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.     

Primary Cardiac Lipoblastoma

Lipoblastoma is a benign adipose tumor in children that has been described in various anatomic locations, most commonly the extremities. We describe the case of a 17-month-old boy diagnosed with cardiac lipoblastoma, a previously unreported primary cardiac tumor in children. Our patient presented with symptoms of coughing, wheezing, and hoarseness and was found to have a large mediastinal mass, which narrowed the left mainstem bronchus and compressed the right atrium and superior vena cava, causing superior vena cava syndrome. Surgical exploration revealed an intrapericardial soft tissue mass arising from the area of the posterior interatrial septum. Grossly, the resected mass was lobulated, pale yellow, and fatty with focal areas of gray myxoid tissue. Microscopically, the tumor consisted of both immature and mature adipocytes, with focal vascular myxoid areas containing lipoblasts, diagnostic of lipoblastoma. Two months after surgery, the patient was in good health without evidence of recurrence. Received April 13, 2000; accepted August 17, 2000.     

Lipoblastoma: MRI appearances of a rare paediatric soft tissue tumour

Lipoblastoma is a rare, benign soft-tissue tumour derived from embryonic fat. Four patients with tumours located in the upper limb are reported, with special reference to imaging techniques and histology. Radical surgical excision is essential to prevent local recurrence and exact imaging techniques are thus crucial. MRI appears to be a reliable preoperative investigation and is the recommended radiological examination. In a child under 3 months of age, images showing a predominantly fatty but inhomogeneous soft-tissue mass are suggestive of lipoblastoma.     

Radiological–pathological correlation in lipoblastoma and lipoblastomatosis

Background Lipoblastoma and lipoblastomatosis are uncommon benign mesenchymal lesions that predominantly occur in infancy and early childhood.Objective To evaluate the imaging and histological features of lipoblastoma and lipoblastomatosis.Materials and methods Retrospective review of the radiological and pathological findings in children with lipoblastoma and lipoblastomatosis treated at a single centre between 1997 and 2004.Results Eight children (median age 18 months) had undergone imaging and surgery at our institution. An infiltrative growth pattern was identified at imaging in two children with lipoblastomatosis, and a well-defined mass in six children with lipoblastoma. In all patients, imaging showed a lesion composed mostly, but not entirely, of fat. There were no recurrences at follow-up of between 1 and 91 months.Conclusion In infancy and early childhood, the identification of a tumour composed mostly of fat should suggest the diagnosis of lipoblastoma or lipoblastomatosis.     

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.     

Rearrangement of HMGA2 in a case of infantile lipoblastoma without Plag1 alteration

Lipoblastoma is a rare benign adipocytic tumor that occurs usually in children. It can be difficult to distinguish a lipoblastoma from other lipogenic tumors. In such cases, the detection of a rearrangement of the PLAG1 gene by fluorescence in situ hybridization analysis is useful for characterizing a lipoblastoma. We present here a novel case of morphological infantile lipoblastoma showing a rearrangement of HMGA2 instead of the classical PLAG1 alteration. HMGA2 is the main target of clonal aberrations encountered in lipomas. This result supports the hypothesis that benign lipomatous tumors harboring PLAG1 or HMGA2 rearrangement could constitute a unique pathogenetic entity.     

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.     

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     

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.     

Characteristics of laparoscopic inguinal hernia recurrences

Purpose  This is so far the largest series of recurrences after laparoscopic inguinal hernia repair. Methods   Video documents of 1,071 laparoscopic inguinal hernia repairs were retrospectively studied with respect to the affected side, anatomical appearance, gender, history and time interval between operation and recurrence. Only indirect, clinically manifest hernias were included. Results  Recurrences occurred in 32 children (3%), 26 boys and 6 girls, aged 62 days to 14 years (median 3). The right side was affected in 21 children, the left in 10 and a bilateral recurrence was noted in 1 child. A total of 25 recurrences occurred medially to the previous suture and 7 laterally. The knot became loose in three cases. An experienced surgeon had only half the recurrences of a less experienced surgeon. The median time interval between surgery and recurrence was 3.8 months. Children up to the age of 2 years had the highest risk of recurrence. The right/left incidence of recurrences roughly was in proportion to the statistical incidence of hernias. Conclusions  Boys had more recurrences than girls. Most recurrences occur medially. The more experienced the surgeon was, the fewer recurrences he had. The stitches at the medial aspect of the hernia, close to the vas, seem to be the most crucial ones.     

