Melanotic Neuroectodermal Tumor of Infancy

Melanotic neuroectodermal tumor of infancy is a specific but unusual tumor of infancy for which only sporadic cases have been reported in the literature. This paper presents a case in an infrequent site, the epididymis, and summarizes the literature on the subject.     

Ossifying renal tumor of infancy

A renal ossifying tumor of infancy is a rare event with few cases having been published, and the etiology has not yet been established. We report on two new cases of this unusual neoplasm. A 2-year-old boy presented with intermittent painless gross hematuria. After several diagnostic procedures, an open pyelolithotomy was performed and the histological diagnosis of renal tumor of infancy was finally made. The history of the second case is very similar. An 8-week-old infant presented with gross hematuria. As in the first case, an open pyelolithotomy was performed and a tumor entirely covered with blood clots was found in the renal pelvis and completely removed. A histological diagnosis of renal ossifying tumor of infancy was made. Using the literature available, the histological criteria and biological behavior are discussed, together with the diagnostic and therapeutic algorithm for this tumor. In infants with gross hematuria and a calcified (non-)invasive mass in the pelvi-calceal system, renal ossifying tumor should be considered in the differential diagnosis. MRI or CT scan offers a good diagnostic guide.     

Melanotic neuroectodermal tumor of infancy in the femur: a case report and review of the literature

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon neoplasm that most often affects the maxilla in the first year of life. MNTI occurring in the long bones is extremely rare, with only 2 cases reported in the medical literature. Here we report a case of MNTI in the right femur of a 5-month-old infant who underwent neoadjuvant chemotherapy followed by limb-salvage surgery.     

Perinatal neuroblastoma of the pancreas

Neuroblastoma is the most common solid tumor in infancy. Arising from the neural crest these tumors are usually located along the sympathetic chain from the neck to the pelvis and in the adrenal medulla. We report the case of a 3-week-old boy presenting with recurrent episodes of colicky pain. After ultrasound examination, magnetic resonance imaging and laboratory data a pancreatic neuroblastoma was suspected. Tumor resection via distal pancreatectomy and histologic investigation confirmed the diagnosis. Surgery is the treatment of choice in children with pancreatic masses, and is usually well tolerated even in the neonatal period. Conclusive diagnosis can be frequently established only by tumor resection or biopsy. Pancreatic neuroblastoma is an extremely rare tumor with only a few cases described in literature.     

Melanotic Neuroectodermal Tumor of Infancy: A Molecular Genetic Study

Melanotic neuroectodermal tumor of infancy is a rare but well-recognized entity in pediatric pathology. However, the relationship of this tumor to other pediatric small cell tumors with neuroectodermal features (such as neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, and desmoplastic small round cell tumor) is undetermined. Molecular genetic studies of melanotic neuroectodermal tumor of infancy have not been reported. We studied three typical cases of melanotic neuroectodermal tumor of infancy in an attempt to link this tumor to other small cell tumors with well-characterized molecular genetic changes. Tests performed included: detection of MYCN gene amplification and deletion of 1p (all 3 cases), and presence of the t(11;22)(q24;q12) and the t(11;22)(p13;q12) translocations (2 of 3 cases). None of these tests yielded positive results. Thus, there is no genetic basis at present to link melanotic neuroectodermal tumor of infancy to neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, or desmoplastic small round cell tumor. Received June 30, 1997; accepted September 19, 1997.     

Chemosensitivity in pediatric dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of fibrohistiocytic spectrum and uncommonly presents in infancy and childhood. The standard treatment is wide-margin surgical excision, but this is difficult to achieve in large tumors and may result in unacceptable cosmetic, structural, and functional consequences. The authors describe a child with recurring DFSP who, after extensive surgery, responded to treatment with vinblastine and oral methotrexate. This is the first reported case in literature of a chemotherapeutic response in pediatric DFSP.     

