1/6. Benign recurring lipoblastoma in an adult versus well differentiated subcutaneous myxoid liposarcoma: clinicopathologic, immunohistochemical and molecular analysis of a unique case.Subcutaneous myxoid liposarcoma (ML) is exceedingly rare, with only two or three cases having been reported. lipoblastoma (LB), a rare fatty tumor that arises in infants and children, is unknown after adolescence. In contrast to ML, LB is often superficial. The two tumors can be difficult to distinguish due to many histologic similarities. We examined a 0.9 cm superficial subcutaneous nodule from the dorsal neck of a 48 year old man that had been growing slowly. Three and one half years later, a 0.4 cm palpable recurrent nodule was excised from the scar. The patient is now free of disease at 7.5 years. Because of these unusual features, we performed clinicopathologic, immunohistochemical and molecular analysis of this unusual tumor to decide if this represented a rare cutaneous ML or an unprecedented example of LB in an adult. The primary featured a thick fibrous pseudocapsule with foci of lymphocytes and infiltrating nests of semi-mature fetal-appearing adipocytic tissue. This surrounded a more immature cellular-but-cytologically-bland myxoid tissue featuring stellate cells and signet lipoblasts. There were fibrous sep at the periphery and the vasculature was rather inconspicuous. The 0.4 cm diameter recurrence was distinctly lobular and had minute satellite nodules. It was composed of uniform fetal-appearing bland myxoid lipoblastic tissue featuring signet ring lipoblasts surrounded by a few spindle cells. In both tumors, lipoblasts expressed S-100 protein. In the primary, 5% of the lesional cells were FXIIIa dendritic stromal histiocytes while in the recurrence, 15% of the lesional cells were FXIIIa dendritic cells. CD34 stained only scattered small capillaries. The Ki67 proliferation index was 1% in the primary and 3% in the recurrence. RT-PCR assay for TLS/FUS-CHOP fusion transcripts was negative despite three repeat tests performed on paraffin sections of the primary tumor in the presence of good m-rna internal controls. We reviewed the clinicopathologic and cytogenetic features of ML and LB. Based on this review and on the growth pattern, anatomic features and molecular data from the present case, we conclude that this tumor may represent the first reported case of adult LB.- - - - - - - - - - ranking = 1keywords = lipoblastoma (Clic here for more details about this article) |
2/6. Myxoid liposarcoma in a 12-year-old girl.A 12-year-old girl developed a myxoid liposarcoma on the inner side of her right thigh. Liposarcomas are extremely rare at this age compared to benign lipoblastomas, which are the most frequent tumors of fatty tissue in childhood. The prognosis of myxoid liposarcoma is good, especially when, as in this patient, the tumor is located in the subcutis where a large and deep excision is possible and often curative.- - - - - - - - - - ranking = 0.25keywords = lipoblastoma (Clic here for more details about this article) |
3/6. 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.- - - - - - - - - - ranking = 2.25keywords = lipoblastoma (Clic here for more details about this article) |
4/6. Cervical lipoblastomatosis producing quadriparesis: case report of surgery with chemotherapy and 10-year follow-up.INTRODUCTION: Cervical lipoblastomatosis is a rare spinal tumour. Management of recurrence and long-term outcome data are not well described. CASE REPORT: A 10-month-old infant presented with an upper extremity weakness. magnetic resonance imaging (MRI) revealed an extradural cervical spinal tumour. It was debulked and histopathology revealed it to be lipoblastomatosis. The infant improved postoperatively. However, 5 months later the patient deteriorated and developed quadriparesis. The patient was managed with a more extensive resection and had chemotherapy. Ten years post-presentation the patient is well having made a full recovery and is living a normal life. Recent MRI shows minimal residual quiescent tumour. CONCLUSION: The treatment of cervical lipoblastomatosis should involve the resection of as much tumour as possible at the first sitting as recurrence can be a problem. In cases of spinal recurrence we recommend aggressive decompression and adjuvant chemotherapy.- - - - - - - - - - ranking = 1.75keywords = lipoblastoma (Clic here for more details about this article) |
5/6. Congenital lipoblastoma of the scalp.lipoblastoma is a unique tumor of infancy and early childhood that can occur congenitally. It commonly occurs in trunk and extremities, but also rarely in head and neck. We have not encountered any report of congenital lipoblastoma of scalp in published literature. Here we describe the case of an infant who presented with a rapidly growing large scalp mass that measured 15 x 15 x 10 cm. At birth, the mass was located on the bregma but progressed to extend into the left upper eyelid and eyebrow. The mass was resected in two stages: the first stage consisted of resecting the scalp mass and the second stage consisted of excising the eyelid extension of the lesion. The histology of both resection specimens was similar and showed lobular adipose tissue separated by fibrous septae, which was consistent with a lipoblastoma. The child is free of recurrence at follow-up 3 years after the resection.- - - - - - - - - - ranking = 1.5keywords = lipoblastoma (Clic here for more details about this article) |
6/6. Tumor karyotype differentiates lipoblastoma from liposarcoma.lipoblastoma is a rare benign pediatric soft tissue tumor that may be difficult to distinguish from a myxoid liposarcoma clinically or histologically. The authors present a case of a progressively growing, locally invasive soft tissue tumor in a child. Tissue culture for cytogenetic study showed a breakpoint in the long arm of chromosome 8. A review of the literature showed seven case reports of lipoblastoma karyotype, six of which had a similar breakpoint in chromosome 8. This distinguishes it from the histologically alike myxoid liposarcoma, the karyotype of which typically contains the clonal anomaly t(12;16)(q13:p11). The authors recommend that when performing a biopsy of a childhood adipose tumor with unusual features, such as progressive or invasive growth, that fresh tissue be submitted for cell culture. The tumor karyotype will, in most cases, aid in differentiating lipoblastoma from myxoid liposarcoma.- - - - - - - - - - ranking = 1.5keywords = lipoblastoma (Clic here for more details about this article) |