Extrapleural solitary fibrous tumour
Definition
An extra-pleural (myo)fibroblastic tumour closely resembling pleural SFT. Previously, these tumours were commonly diagnosed as haemangiopericytomata.
Sites
This is a rare tumour, as common at extra-pleural sites as pleural27, usually found in adults, rarely in children, at a large range of anatomical sites, which include:
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subcutis (40% of cases)21, deep soft tissues12,13,14,15,20,21,22, arm25, back25, nasal cavity9,12,17,21, nasopharynx21, oral cavity18, tongue, larynx, salivary gland4, thyroid, orbit2,7,12,13, meninges11,13, lung16,24, pericardium, mediastinum16,24, breast , peritoneum8,12,24, retroperitoneum12,13,15,22,24, stomach, intestines, liver5, gallbladder28, kidney3,13,19, adrenal glands, periosteum6, spinal cord, testis, prostate21,23, seminal vesicle,23, bladder21,23, vulva22, cervix13, skin
Clinical features
Large tumours may rarely give rise to hypoglycaemia by the secretion of an insulin-like factor. Rarely, there is nail clubbing27.
Macroscopic appearances
Most tumours are well-demarcated. Malignant tumours may have infiltrative margins and show necrosis. The cut surface is usually firm and white; sometimes there is cyst formation or haemorrhage.
Histopathology
The histology is as for that of the pleural tumours. The presence of adipocytes produces overlap with lipomatous haemangiopericytoma and giant cell angiofibroma. A giant cell-rich case has been reported from the gallbladder28.
10-15 % show malignant behaviour. Malignancy is associated with increased cellularity, cytological atypia, 4 or more mitoses per 10 HPF and infiltrative margins. In rare cases, dedifferentiation gives rise to abrupt transition to a high grade sarcoma. Mediastinal, abdominal, retroperitoneum and pelvis are more likely to show aggressive behaviour.
Immunohistochemistry
|
CD34
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95% (78/82)1, 12/1221, 7/922, 16/1618, 3/325,
1/1(giant cell-rich SFT of the gallbladder)28
|
|
bcl-2
|
96% (72/75)1, 12/1218,
1/1(giant cell-rich SFT of the gallbladder)28
|
|
CD99
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31/311, 12/1221, 8/1218,
1/1(giant cell-rich SFT of the gallbladder)28
|
|
vimentin
|
19/1910, 12/1221, 13/1318, 3/325,
1/1(giant cell-rich SFT of the gallbladder)28
|
|
Factor XIIIA
|
10/1018
|
|
muscle-specific actin
|
0/510
|
|
desmin
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0/510
0/1(giant cell-rich SFT of the gallbladder)28
|
|
SMA
|
2/12(rare positive cells)18
, 0/1(giant cell-rich SFT of the gallbladder)28
|
|
S-100
|
0/1010
, 0/1(giant cell-rich SFT of the gallbladder)28
|
|
CD31
|
0/1(giant cell-rich SFT of the gallbladder)28
|
|
CD68
|
0/1010
|
|
CD117
|
0/1(giant cell-rich SFT of the gallbladder)28
|
|
cytokeratin
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0/1910, 0/510,
0/1(AE1/AE3: giant cell-rich SFT of the gallbladder)28
|
|
EMA
|
0/1(giant cell-rich SFT of the gallbladder)28
|
| |
|
Related entities
Possibly part of the same spectrum are:
Differential diagnosis
Very much dependent on anatomical site.
Prognosis
About 10% of extra-pleural tumours develop[ local or distant recurrence. Increased cellularity, more than 4 mitoses per 10 HPF, focal necrosis and cytological atypia correlate with malignant behaviour27. Malignant cases metastasis to lung, bone and liver.
References
0 World Health Organization Classification of Tumours, Tumours of Soft Tissues and Bone, IARC Press 2002.
1 Ordonez NG. Localized (solitary) fibrous tumor of the pleura. Adv Anat Pathol 2000;7:327-340.
2 Dorfman, D. M., To, K., Dickersin, G. R., Rosenberg, A. E., Pilch, B. Z. Solitary fibrous tumor of the orbit. Am J Surg Pathol 1994;18;281-7.
3 Gelb, A. B., Simmons, M. L., Weidner, N. Solitary fibrous tumor involving the renal capsule. Am J Surg Pathol 1996;20:1288-95.
4 Gunhan, O., Yildiz, F. R., Celasun, B., Onder, T., Finci, R. Solitary fibrous tumour arising from sublingual gland: report of a case. J Laryngol Otol 1994;108:998-1000.