Chemotherapy increases amenability of surgical resection in congenital glioblastoma

Brain tumors presenting in infancy, especially during the first 6 months of life, are often very large and highly vascular. It is generally accepted that gross total resection of the tumor affords the best outcome to the patient. However, tumor resection is frequently very challenging due to the?risk of significant bleeding. We report two cases of congenital glioblastoma whose initial surgery was hampered by tumor hypervascularity and excessive blood loss, resulting in subtotal resection. Subsequent carboplatin-based chemotherapy led to a significant reduction in tumor size and vascularity, enabling safe gross total resection at second-look surgery. Based on these findings and a review of the literature, we recommend cytoreductive chemotherapy following diagnostic biopsy for infants presenting with large, highly vascular tumors, such as congenital glioblastoma, in lieu of aggressive upfront surgery, to increase the feasibility and facilitate safe gross total excision at second-look surgery.     

Cervical teratomas. Case report and review (author's transl)]

The finding of a cervical teratoma in a newborn gave rise to a new review of the 131 cases in infancy described in the literature. 13 of the 29 cases reported since 1966 were found in newborns, and a total of 25 cervical teratomas by the end of the first year. The clinical differential diagnosis against other cervical tumors is difficult. With one exception, all teratomas were benign. Because of dangerous respiratory complications and the increasing risk of malign degeneration with age, this tumor has to be removed as early as possible.     

Chemotherapy Increases Amenability of Surgical Resection in Congenital Glioblastoma

Brain tumors presenting in infancy, especially during the first 6 months of life, are often very large and highly vascular. It is generally accepted that gross total resection of the tumor affords the best outcome to the patient. However, tumor resection is frequently very challenging due to the risk of significant bleeding. We report two cases of congenital glioblastoma whose initial surgery was hampered by tumor hypervascularity and excessive blood loss, resulting in subtotal resection. Subsequent carboplatin-based chemotherapy led to a significant reduction in tumor size and vascularity, enabling safe gross total resection at second-look surgery. Based on these findings and a review of the literature, we recommend cytoreductive chemotherapy following diagnostic biopsy for infants presenting with large, highly vascular tumors, such as congenital glioblastoma, in lieu of aggressive upfront surgery, to increase the feasibility and facilitate safe gross total excision at second-look surgery.     

Melanotic neuroectodermal tumor of infancy: report of a case with myogenic differentiation.

An 8-month-old boy presented with a 6-week history of a skull mass of the anterior fontanelle. The mass was excised, and the histopathologic features were diagnostic for melanotic neuroectodermal tumor of infancy. The tumor showed focal myogenin positivity, which has not been previously reported in this tumor. The patient has no evidence of recurrent tumor 10 months after the excision. No adjuvant therapy was given. In addition, we stained a case of melanotic neuroectodermal tumor of infancy obtained from Columbus Children's Hospital; focal myogenin positivity was present.     

Ossifying renal tumor of infancy presenting as a palpable abdominal mass

We report a case of ossifying renal tumor of infancy, which presented as a palpable abdominal mass in an otherwise asymptomatic 10-month-old girl. The tumor was partially calcified and occupied the renal pelvis, causing severe hydronephrosis. The differential diagnosis for a patient this age included Wilms tumor, extra-adrenal neuroblastoma, infection, calculus, calcified hematoma and ossifying renal tumor of infancy. The child underwent heminephrectomy and is currently doing well. Received: 15 October 1997 Accepted: 31 December 1997     

Immature gastric teratoma

Gastric teratoma, a very rare tumor of infancy is essentially benign. Immature (embryonic) elements have been described in only three of the sixty-six cases described previously. This report concerns with one“immature” gastric teratoma seen in infancy.     

Ossifying renal tumor of infancy: findings at ultrasound,CT and MRI

A 4-month-old boy presented with persistent gross hematuria. At ultrasonography, a 3.5-cm echogenic mass with posterior shadowing and tumor vascularity was detected within the right renal pelvis. Precontrast CT showed a slightly hyperattenuating mass in the renal pelvis. At MRI the mass was heterogeneously hypointense on T2-weighted images and isointense on T1-weighted images. Contrast-enhanced CT and MRI both revealed peripheral enhancement of the mass. A histological diagnosis of ossifying renal tumor of infancy was made after open pyelostomy and tumor enucleation. We suggest that ossifying renal tumor of infancy should be considered when a mass with posterior acoustic shadowing and tumor vascularity on US, hyperattenuation on precontrast CT and hypointensity on T2-weighted MRI is seen within the renal pelvis of an infant with hematuria.     

