Resection of Abdominal, Pelvic and Retroperitoneal Tumors.

BACKGROUND: Mesenchymal malignancies are relatively rare tumors with distinct behaviors that are usually surgically removal. However, it is sometimes impossible to perform such surgery according to standardized procedures. In particular, surgical removal of intraperitoneal and retroperitoneal tumors differs among individuals. CASE REPORTS: We present two cases with intra-abdominal and retroperitoneal sarcomas who were treated at our comprehensive oncology center. The first patient was a 36-year-old male who was initially diagnosed with a tumor in the subrenal space that measured 95 × 90 × 140mm, contacted the inferior vena cava and right kidney, and had the same blood supply as the upper pole of the right kidney. Primary histological analysis indicated that the tumor was a schwannoma. After further examinations, the tumor was removed and the right kidney was preserved. A ganglioneuroblastoma was diagnosed based on definitive histological analysis. Adjuvant radiotherapy was administered to the tumor bed. The patient is disease-free at 1 year after resection. The second patient was a 52-year-old male who was diagnosed with a liposarcoma in the retroperitoneal space that measured 50 × 36 × 15cm and weighed 14kg upon resection. Resection involved left-side nephrectomy and adrenalectomy. Adjuvant chemotherapy with IFO/ADM was administered. A recurrence in the tumor bed was resected 31 months after the primary resection. Three new foci appeared in the retroperitoneal space after another 18 months and were removed. Another recurrence in the left funiculus was removed after a further 6 months. The patient has been disease-free for 3 months. CONCLUSIONS: Treatment of soft tissue sarcoma is complex and should be performed at a comprehensive oncology center if possible. Preoperative biopsy is essential. Key words: sarcoma - surgical procedures Supported by MZ CR-RVO (MOÚ,00209805). The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 6. 3. 2018 Accepted: 20. 3. 2018.

High-dose 131I-mIBG as consolidation therapy in pediatric patients with relapsed neuroblastoma and ganglioneuroblastoma: the Japanese experience.

OBJECTIVE: Children with relapsed neuroblastoma have a poor prognosis despite modern multimodality therapy. Novel and more effective therapeutic strategies are required for relapsed neuroblastoma. We retrospectively examined the utility of consolidation therapy with high-dose 131I-meta-iodo-benzyl-guanidine (131I-mIBG) in relapsed neuroblastoma or ganglioneuroblastoma patients with complete response (CR) to induction therapy as demonstrated by diagnostic 123I-mIBG scintigraphy. METHODS: Between December 2009 and 2014, five patients with relapsed neuroblastoma and one with relapsed ganglioneuroblastoma received high-dose 131I-mIBG therapy. Overall and progression-free survival rates at five years after 131I-mIBG therapy were analyzed by the Kaplan-Meier method. RESULTS: During follow-up, three children showed no signs of disease relapse, whereas three died. One child without a relapse died from post-transplant side effects, and two children with a relapse died owing to tumor progression. The 5-year progression-free and overall survival rates after 131I-mIBG therapy were 44% and 67%, respectively. CONCLUSIONS: Consolidation therapy with high-dose 131I-mIBG for patients with 2nd CR showed good overall and progression-free survival. While the risks of radiation exposure must be considered, high-dose 131I-mIBG therapy as consolidation therapy needs to be further investigated.

Case report: renal morbidity and compensatory renal responses associated with flank radiotherapy in two children.

Unilateral kidney irradiation, above renal tolerance, is occasionally necessary in the treatment of flank tumours in childhood. Renal atrophy follows. There is often a gradual increase in the function of the contralateral kidney to compensate for the radiation injury. The development of this compensatory response appears to be influenced by both age and any dose of radiation received. We report the cases of two children who received high dose unilateral kidney radiotherapy as part of the multimodality treatment of a flank tumour and consider the influences on compensatory hypertrophy of the contralateral kidney.

[Iodobenzylguanide-131 therapy as first choice in non-metastasized neuroblastoma]. / Jobenguaan-131-therapie als eerste keus bij het niet gemetastaseerde neuroblastoom.

