of an ileal ulcero-necrotic tumour. After surgical resection, histological examination revealed secondary thymoma. A few months later he underwent cerebral MRI because of neurological symptoms. This revealed a second metastasis located in the brain. Stereotactic radiotherapy led to an improvement. After more than one year of follow-up the patient developed a papillary thyroid carcinoma but there were no signs of recurrence of the thymoma. CONCLUSIONS: Extra-thoracic metastases of thymoma are exceptional but their existence should not be overlooked. Their management is not standardised because of lack of data in the literature. Though surgical excision in oligo-metastatic subjects is a frequently reported therapeutic option, a radiotherapeutic approach, particularly in cerebral situations, could be a credible alternative.

[A Case of Recurrent Small Bowel Cancer That Successfully Responded to Chemoradiation Therapy].

Primary small bowel cancer is a rare entity; thus, it is often found in progress. Therefore, the prognosis is often poor. Because of its low frequency, there are few reports concerning the treatment for small bowel cancer; hence, it is important to examine individual cases in detail. In this study, we present a case of recurrent small bowel cancer that successfully responded to chemoradiation therapy. Case: A 48-year-old woman had anemia. Colonoscopy showed a tumor in the terminal ileum. Because of invasion in the ovaries and uterus, ileocecal resection, hysterectomy, and bilateral adnexectomy were performed. The pathological diagnosis was small bowel cancer with lymph node metastasis, and CapeOX therapy was administered as postoperative adjuvant chemotherapy. Since local recurrence was detected in the right lower quadrant 6 months after the surgery, IRIS plus BV was initiated. Radiation therapy(2Gy×25 times, total 50 Gy)was also administered within the same period(only S-1 administration during radiation). After radiation therapy, the tumor decreased significantly in size and showed CR. Currently, the patient is under observation without treatment, but she has had no recurrence for 6 years after the confirmation of recurrence(6 years and 6 months after surgery). It is extremely rare for chemoradiation therapy to be effective for recurrent small bowel cancer; we report such a case with literatures.

Peptide Receptor Radionuclide Therapy-Induced Gitelman-like Syndrome.

Peptide receptor radionuclide therapy (PRRT) is a molecular-targeted therapy in which a somatostatin analogue (a small peptide) is coupled with a radioligand so that the radiation dose is selectively administered to somatostatin receptor-expressing metastasized neuroendocrine tumors, particularly gastroenteropancreatic. Reported toxicities include myelotoxicity and nephrotoxicity, the latter manifesting as decreased kidney function, often developing months to years after treatment completion. We present a case of PRRT-induced kidney toxicity manifesting as a severe Gitelman-like tubulopathy with preserved kidney function. Because profound hypokalemia and hypocalcemia can lead to life-threatening arrhythmias, we highlight the necessity for careful monitoring of serum and urine electrolytes in patients receiving PRRT.

Treatment with octreotide in patients with well-differentiated neuroendocrine tumors of the ileum: prognostic stratification with Ga-68-DOTA-TATE positron emission tomography.

We investigated the use of Ga-68-DOTA-Tyr3-octreotate (Ga-68-DOTA-TATE) positron emission tomography (PET) and standardized uptake values (SUVs) to predict the effectiveness of treatment with the somatostatin analogue octreotide acetate (Sandostatin LAR) in patients with neuroendocrine tumors (NETs). Thirty patients with well-differentiated NETs of the ileum (grades G1 and G2) were studied with Ga-68-DOTA-TATE. The average SUV of a 50% isocontour volume of interest covering the lesion with maximum uptake (SUV mean) and the maximum SUV (SUV max) were determined. Patients were followed up, and the time to progression was recorded. Twenty-one patients showed progressive disease at the end of the study; nine patients had stable disease. The median progression-free survival (PFS) was 51.0 weeks (95% confidence interval [CI] 26.4-75.6). A cutoff for the SUV max of 29.4 and for the SUV mean of 20.3 could separate between patients with a long PFS (69.0 weeks; 95% CI 9.8-128.2) and a short PFS (26.0 weeks; 95% CI 8.7-43.3) response to octreotide acetate therapy. Patients with high radiotracer uptake had significantly higher PFS with a 2.9-fold higher chance for stable disease after 45 weeks; however, the prognostic performance of SUV max on an individual basis was poor, with a sensitivity of 75% and a specificity of 64%. SUV max and SUV mean of NET tumor lesions in Ga-68-DOTA-TATE PET are important prognostic indices for predicting the response to therapy with octreotide acetate.

