Head and neck lipoblastomas: Report of 3 cases and review of the literature

IntroductionLipoblastoma is a rare benign tumor arising from embryonic white fat which occurs in the early childhood. It usually arises on the extremities and considered as a rare cause of a pediatric head and neck masses. The aim of this study is to shed light on lipoblastomas as a differential diagnosis of rapidly growing soft fatty masses of children in neck and head area.Patients and methodsA retrospective review of 3 patients with lipoblastoma, underwent Surgical resection (case 1 and 2) by cervical approach. The third patient with a facial lipoblastoma was not operated due to the high risk of facial paralysis. Review of literature, diagnostic methods and genetics of lipomatous tumors are discussed.ResultsComplete surgical excision via a cervical approach demonstrated irregular lobules of immature fat cells separated by a loose, myxoid connective tissue. Histology analysis confirmed the diagnosis of lipoblastoma.DiscussionLipoblastoma is a rare childhood tumor, even rarer in head and neck area. The pathogenesis is unknown, though it is believed to arise from altered embryogenesis of human white fat and genetic predisposition, as chromosome 8 abnormalities may be implicated in the development of lipoblastoma. The presumptive diagnosis is performed by imaging. The most important differential diagnosis of lipoblastoma is myxoid liposarcoma. The mainstay of treatment is complete non-mutilating resection of the tumor to avoid recurrence.ConclusionLipoblastoma should be suspected in case of heterogeneous fatty tumor in head and neck area, and included as a differential diagnosis of cervical masses in children younger than 3 years. The mainstay of treatment is complete surgical excision with a good prognosis.     

Lipoblastomatosis of the neck causing hemiparesis: a case report and review of the literature

BACKGROUND: Lipoblastoma and lipoblastomatosis are rare pediatric adipose tumors that sometimes affect the neck or spinal cord. This case is the third report of lipoblastoma extending into the spinal canal, the first report of intradural tumor extension, and the first report of hemiparesis resulting from lipoblastoma compressing the spinal cord. METHODS: A 13-month-old boy was seen by a pediatrician for a firm, supraclavicular neck mass on the left side. After being evaluated by CT and MRI scanning, the tumor was partially resected. RESULTS: Postoperative microscopic examination of the tumor showed adipose cells with mature nuclei and well-formed fat vacuoles interspersed with fibrovascular septa, a finding consistent with maturing lipoblastoma. CONCLUSIONS: Although lipoblastomatous tumors are treated with complete surgical resection when possible, location of these tumors in the neck may dictate partial resection to avoid intraoperative injury to the spinal cord.     

Hibernomas: clinicopathological features, diagnosis, and treatment of 17 cases

Hibernomas are rare benign adipose tumors composed of brown fat cells with granular, multivacuolated cytoplasm admixed with white adipose tissue. They account for 1.6% of benign lipomatous tumors and approximately 1.1% of all adipocytic tumors. They are more common in the third and fourth decades of life. The most common location is the thigh, followed by the shoulder, back, and head and neck. Four histological types have been reported; abundant vascularity is characteristic, and atypias are rare. The treatment of choice for hibernomas is complete surgical excision. Metastases or malignant transformation have not been reported.This article presents a series of 17 patients with hibernomas diagnosed and treated at our institution from January 1986 to December 2009. Six men and 11 women (M:F, 1:2) had a mean age of 38 years (range, 10 months to 64 years). All patients underwent surgical treatment; 14 patients had marginal and 3 had wide excision. Adjuvants such as radiation therapy, chemotherapy, or embolization were not administered for any patient. The most common symptom was a painless palpable mass, followed by a tender or painful mass; in 2 patients, the tumor was an incidental finding. The duration of symptoms ranged from 1 month to 10 years (mean, 27 months). The most common location was the thigh, followed by the buttock, scapula, and neck. The most common histological variant was the typical variant followed by the lipoma-like variant. At a mean follow-up of 5 years (range, 1-9 years), local recurrences were not observed.     

Giant mesenteric lipoma

Mesenteric lipoma is a rare benign tumor of mature fat cells. Asymptomatic abdominal mass, progressive abdominal distension, and intraperitoneal radiolucent fat density mass on computed tomography are the main diagnostic criteria. Main differential diagnosis is lipoblastoma or lipoblastomosis. Treatment is surgical excision. As an unusual case, a 3-year-old boy with a giant mesenteric lipoma is presented in this report.     

