To assess the utility of the current imaging modalities, a six-year retrospective study was conducted from May 1985 through April 1991 on 11 patients with histopathologically confirmed neuroblastoma. There were four males and seven females. Median age at presentation was six years. All patients underwent intravenous urography (IVU), abdominal ultrasonography (US), skeletal surveys and bone marrow aspiration. Computed tomographic (CT) scanning was performed in nine patients and trephine bone biopsy in five. Suprarenal gland was the primary site in six patients, including one bilateral neuroblastoma. Two presented with primary paraspinal mass, one with posterior mediastinal mass, one had a retroperitoneal tumour and one presented as primary cerebral neuroblastoma. IVU showed indirect evidence of suprarenal tumours in five patients. US demonstrated suprarenal mass in six and calcification within the mass in five patients. US also documented retroperitoneal lymphadenopathy in five patients. US-guided fine needle aspiration biopsy was performed in seven patients and it gave correct histological diagnosis in all. CT accurately depicted all the primary tumours and their involvement of the surrounding structures and metastases in the brain and orbits. US proved to be quite accurate in diagnosing primary abdominal tumours despite its limitations but in other sites, CT was of greater help. Solid appearance of a suprarenal mass on US made the diagnosis of neuroblastoma very likely and calcification within the mass further supported the diagnosis.
Neuroblastoma is the most common malignant tumour in infancy originating in about 70% of cases in the adrenal gland. Haemorrhage and necrosis is often seen in neuroblastoma but cyst formation is uncommon. Fistulous communication between an adrenal cystic neuroblastoma and the large bowel has never to our knowledge been reported before.
Our preliminary experience of Somatostatin Receptor Positive Tumour Scintigraphy (SRPTS) in the management of some rare neuroendocrine tumours is highlighted. Six patients were evaluated using SRPTS. A single patient each with Zollinger-Ellison syndrome, recurrent medullary carcinoma of thyroid and Stage IV neuroblastoma. Two patients with phaeochromocytoma, and one patient with suspected insulinoma were evaluated. SRPTS was useful in three of the six patients studied i.e. patient with gastrinoma, recurrent medullary carcinoma of thyroid and metastatic neuroblastoma. SRPTS although expensive is a useful and cost-effective approach of rare endocrine tumours and it role as a first line tool in the diagnosis is discussed.
To study the distribution of presenting features and their prognostic significance in neuroblastoma treated in a single institution in Malaysia. A retrospective study was made of 78 neuroblastoma cases diagnosed and treated in the University Hospital, Kuala Lumpur, Malaysia between June 1982 and February 1997. Diagnosis was established by standard histological criteria. The presenting features were evaluated for their distribution and prognostic influence. Disease-free survival from diagnosis was the outcome variable of interest. The ages ranged from 0.1 to 11 years old (median: 3 years old). The tumor originated from the adrenal glands in 83% and the majority of cases presented in advanced stage (stage III 22%, stage IV 66%). Bone marrow was the commonest site of distant metastasis occurring in 45% of patients. The main presenting signs and symptoms in decreasing order were pallor, fever, abdominal mass, weight loss, and bone/joint pain. Univariate analysis conferred age, initial stage and Hb level as significant prognostic factors. No influence in disease-free survival was found for sex, race, primary site, urinary vanillylmandelic acid level, white cell count and platelet count. Overall 2-year disease-free survival was achieved in 27 (39%) patients. Four patients underwent bone marrow transplant, three of whom achieved 2-year disease-free survival. The results suggest that age, initial stage and hemoglobin level are significant prognostic factors based on univariate analysis. In addition, more Malaysian children presented with adrenal primary site and advanced disease compared to previous reported studies.