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. 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. 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. 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. 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. 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.