Lipoblastoma and lipoblastomatosis in children.

Lipoblastoma and lipoblastomatosis are rare mesenchymal benign tumors of embryonal white fat. They are found primarily in children less than 5 years of age. This tumor presents in two forms: a localized well-circumscribed lesion (lipoblastoma), or a multicentric type (lipoblastomatosis). The authors presents three cases, two of them with a buttock lesion, the last with a paravertebral one. It is recommended a complete but conservative excision of the tumor because there is a natural tendency to involution, although in the first year of life a local reexcision for recurrence is sometimes described.     

A rare case of inguinolabial lipoblastoma in a 13-month-old female

Lipoblastoma is a rare, rapidly growing, benign mesenchymal tumor composed of various stages of maturing adipocytes that most often occurs in children under the age of 3. The common locations are the extremities and the trunk. Presentation in the genitoinguinal area is rare. We report a case of a 13-month-old female infant with a 4-month history of a progressively enlarging left labial mass that encompassed her left labium majora and inguinal region. Pelvic MRI confirmed growth from previous ultrasound size of 3 × 2 × 1 cm to 7 × 2 × 2 cm. Composition was suggestive of adipose tissue. The mass was excised through a left inguinal incision. The final pathology results described a lipoblastoma. Six year follow-up has not revealed any signs or symptoms of recurrence.Circumscribed lipoblastomas should be distinguished from their infiltrative counterpart, diffuse lipoblastoma or lipoblastomatosis, which can be more difficult to excise and thus, more likely to recur. Lipoblastoma should also be distinguished from myxoid liposarcoma, which has malignant features, carries a high risk of recurrence, and requires a more aggressive management protocol. Although rare, lipoblastoma should be considered as part of the differential diagnosis of a rapidly growing vulvar mass in prepubertal children.     

Congenital minor salivary gland sialoblastoma: a case report and review of the literature

Sialoblastoma is the most common epithelial tumor of the salivary gland. We report a case of congenital sialoblastoma arising in a minor salivary gland of the buccal mucosa of a male infant. After radiologic evaluation, an incisional biopsy was performed and then the mass was excised en bloc. Histologic features were both favorable and unfavorable. However, there was no recurrence for 5 months. In spite of a reported histologic grading system, the clinical course of isolated sialoblastoma is considered unpredictable. More published case reports of this rare tumor may enable histologic and clinical correlation in order to accurately predict prognosis.     

Magnetic resonance appearance in a case of lipoblastomatosis

Lipoblastoma-lipoblastomatosis represents a rare, benign tumorous proliferation of adipocytes and their mesenchymal precursor cells. Seen almost exclusively in early childhood, it should be considered in the differential diagnosis of any rapidly expanding soft-tissue mass. We present a case of lipoblastomatosis and its magnetic resonance appearance, which has not been previously described.     

Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature.

Lipoblastoma and lipoblastomatosis are rare benign neoplasms of foetal white fat tissue that occur almost exclusively in infants and children. Two cases are reported and a review of the literature is given. An almost one-year-old girl and a full-term male infant were brought to our hospital with a solid mass in the right thigh. Nine months after total excision of the lipoblastoma, the little girl developed tumour recurrence, with unexpected histopathological maturation. In the case of the little boy, lipoblastomatosis with infiltration of the surrounding muscles, together with involvement of the sciatic and posterior femoral cutaneous nerves was found. Histologically, the tumour showed an unusual inflammatory reaction.The diagnosis of this tumour was made by the pathologist, but the histopathological picture bears a striking similarity to myxoid liposarcoma, and may be indistinguishable. Recent studies describe rearrangements of chromosome 8 q11-q13 region as a new discriminative marker that distinguishes lipoblastoma and lipoblastomatosis from myxoid liposarcoma.     

Benign lipoblastoma arising in the neck

Lipoblastoma and lipoblastomatosis are rare benign tumours of embryonal fat that exhibit a tendency to invade locally but not to metastasise. This condition most often presents before the age of 3 years, affects males more than females, and is typified by a slowly-growing, usually subcutaneous mass arising in a limb. Cases involving the head and neck are extremely rare, with only nine cases in the neck having been described to date. This tenth case arose in a 13-month-old male presenting with a rapidly enlarging lump in the right side of the neck, clinically mimicking a cystic hygroma. Diagnosis is by histology, and treatment involves complete surgical resection. We report this tenth known case of lipoblastoma in the neck and review the literature. Accepted: 4 February 1997     

Chest wall lipoblastomatosis in a 2-year-old girl: a case report and literature review

Lipoblastoma is an uncommon tumor of adipose tissue that usually occurs in infancy and early childhood. They predominantly locate in the extremities, but are less frequently found in the head and neck region, trunk and various other locations. The most common sign is a rapidly growing mass. In spite of their potential for local invasion and rapid growth, they are benign tumors and have no distant metastasis. Although more than 180 cases of lipoblastoma distributed over various parts of the body have been reported, only nine cases were located in the thoracic wall. We present the case of a two-year-old girl with lipoblastomatosis of the chest wall that was identified by histologic examination. In this article, the English literature on this disorder is briefly reviewed.     

