Soft Tissue Pathology

Lipoblastoma

Dr Sampurna Roy MD

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January 2014

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Myxoid Tumours of Soft Tissue

Classification of Soft Tissue Tumour

Gross examination of soft tissue specimen          

A practical approach to histopathological reporting of soft tissue tumours

Grading of soft tissue tumours

Lipomatous tumours

Neural tumours

Myogenic tumours

Fibroblastic/Myofibroblastic tumours

Myofibroblastic tumours

Fibrohistiocytic tumours

ChondroOsseous tumours

Soft TissueTumours of Uncertain Differentiation               

Notochordal Tumour - Chordoma

Extra-adrenal Paraganglioma

Gastrointestinal Stromal Tumour

Lipoma and variants

Angiolipoma

Extrarenal Angiomyolipoma

Chondroid lipoma

Hibernoma

Spindle cell/pleomorphic lipoma

Well differentiated Liposarcoma

De-differentiated liposarcoma 

Myxoid liposarcoma 

Round  cell liposarcoma

Pleomorphic liposarcoma 

Vascular tumours

Angiokeratoma

Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia)

Lobular capillary hemangioma (pyogenic granuloma

Bacillary angiomatosis

Verruga Peruana

Masson's Tumour (Intravascular papillary endothelial hyperplasia)

Acro-angiodermatitis / pseudo-Kaposi's sarcoma

Reactive angioendotheliomatosis

Infantile Hemangioma

Glomeruloid hemangioma

Acquired tufted angioma

Verrucous hemangioma

Cherry angioma/senile angioma

Arteriovenous hemangioma 

Microvenular hemangioma

Targetoid hemosiderotic hemangioma(Hobnail hemangioma)

Kaposiform hemangioendothelioma 

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (Dabska's tumour)

Composite hemangioendothelioma

Kaposi's sarcoma

Epithelioid hemangioendothelioma

Angiosarcoma

Glomus tumour

Hemangiopericytoma

Angiolipoma

Aggressive angiomyxoma

Angiomyofibroblastoma

Angioleiomyoma

Angiomyolipoma

Dermatofibroma (aneurysmal variant)

Spindle cell lipoma (Angiomatoid variant)

Kimura's disease

 

 

Lipoblastoma is a rare benign mesenchymal tumour of embryonal fat that occurs almost exclusively in infants and children.

The term lipoblastoma was first used by Jaffe (Recurrent lipomatous tumors of the groin: liposarcoma and lipoma pseudomixomatodes. Arch Pathol 1926;1:381387).

Vellios et al used the term 'Lipoblastomatosis' for an infiltrating lipoblastoma in the supra-clavicular region, anterior chest wall, and axilla (Lipoblastomatosis: a tumor of fetal fat different from hibernoma: report of a case, with observations on the embryogenesis of human adipose tissue. Am J Pathol 1958;34:11491159).

Age: Usually occurs in infants and young children (between 5 days to 6 years).

Maturing lipoblastomas may be present in older children and rarely in teenagers.

These may take the appearance of fibrolipoma.

Site: Most commonly found in the subcutis of the upper and lower extremities.

Lipoblastomas are also located in less common sites, including head and neck, mediastinum, lung, trunk, mesentery, peritoneal cavity, retroperitoneum, intramuscular, and inguinal canal.

Clinical presentation:

Lipoblastoma presents as a painless nodule or mass.

The disease is presents in two forms:

I Superficial - (embryonal or fetal lipoma)-Solitary subcutaneous circumscribed slow growing lesion.

II Diffuse - (Lipoblastomatosis) - Multicentric, deep-seated and ill-defined, diffuse lesion which arises in skeletal muscle, retroperitoneum, or mesentry.

Lipoblastomatosis may occur in association with hemangiomas, other soft tissue lesions, intestinal neuronal dysplasia and/or macrodactyly.

Gross:

The tumour presents as lobulated, soft, and encapsulated mass with a tan yellow to creamy white cut surface.

The lesion is paler than an ordinary lipoma, with mottled gray myxoid areas.

Microscopic features:

Lipoblastomas is subclassified into the following 4 subtypes, according to their histological features:

(1) the classic type, characterized by a minimal myxoid component consisting of intercellular mucin, spindle cells, and stellate primitive mesenchymal cells together with adipocytic component;

(2) myxoid lipoblastomas contained abundant interstitial mucin, which comprised more than 50% of the specimen.

