Adrenal gland & paraganglia

Adrenal cortical carcinoma

Oncocytic



Last author update: 27 December 2021
Last staff update: 2 December 2022

Copyright: 2002-2022, PathologyOutlines.com, Inc.

PubMed Search: Oncocytic[title] carcinoma adrenal

Maria Tretiakova, M.D., Ph.D.
Page views in 2021: 2,496
Page views in 2022 to date: 2,615
Cite this page: Tretiakova M. Oncocytic. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenocorticalcarcinomaoncocytic.html. Accessed December 9th, 2022.
Definition / general
  • Malignant epithelial tumor of adrenal cortical cells with predominantly oncocytic morphology
Essential features
  • Rare variant of adrenal cortical carcinoma (ACC); less than 70 cases reported
  • Composed entirely or predominantly (> 75%) of oncocytic cells with abundant cytoplasmic mitochondria (Adv Anat Pathol 2014;21:151)
  • Tend to be lower stage, rarely invade adjacent organs and seem to represent more indolent variant of ACC with delayed recurrence and improved survival, despite a larger average size and aggressive morphologic appearance (Surgery 2019;166:524, Turk Patoloji Derg 2015;31:98, Biomedicines 2021;9:175)
  • Positive for inhibin, calretinin, MelanA, synaptophysin and mitochondrial antigen
Terminology
  • Adrenal cortical carcinoma (ACC), oncocytic variant
  • Adrenocortical carcinoma, oncocytic type
  • Oncocytic adrenocortical carcinoma
ICD coding
  • ICD-O: 8370/3 - adrenal cortical carcinoma
  • ICD-10: C74.0 - malignant neoplasm of cortex of adrenal gland
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
Diagnosis
LWB diagnostic algorithm for oncocytic ACC
 1  Major criteria:
    High mitotic rate (> 5 mitoses/50 HPF) 
    Atypical mitotic figures 
    Venous invasion 
 2  Minor criteria:
    Large size (> 10 cm) or weight (> 200 g) 
    Necrosis 
    Sinusoidal invasion  
    Capsular invasion 

  • One major criterion indicates malignancy, minor criteria indicates uncertain malignant potential (borderline) and the absence of all major and minor criteria indicates benign
Laboratory
  • Same as conventional ACC
Radiology description
  • Abdominal computed tomography (CT):
  • On magnetic resonance imaging (MRI):
    • Isointense to hypointense signal on T1 imaging, a hyperintense signal on T2 images and a heterogeneous signal drop on chemical shift (Surgery 2019;166:524)
  • Because oncocytic tumors are lipid poor, CT, MRI or PET imaging are unable to distinguish benign from malignant lesions regardless of size, attenuation or handling of the contrast agent (Surgery 2019;166:524, Asia Ocean J Nucl Med Biol 2018;6:179)
Radiology images

Images hosted on other servers:

CT imaging of oncocytic ACC

F18 FDG PET of oncocytic ACC

Prognostic factors
Case reports
Treatment
  • Surgical (Surgery 2019;166:524):
    • Radical resection - treatment mainstay
    • Lymphadenectomy in 75% of cases
  • Guidelines for adjuvant treatment with mitotane, cytotoxic chemotherapy and radiation therapy are similar to conventional ACC but data is limited
Gross description
Gross images

Contributed by Maria Tretiakova, M.D., Ph.D.
Large lobulated yellow mass

Large lobulated yellow mass

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Maria Tretiakova, M.D., Ph.D.
Oncocytic ACC with confluent necrosis

Oncocytic ACC with confluent necrosis

Diffuse discohesive growth

Diffuse discohesive growth

Trabecular pattern

Trabecular pattern

Capsule invasion

Capsule invasion

Pleomorphic morphology

Pleomorphic morphology


Nuclear pleomorphism

Nuclear pleomorphism

Abundant mitoses

Abundant mitoses

Fat invasion & positive margin

Fat invasion & positive margin

Regional node involvement

Regional node involvement

Disrupted reticulin network

Disrupted reticulin network

Electron microscopy description
  • Abundant mitochondria
Molecular / cytogenetics description
  • Compared to conventional ACC oncocytic variant characterized by:
    • Similar but less common somatic mutations of ACC driver genes
    • Lower mutation burden of β catenin and p53 / Rb pathways (Turk Patoloji Derg 2015;31:98)
    • Reduced expression of miR-483-3p, miR-483-5p and miR-210 compared to other ACC variants (Hum Pathol 2014;45:1555)
  • Common deletion in mitochondrial DNA (mtDNA 4977 bp) (Am J Surg Pathol 2011;35:1882)
Sample pathology report
  • Adrenal gland, left, adrenalectomy:
    • Adrenal cortical carcinoma with the following features:
      • Tumor size: 15 cm x 11 cm x 10 cm
      • Tumor (gland) weight: 450 g
      • Tumor extent: organ confined
      • Histologic type: pure oncocytic variant
      • Histologic grade: low grade
      • Necrosis: present
      • Lymphovascular invasion: absent
      • Margins: free of tumor
      • pTNM, AJCC 8th edition: pT2 NX MX
    • Ancillary studies: Ki67 mitotic rate 7%
    • Reticulin stain: disruption of reticulin framework
Differential diagnosis
Board review style question #1

A 47 year old woman presents to a clinic complaining of abdominal distention and constipation. Imaging studies reveal a low fat heterogeneous enhancing 14 cm mass in the left adrenal gland with central necrosis. An adrenalectomy was done and representative morphologic picture of pure oncocytic neoplasm is shown in the photo. Which one of the listed criteria will be sufficient for the diagnosis of adrenal cortical carcinoma?

  1. Capsular invasion
  2. Presence of necrosis
  3. Sinusoidal invasion
  4. Tumor weight 350 g
  5. Venous invasion
Board review style answer #1
E. Venous invasion. Oncocytic adrenocortical tumors should be diagnosed using modified scoring Lin-Weiss-Bisceglia (LWB) system. Presence of 1 major criteria indicates malignancy, minor criteria indicates uncertain malignant potential (borderline) and the absence of all major and minor criteria indicates benign. From all listed answers, only venous invasion belongs to major criteria and would quality this tumor as adrenocortical carcinoma. (Hum Pathol 2011;42:489, Adv Anat Pathol 2014;21:151)

Comment Here

Reference: Oncocytic variant
Board review style question #2
When comparing to conventional adrenal cortical carcinoma (ACC), which statement is accurate in characterizing oncocytic ACC variant?

  1. Typically smaller at presentation
  2. Affects male patients more often
  3. More aggressive with higher metastatic rate and shorter survival
  4. Difficult to distinguish from benign oncocytic tumors on imaging
  5. Requires different therapy approach
Board review style answer #2
D. Difficult to distinguish from benign oncocytic tumors on imaging. Because all oncocytic tumors are lipid poor, CT, MRI or PET imaging are unable to distinguish benign from malignant lesions regardless of size, attenuation or handling of the contrast agent. Answers A and C are incorrect, because oncocytic ACC, despite larger size at presentation and aggressive morphologic appearance, tends to be lower stage, less frequently metastasizes and seems to represent more indolent variant of ACC with delayed recurrence and improved survival. Answers B and E are false since patient population and therapy approaches are similar for both conventional and oncocytic ACC. (Surgery 2019;166:524, Asia Ocean J Nucl Med Biol 2018;6:179, Turk Patoloji Derg 2015;31:98, Biomedicines 2021;9:175)

Comment Here

Reference: Oncocytic variant
Back to top
Image 01 Image 02