Solitary fibrous tumour of the pleura

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Solitary fibrous tumour of the pleura
Diagnosis in short

Pleural solitary fibrous tumour. H&E stain.

LM spindle cells usually with a bland cytology, patternless pattern, +/-ropy collagen
LM DDx CD34 +ve, CD99 +ve, BCL2 +ve, S-100 -ve
Site pleura of lung

Syndromes Doege-Potter syndrome

Signs +/-impaired respiratory function (due to mass effect)
Prevalence common for site, uncommon overall
Prognosis usu. benign
Treatment surgery

Solitary fibrous tumour of the pleura, also pleural solitary fibrous tumour, is a relatively common chest wall tumour.

The solitary fibrous tumour in general is dealt with in the solitary fibrous tumour article.

General

  • Uncommon overall[1] ~ 800 cases reported.[2]
    • Common for the anatomical site.
  • Usually benign ~ 80% of the time.[2]
  • Slow growing.[1]
  • Elderly.
  • May be large - impair respiratory function.[3]
  • May be associated with hypoglycemia.
    • Known as Doege-Potter syndrome.[4]

Gross/radiology

  • Chest wall mass.
    • May be "large".[2]

Microscopic

Features:

  • Spindle cells - usually bland cytology.
  • Patternless pattern.
  • +/-Ropy collagen.
  • Staghorn vessels.

Suggestive of malignant:[3]

  • Mitotic rate >4/10 HPF.

DDx:

Images

www:

IHC

Features:

  • CD34 +ve (19/23 cases[6]).
  • Ki-67 low (<5%).[3]
  • Vimentin +ve (23/23 cases[6]).
  • BCL2 +ve (23/23 cases[6]).
  • CD99 +ve (23/23 cases[6]).
  • SMA -ve/+ve (7 +ve/23 cases[6]).

Others:

  • WT1 -ve (+ve in 1 of 10 cases[7]).
  • Desmin -ve.[5]
  • CD117 -ve.[5]

Suggested stains:[citation needed]

  • CD34, CD99, BCL2, S-100, Ki-67.

Sign out

LEFT LUNG ("LARGE PLEURAL BASED MASS"), BIOPSY:
- SOLITARY FIBROUS TUMOUR.

Micro

The sections show cellular fibrous tissue with a patternless pattern. Rare staghorn vessels are present. No mitotic activity is readily apparent. No significant nuclear atypia is identified. No necrosis is apparent.

See also

References

  1. Jump up to: 1.0 1.1 Mordenti, P.; Di Cicilia, R.; Delfanti, R.; Capelli, P.; Paties, C.; Cavanna, L.. "Solitary fibrous tumors of the pleura: a case report and review of the literature.". Tumori 99 (4): e177-83. doi:10.1700/1361.15120. PMID 24326857.
  2. Jump up to: 2.0 2.1 2.2 Robinson, LA. (Oct 2006). "Solitary fibrous tumor of the pleura.". Cancer Control 13 (4): 264-9. PMID 17075563.
  3. Jump up to: 3.0 3.1 3.2 Abe, M.; Nomori, H.; Fukazawa, M.; Sugimura, H.; Narita, M.; Takeshi, A. (Oct 2013). "Giant Solitary Fibrous Tumor of the Pleura Causing Respiratory Insufficiency: Report of 3 Cases.". Ann Thorac Cardiovasc Surg. PMID 24088920.
  4. Roy, TM.; Burns, MV.; Overly, DJ.; Curd, BT. (Nov 1992). "Solitary fibrous tumor of the pleura with hypoglycemia: the Doege-Potter syndrome.". J Ky Med Assoc 90 (11): 557-60. PMID 1474302.
  5. Jump up to: 5.0 5.1 5.2 Zhu, Y.; Du, K.; Ye, X.; Song, D.; Long, D. (Jun 2013). "Solitary fibrous tumors of pleura and lung: report of twelve cases.". J Thorac Dis 5 (3): 310-3. doi:10.3978/j.issn.2072-1439.2013.05.19. PMID 23825765.
  6. Jump up to: 6.0 6.1 6.2 6.3 6.4 Chen, HJ.; Zhang, HY.; Li, X.; Guo, LX.; Wei, B.; Guo, H.; Bu, H.; Yang, K. et al. (Sep 2004). "[Solitary fibrous tumor: the clinicopathologic and immunohistochemical characteristics of 26 cases].". Sichuan Da Xue Xue Bao Yi Xue Ban 35 (5): 675-9. PMID 15460417.
  7. Bing, Z.; Pasha, TL.; Acs, G.; Zhang, PJ. (Jul 2008). "Cytoplasmic overexpression of WT-1 in gastrointestinal stromal tumor and other soft tissue tumors.". Appl Immunohistochem Mol Morphol 16 (4): 316-21. doi:10.1097/PAI.0b013e31815c2e02. PMID 18528287.