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.     

A thoracic wall lipoblastoma in a 3-month-old infant: A case report and review of the literature

Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infancy and early childhood. Lipoblastoma is present in 2 forms which are pathologically identical: circumscribed and diffuse. It is typically located in the extremities, and less frequently in head-neck region, trunk, and various organs. Lipoblastoma is a tumor with good prognosis with no reported metastases, despite its potential for local invasion and rapid growth. Our patient was a 3-month-old boy who was brought to our clinic for rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the lipomatous mass was removed by local resection. In addition to the patient's age, histologic and cytogenetic analyses assisted the diagnosis of diffuse lipoblastoma. In the postoperative period, the thorax wall was unaffected, and after 6 years of follow-up no recurrence was observed. In the English literature, 8 cases of thoracic wall lipoblastoma have been previously reported, and only 3 of diffuse form. Here, we report, at our knowledge, the fourth case of thoracic diffuse lipoblastoma, in which cytogenetic analysis showed a previously undescribed karyotype aberration involving chromosomes 8, 13, and 16.     

Liposarcoma of hepatic hilum in childhood: report of a case and review of the literature

Liposarcoma in childhood has seldom been documented. The rare occurrence of this tumor has resulted in a lack of information about its natural history, prognosis, and management. The differential diagnosis with lipoblastomatosis is obligatory. A 2 year 4 month-old white male child presented at the Hospital das Clinicas of Medical School of Ribeir?o Preto, University of S?o Paulo, with fever, jaundice, and coluria secondary to a tumoral mass of the hepatic hilum causing obstruction of the biliary tree. Histologic and immunohistochemical study at autopsy disclosed a liposarcoma. A literature review found 64 other reported cases. Special attention was given to age, sex, anatomic localization, and histologic aspects of the literature cases. Liposarcoma involving the hepatic hilum and causing obstruction of the biliary tree in childhood was not previously reported in the literature.     

Clinical and pathologic considerations in a case of inflammatory myofibroblastic tumor of the spleen

Inflammatory myofibroblastic tumors, also called inflammatory pseudotumors, are rare pseudosarcomatous proliferations. Their behavior is generally not aggressive, but in most instances they can simulate malignant neoplasms, from which they are impossible to distinguish before excision. One case of myofibroblastic tumor of the spleen in a 5-year-old boy is described: the patient was treated with a partial splenectomy, which was found to be the best approach for diagnosis and treatment. The purpose of this report is to call attention to this entity with peculiar histologic and immunohistochemical characteristics, which has to be included in the differential diagnosis of splenic lesions and may be excised by a nonaggressive procedure.     

Lipoblastoma/lipoblastomatosis: a clinicopathologic study of 16 cases in Taiwan

Lipoblastoma/lipoblastomatosis is an uncommon benign lipomatous tumor affecting mainly infants and children. It presents as a painless mass located frequently in upper and lower extremities. The present study was conducted to characterize the clinicopathologic features of lipoblastoma/lipoblastomatosis in a Chinese population. A retrospective survey of 16 cases of pathologically confirmed lipoblastoma/lipoblastomatosis from 1988 to 2002 was performed. The available clinical information and histological sections were reviewed. The study involved 10 males and 6 females ranging in age from 5 to 49 months (median, 11.5 months). The patients presented with mass involving neck (n=5), inguina (n=3), feet (n=2), arm (n=1), leg (n=1), thorax (n=1), mesentery (n=1), buttock (n=1), and presacral region(n=1). The lesions were removed surgically. Histologically, the majority (11/16) of primary tumors were of diffuse type. Follow-up ranging from 5 to 125 months was available in 13 patients. Four patients experienced a recurrence of tumors in the neck (n=2) and lower extremities (n=2) 11–84 months after operation. The most common site of involvement of lipoblastoma/lipoblastomatosis in our series was the neck. A follow-up period of more than 3 years is recommended for patients with this condition.     

Leiomyoma of the esophagus in children - case report and review of the literature

The leiomyoma of the esophagus is very rare in children, contrary to what has been observed in adults, with only 26 cases reported up to now. In this paper we present the case of a 9 year-old child in which the tumor was located in the abdominal esophagus, being progressive dysphagia its most important manifestation. Diagnosis was done during the surgical procedure, and the treatment involved sub-total esophagotomy and pyloroplasty, with ulterior esophageal substitution for a colon segment. The only post-surgical complication was dumping which resulted from the pyloroplasty. Its symptoms receded with appropriate diet. The review of the literature shows that leiomyomas in children may be diffuse or located, the former being the more common case (90%). Twelve children presented association with Alport's syndrome (nephropathy with hematuria, deafness and cataract) while others had hiatus hernia, esophageal peptic ulcer and cholelithiasis. In two children the tumor affected the tracheo-bronchial tree, and in four girls there was vulvar or perianal hypertrophy. Finally, there is a rarer kind of tumor in the esophageal muscular layer called benign idiopathic hypertrophy, which has been described for six children. Its basic histological characteristic is non neoplastic hyperplasia of the cells of the internal muscular layer. We conclude that the leiomyomas of the esophagus, although rare, must be considered in the differential diagnosis of the mediastinal mass and the esophageal stenosis in children.     

