Outcomes of pediatric nephroblastoma in southern Thailand.

BACKGROUND AND AIMS: The outcomes of nephroblastoma (Wilms tumor, WT) in our institute and other developing countries are notably below results in most developed countries. The objective of this study was to review treatment outcomes of pediatric nephroblastoma in southern Thailand during the past decade and attempt to isolate factors associated with a poor prognosis. PATIENTS AND METHODS: The records of 34 WT patients, aged less than 16 years, who were operated on in Songklanagarind Hospital, Thailand, during the period from January 1996 to December 2007 were reviewed. The management protocol followed the scheme of the US National Wilms Tumor Study Group (NSWTG). RESULTS: Thirteen cases (38.2%) were diagnosed as stage I, 4 (11.8%) as stage II, 13 (38.2%) as stage III and 2 (5.9%) as stage IV. Two cases with bilateral disease (stage V) had stage I tumors in both kidneys. Four-year overall survival (OS) and event free survival (EFS) rates were 65.2% and 52.7%, respectively. Univariate analysis by Log-rank test revealed statistically significant associations between OS and nodal status (p-value <0.01), manifestation of gross hematuria (p-value 0.02), and tumor size of 10 centimeters or more (p-value 0.02). Multivariate analysis found only the nodal status to be independently associated with OS at a Hazard Ratio of 16.6 (p-value <0.01). Eight of 13 stage I cases and 6/13 stage III cases had relapsed, with two-year post-relapse survival of 42.8%. Significantly poorer outcome was found in cases with early relapse within 200 days after enrolment (p-value 0.02). CONCLUSION: The poor outcome of pediatric nephroblastoma in southern Thailand seems to be related at least in part to failures in primary treatment in stage I patients. Large tumor size and gross hematuria were associated with risk of a poorer outcome.

A unique case of transient spontaneous regression complicated with tumor lysis syndrome of T-cell lymphoblastic lymphoma in HIV-infected patient without antiretroviral therapy.

Spontaneous regression in high-grade non-Hodgkin lymphoma is rare. Herein, the authors report the case of a 26-year-old asymptomatic HIV-infected patient presenting with bleeding per gum after a dental extraction. Initially, a complete blood count showed lymphoblasts and thrombocytopenia. Laboratory investigations were compatible with acute tumor lysis syndrome. Without any steroid or chemotherapy, both clinical and laboratory abnormalities were spontaneously returned to normal limits. However, three weeks later he developed generalized lymphadenopathy. A submandibular gland biopsy revealed to be T-cell lymphoblastic lymphoma. This was followed by the second episode of spontaneous tumor lysis syndrome and spontaneous regression of lymphadenopathy again. At this time, he was treated with cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) with whole brain irradiation. During seven months of chemotherapy, the physical examination and blood chemistry were normal. Unfortunately, after seven courses of CHOP, the disease rapidly progressed and ultimately lead to his death.

Primary pigmented nodular adrenocortical disease with synaptophysin immunoreactivity in two thai children.

This paper reports the cases of two Thai children diagnosed with primary pigmented nodular adrenocortical disease (PPNAD). The first was a thirteen and a half year old male who presented with Cushing syndrome for three years and severe osteoporosis. He had hypercortisolemia, a non-suppressible dexamethasone suppression test, and low serum ACTH. A CT scan showed slight enlargement of both adrenal glands. A bilateral adrenalectomy was performed. The second case was a thirteen-year old female who presented with mild Cushing syndrome for one year with paradoxical response to high-dose dexamethasone suppression test. An MRI revealed suspected microadenoma of the left adrenal gland. Blood sampling showed a higher cortisol level from the left adrenal vein than from the right. A left adrenalectomy was performed, followed by a right adrenalectomy four months later. The pathologies were compatible with PPNAD. Immunostaining for synaptophysin was done in both patients. The cells in the adrenocortical micronodules of both cases were stained intensely with antiserum for synaptophysin, whereas the surrounding adrenal cortex did not.