(3) lipoma-like lipoblastomas lacked a myxoid component and are composed predominantly of mature adipocytes with scattered monovacuolated and multivacuolated lipoblasts; and

(4) hibernoma-like lipoblastomas lacked a myxoid component and are composed predominantly of multivacuolated lipoblasts, some of which had central nuclei and granular eosinophilic cytoplasms.

Summary of microscopic features:-

Lobulated tumour composed of mature and immature adipocytes.

Immature fat cells resemble embryonic white fat.

A mixture of lipoblasts in different stages of maturation- from univacuolated spindle mesenchymal cells within the myxoid matrix to larger and more vacuolated cells to the mature adipocytes may be present.

There are large cells with granular cytoplasm resembling hibernoma cells.

There are no atypical mitotic figures and no nuclear atypia.

The cells are present in a stroma which is often myxoid with a rich delicate plexiform vascular network.        

Cytogenetics:

Shows rearrangements of chromosome 8. 

Differential diagnosis:

Confusion with myxoid liposarcoma, well-differentiated liposarcoma and typical lipomas may occur.

Myxoid liposarcoma is rare under the age of 10 years.

In lipoblastoma there is prominent lobulation,with maturation at the center of the lobules.

In myxoid liposarcoma, maturation is at the periphery.

Cytologic features suggestive of malignant potential, such as nuclear atypia, mitotic figures, hyperchromasia, and hypercellularity, are relatively uncommon in lipoblastoma.

Lipoblastomas have been reported to exhibit karyotypic abnormalities involving chromosome 8, while lacking the translocation associated with myxoid liposarcoma.

Treatment :

Depending on the size and location the tumour may compress adjacent structures and interfere with their functions.

Complete excision is the treatment of choice.

The diffuse form can be difficult to excise completely which may lead to recurrence of the tumour.

A followup of at least two years is suggested.         

                                                          

Abstracts:

Lipoblastoma / lipoblastomatosis: a clinicopathologic study of 25 tumours.Am J Surg Pathol.1997;21:1131-1137

Lipoblastoma and lipoblastomatosis: a clinico- pathological  study of 14 cases.Histopathology 1993 ; 23: 527-533

Benign recurring lipoblastoma in an adult versus well differentiated subcutaneous myxoid liposarcoma: clinicopathologic, immunohistochemical and molecular analysis of a unique case.Pathol Res Pract 1999;195(11):787-92

Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature. Eur J Pediatr Surg. 2001 Oct;11(5):342-9

Primary cardiac lipoblastoma.Pediatr Dev Pathol 2001 May-Jun;4(3):276-80

An unusual posterior mediastinal lipoblastoma with spinal epidural extension presenting as a painful suprascapular swelling: case report and a brief review of the literature.J Clin Neurosci 2002 Mar;9(2):204-7

Lipoblastoma: the least well known of adipose tumors. Cir Pediatr. 2004 ;17(4): 175-8

Intrascrotal lipoblastoma with a complex karyotype: a case report and review of the literature.Arch Pathol Lab Med. 2004 Jul;128(7):797-800

Lipoblastoma and lipoblastomatosis in infancy and childhood: histopathologic, ultrastructural,and cytogenetic features.Ultrastruct Pathol 2001;25(4):321-33

Lipoblastoma.J Pediatr Surg 2001;36(6):905-7

Lipoblastoma: an embryonal tumor of soft tissue related to organogenesis. Semin Diagn Pathol 1994;11:98-103.

Cytogenetic findings in pediatric adipose tumors: consistent rearrangement of chromosome 8 in lipoblastoma. Genes Chromosome Cancer. 1993;6(1):24-9.

Two rapidly growing fatty tumors of the upper limb in children: lipoblastoma and infiltrating lipoma. J Hand Surg [Am] 1995;20:2023

Tumor karyotype differentiates lipoblastoma from liposarcoma. J Pediatr Surg 1997;32:1771-1772.

Benign lipoblastoma and myxoid liposarcoma: a comparative light- and electron-microscopic study.Am J Surg Pathol 1980 Apr;4(2):163-74

Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature.Can J Surg 1998 Dec;41(6):455-8

Evidence of involvement of the PLAG1 gene in lipoblastomas. Int J Oncol. 2000;11071110.

Lipoblastoma: better termed infantile lipoma? Pediatr Surg Int.2000;16: (5-6): 458-461

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