Pulmonary sequestration is a complex anomaly involving the pulmonary parenchymal tissue and its vasculature. It presents as a cystic mass of nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation (CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neuroblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.
Abdomen ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Diagnosis ; Lung ; Neuroblastoma ; Phaseolus ; Thorax ; Ultrasonography

Neuroblastomas are derived from the neural crest ectoderm, and are the most common solid abdominal masses of infancy. Congenital neuroblastoma, however, is rare. We report a rare case of congenital neuroblastoma with multiple metastases found at autopsy, performed at 2 days after birth. He was born by cesarian section and weighed 2,350 g. His respiration was weak and abdomen was distended. The patient died 2 days after birth. Postmortem examination revealed a relatively well demarcated ovoid mass, in the left adrenal, with necrosis and hemorrhage. Multiple small metastatic tumor nodules in the liver, lung, kidney, brain, rib, thyroid glands, and spleen, were noted. The histopathological pictures confirmed the diagnosis of neuroblastoma of the adrenal with multiple metastasis.
Adrenal Gland Neoplasms/congenital/*diagnosis/pathology ; Autopsy ; Chromogranins/biosynthesis ; Cytoplasm/metabolism ; Human ; Immunohistochemistry ; Infant, Newborn ; Male ; Necrosis ; Neoplasm Metastasis ; Neuroblastoma/congenital/*diagnosis/pathology ; Phosphopyruvate Hydratase/biosynthesis

OBJECTIVE: To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. MATERIALS AND METHODS: We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. RESULTS: All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. CONCLUSION: Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas.
Adrenal Gland Diseases/*diagnosis ; Adrenal Gland Neoplasms/*congenital/*diagnosis ; Catha ; Cysts/*diagnosis ; Diagnosis, Differential ; Female ; Hemorrhage/*diagnosis ; Humans ; Infant ; Infant, Newborn ; Male ; Neuroblastoma/*congenital/*diagnosis ; Tomography, X-Ray Computed ; Ultrasonography

OBJECTIVE: Neuroblastoma is a very common pediatric malignant tumor and sometimes involves the spinal cord to result in neurological deficits. The authors perform a retrospective analysis of the 12 cases of pediatric neuroblastoma with spinal involvement to assess the characteristics and surgical outcome. METHODS: We retrospectively reviewed the 12 cases of pediatric neuroblastoma with spinal involvement which underwent surgery from 1988 to 2002 in our hospital. All the cases were confirmed by pathologic diagnosis and reviewed about initial presentation, the location of tumor, treatment, outcome and complication. RESULTS: The ratio of male to female was 1: 2 and mean age was 3.5 years(0.3-13.6). The chief complaint was motor weakness in 7 cases, mass in 2, urinary incontinence in one and 2 cases were asymptomatic. The tumor involved thoracic level in 5 cases, thoracolumbar level in 3, cervicothoracic level in 2, cervical and lumbar levels in one case each. All the cases underwent surgery, four had chemotherapy and four had both chemotherapy and radiotherapy. After 33.9 months of mean follow up, in all of the 4 cases whose intraspinal tumor was gross-totally resected, neurological status improved. Of the 4 cases with subtotal resection, all except one showed progression or no neurologic improvement. Postoperative spinal deformity has developed in 5 cases. Three cases expired because of chemotherapy complication and tumor progression. CONCLUSION: Active surgery about the spinal involvement of pediatric neuroblastoma shows neurological improvement regardless of the survival. It seems to be helpful to the quality of life in pediatric patients.
Congenital Abnormalities ; Diagnosis ; Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Neuroblastoma* ; Quality of Life ; Radiotherapy ; Retrospective Studies ; Spinal Cord ; Urinary Incontinence

Palpation of an abdominal mass in an infant or child presents a challenging problem in diagnosis and treatment. We reviewed the data on 166 patients under age 15 years who admitted to Ped. Dept. of PMC due to palpable abdominal mass in Jan. 1972-July 1977. The results are as follows 1. Of the 57 surgical cases, pathologically confirmed abdominal tumors were 39 cases. Of the 20 retroperitoneal tumors, Wilms tumors were 13 cases, neuroblastomas were 3 cases, polycystic kidney was 1 case, and retroperitoneal teratoma was 1 case. Of the 19 intraperitonel tumors, hepatomas were 3 cases, hepatoblastoma was 1 case, choledocal cysts were 3 cases, mesenteric and omental cysts were 4 cases, malignant lymphomas were 4 cases and ovarian cysts were 3 cases. Other surgical diseases were 2 cases of ascariasis and 2 cases of bezoar. 2. Medical cases were as follows : 35 cases of leukemia, 15 cases of infectious hepatitis, 14 cases of congenital syphilis 7 cases of liver cirrhosis 7 cases of lirerabscesses, 4 cases of miliary Tbc., 3 cases of congenital spherocytosis, 1 case of cryptococosis, I case of osteopetrosis and 1 case of erythroblastosis fetalis. 3. In age distribution, almost all cases(94%) of Wilms tumor and neuroblastoma were under age of 4 and half of medical cases in infancy were congenital syphilis. 4. Of the pathologically confirmed 39 abdominal tumors, 20 cases were retroperitoneal tumor and 19 cases were intraperitoneal tumor. Of the 20 retroperitonel tumor cases, 16 cases were renal origin, 3 cases were adrenal origin, and 1 case was teratoma. Of the 19 intraperitoneal tum orcases, 8 cases were hepatobiliary origin, 3 cases were ovary origin, 4 cases were omental and mesenteric origin and 4 cases were lymphatics origin. 5. Of the 39 abdominal tumor cases, 34 cases (87%) visited the hospital with the chief complaint of palpable abdominal mass. But, of the 94 medical cases, only 16 cases(17%) visited the hospital with the chief complaint of palpable abdominal mass. 6. In some cases presumptive diagnosis on the base of history taking, physical examination, chest X-ray, simple abdominal X-ray. Peripheral blood findings on admission were uncorrect. In 1 case of Wilms tumor, we suspected liver abscess on admission. In 1/3 case of intraperitoneal tumors, we suspected retroperitoneal tumor on admission. We suspected lymphoma on admission in 1 case of ascariasis and 1 case of bezoar. We misdiagnoed 1 case of miliary Tbc. As hepatoma, 1 case of liver cirrhosis as retroperitoneal tumor and 1 case of congenital syphilis as retroperitoneal tumor on admission. 7. In the treatment of malignant abdominal tumor, we tried all possible measures such as surgery, chemotherapy, and radiation therapy. But prognosis of all malignant abdominal tumors were very poor. Only 1 case of Wilms tumor and 1 case of hepatoblastoma were survived at the time of review.
Age Distribution ; Ascariasis ; Bezoars ; Carcinoma, Hepatocellular ; Child* ; Diagnosis ; Drug Therapy ; Erythroblastosis, Fetal ; Female ; Hepatitis A ; Hepatoblastoma ; Humans ; Infant* ; Infant, Newborn ; Leukemia ; Liver Abscess ; Liver Cirrhosis ; Lymphoma ; Neuroblastoma ; Osteopetrosis ; Ovarian Cysts ; Ovary ; Palpation ; Physical Examination ; Polycystic Kidney Diseases ; Prognosis ; Syphilis, Congenital ; Teratoma ; Thorax ; Wilms Tumor