Granular cell tumor (GCT) is a slow-growing benign neoplasm that can be found in any organ. Pediatric laryngotracheal GCT is rare. We experienced a 6-year-old boy suffering from a barking cough and symptoms of stridor and croup for one month. Head and neck computed tomography revealed a protruding mass that occluded 60% of the airway lumen. Under the impression of hemangioma or papilloma, excision revealed a submucosal non-encapsulated mass. Histologically, the mass was composed of sheets of large polyhedralshaped tumor cells containing plump eosinophilic granular cytoplasm and centrally placed, small, bland-appearing nuclei. The tumor cells were positive for S-100 protein, and voluminous eosinophilic cytoplasm was stained by diastase-resistant periodic acid-Schiff. The present report describes a unique case of a huge pediatric laryngeal GCT extending to the subglottic trachea. We also review the clinical course of pediatric laryngotracheal GCT and emphasize the importance of diagnosing GCT in children.
Citations
Citations to this article as recorded by
Pediatric granular cell tumor of the larynx: A case report and literature review Jing Ke, Junwei Xiong, Juhong Zhang, Haiyu Ma, Wei Yuan Journal of Cancer Research and Therapeutics.2023; 19(4): 1070. CrossRef
The pathogenetic mechanism of focal segmental glomerulosclerosis (FSGS) is not known. Some authors suggest glomerular hypertrophy may precede the development of FSGS in patients with minimal lesion. It was recently reported that the size of nonsclerotic glomeruli in adults with FSGS is significantly larger than that of cases with minimal lesion. It is not clear whether glomerular hypertrophy observed in adults with FSGS is also seen in children with FSGS. Thus, we have analyzed 37 renal biopsies from children with FSGS by morphometry and the data were compared with 37 renal biopsies from age- and sex-matched patients with minimal lesion. The number of glomeruli submitted for morphometric analysis was 22.6+/-14.2 in cases with FSGS and 30.9+/-11.4 in cases with minimal lesion. Mean glomerular volume (MGV) in FSGS group was significantly larger than that of minimal lesion [(13.1+/-3.9) x10(5) microm3 vs.
(10.1+/-1.9) x10(5) microm3, p<0.001]. The relative interstitial volume of renal cortex in patients with FSGS was significantly larger than that of minimal lesion [(0.106+/-0.051) microm3/microm3 vs. (0.029+/-0.012) microm3/microm3, p<0.0001]. In FSGS, the percentage of glomeruli with FSGS was significantly correlated with relative interstitial volume of renal cortex (r=0.79, p<0.0001). As is the case for adult FSGS, MGV of children with FSGS is significantly larger than that of minimal lesion. Thus, the presence of glomerular hypertrophy observed in biopsies with minimal lesion nephropathy seems to be an indication that the coexistent FSGS lesions are undetected due to sampling problems.
Microvillous inclusion disease (MID) or congenital microvillous atrophy is a rare cause of intractable, persistent secretory diarrhea shortly after birth or at birth. The prognosis of MID is very poor and most of the reported patients have died within 6.5 years. Diagnosis is based on the ultrastructural demonstration of intracytoplasmic inclusion of microvilli. There have been several cases reported in the literature. To evaluate the clinicopathologic features of MID, we have reviewed 4 cases of MID which was confirmed by the ultrastructural study of the duodenal biopsy. All patients suffered from life-threatening diarrhea since birth. Many other radiologic or laboratory findings were of no value in the evaluation of causative agents. One of the patients died of the disease and remaining patients have been alive on intravenous fluids or total parenteral nutrition. Histological findings of all cases were similar and characterized by varying degrees of mucosal atrophy and chronic inflammatory cell infiltration in the duodenal mucosa. PAS stain revealed a discontinuous brush border over the atrophic villous surface with or without small vacuoles in the cytoplasm of the surface epithelium. Ultrastructural changes were found mainly in the surface epithelium of the duodenal mucosa and characterized by the presence of membrane bound inclusions lined by intact or degenerating microvilli, as well as degeneration of surface epithelial cells with loss of microvilli, or with sparse, short microvilli.
PURPOSE: There is no specific treatment guidelines for Henoch-Schonlein (HS) nephritis. Therefore we performed this study to observe the effect of long term steroid therapy combined with azathioprine METHODS: Treatment protocols; 1) Steroid pulse therapy: methylprednisolon 30 mg/kg/dose, maximum 1 gm, intravenously 6 times for alternate day. 2) Oral steroid was given 2 mg/kg/day for 1 month, 1 mg/kg/day for following I month and alternate day oral steroid combined with azathioprine 2 mg/kg/day for 2 years. RESULTS: Time period from HSP to onset of HS nephritis was between 2 weeks to 5 months with mean 7.4+/-7.4 weeks. Clinical remission were seen in 4 cases out of 5 (80%). Mean time period with disappearance of proteinuria and microscopic hematuria were 5+/-2.4 month and 13.3+/-2.9 month respectively. On pathologic findings by ISKDC, 3 cases were grade IIIb, 2 cases were grade IV in first kidney biopsies and showed pathologic improvement in follow up kidney biopsies after 2 years treatment. CONCLUSION: As there no definitive treatment for HS nephritis so far, our study of long term oral steroid therapy with azathioprine was effective in clinical and histologic aspect. Therefore further study in HS nephritis with in a large group will be needed in the future.