[Histological findings in an irradiated choroidal melanoma]. / Histologische Diagnostik eines bestrahlten Aderhautmelanoms.

BACKGROUND: Histological findings of choroidal melanomas after proton beam irradiation have been reported for complicated cases after enucleation. We present specimens of a tumor after transretinal probe excision. PATIENT AND METHODS: One year after irradiation, the biopsy was examined histologically. RESULTS: The specimens showed pigmented, spindle-shaped cells staining positively for Melan-A and HMB-45. Ki-67 showed low proliferation. Caspase-3 staining was normal. CONCLUSIONS: The melanoma still contained vital and even single proliferating cells, but regressed afterwards without additional therapy.

Lymphocytic toxicity in patients after peptide-receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE and 90Y-DOTATOC.

PURPOSE: Peptide-receptor radionuclide therapy (PRRT) with somatostatin analogs is an efficient new tool in patients with neuroendocrine tumors, with low risk of toxicity. Since lymphocytes express somatostatin receptors, the aim of this study was to evaluate lymphocytic toxicity after PRRT. METHODS: From May 2005 to May 2007, 16 patients affected by neuroendocrine tumors received PRRT with (90)Y-DOTATOC (9), (177)Lu-DOTATATE (5), or both (2). Absolute count, percentage of leukocytes and lymphocytes, and lymphoid subsets (B, T, and NK) were tested at baseline and until 90 days after treatment. RESULTS: A significant lymphoid toxicity (G2-3), mainly affecting B-cells, was observed. It was particularly evident after (90)Y-DOTATOC. Toxicity resulted in being transient and resolved completely at the end of the follow-up (90 days). CONCLUSION: Lymphocyte toxicity in PRRT is mainly due to the selective targeting on B-cells. The relative sparing of T-lymphocytes could explain the absence of clinical side-effects in these patients, such as increased risk of infections. These findings open interesting perspectives in the treatment of B-cell lymphoproliferative disorders.

Clinical outcome in patients with small-intestinal non-Hodgkin lymphoma.

The clinical features and outcome of small intestinal lymphoma remain unclear. We retrospectively analyzed 23 patients who had non-Hodgkin lymphoma with a small intestinal lesion. With a median follow-up of 37 months, the 5-year overall survival and failure-free survival (FFS) were 64% and 60%, respectively. In a univariate analysis, a worse performance status at the start of treatment and the occurrence of abdominal symptoms or perforation during treatment were associated with poor survival. Perforation often resulted in a dismal prognosis in patients with uncontrollable lymphoma, but not in patients with lymphoma in remission. The role of surgery in small intestinal lymphoma remains equivocal. In the current study, surgery before other therapies favorably influenced FFS, and all patients who underwent complete resection of the small intestinal lesion had extremely favorable results. Further studies are warranted to establish optimal therapeutic strategies.

Chemotherapy in metastatic malignant triton tumor: report on two cases.

Malignant triton tumor (MTT) is a rare, highly malignant nerve sheath tumor with rhabdomyoblastic differentiation. Initial debulking surgery followed by adjuvant therapy is the current treatment of choice, but has very limited efficacy when optimal cytoreduction is not achieved by surgical procedure. Neoadjuvant therapy for MTT, to potentially facilitate subsequent surgery, eradicate micrometastatic lesions and, therefore, improve the therapeutical outcome, has never before been presented in literature. Here, we report on the multimodal management of two cases of advanced and metastatic MTT. Treatment modalities involved neoadjuvant and adjuvant chemotherapy, surgical resection, and radiation. In both cases, integrated Positron Emission Tomography/Computed Tomography (PET/CT) emerged as an important diagnostic tool for the reliable assessment of MTT response and metabolic remission.

