Difference between revisions of "Adipocytic tumours"

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===General===
===General===
*May be syndromic - typically [[autosomal recessive]].<ref name=pmid25671454>{{cite journal |vauthors=Garib G, Siegal GP, Andea AA |title=Autosomal-dominant familial angiolipomatosis |journal=Cutis |volume=95 |issue=1 |pages=E26–9 |date=January 2015 |pmid=25671454 |doi= |url=}}</ref>  
*May be syndromic - typically [[autosomal recessive]].<ref name=pmid25671454>{{cite journal |authors=Garib G, Siegal GP, Andea AA |title=Autosomal-dominant familial angiolipomatosis |journal=Cutis |volume=95 |issue=1 |pages=E26–9 |date=January 2015 |pmid=25671454 |doi= |url=}}</ref>  
*Can be seen in the context of [[Birt–Hogg–Dubé syndrome]].<ref name=pmid8734663>{{cite journal |vauthors=Chung JY, Ramos-Caro FA, Beers B, Ford MJ, Flowers F |title=Multiple lipomas, angiolipomas, and parathyroid adenomas in a patient with Birt-Hogg-Dube syndrome |journal=Int J Dermatol |volume=35 |issue=5 |pages=365–7 |date=May 1996 |pmid=8734663 |doi=10.1111/j.1365-4362.1996.tb03642.x |url=}}</ref>
*Can be seen in the context of [[Birt–Hogg–Dubé syndrome]].<ref name=pmid8734663>{{cite journal |authors=Chung JY, Ramos-Caro FA, Beers B, Ford MJ, Flowers F |title=Multiple lipomas, angiolipomas, and parathyroid adenomas in a patient with Birt-Hogg-Dube syndrome |journal=Int J Dermatol |volume=35 |issue=5 |pages=365–7 |date=May 1996 |pmid=8734663 |doi=10.1111/j.1365-4362.1996.tb03642.x |url=}}</ref>
*[[Painful skin lesion]].
*[[Painful skin lesion]].



Revision as of 22:27, 9 October 2021

Adipocytic tumours fall into the grouping soft tissue lesions and includes things that are very common (e.g. lipoma) and everything from benign to malignant.

Overview

This grouping includes a number of tumours, which can be divided based on their behaviour into benign, intermediate and malignant.

Benign

Benign adipocytic tumours:[1]

Intermediate

Intermediate adipocytic tumours:[1]

  • Atypical lipomatous tumour.

Malignant

Malignant adipocytic tumours:[1]

  • Dedifferentiated liposarcoma.
  • Myxoid liposarcoma.
  • Pleomorphic liposarcoma.
  • Mixed-type liposarcoma.
  • Liposarcoma NOS.

Detail section

Normal mature fat

Microscopic

Features:

  • Adipocytes of approximately equal size.
  • Not vascular.
  • No nuclear hyperchromasia.

Notes:

  • May have nuclear pseudoinclusions (Lockhern cell).[2]
    • There is some suggestion this is not benign.[3]

IHC

Lipoblastoma

General

  • Rare paediatric tumour.[4]

Usual presentation:[4]

  • Painless neck mass.

Microscopic

Features:

  • Nests of cells in the dermis with abundant pale cytoplasm - vaguely resemble adipocytes.
    • Smaller than mature adipocytes.

DDx:

Images:

Lipoblastoma like tumor

Lipoblastoma like tumor Lipoblastoma like tumor Lipoblastoma like tumor Lipoblastoma like tumor


Lipoblastoma like tumor in 26 yo woman, tumor of pelvis. A. Lobulated appearance at low power. B. Cytologically bland lipoblasts with a myxoid stroma. C. Numerous thin-walled branching blood vessels. D. Loose collagenous stroma. The morphologic features, combined with molecular findings of negative DDI T3 gene rearrangement and lack of MDM2 amplification permit the diagnosis.

Lipoma

Pleomorphic lipoma

Spindle cell lipoma

Hibernoma

Atypical lipomatous tumour

  • AKA well-differentiated liposarcoma, abbreviated WDLPS.[6]
  • Abbreviated ALT/WDLPS.

General

  • Atypical lipomatous tumour is a term used to save people with a (curable) peripheral liposarcoma from getting denied life insurance.

Microscopic

Features:[7]

  • Large adipocytes.
  • Atypical lipoblasts - focal, scattered:
    • Nuclear hyperchromasia.
    • +/-Multinucleated.

Liposarcoma

Angiolipoma

General

Microscopic

Features:

  • Adipose tissue.
  • Small blood vessels (clustered).

DDx:

See also

References

  1. 1.0 1.1 1.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 601. ISBN 978-0781765275.
  2. URL: http://journals.lww.com/amjdermatopathology/Citation/2004/12000/Original_Observation_to_Rediscovery__Nuclear.9.aspx. Accessed on: 18 April 2011.
  3. URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970574-5. Accessed on: 18 April 2011.
  4. 4.0 4.1 Pham, NS.; Poirier, B.; Fuller, SC.; Dublin, AB.; Tollefson, TT. (Jul 2010). "Pediatric lipoblastoma in the head and neck: a systematic review of 48 reported cases.". Int J Pediatr Otorhinolaryngol 74 (7): 723-8. doi:10.1016/j.ijporl.2010.04.010. PMID 20472310.
  5. Nagano, A.; Ohno, T.; Nishimoto, Y.; Hirose, Y.; Miyake, S.; Shimizu, K. (2011). "Lipoblastoma mimicking myxoid liposarcoma: a clinical report and literature review.". Tohoku J Exp Med 223 (1): 75-8. PMID 21212605.
  6. Creytens, D.; van Gorp, J.; Savola, S.; Ferdinande, L.; Mentzel, T.; Libbrecht, L. (Jul 2014). "Atypical spindle cell lipoma: a clinicopathologic, immunohistochemical, and molecular study emphasizing its relationship to classical spindle cell lipoma.". Virchows Arch 465 (1): 97-108. doi:10.1007/s00428-014-1568-8. PMID 24659226.
  7. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 605. ISBN 978-0781765275.
  8. Garib G, Siegal GP, Andea AA (January 2015). "Autosomal-dominant familial angiolipomatosis". Cutis 95 (1): E26–9. PMID 25671454.
  9. Chung JY, Ramos-Caro FA, Beers B, Ford MJ, Flowers F (May 1996). "Multiple lipomas, angiolipomas, and parathyroid adenomas in a patient with Birt-Hogg-Dube syndrome". Int J Dermatol 35 (5): 365–7. doi:10.1111/j.1365-4362.1996.tb03642.x. PMID 8734663.