1/54. Malignant melanoma showing ganglioneuroblastic differentiation: report of a unique case. We report a case of metastatic malignant melanoma in an inguinal lymph node, expressing ganglioneuroblastic differentiation. This was characterized by the presence of discrete nests and islands of large ganglion cells with abundant cytoplasm and eccentric nuclei with prominent nucleoli admixed with smaller primitive neuroblasts. The cells were separated by pale pink fibrillar material representing neuritic cell processes. These foci of ganglioneuroblastoma were seen over a background of an otherwise typical metastatic epithelioid, focally melanotic, malignant melanoma. immunohistochemistry showed positivity for neurofilament, synaptophysin, chromogranin, vasoactive intestinal peptide, and glial fibrillary acidic protein in the areas with ganglioneuroblastic differentiation, but not in the melanocytic component. Conversely, HMB45 positivity was expressed by the melanocytic cells only. S-100 protein and Melan-A, a putative melanocytic marker, showed positivity in both melanocytic and ganglioneuroblastic components. Ultrastructurally, neuritic cell processes and dense core neurosecretory granules were identified in the ganglionic and neuroblastic cells. A subsequent nodal metastasis in the same region showed focal neuroblastic differentiation without the ganglionic element. No evidence of neuronal or ganglionic differentiation was seen in the primary skin melanoma. ( info) |
2/54. Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction. After a 3-year history of severe constipation, a 16-year-old girl required surgery to be relieved of impacted stools. Histologic examination showed ganglionitis in the myenteric plexus of the large bowel and ileum, whereas the submucosal plexus was spared. At this time, antineuronal nuclear antibodies (ANNA-1, anti-Hu) were found at high titer in the serum of the patient. One and a half years earlier, a paravertebral ganglioneuroblastoma had been removed. Histologic examination had shown undifferentiated neuroblasts and morphologically mature ganglion cells with both cell types embedded in an inflammatory infiltrate morphologically similar to the lymphoplasmocytic infiltration seen in the myenteric plexus. The patient's serum was found to bind to nuclei of mouse intestinal tract neurons, thus fulfilling defining criteria for ANNA-1. The serum also reacted with antigens of defined molecular weight in a Western blot, thus fulfilling defining criteria for anti-Hu. Expression of the Huantigen could be visualized in the nuclei of the patient's tumor cells by immunohistochemistry. These tests showed that an antitumor inflammatory response was the cause of the bowel disease. This is the first report of a tumor from the neuroblastoma group that caused paraneoplastic intestinal pseudoobstruction. Ganglionitis and subsequent aganglionosis are the hallmark of the morphologic diagnosis which cannot be obtained by suction biopsy in patients with intact submucosal plexus. Instead, serum testing for autoantibodies can reveal the etiology. ( info) |
| A 57-year-old male with pineal ganglioneuroblastoma was reported. The tumor was composed of a predominant ganglioneuromatous component and a small neuroblastic component. Primary ganglioneuroblastoma of the pineal region is extremely rare. To our knowledge, only three cases have been documented. This case may have a good prognosis because there is a predominant ganglioneuromatous component defined by both neurofilament triplet proteins and synaptophysin. The clinical significance of the neuronal differentiation in the present case and pathological considerations are discussed. ( info) |
4/54. Results of surgery for a compound adrenal tumor consisting of pheochromocytoma and ganglioneuroblastoma in an adult: 5-year follow-up. A rare, compound adrenal tumor consisting of ganglioneuroblastoma and pheochromocytoma was completely resected in an adult woman. Most of the tumor was occupied by the ganglioneuroblastoma component. This ganglioneuroblastoma was an intermixed tumor, which is known to have a favorable prognosis in children. Based on the lack of spread, the resectability of the tumor, and the histology of the ganglioneuroblastoma, no adjuvant therapy was employed. There was no evidence of recurrence at the 5-year follow-up. This suggests that adjuvant therapy may not be necessary in these compound tumors. ( info) |
| A four and one-half year-old child presented with a several month history of shoulder pain. Her workup revealed a large, homogeneous tumor in the apex of the chest. Surgical resection was performed demonstrating ganglioneuroblastoma. This case illustrates an unusual cause of joint discomfort in children. ( info) |
6/54. Malignant peripheral nerve-sheath tumor arising in a previously irradiated neuroblastoma: report of 2 cases and a review of the literature. BACKGROUND: Only ten cases of the rare occurrence of a malignant peripheral nerve-sheath tumor (MPNST) arising in a ganglioneuroma either de novo or at a site of previous irradiation have been reported. patients AND methods: We present two children who at the age of 19 months and 6 months were diagnosed with a cervicothoracic ganglioneuroblastoma and a retroperitoneal neuroblastoma, respectively. They both received radiation therapy as part of the treatment of their disease. RESULTS: Following a 12-year interval, MPNST arose inside a benign ganglioneuroma in both patients. We illustrate the imaging findings in these two cases and review the cases reported in the literature to increase awareness of this association among radiologists. ( info) |
| A 39-year-old lady presented with low back pain and neurogenic claudication. Magnetic resonance imagining revealed an intradural neoplasm in the cauda equina region. The patient underwent lumbar laminectomy and total excision of the neoplasm. biopsy showed it to be a ganglioneuroblastoma, which is rare in the spinal canal and so far does not appear to have been reported in the region of the cauda equina. Its management is discussed. ( info) |
8/54. So-called malignant and extra-ventricular neurocytomas: reality or wrong diagnosis? A critical review about two overdiagnosed cases. Central neurocytoma (CN) is described as a rare intra-ventricular benign neuronal tumor of the brain. Two primary tumors first diagnosed as malignant and extra-ventricular neurocytomas are reported here. Histologically, the tumor of the first patient, a forty-one-year-old man, consisted of monotonous cells with round nuclei, but no fibrillar background. The second tumor, in a nineteen-year-old girl, showed areas of moderately pleomorphic round cells, with numerous rosettes and ganglion cell differentiation, in an abundant fibrillary network. Both presented calcifications. Mitoses were more frequent in recurrences and spinal locations than in the primaries. All tumors stained strongly for synaptophysin, and GFAP was partly positive in the first case only. patients received post-surgical radiotherapy and were still alive eight and six years, respectively, after initial surgery. The interpretation of atypical cases, such as ours is not easy: the diagnoses finally retained were oligodendroglioma in the first case and ganglioneuroblastoma in the second case. Furthermore, neurocytomas atypical either by their unusual topographical or histological presentation or by their poor prognosis, have been frequently entitled in this way on synaptophysin positivity. So, we were prompted to reassess the entity of CN, seventeen years after the first description, to re-appreciate the reality of anatomo-clinical variants and to discuss the value of synaptophysin positivity in these tumors. In conclusion, it seems preferable to individualize true classical CN, which has a favorable outcome, from so-called extra-ventricular, atypical and anaplastic, clinically malignant neurocytomas for which complementary treatment is required. ( info) |
| A case of retroperitoneal ganglioneuroblastoma in a 60-year-old man is reported. This retroperitoneal tumor was surgically removed and pathologic diagnosis was ganglioneuroblastoma. ganglioneuroblastoma usually occurs in children and is extremely rare in adults. The characteristics are described of an unusual tumor based on the published reports. ( info) |
10/54. 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. ( info) |