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.     

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.     

Analysis of clinical features of lipoblastoma in children

Objective: A lipoblastoma is pathologically benign but often recurs. Due to its rarity, studies are scarce. The purpose of this study was to investigate the clinical characteristics of lipoblastoma occurring in children and to detect any correlations with the expression of Ki-67. Participants: From 1998 to 2010, 33 patients were diagnosed with lipoblastoma at Seoul National University Children's Hospital. Methods: Ki-67 immunohistochemistry staining of the tumor tissue was performed. Results: A total of 33 patients (64% males) were enrolled in the study, with a mean age of 28 month. Eleven and 22 lesions were deep and superficial, respectively. Complete excisions were performed for 30 patients, and three underwent incomplete excisions. Two patients who underwent incomplete excision subsequently underwent a second operation due to tumor regrowth, and one patient had a recurrence despite complete excision. There was no statistically significant correlation observed between the tumor size or recurrence and the expression of Ki-67. Conclusions: Lipoblastoma requires an accurate diagnosis and operative resection to alleviate the symptoms induced by its growth. Incompletely resected tumor may regrow; therefore, complete excision is the treatment of choice. Continuous follow-up is needed to monitor for recurrence of disease, even after a complete excision.     

Detection of a deep lipoblastoma by MRI and ultrasound

Lipoblastoma is a rare benign tumor of the soft tissue occurring predominantly during the first two years of life. We report here the application of MRI and ultrasound to the detection of a soft tissue lipoblastoma. By MRI and ultrasound, we precisely evaluated the extent of the tumor, the presence of atypical areas of cystic and mucoid degeneration within the tumor, and its lack of vascularity. Our patient's lipoblastoma did not demonstrate the signal characteristics of a fatty tumor on MRI.     

Lipoblastoma: better termed infantile lipoma?

Lipoblastoma is a rare adipose tumor occurring exclusively in childhood. There have been no reports of metastases, making the designation “blastoma” confusing, since this term is usually reserved for malignant tumors. Two recent cases treated at our institution confirm its benign nature. In addition, a review of the literature supports the idea that the tumor may more accurately be described as an “infantile lipoma”. Infantile lipoma better reflects many of the tumor's characteristics such as, its early occurrence, it's ability to mature into a simple lipoma, it's cellular composition of mainly mature adipocytes, and its benign course. Although lipoblastoma is an uncommonly encountered tumor, making an effort to change its name to infantile lipoma will result in a more a accurate term that will facilitate treatment. Accepted: 18 March 1998     

A novel t(3;8)(p13;q21.1) translocation in a case of lipoblastoma

Lipoblastoma is a rare benign neoplasm of embryonic white fatty tissue primarily found in the extremities of children <3 years old (Batanian et al., Cancer Genet Cytogenet 125(1):10-13, 2001; McVay MR et al., J Pediatr Surg 41(6):1067-1071, 2006; Kamal et al., J Pediatr Surg 46(7):E9-E12, 2011). Translocations affecting the 8q11-13 region are commonly reported with lipoblastoma and proper diagnosis requires cytogenetic analysis to distinguish it from malignant myxoid liposarcoma (Miller et al., J Pediatr Surg 32(12):1771-1772, 1997; Morerio et al., Pediatr Blood Cancer 52(1):132-134, 2009). We describe an additional case of lipoblastoma containing a new translocation t(3;8)(p13;q21.1), which has not previously been reported in a healthy asymptomatic child.     