Lipoblastoma

BACKGROUND/PURPOSE: Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat that occurs almost exclusively in infants and children. This study was done to determine the clinical and pathologic characteristics of lipoblastoma. METHODS: Nine cases of pathologically proven lipoblastoma from 1979 to 1997 were reviewed. There were 6 boys and 3 girls ranging in age from 3 months to 29 months. RESULTS: A soft tissue mass was the chief complaint in 7 patients, abdominal distension in 1 patient with a retroperitoneal mass, and defecation difficulty in 1 patient with a perirectal mass. In 4 patients, tumors occurred on the back. Other tumor location includes the neck, scrotum, retroperitoneum, perirectal area, and buttock in 1 patient each. Lesions measured 2.3 to 19.5 cm. Complete excision was done in 8 patients. One perirectal tumor was removed by both the posterior sagittal approach and the intraabdominal approach but incompletely resected. Two tumors located on the back recurred with intraspinal extension 12 months and 18 months after resection. Second resection and second resection with laminectomy were done. Leg pain and urinary incontinence developed in 1 patient but improved on conservative treatment. CONCLUSIONS: Lipoblastoma is a benign neoplasm but can frequently recur (25%) in spite of complete excision. Lipoblastomas occurring on the back had a high recurrence rate (50%) and associated with intraspinal extension. J Pediatr Surg 36:905-907.     

Lipoblastoma of the Back; A Case of a 7-month-old Boy

Lipoblastomatous tumours are rare, benign tumours of embryonic adipose tissue which primarily occur in children younger than 3 years. Most common, these tumours can be found in the extremities, yet cases involving other locations, such as the trunk, face, neck and mediastinum, have been described and they have an excellent prognosis despite its potential to local invasion and rapid growth.

In medical literature two types are known, the focal well circumscribed lipoblastoma, and the more diffusely infiltrating lipoblastomatosis.

The authors report a case of a 7-months old Ethiopian boy with a congenital lipoblastoma on the back. Despite surgical intervention, we were confronted with recurrences.

In conclusion, we stress the morbidity of possible surgical management and the difficulty in decision-making. Therefore, awareness in young children with a fast growing lipomatous mass for this clinical and histopathological entity is crucial.     

Cytogenetic analysis in the diagnosis and management of lipoblastomas: results from a single institution

Background

Lipoblastomas are rare, benign, soft tissue tumors that occur primarily in young children. Treatment includes complete excision and surveillance for recurrence. Lipoblastomas can be indistinguishable from other benign lipomatous tumors and liposarcomas. Cytogenetic analysis can provide the definitive diagnosis in questionable cases, because benign and malignant lipomatous tumors exhibit specific nonrandom cytogenetic abnormalities. The purpose of the present study was to discuss the disease management and outcomes in a large contemporary group of patients with lipoblastoma.

Materials and methods

A retrospective chart review of patients diagnosed with lipoblastoma presenting from 2000–2011 was conducted. The data from these patients were compared with data from a previously published historical group of patients (1985–1999) from the same children's hospital.

Results

We identified 37 patients in the contemporary cohort group and compared them with 25 patients from the historical group. The tumor involvement sites were similar. The current cohort group had a lower recurrence rate, although this might have been underestimated owing to a shorter follow-up period (median 1.4 y, range 2 wk to 11.0 y). Preoperative imaging findings led to an incorrect diagnosis in 62% of the patients. Cytogenetic analysis was used to help determine the final diagnosis in 50% of the cases. In 39% of cases, translocations involved the long arm of chromosome 8, the most common anomaly in lipoblastoma.

Conclusions

Lipoblastomas are rare tumors in young children that can be misclassified as other malignant or benign lipomatous tumors with markedly different outcomes and treatments. We recommend that cytogenetic analysis be routinely used for all pediatric lipomatous tumors to provide an accurate diagnosis and guide appropriate therapy and follow-up.     

Ossifying fibromyxoid tumor of the trunk mimicking hydatid cyst: A case report

IntroductionOssifying fibromyxoid tumor (OFMT) is a rare lesion that generally occurs in the soft tissues of proximal limbs, head or neck and presents as a slowly growing mass. Abdominal or trunk locations are extremely rare.Presentation of caseWe report a case of 50-year-old man who presented with a painless, slow growing epigastric mass for 5 years. Radiologic assessment revealed a well circumscribed median subcutaneous parietal mass lesion present in front of the xiphoid process suspicious of a calcified hydatid cyst. Diagnosis of OFMT was made on histopathological examination of the resected specimen.DiscussionOFMT most often presents as a single swelling arising from the subcutaneous soft tissues or skeletal muscles of the extremities. Multifocal presentation is exceedingly rare. Radiologically, a peripheral shell of bone is seen in more than 50% cases. On MRI, myxofibrous stroma appears isointense to muscle on T1 and of intermediate to high signal intensity on T2. Surgical excision is the mainstay of treatment. Histologically, the tumor has a thick fibrous capsule with a complete or partial underlying layer of metaplastic woven or lamellar bone. Tumor is composed of uniform round, ovoid, or spindle-shaped cells arranged in nests and cords embedded in a variably myxoid and collagenous Alcian blue-positive stroma. On immunochemistry, the tumor cells are positive for S100 protein and desmin in 90% and 50% cases respectively.ConclusionOFMT is a rare soft tissue tumor with malignant potential often misdiagnosed as a benign lesion. Complete surgical excision should be performed to prevent local recurrence.     

Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease

BACKGROUND: Simultaneous malignancies in the field of radiation for Hodgkin's disease is an extremely rare event. A unique case of concurrent thyroid and neck mass in the postirradiation field of a young patient with Hodgkin's disease is presented. METHODS AND RESULTS: Thyroidectomy and excision biopsy of the neck mass were performed. A 1.5-cm papillary thyroid carcinoma was identified in thyroidectomy and an initial diagnosis of undifferentiated malignant neoplasm was rendered on the neck mass biopsy. Subsequent surgical excision of the neck mass and immunohistochemical analysis revealed malignant peripheral nerve sheath tumor. CONCLUSION: Concurrent malignancies in the field of treatment of Hodgkin's disease may occur. Rare malignancies including malignant peripheral nerve sheath tumor may be encountered along with the more common papillary thyroid carcinoma.     

Lipoblastoma arising from the submandibular region

A lipoblastoma is a rare, benign tumor arising from embryonic white fat. The tumors occur primarily in infancy and early childhood and commonly arise from the limbs and the trunk, but neck involvement is extremely rare. Our case arose in a 22-month-old male presenting with a rapidly enlarging soft mass in the right submandibular area. Lipoblastoma was diagnosed by histologic evaluation, the mass was completely removed, and there was no recurrence at 1-year follow-up.     

Acute respiratory distress caused by a giant mediastinal lipoblastoma in a 16-month-old boy

Lipoblastoma is a rare benign tumor arising from embryonic fat; it occurs mainly in the extremities and almost exclusively in infants and children younger than 3 years. We present a case of giant mediastinal lipoblastoma in a 16-month-old boy who presented with acute respiratory distress. The mass was completely excised through a left posterolateral thoracotomy. The postoperative course was uneventful, and the pathologic final diagnosis was lipoblastoma. Although extremely rare, mediastinal lipoblastoma can be life threatening; therefore, it should be included in the differential diagnosis of mediastinal mass in younger subjects.     

Intraskrotales Lipoblastom im Kindesalter

Based on a case report of an intrascrotal lipoblastoma in childhood, we present the preoperative diagnostic algorithm and the main differential diagnoses in testicular and paratesticular tumors as well as their surgical management. An 8-week-old infant was admitted with a scrotal tumor known since birth. MRI showed a pinnate vessel supply, originating in the left internal iliac artery. The tumor was exposed operatively and could be completely removed. Intrascrotal lipoblastoma are rare. Like most testicular and paratesticular tumors in childhood lipoblastomas are benign. Today ultrasound and Doppler sonography are basic tools for diagnosis and surgical planning in testicular and scrotal tumors in childhood. In some cases MRI can provide additional important information for surgical planning. In contrast to adults testis sparing surgery is favoured in children.     

Is surgical treatment of lipoblastoma always necessary?

Lipoblastoma is an uncommon, benign mesenchymal tumor with an excellent prognosis despite its potential to local invasion and rapid growth. However, in the literature, a spontaneous resolution has never been reported, and, consequently, the need for a complete surgical excision has never been questioned. The authors report a case of a 2-day-old boy with congenital diffuse lipoblastoma in the left thigh, which forced us to withhold from surgical treatment to avoid the risk of mutilation in a patient so young. The lesion was followed-up by imaging, and a complete spontaneous resolution of the diffuse lipoblastoma was shown by magnetic resonance imaging (MRI) at 1-year follow-up. In the literature, a complete surgical excision is recommended. The results of this case suggest that a "wait and see" approach is justified at least in infants with huge invasive lesions requiring a mutilating excision.     