Three children with a localized pelvic neuroblastoma are presented. Because of progression and clinical signs of compression of adjacent structures therapy was necessary. Targeted radiotherapy with 131I-MIBG was used as the first mode of therapy. This new strategy proved to be feasible, effective and non-toxic. Application of 131I-MIBG as first-line therapy should be considered more often in neuroblastoma patients.

[Ganglioneuroblastoma in the adult]. / Ganglioneuroblastome de l'adulte.

BACKGROUND: Ganglioneuroblastoma is commonly observed in children but rarely in adults. Therapy and prognosis are not well defined in adults. CASE REPORT: We report the case of a female adult who developed a ganglioneuroblastoma of the left adrenal gland with liver metastasis. Surgical treatment combined with radio and chemotherapy led to complete remission. DISCUSSION: The outcome in our patient and data reported in the literature suggest that a multidisciplinary approach is necessary and that initial surgical resection should be performed whenever possible. Adjuvant radiotherapy or chemotherapy have been shown to be somewhat effective in children but their true impact in adults remains to be ascertained. Follow-up after resection should include physical examination, assay of adrenal derivatives in plasma and urine and imaging.

Ganglioneuroblastoma of filum terminale: case report.

OBJECT: Neuroblastic tumors can be classified as neuroblastoma, ganglioneuroblastoma (GNB), or ganglioneuroma. Ganglioneuroblastomas consist of small, round, immature neuroblast cells and matured ganglion cells. They are most commonly found in the mediastinum and retroperitoneum; intraspinal GNBs are extremely rare. There are only 5 cases of intraspinal GNB reported in the English literature. The authors report a case of GNB of the filum terminale. The duration of follow-up after the initial treatment is longer than in any other published reports. METHODS: A 36-year-old woman underwent resection of an intradural extramedullary tumor at L1-2 in 1993. Pathological diagnosis was GNB. After surgery, her symptoms resolved and she recovered to a normal condition. In 2009, when she was 53 years old, she presented to the hospital with paralysis of both legs. Magnetic resonance imaging suggested recurrence of spinal tumor. She underwent subtotal resection of the tumors, followed by 4 weeks of radiation therapy. RESULTS: Neurological symptoms improved, and, after radiation therapy, the patient was able to walk with a crutch. Histological investigation of the excised tumor indicated that it was a nodular type GNB, which was consistent with the diagnosis from the time of the initial surgery in 1993. Follow-up MRI studies showed no growth of residual tumors in the 3 years following the surgery. CONCLUSIONS: The authors present a rare case of spinal GNB. The duration of follow-up after the initial surgery in 1993 represents the longest description of clinical course after treatment for spinal GNB.

Disaloganglioside GD2 loss following monoclonal antibody therapy is rare in neuroblastoma.

BACKGROUND: Gangliosicle GD2 is abundant on human neuroblastoma (NB). Monoclonal antibody 3F8 targeted to GD2 may have imaging and therapeutic potential. Antigen-negative clones can escape immune-mediated attack leading to clinical resistance or recurrence. PROCEDURE: Among 95 evaluable patients treated intravenously with 3F8 (94 Stage 4, 1 Stage 3), 66 received nonradiolabeled 3F8, 11 received 131-iodine-labeled-3F8 (8-28 mCi/kg) with autologous bone marrow rescue, and 18 received both forms of treatment. Prior to treatment, 90 patients tested positive for GD2 reactivity by bone marrow immunofluorescence (n = 68), tumor immunohistochemistry (n = 20), or diagnostic radioimmunoscintigraphy (n = 2). RESULTS: Of 62 patients who had refractory or recurrent neuroblastoma following 3F8 treatment, 61 (98%) tested positive for GD2 reactivity by bone marrow immunofluorescence (n = 51) or tumor immunohistochemistry (n = 10). The sole tumor that lost GD2 expression underwent phenotypic transformation into a pheochromocytoma-like tumor. CONCLUSIONS: The persistence of GD2 expression in refractory or recurrent NB suggests that complete antigen loss is an uncommon event and cannot account for treatment failure.