Melanotic neuroectodermal tumor of infancy: clinical, radiological, and pathological features

We present a case of a 4-month-old female infant with a maxillary melanotic neuroectodermal tumor of infancy (MNTI) and review the pooled data from previous publications on this entity. The literature to date comprises 378 reported cases from 1918 to the present, from which data on the presence or absence of metastatic disease was available in 311, and on the presence or absence of local recurrence in 165. These pooled data suggest a local recurrence rate of 36% with metastasis occurring in 7% of cases. At present, the optimal management includes complete surgical excision with clear margins, but there are no reliable histopathological or molecular features to predict the biological behavior in individual cases.     

The management of benign renal tumors of infancy. Case report of a congenital mesoblastic nephroma (leiomyomatous hamartoma).

A case of a benign renal tumor found in an infant of 11/2 months is reported. Its clinical, radiological and operative findings were similar to those described previously as Wilms' tumors. Only pathological examination revealed its benignancy. In view of the occurrence of benign congenital mesoblastic nephroma which is evidently not rare in infancy, and because of the side effects of adjuvant cytotoxic and X-ray therapy, the authors feel that the immediate therapeutic approach to a solid intrarenal tumor in infancy should be surgical only. Further therapy should depend on pathological findings.     

Congenital mesoblastic nephroma 50 years after its recognition: A narrative review

Congenital mesoblastic nephroma (CMN) is a rare pediatric renal tumor with low malignant potential that most commonly occurs early in infancy. Treatment strategies are based on the few published CMN series, while a significant number of CMN patients have been described in case reports. The aim of this narrative review was to create an up‐to‐date overview of the literature. Complete surgical removal is curative in most cases. The risk of treatment‐related mortality (both surgery‐ and chemotherapy‐related) is relatively high in the first weeks of life, indicating that these young patients deserve special attention with respect to timing and type of treatment.     

Infantile chest hamartoma--case outcome aged 11.

Chest wall hamartoma of infancy is a rare lesion, usually presenting in the first year of life. Recent literature has recommended conservative management of asymptomatic children, yet most continue to undergo surgical resection irrespective of their symptom status. We report a case of spontaneous regression of a chest wall hamartoma of infancy, supporting recommendations for conservative management in asymptomatic children.     

Infantile chest hamartoma--case outcome aged 11

Chest wall hamartoma of infancy is a rare lesion, usually presenting in the first year of life. Recent literature has recommended conservative management of asymptomatic children, yet most continue to undergo surgical resection irrespective of their symptom status. We report a case of spontaneous regression of a chest wall hamartoma of infancy, supporting recommendations for conservative management in asymptomatic children.     

Long‐term outcome for kaposiform hemangioendothelioma: A report of two cases

Kaposiform hemangioendothelioma (KHE) is a rare aggressive vascular tumor of skin and deep soft tissues that typically presents in infancy and may be associated with a potentially life‐threatening coagulopathy known as Kasabach–Merrit phenomenon (KMP). Recent advances in medical therapy have successfully treated many patients. However, our knowledge regarding the natural history of these lesions and optimum surveillance strategies remains rudimentary. We report two young women who had KHE with KMP treated in infancy and presented in adolescence with comorbidities related to their KHE tumor. This presentation supports the need for long‐term surveillance in these patients.     

Melanotic neuroectodermal tumor of infancy

Melanotic neuroectodermal tumor of infancy is an uncommon neoplasm occurring primarily in the child one year or less in age [1]. Difficulty in deciding the cellular origin of this tumor has led to numerous names, including congenital melanocarcinoma, melanotic epithelial odontoma, melanotic ameloblastoma, and retinal anlage tumor, to list a few [2]. Electron microscopy and histochemical studies, however, have now established the neural crest as the most likely origin [2]. The most frequent site of occurrence is the maxilla followed by the skull, the brain and the mandible. The genital organs are the most frequent extracranial site [1]. Within the skull, there is a predilection for the anterior fontanel. The following is a case report of a young child with melanotic neuroectodermal tumor of infancy arising at the anterior fontanel. Included is a discussion of magnetic resonance (MR) findings, which to our knowledge, have not been previously reported in this tumor.