Outcome of surgery in patients with solitary fibrous tumors of the pleura

Abstract

The essential step in the treatment of a patient with a diagnosis of benign solitary fibrous tumor of the pleura is complete resection. These patients should be followed up for a long time because of the possibility of late recurrence. Due to the rarity of these tumors, there has been no systematic assessment of the role of adjuvant therapy for benign solitary fibrous tumor of the pleura.

Mesenchymal/non-epithelial mimickers of neuroendocrine neoplasms with a focus on fusion gene-associated and SWI/SNF-deficient tumors

“…Regarding the chromograninA labeling, our data as well as the data from literature revealed a striking dichotomy of synaptophysin and chromograninA expression. Synaptophysin-chromograninA co-expression was observed in 34% of our MN-NEs and in 5/92 (5%) neoplasms from the literature [ 2 – 4 , 12 , 15 , 18 , 19 , 23 , 30 , 33 , 34 , 36 , 38 – 42 , 44 , 46 , 47 , 49 , 50 , 54 ] (see Table 1 and 2 ), suggesting that the dichotomy in the synaptophysin-chromograninA expression is a common finding in these neoplasms and contrasts sharply with the expression rates in PanNETs, in which chromograninA labels 91% of the synaptophysin positive tumors [ 52 ]. Among our chromograninA, negative MN-NEs were SFT, ES, SMARCA4-deficient neoplasm, melanoma, and chordoma.…”

“…The other feature of MN-NEs, the patchy expression of synaptophysin and the absence or focality of chromograninA staining, are also rare in NECs, and particularly in NETs, which instead show a diffuse and usually intense staining. The complete absence of chromograninA should always arouse suspicion against the diagnosis of 40,49 0 (0/23) 0 (0/23) 0 (0/23) 0/0 unspecified 50 (1/2) 0 (0/2) 0 (0/2) 0/1 Soft tissue/retroperitoneum Alveolar soft part sarcoma 36,53,26,16,16,31,20 0 (0/7) NA NA NA Soft tissue 0 (0/5) 0 (0/4) 0 (0/4) 0/0 Case reports: soft tissue, head and neck, prostate, mediastinum Solitary fibrous tumor 30,33,19 17 (4/23) 0 (0/23) 0 (0/23) 0/4 Central nerve system 0 (0/28) 0 (0/28) 0 (0/28) 0/0 Mostly extrapleural soft tissue 0 (0/13) 0 (0/13) 0 (0/13) 0/0 Pleura Epithelioid sarcoma 23 60 In conclusion, MN-NEs represent only a small group among the various NEN mimickers, but have been increasingly noticed in recent years and are particularly found among new entities of mesenchymal tumors that share an epithelioid-mesenchymal morphology, show a variegated immunophenotype, are characterized by gene fusion alterations in the CREB family or mutations of SMARC genes, and can occur as pancreatic primaries. All these tumors may cause diagnostic problems in the distinction from NECs and to a minor degree also NETs.…”

“…In these neoplasms, synaptophysin was recorded in approximately half to two-thirds of the cases, making synaptophysin an important “pitfall marker” in these neoplasms. Synaptophysin expression was also relatively common in EWS (on average 23%) [ 34 , 40 , 45 ] and melanoma (on average 35%) [ 47 , 50 ], while in SFT [ 19 , 30 , 33 ], chordoma [ 39 ], and the pancreatic SEMN [ 11 ], synaptophysin expression seems to be rare. A new finding is the synaptophysin positivity in one of our two ASPS [ 16 , 17 , 20 , 21 , 26 , 36 , 53 ] and the epithelioid mesenchymal neoplasm with FUS-CREM gene fusion [ 6 , 9 , 35 ].…”

Benign Solitary Fibrous Tumor of the Pleura

“…The primary treatment approach for SFTP is surgical resection, with the completeness of the initial resection being crucial in preventing recurrence [ 3 ]. VATS has been used in the surgical treatment of pedunculated and free-moving tumors originating from the visceral pleura, demonstrating its effectiveness in managing such cases with a favorable long-term prognosis and a minimally invasive approach [ 16 ].…”

“…It is a slow-growing, localized fibrous tumor. Total en bloc resection is pivotal in treating a patient diagnosed with benign SFTP, which is linked to an excellent prognosis [ 3 ]. Despite being initially characterized as a pleura-based lesion, the SFTP has since been acknowledged as a widespread neoplasm that can manifest in various anatomical locations [ 4 ].…”