Extrapleural solitary Definition ‘A ubiquitous mesenchymal tumour of fibro blastic type, which shows a prominent haemangiopericytomavike branching vas cular pattern Inthe past, most cases were termed *haemangiopericytoras.” ICD-0 code Solitary fiorous tumour Be15/1 Malignant solitary fibrous tumour 8815/3 ‘Synonyms Haemangiopericytoma (obsolete): giant coll angiofibroma fibrous tumour Fat-lorming variant: ipomatous haeman- gopericytoma Epidemiology Extrapleural solitary fibrous tumours (GF Is) are uncommon mesenchymal neo- plasms that are anatomically ubiquitous and occur most often in middle-aged adults aged 20-70 years, wih cases in children and adolescents being rare. Males and females are affected equally, except in the case of the fat-lorming SFT variant, which tends to slightly predomi nate in males (male to female ratio, 3: 2) ne Ber yied Fg 2072 Esra lay foes una. A Te tour eens os awe eumsbedut nencapsed mass. B Tati strong mmuroreactiy ol the nour cs for CDSS, but isis ot speci, A Fig. 2479 gel str Roeus know Noe ptanes achive B Stor an ptvaraayaritch ‘we canon. C Kota ype depen of calogen rogue 8 Fil fibroblasic wmours (Tye and sping cls wih her veal nce, CDM. Fletcher JA, Bridge weC. Lee Fig 3.074 Exraniowa solar feos tumour. Gros, the lesion wel deoealed end shows mull whith appoaranco on cu eocton Sites of involvement SFTs may be found al any location: 40% are found in subcutaneous tissue, while others arise in deep soft tissue of extrem- ities or in the head and neck region (esp- ‘ecially the orbit), thoracic wall, media stinum, pericardium, reroperitoneum, and abdominal cavity. Other locations de- scribed include meninges, spinal cord, salivary gland, lung, thyroid, liver, the gas~ trointestinal act, adrenal, kidney, urinary bladder, prostate, spermatic cord, testis, bbone, and skin 896,983,1119,1121,1827, 2039, 20882681 The fat-forming variant of SFT often al- fects the deep solt tissues of a wide vari- ety of anatomical locations, including the lower extremity (especially the thigh). trunk, abdorninopelvic regions (especially retroperitoneum), and head and neck. Clinical features ‘Most tumours present as well-delineated, slowly growing, painless masses. Large tumours may give rise to compression symptoms, especially in the nasal cavity, frbil and meninges. Malignant tumours. are often locally infiltrative (924,925, 1121,2690), Rarely, large tumours may be the source of paraneoplastic syndromes such as hypoglycaemia due to the pro- duction of IGF2 (2645), Macroscopy Most SF's are well circumscribed, often Parially encapsulated masses, measuring between 1 and 250m (median, §-8¢m;ig. 3074 Magnan extol soar Sa unow. A Hyperceulariy and marked cogil apa. Note the. chil. CHypeteeuoty, mares oylgcal ap, and res ofunour rere ‘generally salle in the head and neck re- gion). On section, they frequently have a multinodular, whitish and firm appearance; myxoid and haemorhagic changes are Infrequent (924,1121,1827,2039,2684, 2830], Tumour necrosis and infiltrative ‘margins (about 10% of cases) are mostly ‘observed in locally aggressive or malig ‘nant tumours (925,1121,2830). Histopathology Typical SFTs show a patterniess architec: ture characterized by a combination of hypocollular and hyporcellular areas sep: ‘arated by thick bands of hyalnized, some- times Keloidal, collagen and thin-walled Fig. 3.076 Cohiar solitary frou inouc (SFT). A Nolte evanly cso cebary (rn corast wal SFT) andthe pempent banding vascar pate. B Even inthe tore so aces, tumour cls are ranges around numerous in-waled vessel. Tour branching haemangiopericytoma-ike ves: sels. There may also be perivascular hyalinization. Tumour cells are ovoid to spindle-shaped with limited pale cyto- plasm having indistinct borders and cis- persed chromatin within vesicular nuclei Rarely, epithelioid or rhabdoid cells can be found focally. Myxoid change, areas of fibrosis and interstitial mast cells are com> monly observed. Mitoses are generally scarce, rarely exceeding 3 mitoses per 10 HPF. Some SFTs may contain