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.     

Intracranial metastasis in fibrolamellar hepatocellular carcinoma

Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver malignancy in adolescents and young adults. Surgery is the mainstay of therapy for primary and metastatic disease. Most patients relapse, with development of both local and distant metastases. Brain metastases from solid tumors are rare in the pediatric and young adult population. Here, we document three patients with brain metastases from FLHCC, confirmed by histology and molecular characterization of the chimeric fusion DNAJB1–PRKACA, each necessitating neurosurgical intervention. These observations highlight the ability of FLHCC to metastasize to the brain and suggest the need for surveillance neuroimaging for patients with advanced‐stage disease.     

Clinical aspects of the rhabdoid tumor of the kidney: A report of the national wilms' tumor study group

Rhabdoid tumor of the kidney (RTK), originally described as a monophasic sarcomatous variant of Wilms' tumor, is now recognized as a highly malignant, non-Wilms' tumor possibly of neuroectodermal origin. Twenty-one National Wilms' Tumor Study patients with this tumor were treated in the years 1969 through 1978. Mean patient age was 18 months with 16 of the 21 younger than 2 years at diagnosis. Two patients were Stage I, 10 Stage II, 5 Stage III, and 4 Stage IV. One patient only is continuously disease free and another is surviving disease free following excision of bilateral pulmonary metastases. One patient died of sepsis early during therapy. Thus 18 of the 19 patients who relapsed died, 15 within 1 year of diagnosis, all with progressive tumor growth. The rapid appearance of metastases (mean 4 months), often to multiple sites, and short subsequent survival signal a very malignant tumor resistant to current treatment stratagems.     

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.     

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.     

Embryoma (or Embryonal Tumor) of the Parotid Gland: Report of two Cases

Embryoma is the name applied by Vawter and Tefft to congenital tumors of the parotid gland. These tumors are highly uncommon, and they must not be confused with malignant tumors—i.e., cylindroma, embryonal carcinoma, or teratoma. Alone, complete surgical exeresis is necessary without radiation or chemotherapy. Two observations of embryoma are reported because of the diagnostic and therapeutic problems involved.     

Presacral Mesenchymoma: a Case Report

A 4-year-old girl with a presacral benign mesenchymoma is reported. The nature of the tumor was not recognized preoperatively or intraoperatively. There was evidence of pericapsular infiltration of the soft tissues of the pelvis but not signs of malignancy. A follow-up examination after 3 months indicates that the patient has no clinical evidence of recurrence. Review of the English literature shows the rarity of benign mesenchymoma in childhood and the lack of reports of its occurrence in the pelvis.     

Tumor biology,biomarkers, and liquid biopsy in pediatric renal tumors

The expansion of knowledge regarding driver mutations for Wilms tumor (WT) and malignant rhabdoid tumor of the kidney (MRT) and various translocations for other pediatric renal tumors opens up new possibilities for diagnosis and treatment. In addition, there are growing data surrounding prognostic factors that can be used to stratify WT treatment to improve outcomes. Here, we review the molecular landscape of WT and other pediatric renal tumors as well as WT prognostic factors. We also review incorporation of circulating tumor DNA/liquid biopsies to leverage this molecular landscape, with potential use in the future for distinguishing renal tumors at the time of diagnosis and elucidating intratumor heterogeneity, which is not well evaluated with standard biopsies. Incorporation of liquid biopsies will require longitudinal collection of multiple biospecimens. Further preclinical research, identification and validation of biomarkers, molecular studies, and data sharing among investigators are crucial to inform therapeutic strategies that improve patient outcomes.     

Intracardiac teratoma in an infant

Summary We report the only infant known to us who has survived surgery for a primary right ventricular teratoma. Included is a review of other reported cases of teratoma of the heart in infancy and childhood.     

Irinotecan for relapsed Wilms tumor in pediatric patients: SIOP experience and review of the literature—A report from the SIOP Renal Tumor Study Group

While irinotecan has been studied in various pediatric solid tumors, its potential role in Wilms tumor (WT) is less clear. We evaluated response and outcome of irinotecan‐containing regimens in relapsed WT and compared our results to the available literature. Among 14 evaluable patients, one complete response (CR) and two partial responses (PRs) were observed in patients with initial intermediate‐risk (CR and PR) and blastemal‐type histologies (PR). Two patients were alive at last follow‐up showing no evidence of disease. Our results and the reviewed literature suggest some effectiveness of irinotecan in the setting of relapsed WT.     

Inflammatory pseudotumor of the liver masquerading as a metastasis in a child treated for nephroblastoma

A 31/2-year-old boy presented with a palpable hepatic tumor thought on clinical and radiological grounds to be a metastasis but which was found to be an inflammatory pseudotumor on histological examination. Eighteen months previously he had received chemotherapy and radiotherapy for a stage IV Wilms' tumor, which had been surgically excised 4 months after commencing treatment. This case illustrates the importance of obtaining a histological diagnosis in the management of patients with malignant tumors.     

Successful excision of an atrial fibroma in a four-month-old male infant

Summary This report presents an infant who was initially admitted with a pericardial effusion. Subsequent evaluation led to the diagnosis of a primary cardiac tumor. Excision of the tumor which arose from the atrial septum was possible only by removing most of the free right atrial wall and a portion of the septal leaflet of the tricuspid valve. Reconstruction of the tricuspid valve leaflet by reattachment of chordae and a partial annuloplasty was done with the right atrial wall being replaced using pericardial tissue. The histology of the tumor was compatible with a fibroma, a type not previously reported in this location in the pediatric age group. Twenty-one months following operation, the child was restudied and the catheterization revealed mild to moderate tricuspid regurgitation and no recurrence of the tumor. The child is now 4 years of age and remains asymptomatic with normal growth and development and no evidence of fibromatosis.