Pigmented spindle cell carcinoid tumour of the thymus with ectopic adrenocorticotropic hormone secretion: report of a rare variant and differential diagnosis of mediastinal spindle cell neoplasms

Histopathology. 2002 Feb;40(2):159-65. doi: 10.1046/j.1365-2559.2002.01339.x.

Abstract

Aims: A variety of histological variants of thymic carcinoid tumour have been described. A rare case of pigmented spindle cell carcinoid tumour of the thymus is documented and compared with the reported cases of thymic pigmented carcinoid tumour in the literature, with a discussion of the differential diagnosis of spindle cell tumours of the mediastinum.

Methods and results: A thymic tumour with ectopic adrenocorticotropic hormone (ACTH) secretion was resected from a 24-year-old man suffering from Cushing's syndrome. Histological, immunohistochemical, and ultrastructural studies revealed an ACTH-producing spindle cell carcinoid tumour harbouring pigmented melanocytes. Among four thymic pigmented carcinoid tumours reported before, only one was similar to the present case by being also an ACTH-secreting pigmented spindle cell thymic carcinoid tumour. The clinicopathological features of this tumour distinguish it from a spindle cell thymoma, spindle cell thymic carcinoma, and other mediastinal spindle cell tumours.

Conclusions: This case illustrates an extremely rare variant of thymic carcinoid tumour exhibiting a spindle cell morphology and harbouring pigmented melanocytes. Awareness of this histological variant is important in the differential diagnosis of spindle cell tumours of the mediastinum.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenocorticotropic Hormone / metabolism*
  • Adult
  • Biomarkers, Tumor / metabolism
  • Carcinoid Tumor / complications
  • Carcinoid Tumor / metabolism*
  • Carcinoid Tumor / pathology
  • Carcinoid Tumor / surgery
  • Carcinoma / pathology
  • Cushing Syndrome / complications
  • Cushing Syndrome / pathology
  • Diagnosis, Differential
  • Humans
  • Immunoenzyme Techniques
  • Male
  • Melanocytes / metabolism
  • Melanocytes / pathology
  • Sarcoma / pathology
  • Secretory Vesicles / ultrastructure
  • Thymoma / pathology
  • Thymus Neoplasms / complications
  • Thymus Neoplasms / metabolism*
  • Thymus Neoplasms / pathology
  • Thymus Neoplasms / surgery
  • Tomography, X-Ray Computed

Substances

  • Biomarkers, Tumor
  • Adrenocorticotropic Hormone