Sialoblastoma: A Case Report and Review of the Literature on Congenital Epithelial Tumors of Salivary Gland Origin

The histologic, immunohistochemical, and ultrastructural features of a congenital epithelial tumor of the parotid were studied. The tumor was characterized by solid nests of epithelial cells intermingled with proliferating ductal structures lined by a double layer of cells. Immunoperoxidase staining for cytokeratin, vimentin, actin, and S-100 protein showed the presence of cytokeratin in the ductal cells as well as the presence of vimentin, actin, and S-100 protein in the outermost layer of the ducts. The solid nests were focally reactive to S-100 and vimentin. Ultrastructural examination revealed myoepithelial cells with replication of basement membrane material. The tumor recurred 17 months after excision without lymph node involvement or metastasis. The term “sialoblastoma” is favored. Review of the literature on congenital, epithelial salivary gland tumors showed that a few cases recurred locally and only one case had regional lymph node involvement. No distant metastasis has been reported.     

Pathologic Characterization of a Renin-Secreting Juxtaglomerular Cell Tumor in a Child and Review of the Pediatric Literature

We describe a rare case of renal hypertension in a 15-year-old caused by juxtaglomerular cell tumor and compare our findings with those of 20 children reported in the literature. These tumors are usually encapsulated and composed microscopically of polyhedral cells with bland nuclei separated by fibrovascular septa. Characteristic renin granules can be demonstrated by Bowie's stain or electron microscopy. These tumors are benign. Pathologists should recognize the morphologic characteristics of these tumors when dealing with renin-producing neoplasia.     

Granular Cell Tumor of the Thyroid Gland in a Girl Receiving High-Dose Estrogen Therapy

We describe the first case of a granular cell tumor of the thyroid. The tumor appeared while a girl was receiving high-dose estrogen therapy for tall stature. The tumor, however, did not contain estrogen or progesterone receptors. Although granular cell tumors occur twice as commonly in women as in men, the results of this case study do not provide evidence that tumor growth is mediated by direct estrogen or progesterone stimulation.     

Perinatal (Congenital and Neonatal) Neoplasms: A Report of 110 Cases

One hundred ten congenital and neonatal tumors encompassing a 25-year period are described and compared with similar published cases. Forty percent are classified as histologicallly malignant, and 65% of neonates with malignancies died. The types, frequency, and clinical features of neoplasms encountered in the perinatal period are markedly different from those observed in older children and adolescents. Their biological behavior and response to therapy are also dissimilar. Leukemia was responsible for the largest number of deaths followed by neuroblastoma and brain tumors.     

Hemangiopericytoma in infants and children

Seven cases of hemangiopericytoma in infants and children were managed in the past 10 yr at our Institute. All the children were males and presented with a painless tumor of varying duration, over the extremities or head and neck region. Five of them had the tumors present since birth. Five patients were treated by surgical excision, one received radiotherapy and an other received chemotherapy as the primary treatment. These children were followed for period varying from 1–10 yr. One child died and another had local recurrence in this series. Wide local excision of the tumor is the choice of treatment of these rare tumors. If the histology is suggestive of malignancy or if the tumor is incompletely excised, postoperative radiotherapy is advocated, Chemotherapy is not effective for these tumors.     

Imaging of pediatric abdominal soft tissue tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper

This paper provides imaging recommendations for pediatric abdominal tumors that arise outside of the solid viscera. These tumors are rare in children and have been categorized in two groups: abdominal wall and peritoneal tumors (desmoid tumor and desmoplastic small round cell tumor) and tumors that arise from the gastrointestinal tract (gastrointestinal stromal tumor and gastrointestinal neuroendocrine tumor). Authors offer consensus recommendations for imaging assessment of these tumors at diagnosis, during follow-up, and when off-therapy.     

NONTERATOID MEDULLOEPITHELIOMA OF THE RETINA WITH ELECTRON MICROSCOPIC AND IMMUNOHISTOCHEMICAL CHARACTERIZATION

Medulloepitheliomas are rare intraocular tumors, predominant in children, and originate mainly from undifferentiated nonpigmented epithelium of the ciliary body. These tumors rarely involve the optic nerve or the retina. They are classified as nonteratoid and teratoid types; the latter contains heterologous tissues. The teratoid variant of medulloepitheliomas involving the optic nerve or the retina is reported in four patients only. We describe the first case of a benign nonteratoid medulloepithelioma of the retina in a 3 1/2-year-old girl with immunohistochemical and electron microscopic characterization.     