Giant lipoma of the thumb

While MRI is recognized to be the gold standard examination to diagnose giant lipomas and exclude liposarcomas, there is insufficient knowledge about how to clarify the degree of malignancy of intermediate lesions. We report here the case of a digital giant lipoma, where MRI showed a benign polylobulated lipomatous tumor, but that presented nuclear atypia on conventional histological examination suggestive for an atypical lipoma or well-differentiated liposarcoma. To exclude such a lipomatous tumor necessitating a more aggressive surgical excision, complementary cytogenetics with fluorescence in situ hybridization study for MDM2 and CDK4 genes was required. A literature review of the diagnostic approach for lipomatous tumors of the extremities, including MR images, histological examination, and new cytogenetic techniques, is performed. Level of Evidence: Level V, diagnostic study.     

Multiple intramedullary lipomas with conal intramedullary dermoid: magnetic resonance appearances.

BACKGROUND CONTEXT: Intramedullary fat-containing benign childhood tumors of the cord include lipomas, dermoid cysts, and teratomas. These are embryonal tumors. Most intramedullary fat-containing tumors are solitary. Multiple intramedullary lipomas are rare and may represent a spinal lipomatous malformation. The presence of another intramedullary dermoid tumor in the same case is rare. PURPOSE: The intent of this case report is to look at magnetic resonance features and possible mechanisms of association of these fat-containing intramedullary tumors. STUDY DESIGN/SETTING: A 3-year-old male child presented with spastic quadriplegia. METHODS: Magnetic resonance imaging (MRI) of spine was done on a 1.5-T scanner in different planes. RESULTS: MRI showed multiple intramedullary spinal lipomas with an intramedullary dermoid involving the conus, cord atrophy, and subarachnoid fat droplets. CONCLUSION: Multiple intramedullary lipomas with an intramedullary dermoid represent a form of spinal lipomatous malformation. Both may represent embryogenic mesenchymal inclusions and hamartomatous growth, which can be accurately diagnosed with MRI.     

Diagnostic and therapeutic approaches to carotid body tumours: report of three cases and review of the literature

BACKGROUND: Carotid body tumour is a rare neoplasm of the carotid body. Three cases of carotid body tumour presenting as a painless progressive mass in the neck region are reported here. A review of the relevant literature regarding carotid body tumours is also presented. METHODS: Angiographic features were diagnostic of carotid body tumour and complete surgical excision was done. RESULTS: There was no mortality and minimum morbidity. There were no malignant tumours. All three patients belong to the high-altitude area of Himachal Pradesh. CONCLUSIONS: A high degree of clinical suspicion of upper posterior triangle neck masses and an accurate diagnostic work-up are needed for operative planning.     

Gigantic thymolipoma

A 48-year-old woman with an abnormal shadow in chest radiography during an annual physical examination was found by chest computed tomography to have a large fatty mass lesion found to be diagnosed as a gigantic lipoma. Histopathological diagnosis was found to be benign thymolipoma consisting of mature fatty tissue and hyperplastic thymic tissue structures with Hassall,s corpuscles. Although the diagnosis is supported by imaging studies that demonstrate fat and soft tissue within the tumor, variations occur in computed tomography appearance. We suggest that surgical excision be considered when a gigantic intrathoracic lipomatous mass is in scanning as in this case.     

Gigantic thymolipoma

A 48-year-old woman with an abnormal shadow in chest radiography during an annual physical examination was found by chest computed tomography to have a large faty mass lesion found to be diagnosed as a gigantic lipoma. Histopathological diagnosis was found to be benign thymolipoma consisting of mature fatty tissue and hyperplastic thymic tissue structures with Hassall,s corpuscles. Although the diagnosis is supported by imaging studies that demonstrate fat and soft tissue within the tumor, variations occur in computed tomography appearance. We suggest that surgical excision be considered when a gigantic intrathoracic lipomatous mass is in scanning as in this case.     

Lipoblastoma--a rare paediatric foot tumour

Lipoblastoma, a rare benign tumour arising from embryonic fat, is usually found in areas of abundant adipose tissue. Various reports describe a predilection of lipoblastoma for sites with primitive adipose tissue such as axilla, neck, retroperitoneum and prevertebral soft tissue. The plantar aspect of the foot is an extremely rare site due to scarcity of fatty tissue. Differential diagnosis includes lipomas, fibromyxolipomas and liposarcomas. Age of presentation, chromosomal markers and histopathological examination help in arriving at final diagnosis. Radical surgeries are not advocated; however, complete excision is necessary to avoid recurrence.     

Lipoblastoma with unique localization requiring tracheal and esophageal resection

Lipoblastoma and lipoblastomatosis are rare tumors of infancy. They originate from embryonic fat and localize in soft tissues. We present the case of a lipoblastoma of the neck with localization in tracheal and esophageal walls that required an extended laryngotracheal and esophageal resection. To our knowledge, this is the first report of such localization of this tumor.