ANAPLASTIC LARGE CELL LYMPHOMA WITH PRIMARY INVOLVEMENT OF SKELETAL MUSCLE: A Rare Case Report and Review of the Literature

Primary skeletal muscle ALCL is very rare. Here the authors report a case of skeletal muscle ALCL that was proven pathologically. A 14-year-old boy presented with a persistent fever, chills, night sweats, headache, and significant weight loss. A CT scan of the abdomen showed a hazy mass about 3.2 × 1.2 cm in his left sacrospinalis. Ultrasonography revealed a low-echo and irregular mass in the left lumbar muscle measuring 8 × 1.4 × 3.6 cm in size and a similar mass 8 × 3.5 × 3.7 cm in size in the femoral muscle of the left thigh. MRI demonstrated an abnormal mass signal 4 × 3 × 9 cm in size infiltrating the left sacrospinalis muscle. The biopsy specimen was taken from the femoral muscle of the left thigh at surgery. Histopathological examination revealed a diffuse infiltration of large and atypical cells with pleomorphic nuclei and abundant cytoplasm. Immunohistological staining showed these atypical cells were positive for CD30 (Ki-l), anaplastic lymphoma kinase (ALK), epithelial membrane antigen (EMA), CD3, CD45RO, and CD68. The morphology and immunophenotype were consistent with CD30-positive, ALK-positive, and ALCL of T-cell lineage. The patient's condition was diagnosed as CD30-positive primary skeletal muscle ALCL.     

Ruptured giant intrathoracic lipoblastoma in a 4-month-old infant: CT and MR findings

Background. We describe a 4-month-old infant with a ruptured intrathoracic lipoblastoma arising from the parietal pleura and associated with a pleural effusion.¶Objective. The clinical presentation was rapidly evolving respiratory distress. The chest radiograph showed a large mass and a pleural effusion in the right thoracic cavity. CT demonstrated an inhomogeneous low-attenuation mass which was 7 cm in diameter and which showed areas of enhancement after intravenous contrast medium. MRI showed a fatty intrathoracic mass with intratumoral streaks and whorls, which were attributed to loose fibrovascular connective tissue on pathological examination.¶Results. Thoracotomy and pathological examination revealed a ruptured intrathoracic lipoblastoma arising from the parietal pleura.¶Conclusion. The pleural effusion might have suggested rupture of the tumour.     

Lipoblastoma in a four-year-old African child

A four-year old Sudanese child presented with a growing mass in the medial aspect of the right thigh. The mass appeared during the neonatal period. On clinical examination a diagnosis of lipoblastoma was entertained on the basis of the patient's age and the clinical features of the mass. The tumor was completely excised surgically. The clinical diagnosis of lipoblastoma was confirmed pathologically. Follow-up of the patient for 6 months postoperatively showed no evidence of recurrence.     

A left-sided asymptomatic supraclavicular cystic mass in a 14-year-old girl

A 14-year-old girl was referred to our department with a 3-month history of an asymptomatic swelling in the left cervical triangle. The girl, an active ski jumper, was used to carrying her skis on the left shoulder. There was a history of many minor falls and contusions of her body. The left posterior cervical triangle was enlarged by an elastic, partially fluctuating and clearly delineated swelling with 4–6 cm in diameter. The trapezius was elevated and the overlaying skin was intact. It was clear that the mass was spreading infraclavicularly. Nevertheless, the distal boundaries of the mass were not quite clear (Fig. 1). The infraclavicular spreading was confirmed with an ultrasound examination which revealed a hypoechogenic echo-free mass of the size of 7–10 × 3.6 cm. The mass was sonocompressible, clearly outlined and there were no signs of any infiltrating growth. No internal perfusion was found by colour-coded Doppler investigation. The blood count or biochemical tests did not reveal any pathological findings.