Pediatric ovarian tumors: a review of 67 cases

BACKGROUND: Ovarian tumors are uncommon but important childhood neoplasms. PROCEDURE: We reviewed records of 67 pediatric patients presenting to three pediatric referral centers from 1980 to 2003. RESULTS: Thirty patients had benign tumors. Thirty-seven patients had malignant tumors: 11 immature teratomas, seven malignant mixed germ cell tumors, seven juvenile granulosa cell tumors, five dysgerminomas, two endodermal sinus tumors, two serous papillary cystadenocarcinomas, one small cell carcinoma, one anaplastic sex-cord tumor, and one undifferentiated sarcoma. More than half presented with abdominal pain. Forty-six percent had an abdominal mass at the time of presentation. Other signs and symptoms included poor appetite (15%), urinary symptoms/urinary infection (9%), menstrual changes (9%), and weight loss (6%). Precocious puberty was noted in seven patients. Torsion was seen more often in patients with benign tumors (23 vs. 8%); two patients had both torsion and acute appendicitis. The neoplasm was an incidental finding in 12 patients. CONCLUSIONS: Fifty-five percent of the 67 ovarian tumors presenting to our centers were malignant. Pain was the most common symptom, although presence of an abdominal mass was frequent, and other symptoms non-specific. Almost all neoplasms presented as unilateral masses and rarely were metastatic at diagnosis. Ovarian tumors must be considered in the differential diagnosis of young girls with abdominal pain, mass, or other non-specific symptoms.     

Intraarterial management of giant-cell tumors of the spine in children

Transcatheter arterial management of giant cell tumors of the spine was performed in two female patients aged 12 and 15 who had failed to respond to conventional therapy. Response was determined clinically by alleviation of pain and radiographically by regression and/or calcification of the tumor. Minor side effects included transient nausea, elevated body temperature, and pain.     

Rat Renal Mesenchymal Tumor as an Experimental Model for Human Congenital Mesoblastic Nephroma: II. Comparative Pathology

The morphological features of kidney neoplasms induced by ethylnitrosourea (ENU) with or without prior irradiation were examined with a view to comparing them with human renal tumors of childhood. The rat renal tumors consisted of poorly differentiated, highly mitotic mesenchymal cells frequently attempting to differentiate along fibroblastic and myofibroblastic lineages. Though the host renal tubules were frequently entrapped in these tumors, immature tubules and islands of epithelial cells occasionally were seen to form an integral part of the neoplasms. Rarely, adenomatous areas surrounded by mesenchymal proliferation were observed. None of the tumors had the blastemal component that is the hallmark of nephroblastoma. The rat mesenchymal tumors resembled the human congenital mesoblastic nephroma.     

Solitary Intestinal Fibromatosis with Perinatal Bowel Obstruction

Three newborn male infants presented with bowel obstruction in the first day of life and at surgery were found to have solitary tumors involving the small or large intestine. Histologic examination in each case showed a transmural infiltrative spindle cell lesion having the morphologic features of fibromatosis. Ultrastructural studies in one case revealed the tumor to be composed of myofibroblasts. The patients are all alive and well without recurrences 26 months to 10 years after surgery. Only 3 previous cases of solitary congenital fibromatosis of the intestinal tract have been reported. Some of the other congenital spindle cell tumors cited in the literature under various names have morphologic and biologic similarities to our cases and may in fact be examples of congenital fibromatosis. The appropriate treatment of this unusual lesion is local excision, and the prognosis is excellent.     

Primary testicular and paratesticular tumors of childhood

Testicular and paratesticular neoplasms are uncommon tumors of childhood. Consequently, the experience gained with regard to their optimal management is limited in any given children's cancer centre. Here we review the classification, diagnosis, and staging of testicular and paratesticular neoplasms and subsequently discuss the more frequently occurring ones: germ cell tumors, gonadal stromal tumors, gonadoblastoma, tumors of the supporting tissue, lymphomas and leukemias, tumor-like lesions, secondary tumors, and tumors of the adnexa. © 1994 Wiley-Liss, Inc.     

How to manage an unresectable or recurrent sialoblastoma

Only 2–5% of all salivary gland tumors occur in children. Sialoblastoma is an extremely rare salivary gland tumor diagnosed at birth or shortly thereafter with significant variability in histological range and clinical course, so that it may be difficult to predict the most appropriate therapy. In cases where surgical removal is not curative or technically feasible, chemotherapy may be attempted. We report herein a patient with progression of a huge partially resected sialoblastoma who was successfully treated with chemotherapy. Systemic chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) seems to be an effective adjuvant or neoadjuvant treatment option for unresectable or recurrent sialoblastoma. Pediatr Blood Cancer. 2010;55:374–376. © 2010 Wiley–Liss, Inc.     

Askin's tumor in children: report of two cases

Two cases of Askin's tumor of the thoracopulmonary region in children (a 6-year-old female and a 5-year-old male) are presented. Primary surgery was unfeasible and despite aggressive chemotherapy both died within 304 and 300 days, respectively, from the onset of the disease. Although rare, Askin's tumor should be considered in the differential diagnosis of any thoracopulmonary mass in childhood and adolescence through appropriate histology and immunohistochemistry of incisional biopsies. Its prognosis is poor unless radical surgery is performed as soon as possible: unresectable tumors can be treated with chemotherapy, but prolonged chemotherapy yields potentially lethal complications. We conclude that only early diagnosis and cytogenetic recognition can give children with Askin's tumor a chance of curative surgery.     

