Difference between revisions of "Pheochromocytoma"

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Line 15: Line 15:
| Gross      =
| Gross      =
| Grossing  =
| Grossing  =
| Site      = [[adrenal gland]] (same tumour at other sites known as ''[[paraganglioma]]'')
| Site      = [[adrenal gland]] (same tumour arising at other sites known as ''[[paraganglioma]]'')
| Assdx      =
| Assdx      =
| Syndromes  = [[Multiple endocrine neoplasia]] 2A and 2B, [[von Hippel-Lindau syndrome]],[[Neurofibromatosis]] type 1, familial paraganglioma syndromes (several)
| Syndromes  = [[Multiple endocrine neoplasia]] 2A and 2B, [[von Hippel-Lindau syndrome]],[[Neurofibromatosis]] type 1, familial paraganglioma syndromes (several)

Revision as of 03:00, 23 August 2014

Pheochromocytoma
Diagnosis in short

Paraganglioma - has identical histologic appearance as pheochromocytoma. H&E stain.
LM DDx adrenocortical carcinoma, paraganglioma
IHC chief cells: chromogranin A +ve, synaptophysin +ve; sustentacular cells: S-100 +ve
Site adrenal gland (same tumour arising at other sites known as paraganglioma)
Syndromes Multiple endocrine neoplasia 2A and 2B, von Hippel-Lindau syndrome,Neurofibromatosis type 1, familial paraganglioma syndromes (several)
Clinical history hypertension (classic Hx), paroxysms of tachycardia, headache, anxiety, hypertension
Prevalence uncommon
Prognosis usually benign
Clin. DDx other adrenal gland masses, renal cell carcinoma, other abdominal masses

Pheochromocytoma is a tumour of the adrenal gland medulla. It may be benign or malignant.

General

  • Considered to be a paraganglioma.[1]
  • Literally means "dusky" (pheo) "colour" (chromo) - dull appearance on gross.
  • Tumour arises from adrenal medulla - chromaffin cells.[2]

Memory device - the rule of 10s:[2]

Clinical

  • Classic finding: hypertension.
  • Paroxysms (i.e. episodes) of tachycardia, headache, anxiety, hypertension.

Laboratory findings (urine):

  • Vanillylmandelic acid (VMA).
  • Metanephrines.

Microscopic

Features:[3]

  • Chief cells:
    • Usu. polygonal cells, may be spindled.
    • Arranged in cell nests - "Zellballen" (literally cell balls) - key feature.
    • Stippled chromatin (AKA salt and pepper chromatin) - coarsely granular chromatin.
    • Granular cytoplasm, often basophilic - important.
  • Sustentacular cells (structural support cell).
  • Often haemorrhagic - highly vascular.
  • +/-Nuclear pleomorphism.

Notes:

  • The nested architecture (Zellballen) is useful for differentiating from ACC.
  • Metastasis sole criteria of malignancy.[2]
  • Surrounding adrenal cortex is typically compressed.[4]

DDx:

Images

  • Carotid body tumour - low mag. (WC/Nephron)

  • Carotid body tumour - high mag. (WC/Nephron)

Pheochromocytoma versus adrenal cortical carcinoma

  • Pheochromocytoma and adrenal cortical carcinoma overlap histologically.[5]

Favour pheochromocytoma:

  • Small chickenwire-pattern blood vessels, nests, salt-and-pepper chromatin, red blood cell extravasation.

Favour adrenal cortical carcinoma:

  • Nucleolus, sheeting.

Malignant pheochromoctyoma

  1. Robbins says metastases are the sole criteria of malignancy.[2]
  2. Thompson suggests one can differentiate benign from malignant with the aid of the following:[6]
    • Marked nuclear atypia.
    • Invasion:
      • Capsular.
      • Vascular.
    • Necrosis.
    • Cellular monotony.
    • Mitoses:
      • Rate.
      • Atypical mitosis.

IHC

  • Chief cells:
    • Chromogranin A +ve.
    • Synaptophysin +ve.
  • Sustentacular cells:
    • S100 +ve.

Electron microscopy

  • Membrane-bound secretory granules.

Sign out

ADRENAL MASS, RIGHT, ADRENALECTOMY:
- PHEOCHROMOCYTOMA.
- SURGICAL MARGIN NEGATIVE FOR PHEOCHROMOCYTOMA.

COMMENT:
The tumour cells stains for chromogranin and synaptophysin. S-100 marks the sustentacular cells.
Inhibin is negative in the tumour cells. The immunostaining pattern is consistent with a 
pheochromocytoma.

Micro

The sections shows a partially hemorrhagic lesion in the medulla of the adrenal gland that is arranged in nests (Zellballen). The tumour cells have abundant grey/blue granular cytoplasm, and nuclei with granular chromatin (salt and pepper chromatin). The lesion is surrounded by a compressed rim of adrenal cortex and fibrosis tissue. The core of the lesion is fibrotic and has clusters of hemosiderin-laden macrophages.

There is no capsular invasion. Vascular invasion is not identified. There is no necrosis. Mitotic activity is not appreciated.

The adrenal cortex is unremarkable.

See also

References

  1. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  2. ↑ 2.0 2.1 2.2 2.3 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 586. ISBN 978-1416054542.
  3. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1161. ISBN 978-1416031215.
  4. ↑ URL: http://www.pathpedia.com/Education/eAtlas/Histopathology/Adrenal/Pheochromocytoma.aspx. Accessed on: 27 May 2013.
  5. ↑ Sangoi, AR.; McKenney, JK. (Mar 2010). "A tissue microarray-based comparative analysis of novel and traditional immunohistochemical markers in the distinction between adrenal cortical lesions and pheochromocytoma.". Am J Surg Pathol 34 (3): 423-32. doi:10.1097/PAS.0b013e3181cfb506. PMID 20154585.
  6. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 259. ISBN 978-0443066856.
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