Primary follicular lymphoma of the small intestine.

While nodal follicular lymphoma is prevalent in western countries, primary extranodal presentation is rare. We present the clinico-pathological and radiologic features of a patient with primary follicular lymphoma of the small intestine presenting with intestinal obstruction. This is followed by the discussion on the frequency and staging systems for primary gastrointestinal lymphomas, and the relevance of monoclonal anti-CD20 antibody therapy.

[Therapeutic management of primary digestive lymphomas at Yaoundé: analysis of a series of 46 cases]. / Prise en charge thérapeutique des lymphomes digestifs primitifs à Yaoundé: analyse d'une série de 46 cas.

The aim of this study was to identify the principal deficiencies and difficulties associated with the treatment of primary digestive lymphomas at Yaounde. We analyzed the medical records of 46 patients treated at Yaounde General Hospital over a period of 7 years (January 1989 to December 1996). The study population consisted of 24 men (52%) and 22 women (48%), giving a sex ratio of 1.08. The mean age of the patients was 49 years (range: 18 to 80 years). The treatment of these patients was not always optimal, due largely to socioeconomic and technical factors. We observed cases of very late diagnosis in which the histological data were imprecise or incomplete, in the absence of modern histological techniques. Assessment of the expansion of tumors was frequently inadequate, due to the absence of scanners or the inability to pay for their use. These problems, and the high cost of chemotherapy and radiotherapy, have severely limited the efficacy of treatment, resulting in poorer responses to treatment than reported elsewhere. In conclusion, national solidarity and cooperation at the regional and international levels seem to be necessary in these clinical conditions, to overcome these obstacles and to improve the quality of treatment for patients in general, and for those with primary digestive lymphomas in particular.

[Primary ileocecal lymphoma: report of 30 cases].

OBJECTIVE: To investigate the best therapeutic method for primary ileocecal non-Hodgkin's lymphoma. METHOD: Clinicopathologic features of 30 patients who had been treated from June 1964 to September 1993 were analyzed retrospectively. RESULT: Twenty-eight patients (93%) underwent resection of their disease. Eleven patients had the resection followed by chemotherapy. Eleven patients had the resection followed by chemotherapy and radiotherapy. All had pathologic evidence. Twenty-nine patients were categorized as B-cell type NHL. One had T-cell lesion. Eleven patients had stage I disease, 11 stage II, 4 stage III, and 4 stage IV. The 5-year survival rate was 36.67%. CONCLUSION: The prognostic factors are stage of the disease and treatment modality. Adequate initial surgery combined with radiotherapy and chemotherapy may provide optimum therapy for patients with primary ileocecal lymphoma.

Clinical stage IB endometrial adenocarcinoma with an isolated small bowel metastasis.

Although patients with widely disseminated endometrial adenocarcinoma can have tumor involving the intestine, a focal metastasis to the small bowel with no other evidence of extra-uterine spread has not been reported. A patient with a clinical stage IB, grade 2 superficially invasive endometrial adenocarcinoma was found to have an isolated distant metastasis to the small intestine. The patient has been asymptomatic and disease free for almost 4 years following postoperative treatment with whole abdominal radiation therapy on a Gynecologic Oncology Group protocol. The finding of a solitary small bowel metastasis was not predictable based on the current understanding of the biologic behavior of endometrial adenocarcinomas. This case emphasizes the need for a thorough abdominal exploration in patients with endometrial adenocarcinoma by surgeons trained to detect and resect abdominopelvic tumors, and it validates the need for innovative treatment protocols for women with gynecologic malignancies.

[Ultrasonic findings in aggressive abdominal fibromatosis]. / Sonographische Befunde bei aggressiver abdomineller Fibromatose.

A case of aggressive abdominal fibromatosis is reported. Ultrasonography revealed multiple inhomogenous, hypoechoic tumours within the mesenterium and the anterior abdominal wall. Some of these lesions had a hyperechogenic margin.