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intrahepatic cholangiocarcinoma

Tuesday 28 August 2007

Definition: Adenocarcinoma arising from intrahepatic bile duct epithelial cells.

Synopsis

- 10% of primary liver cancers
- High prevalence in southeast and eastern Asia.
- Usually age 60+ years; no gender preference; but mean age 40 years in those with primary sclerosing cholangitis or chronic inflammatory bowel disease
- Although previously thought to be not related to cirrhosis yet Japanese series suggest that HCV related cirrhosis is a major risk factor for cholangiocarcinoma in Japan.

Laboratory:

Normal AFP, occasional hypercalcemia.
- Adenocarcinoma with glandular and tubular structures, mucin production and dense desmoplasia; epithelial cells are anaplastic, cuboidal to columnar with eosinophilic cytoplasm and round central nuclei, tumor cells are heterogeneous even within the same gland but resemble bile duct cells, not hepatocytes; spread along hepatic plates, duct walls, via nerves (81% perineural)(3), but not sinusoidal; the stroma usually abundant may be circumferential around glands; associated with neutrophils; variable vascular invasion; no bile production(4).

- Patterns:

  • glandular pattern
  • tubular pattern
  • adenosquamous pattern
  • mucoepidermoid pattern
  • sarcomatous pattern
  • colloid pattern

- Variants

  • intrahepatic clear cell cholangiocarcinoma
  • signet ring cells
  • osteoclast giant cells
  • rhabdoid
  • clear cell
  • lymphoepithelioma-like pattern

Special stains

- Mucin (+)

Immunohistochemistry

- CK7 (+)
- CK20 (-)
- CEA monoclonal (+), polyclonal (+) (cytoplasmic and luminal, not canalicular)

Gene mutations

- KRAS (Ki-ras) mutations

Differential diagnosis

- hepatocellular carcinoma (tubular type) [Mucin (-) , CEA Monoclonal (-) polyclonal (+) (canalicular not cytoplasmic), AFP (+) and HepPar (+)]

- metastatic GI adenocarcinoma:

- metastasis mammary cvarcinoma [GCDFP (+) ER (+/-)]
- epithelioid hemangioendothelioma [vascular markers+, mucin-]
- benign bile duct proliferations [smaller, no atypia, non- infiltrating]

Differential diagnosis

The finding of foci of in-situ carcinoma, dysplasia or perineural/vascular invasion can be very valuable to differentiate a primary carcinoma form a metastasis.

Prognosis

- poor prognosis
- death usually within 6 months
- 5-year survival in resectable cases is 30%
- 50-75% metastasize to regional lymph nodes, lungs, vertebrae, adrenals, brain, elsewhere at autopsy.

Variants

- intrahepatic cholangiocarcinoma metastatic to the ovary (18043033)

See also

- cholangiocarcinomas

References

- Khunamornpong S, Siriaunkgul S, Suprasert P, Pojchamarnwiputh S, Na Chiangmai W, Young RH. Intrahepatic cholangiocarcinoma metastatic to the ovary: a report of 16 cases of an underemphasized form of secondary tumor in the ovary that may mimic primary neoplasia. Am J Surg Pathol. 2007 Dec;31(12):1788-99. PMID: 18043033