5 Kottke-Marchant, K., Hart, W. R., Broughan, T. Localized fibrous tumor (localized fibrous mesothelioma) of the liver. Cancer 1989;64:1096-102.
6 O'Connell, J. X., Logan, P. M., Beauchamp, C. P. Solitary fibrous tumor of the periosteum. Hum Pathol 1995;26:460-2..
7 Westra, W. H., Gerald, W. L., Rosai, J. Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol 1994;18:992-8.
8 Young, R. H., Clement, P. B., McCaughey, W. T. Solitary fibrous tumors ('fibrous mesotheliomas') of the peritoneum. A report of three cases and a review of the literature. Arch Pathol Lab Med 1990;114:493-5.
10 Brunnemann, R. B., Ro, J. Y., Ordonez, N. G., Mooney, J., El-Naggar, A. K., Ayala, A. G. Extrapleural solitary fibrous tumor: a clinicopathologic study of 24 cases. Mod Pathol 1999;12:1034-42.
11 Carneiro, S. S., Scheithauer, B. W., Nascimento, A. G., Hirose, T., Davis, D. H. Solitary fibrous tumor of the meninges: a lesion distinct from fibrous meningioma. A clinicopathologic and immunohistochemical study. Am J Clin Pathol 1996;106:217-24.
13 Hasegawa, T., Matsuno, Y., Shimoda, T., Hasegawa, F., Sano, T., Hirohashi, S. Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior. Hum Pathol 1999;30:1464-73.
14 Suster, S., Nascimento, A. G., Miettinen, M., Sickel, J. Z., Moran, C. A. Solitary fibrous tumors of soft tissue. A clinicopathologic and immunohistochemical study of 12 cases. Am J Surg Pathol 1995;19:1257-66.
15 Vallat-Decouvelaere, A. V., Dry, S. M., Fletcher, C. D. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol 1998;22:1501-11.
16 van de Rijn, M., Lombard, C. M., Rouse, R. V. Expression of CD34 by solitary fibrous tumors of the pleura, mediastinum, and lung. Am J Surg Pathol 1994;18:814-20.
18 Alawi, F., D. Stratton, et al. (2001). "Solitary fibrous tumor of the oral soft tissues: a clinicopathologic and immunohistochemical study of 16 cases." Am J Surg Pathol 25(7): 900-10.
19 Wang, J., L. M. Weiss, et al. (2004). "Usefulness of immunohistochemistry in delineating renal spindle cell tumours. A retrospective study of 31 cases." Histopathology 44(5): 462-71.
21 Mentzel, T., T. C. Bainbridge, et al. (1997). "Solitary fibrous tumour: clinicopathological, immunohistochemical, and ultrastructural analysis of 12 cases arising in soft tissues, nasal cavity and nasopharynx, urinary bladder and prostate." Virchows Arch 430(6): 445-53.
22 Nielsen, G. P., J. X. O'Connell, et al. (1997). "Solitary fibrous tumor of soft tissue: a report of 15 cases, including 5 malignant examples with light microscopic, immunohistochemical, and ultrastructural data." Mod Pathol 10(10): 1028-37.
23 Westra, W. H., R. T. Grenko, et al. (2000). "Solitary fibrous tumor of the lower urogenital tract: a report of five cases involving the seminal vesicles, urinary bladder, and prostate." Hum Pathol 31(1): 63-8.
24 Goodlad, J. R. and C. D. Fletcher (1991). "Solitary fibrous tumour arising at unusual sites: analysis of a series." Histopathology 19(6): 515-22.
25 Hasegawa, T., T. Hirose, et al. (1996). "Solitary fibrous tumor of the soft tissue. An immunohistochemical and ultrastructural study." Am J Clin Pathol 106(3): 325-31.
26 Chan, J. K. (1997). "Solitary fibrous tumour--everywhere, and a diagnosis in vogue." Histopathology 31(6): 568-76.
27 Graadt van Roggen JF, Hogendoorn PCW. Solitary fibrous tumour: the emerging clinicopathologic spectrum of an entity and its differential diagnosis. Current Diagnostic Pathology 2004;10:229-235.
28 Lazure T, Dimet S, Ndiaye N, et al. Giant cell-rich solitary fibrous tumour of the gallbladder. First case report. Histopathology 2007; 50:805-7
This page last revised 18.5.2007.
©SMUHT/PW Bishop