Critical review of the management of primary central nervous nongerminomatous germ cell tumors

Multimodal strategies have significantly improved the outcomes for patients with central nervous system nongerminomatous germ cell tumors. Two large cooperative group studies have recently reported much improved outcomes compared with historical series. However, a substantial proportion of patients still attain inadequate responses to initial chemotherapy prior to irradiation, with adverse impact upon survival; optimal induction chemotherapy regimens and radiotherapy strategies are as yet unidentified. Outcomes for patients with relapsed disease remain poor. There is an obvious need to incorporate molecular studies within prospective clinical trials that will likely lead to the incorporation of targeted, more effective future treatment strategies.     

Diagnostic accuracy of neuroimaging in pediatric optic chiasm/sellar/suprasellar tumors

Preoperative diagnosis for tumors arising in the optic chiasm/sellar/suprasellar region in children is helpful to determine surgical necessity and approach, given the high operative risk in this area. We evaluated the ability to differentiate tumor type by preoperative neuroimaging. Thirty‐eight of 53 tumors were correctly diagnosed by neuroimaging based on final pathologic diagnosis (prediction accuracy 72%). Prediction accuracies were 87% (20/23) for craniopharyngioma, 79% (11/14) for optic pathway glioma, 64% (7/11) for germ cell tumor, and 0% (0/5) for Langerhans cell histiocytosis. Diagnosis of optic chiasm/sellar/suprasellar tumors in children by imaging alone should be considered when biopsy is considered high risk.     

TESTICULAR GERM CELL TUMORS IN CHILDHOOD: Treatment Results of 52 Patients

We analysed the treatment results of 52 children with testicular germ cell tumors. Histopathological diagnoses were endodermal sinus tumor (63.4%), embryonal carcinoma (28.8%), teratocarcinoma (5.7%), and mixed tumors (2.1%). Radical inguinal orchiectomy was performed in 42 patients and retroperitoneal lymph node dissection in 10 (3/10 positive). Overall survival rates were: whole group: 71.2%; stage I: 89.7%; II: 68.5%; III: 31.2%; IV: 30% (p =. 001). Five-year overall survival rates were 85.8% and 100% for stage I patients who received chemotherapy or not (p =. 27); BEP regimen: 85.7%; classical VAC: 67.9%; vinblastine + bleomycin: 63.6%. Chemotherapy is not required in stage I. BEP regimen is effective in testicular germ cell tumors.     

Congenital glioblastoma

We describe the case a 2‐day‐old female with congenital glioblastoma. Total resection was followed by adjuvant and high dose chemotherapy, as indicated by the current Italian infant protocol. The child is alive and well 18 months after diagnosis. A review of 67 selected congenital brain tumors showed the mortality rate was 82%. Even though the majority of patients had glioblastoma, only 5/67 had received adjuvant therapy. To ensure optimal outcomes, we recommend total or subtotal surgical resection, followed by adjuvant and high dose chemotherapy. Given the lack specific protocols for congenital brain tumors an international consensus seems to be needed, starting with congenital glioblastoma. Pediatr Blood Cancer 2009;53:124–126. © 2009 Wiley‐Liss, Inc.     

Mandibular and para-mandibular tumors in children

Sixteen cases of mandibular tumors or paramandibular soft tissue tumors with mandibular involvement are reported. These include such rare mandibular tumors or tumor-like conditions as melanotic progonoma, intraosseous haematoma secondary to von Willebrand's disease, post-irradiation osteosarcoma, monostotic eosinophilic granuloma, aneurysmal bone cyst and osseous hemangiopericytoma. Three cases of cherubism, one of fibrous dysplasia or aggressive fibromatosis and one of central giant cell reparative granuloma are also reported. The soft tissue tumors comprise round cell sarcoma, parotid adeno-carcinoma with generalised metastases, embryonal rhabdo-myo-sarcoma, neuro-fibro-sarcoma and congenital cystic hygroma. In all the cases the disease was well advanced when the patient presented for X-ray examination. The specific X-ray diagnosis of mandibular and paramandibular tumors in childhood is more difficult than that of similar tumors in other parts of the body.Presented at the 24-th Annual Meeting of the American Society for Paediatric Radiology, San Francisco, California, USA, March 22–27, 1981; Presented at the 18-th Annual Meeting of the European Society of Paediatric Radiology, Oslo, Norway, May 20–22, 1981 and at the 32-nd Annual General Meeting of the Royal Australasian College of Radiologists, Christchurch, New Zealand, September 20–25, 1981