儿童脂肪母细胞瘤病3例报告并文献复习

目的探讨脂肪母细胞瘤病的临床特点及诊疗方式。方法回顾性分析2011年12月至2014年4月在本院住院治疗的3例脂肪母细胞瘤病患儿的临床资料,3例均为男性,年龄分别为1岁3个月,4岁6个月,12岁1个月,平均5岁11个月。患儿均以无痛性肿物就诊。结果 3例患儿均行手术治疗,1例接受2次手术。术后病理诊断脂肪母细胞瘤病。随访平均约1年,无死亡病例,1例未复发,2例仍有残留或者复发。结论脂肪母细胞瘤病多表现为无痛性肿物,治疗为手术切除,但手术难切除干净,复发率很高,需要长期随访。     

Pulmonary cystic images as a presentation of a pleuropulmonary blastoma]

A five-month-old infant presented with a sudden choking cough and mild dyspnea. Bilateral cystic pulmonary images were found on a chest X-ray, leading to exploratory surgery. A pleuropulmonary blastoma, a rare malignant embryonic tumor of childhood, was diagnosed on histologic examination. This case illustrates the necessity of performing surgical resection and histologic evaluation of any pulmonary cystic malformations, particularly when bilateral.     

Lipoblastoma of the neck

Lipoblastoma and lipoblastomatosis are benign tumours arising from embryonal fat cells. These rare tumours essentially occur in infancy and early childhood. The males are affected more than females often in the ratio of 3∶1. These tumours usually arise in extremities. The involvement of neck is rare with only 10 cases reported so far in English literature. We herein report lipoblastoma of the neck in a 3 year old girl.     

Giant abdomino-pelvic adipose tumors of childhood

Adipose tumors are rare in pediatric age and are commonly represented by benign forms: lipoma and lipoblastoma/lipoblastomatosis. Generally localized in subcutaneous tissue of the trunk and extremities, they can exceptionally occur as giant intra-peritoneal or pelvic masses. These tumors could reach considerable size prior to diagnosis since they are asymptomatic. The authors report a case series of three giant abdomino-pelvic adipose tumors.     

Congenital hemangiopericytoma: two case reports

Congenital hemangiopericytoma is a rare tumor consisting of capillaries surrounded by pericytes. Only histologically can the tumor be distinguished from other vascular hamartomas. For a long time the congenital type was considered benign; however, metastases have recently been described. A histologic diagnosis of all soft-tissue sarcomas should be made. We describe two patients with congenital hemangiopericytomas in whom the diagnosis was not suspected until after histologic examination. Long-term follow-up is indicated. Accepted: 4 February 1997     

ALK-positive diffuse large B-cell lymphoma

Anaplastic lymphoma kinase (ALK) positive diffuse large B-cell lymphoma (DLBCL) is a rare subtype of non-Hodgkins lymphoma. Five such cases have been described in children. We present a 9-year-old boy, in whom diagnosis of DLBCL has been established in addition to congenital multiple enchondromatosis. Immunohistopathological evaluation of tumor biopsy established the final diagnosis of ALK + DLBCL. The clathrin gene (CLTC)-ALK fusion underlying aberrant expression of ALK in the present case was demonstrated by interphase fluorescence in situ hybridization (FISH) using break-apart rearrangement probes for ALK and CLTC. The disease in this patient was highly resistant to applied chemotherapy regimens and to radiotherapy. Analysis of the disease course in our patient and review of other cases reported previously show that ALK + DLBCL can be an aggressive malignancy that can be cured with conventional chemotherapy protocols only at stage of localized disease.     

Congenital epulis

Epulis is a rare tumor of the newborn, also known as granular cell tumor of the newborn or Neumann's tumor. This tumor arises from the mucosa of the gingiva, most commonly from the anterior part of the maxillary alveolar ridge, and is typically seen as a mass protruding out of the newborn child's mouth, which may interfere with respiration or feeding. Epulis is seen only in the newborn and is a different entity from other granular cell tumors. The tumor has a marked female preponderance of 8:1. The recommended treatment is prompt surgical resection. Recurrences of the tumor and damage to future dentition have not been reported, suggesting that radical excision is not warranted. A newborn female with such a mass is described. The tumor was resected using a carbon dioxide laser; the postoperative course was uneventful. On histologic examination, it was composed of diffuse sheets and clusters of polygonal cells containing small round to oval nuclei and abundant coarsely granular cytoplasm. The tumor cells stained positive for vimentin, and negative for S100-protein, actin, desmin, laminin, keratin, estrogen, and progesterone receptors. Electron microscopic examination showed granular cells containing heterogeneous electron-dense granules, lysosomes, and cytoplasmic lipid droplets. The clinical and microscopic features of such tumors are reviewed.