Adrenal cortical neoplasm, especially carcinoma, is extremely rare in pediatric patients. We describe here a rare pediatric case of adrenal cortical neoplasm. A 2-year-old girl presented with an enlarged clitoris. The other physical findings and laboratory tests were nonspecific. The magnetic resonance imaging showed a 4 cm-sized heterogeneously enhancing soft tissue mass with calcification in the left adrenal gland. The mass was removed by laparoscopic operation. Grossly, several fragments of reddish tan soft tissue were present, and they weighed 19 gm in total. Microscopically, there were capsular invasion, diffuse/solid growth pattern with focal necrosis, high cellularity, cytoplasmic eosinophilia, marked nuclear pleomorphism, high N/C ratio, prominent nucleoli, atypical mitotic figures and calcifications, which all suggested adrenal cortical neoplasm of histologic malignancy. On immunohistochemistrical staining, there were positive reactivities to pancytokeratin, cytokeratin 7/20, CEA, inhibin and p53. The Ki-67 labeling index was about 6%. All these findings were indicative of adrenal cortical neoplasm of histologic malignancy.
Biliary cystadenoma of the liver is a rare multilocular cystic neoplasm of biliary origin. it occurs most often in middle aged women and rarely in children. Histogenesis of this tumor is uncertain. It may be developmental in origin arising from aberrant hamartomatous bile ducts or ectopic rests of embryonic biliary cystadenoma of the liver discovered at 8 month of intrauterine fetal life. This case supports its congenital theory.
The posterior fossa, containing roughly 6ne fourth of the intracranial contents, is the site of about 30-35% of the intracranial tumors. The incidence of primary tumors in the posterior fossa is quite different from that of the cerebivm. We analysed 124 cases of posterior fossa tumor, over a 10 year period, to understand the status of posterior fossa tumor and its histologic characteristics.
Medulloblastoma was most common(37cases, 29.8%), followed by astrocytoma, hemangiobla-stoma, ependymoma, meningioma, metastatic tumor, arteriovenous malformation and choroid plexus papilloma in descending order of frequency. Tumors were found most frequently between the ages of two and ten years(28.2%) and sixty seven(54.0%) cases were diagnosed before the age of fifteen. The ratio of male to female was 60 : 64. Astrocytoma revealed a characteristic juvenile pilocytic type and a microcystic change. Hemangioblastoma showed higher frequency(17.7%) than previous reports and the origin of tumor cells is still equivocal. Other tumors revealed the same histologic features as other intracranial tumors.
Thirty cases of rhabdomyosarcoma in patients under the age of 15 years were reviewed retrospectively to find out characteristics of histologic subtypes related to prognosis.
Histologic diagnosis were embryonal in 21(70%), alveolar in 7(23%) and mixed type in 2(7%). Cellular anaplasia was noted in 6 cases. Round to polygonal cells with abundant eosinophilic cytoplasmic rim or globules were most characteristic cytologic feature or rhabdomyosarcomas, being followed by myofibrils. Myxoid and edematous stroma in embryonal type and alveolar pattern in alveolar type wee nost characteristic growth pattern. Immunohistochemically, tumors were positive for desmin, actin and myoglobin in 97%, 27%, and 40%, respectively. Consequently, characteristic cytologic features are important to differentiate rhabdomyosarcomas form other kinds of small round cell tumor and growth pattern is more critical in categorizing histologic subtypes. Desmin is far more useful than actin or myoglobin in the diagnosis of rhabdomyosarcoma.
Primitive neuroectodermal tumor (PNET) occuring in the brain (especially in the cereburm) of young individuals is a rare and highly malignant neoplasm. The authors analyzed 5 cases of primitive neuroectodermal tumor, operated from January 1986 to September 1987. They had characteristic clinical, radiologic and pathologic features. Patient's age ranged from 2 years to 14 years old (mean 8.2 years old) and there were 3 boys and 2 girls. They were rapidly growing tumor, with a brief duration of symptoms taking a rapidly progessive course. Computerized tomographic findings were characteristic, revealing the large, irregular, typically iso-to hyper-dense mass lesions with calcification and cystic or necrotic areas, and showing dense heterogeneous contrast enhancement of the mass in the cerebral hemisphere.
Grossly all of the 5 cases showed hemorrhage and necrosis with sharp border and 2 cases showed calcification and cystic change grossly. Microscopically, they were predominantly composed of undifferentiated small dark cells with evidence of focal differentiation along glial and or neuronal lines. Four cases showed glial differentiation and three cases showed neuronal differentiation. Mesenchymal components were predominant in 3 cases.