Lymph nodes - not lymphoma
Neoplasms (not lymphoma)
Lymphangiomyomatosis

Author: Sheren Younes, M.D. (see Authors page)

Revised: 25 June 2018, last major update February 2016

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Lymphangiomyomatosis[TI]
Cite this page: Younes, S. Lymphangiomyomatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphnodeslymphangiomyomatosis.html. Accessed July 17th, 2018.
Definition / general
  • Multisystem disorder affecting mainly middle age females, causing pulmonary and extrapulmonary disease
  • Its pathologic features result from the proliferation of neoplastic cells (LAM cells), which have characteristics of both smooth muscle cells and melanocytes
  • It was originally classified as benign but now is considered a "low grade, destructive metastasizing neoplasm" (Am J Respir Crit Care Med 2012;186:1210)
Terminology
  • Also called lymphangioleiomyomatosis
Epidemiology
  • Estimated median transplant free survival time for LAM patients in the U.S. is 29 years from onset of symptoms and 23 years from diagnosis
  • About 80% of women with TSC develop cystic disease in lungs (Chest 2013;144:578), compared to 13% of male patients (Clin Radiol 2011;66:625)
  • Relationship between pulmonary and extrapulmonary LAM needs further investigation
Etiology
  • Associated with tuberous sclerosis complex (TSC), an autosomal dominant disorder caused by mutations in the TSC1 or TSC2 genes, characterized by mental retardation, autism, seizures, facial angiofibroma, cortical tubers, cardiac rhabdomyoma, asrocytoma
Diagrams / tables

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Diagnostic criteria

Case reports
Microscopic (histologic) description
  • Single or multiple lymph nodes, median size is 3.5 cm
  • Mostly affecting nodal parenchyma but can be seen in hilum, subcapsular sinuses or extranodal extension
  • LAM cells are either spindle or epitheliod
  • Epithelioid cells are arranged in nests or swirls, separated by clefts which resemble lymphatic spaces
  • Spindled cells show fascicular growth with some nesting and less prominent lymphatic channels
  • No atypia, no necrosis, no mitosis
Microscopic (histologic) images

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Lymph node

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Involving pelvic lymph node



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HMB45-

Cytology description
Positive stains
Differential diagnosis