Adrenal gland & paraganglia

Adrenal cortical carcinoma

Myxoid



Last author update: 16 February 2022
Last staff update: 2 December 2022

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PubMed Search: Myxoid[TIAB] carcinoma adrenal

Maria Tretiakova, M.D., Ph.D.
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Cite this page: Tretiakova M. Myxoid . PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenocorticalcarcinomamyxoid.html. Accessed December 9th, 2022.
Definition / general
  • Rare variant of malignant epithelial tumor of adrenal cortical cells with extracellular myxoid component
Essential features
Terminology
  • Adrenal cortical carcinoma (ACC), myxoid variant
  • Adrenocortical carcinoma, myxoid type
  • Myxoid adrenocortical carcinoma
ICD coding
  • ICD-O: 8370/3 - adrenal cortical carcinoma
Epidemiology
Sites
Pathophysiology
Etiology
Diagrams / tables

Images hosted on other servers:

Survival analysis comparing myxoid ACC to other variants

Clinical features
Diagnosis
Radiology description
Radiology images

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Myxoid ACC on CT, MRI and PET / CT imaging

Prognostic factors
  • Myxoid ACC has slightly worse prognosis than conventional ACC (Horm Cancer 2011;2:333)
  • Overall median survival for myxoid ACC is 29 months (Biomedicines 2021;9:175)
  • Behavior of myxoid ACC is difficult to predict: even tumors with low Weiss score could have fatal outcome (Histopathology 2018;72:82)
  • p53 / Rb1 pathway activation was associated with high tumor stage, high Ki67 index, aggressive disease status and shorter disease free survival
  • Overexpression of miR-483-3p, miR483-5p and miR-210 indicate poor prognosis (similar to conventional ACC) and are associated with male sex, presence of necrosis, high Ki67 proliferation index, mitotic count and increased SF1 expression on IHC (Hum Pathol 2014;45:1555)
Case reports
Treatment
  • Surgical: radical resection, treatment mainstay (Surgery 2019;166:524)
  • Guidelines for adjuvant treatment with mitotane, cytotoxic chemotherapy and radiation therapy are similar to conventional ACC
Clinical images

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Robot assisted laparoscopic adrenalectomy

Gross description
Gross images

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Gelatinous and solid whitish appearance

Obvious areas of hemorrhage and necrosis

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Maria Tretiakova, M.D., Ph.D.

ACC with predominant myxoid component

Delicate arborizing cords

Mucin pools with floating cells

Pseudoglandular architecture

Complex architecture


Adjacent benign adrenal

Focal myxoid pattern

Transition to myxoid area

Microcystic architecture

Positive margin


pT3 myxoid ACC

Mitoses

Normal reticulin network

Loss of reticulin framework

Positive stains
Electron microscopy description
Molecular / cytogenetics description
  • Molecular alterations are similar to conventional ACC, including high mutation burden and massive DNA loss followed by whole genome doubling (Histopathology 2018;72:82)
  • Most frequently affected by somatic mutations, copy number alterations and epigenetic silencing were p53 (21%), ZNRF3 (19%), CDKN2A (15%), CTNNB1 (16%), TERT (14%) and PRKAR1A (11%) (Cancer Cell 2016;30:363)
  • Another study of 7 myxoid ACC showed very frequent gain of TERT (43%) and CDK4 (86%) and loss of RB1 (28%), CDKN2A (28%), ZNRF3 (14%) (Mod Pathol 2018;31:1257)
  • High expression of miR-483-3p, miR483-5p and miR-210 was similar to conventional ACC (Hum Pathol 2014;45:1555)
  • EGFR protein overexpression without mutations or amplification; increased EGFR copy number in one case (Am J Clin Pathol 2011;136:783)
Sample pathology report
  • Adrenal gland, left, adrenalectomy:
    • Adrenal cortical carcinoma with the following features:
      • Tumor size: 12 cm x 10 cm x 9 cm
      • Tumor (gland) weight: 300 g
      • Tumor extent: capsular invasion
      • Histologic type: myxoid variant (30% tumor)
      • Histologic grade: high grade
      • Necrosis: present
      • Lymphovascular invasion: present
      • Margins: positive
      • pTNM, AJCC 8th Edition: pT3 NX MX
    • Ancillary studies: Ki67 mitotic rate 25%
    • Reticulin stain: disruption of reticulin framework
Differential diagnosis
Board review style question #1

Which of the following features is characteristic for myxoid variant of adrenocortical carcinoma (ACC), shown in the picture?

  1. Has unique immunohistochemical, molecular and genomic profiles
  2. More common than other variants of ACC
  3. More indolent than conventional ACC
  4. Must have ≥ 50% extracellular mucinous component
  5. Should be distinguished from metastatic mucin producing carcinoma
Board review style answer #1
E. Should be distinguished from metastatic mucin producing carcinoma. Myxoid variant of adrenocortical carcinoma (ACC) is characterized by the presence of extracellular myxoid component. In some cases, tumors exhibit striking resemblance with myxoid tumors, adenoid cystic and other mucin producing carcinomas, which have to be ruled out in the right clinical context. Answer A is incorrect because there is a substantial overlap in immunohistochemical, molecular and genomic profiles of myxoid and conventional ACC variants. Answer B is incorrect because myxoid ACC is less common than conventional or oncocytic variants of ACC with only ~50 cases reported to date. Answer C is incorrect because compared with conventional ACC, myxoid variant has more aggressive clinical behavior with higher rate of tumor recurrence, metastases and shorter overall survival. Answer D is incorrect because myxoid extracellular matrix is variably abundant from 5 - 90% of tumor volume.

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Reference: Adrenal cortical carcinoma - Myxoid variant
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