giant mult- nucleate stromal cells and pseudo-vascu- lar spaces (416,1121,2088}, being for- ‘merly known as "giant cell angiofibroma’. Malignant SFTs are usually hypercellular ‘er CDi vient. Alesion suchas hs would have bean belod"haomangiopariyoa no past. typical ios B Moder cellar ra wi bk ie 4 Cogs lesions, showing increased mitoses (> 4 mitoses per 10 HPF), variable cytological alypia, tumour necrosis, and/or infitrative margins (925,1121, 2830}, of which mi- toses seem to be most prognostic {998}. Rare cases show abrupt transition from conventional benign-appearing SFT to high-grade sarcoma, likely representing & form of dedifferentiation {1963}. Lesions may show cytological atypia in the ab- ‘sence of mitoses or necrosis. Fat-lorming SFT is an uncommon, usually slowly growing, mesenchymal neoplasm closely resembling typical or cellular SFT with, in addition, a variably prominent adipocytic component (883,1061,2034) Is are smal with monomorphic nucle and eosbephiceveptasm. C Ouse psitvy Extrapleural solitary fibrous tumour BYmemandopercyloma-ke branching veselsB Aninimate admire of bland spindle cals and make aoc. Morphologically, itis a well-demarcated neoplasm consisting of a varying combi- ‘ation of pattemless cellular afeas, promi- nent naemangiopericytomaike vessels, variably collagerized extracellular marix, ‘and admixed mature asipocytes, which range from being singly scatteredta being tho predominant component. A small sib- 8et of fat-lorming SFIs show malignant his- ‘ological features (as Seen in “classical” ma- lignant SFTs);ofnote, ipoblasts andlor atyp- ical lipomatous tumourike areas can be found in these malignant tumours, closely mimicking iposarcoma (141,106 11585). Immunophenotype Tumour calls are characteristically im- ‘munoreactive for CD34 (90-95% of cases) 1396, 1827,2039,2684,2830); 20-35% of ‘cases are variably postive for EMA and ‘SMA. Focal and limited reactivity for $100 protein, Keratine and/or desmnin has also ‘occasionally been reported (924,2830} Fat-forming SFT has a similar immuno- phenotype (1061, 1585}. 82 Genetics SFTs are karyotypicaly diverse, with aber- rations detected primarily in tumours > 10cm in diameter. Recurrently involved breakpoints are few, although structural rearrangements of 2p21, 4413, 6p11, ‘9p22-23, 9q22, 9q31-32, 12q15, and. 112924 have each been identified in more than one SFT (2691,2768}. With respect to imbalances, gain of chromosomes 5, 8, and 21 and loss of 1 oF 13q are most prominent [1741, 189, 1963). 1253muta- tions are rare, although TP53 overexpres- sion has been obsetved in the high-grade ‘component of benign-appearing SFTs ex hibiting an abrupt transition to nondistinc- tive high-grade sarcoma [1951,1963). Mu- tation testing of various genes encoding proteins with potential for targeted therapy has been negative, with the exception of rare isolated cases exhibiting missense gain-of-function mutations involving the enzymatic tyrosine kinase domain of POGFRA {1078,2465.2634). IGF? loss of Imprinting corresponds with IGF2 overex- pression in some SFTs (1078), Prognostic factors Although most cases are benign, the be- haviour of SFT can be unpredictat About 10% behave aggressively, and lo- cal or distant recurrence can occur many years alter primary resection (924,121, 1281,2830). Although there is no strict correlation between morphology and be- haviour, malignant hislology (especially high mitotic counts) remains the best in- dicator of poor oulcome {520,998}; most (but not all) histologically benign SFTs prove to be non-recurring and non-metas- \asizing lesions, and most histologically malignart tumours behave aggressively Lesions located in the mediastinum, ab- omen, pelvis, retroperttoneum, andor meninges also tend to behave more ag- gressively than those in the limbs [520,924,925,1121,2830}. Tumour size > 10cm and positive surgical margins also predict poorer prognosis {998} Metastases are most frequently observed in lungs, bone and liver {2830}. The be- haviour o! SFT with atypia alone is unpre- dictable