PNST Dotaz Zobrazit nápovědu
OBJECTIVE: To describe the clinical and sonographic characteristics of benign, retroperitoneal, pelvic peripheral-nerve-sheath tumors (PNSTs). METHODS: This was a retrospective study of patients with a benign, retroperitoneal, pelvic PNST who had undergone preoperative ultrasound examination at a single gynecologic oncology center between 1 January 2018 and 31 August 2022. All ultrasound images, videoclips and final histological specimens of benign PNSTs were reviewed side-by-side in order to: describe the ultrasound appearance of the tumors, using the terminology of the International Ovarian Tumor Analysis (IOTA), Morphological Uterus Sonographic Assessment (MUSA) and Vulvar International Tumor Analysis (VITA) groups, following a predefined ultrasound assessment form; describe their origin in relation to nerves and pelvic anatomy; and assess the association between their ultrasound features and histotopography. A review of the literature reporting benign, retroperitoneal, pelvic PNSTs with preoperative ultrasound examination was performed. RESULTS: Five women (mean age, 53 years) with a benign, retroperitoneal, pelvic PNST were identified, four with a schwannoma and one with a neurofibroma, of which all were sporadic and solitary. All patients had good-quality ultrasound images and videoclips and final biopsy of surgically excised tumors, except one patient managed conservatively who had only a core needle biopsy. In all cases, the findings were incidental. The five PNSTs ranged in maximum diameter from 31 to 50 mm. All five PNSTs were solid, moderately vascular tumors, with non-uniform echogenicity, well-circumscribed by hyperechogenic epineurium and with no acoustic shadowing. Most of the masses were round (n = 4 (80%)), and contained small, irregular, anechoic, cystic areas (n = 3 (60%)) and hyperechogenic foci (n = 5 (100%)). In the woman with a schwannoma in whom surgery was not performed, follow-up over a 3-year period showed minimal growth (1.5 mm/year) of the mass. We also summarize the findings of 47 cases of benign retroperitoneal schwannoma and neurofibroma identified in a literature search. CONCLUSIONS: On ultrasound examination, no imaging characteristics differentiate reliably between benign schwannomas and neurofibromas. Moreover, benign PNSTs show some similar features to malignant retroperitoneal tumors. They are solid lesions with intralesional blood vessels and show degenerative changes such as cystic areas and hyperechogenic foci. Therefore, ultrasound-guided biopsy may play a pivotal role in their diagnosis. If confirmed to be benign PNSTs, these tumors can be managed conservatively, with ultrasound surveillance. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
- MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory nervové pochvy * diagnóza patologie chirurgie MeSH
- nádory pánve * MeSH
- neurilemom * diagnostické zobrazování patologie MeSH
- neurofibrom * diagnóza patologie chirurgie MeSH
- retroperitoneální nádory * diagnostické zobrazování MeSH
- retrospektivní studie MeSH
- ultrasonografie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
Tumory vycházející z obalů periferních nervů tvori významnou část všech tumorů periferních nervů. V letech 998-2000 jsme na naši klinice operovali 7 těchto pacientů - 3 ženy, 4 muže. Konečná diagnóza byla stanovena na základě histologického vyšetření. U všech pacientů byla provedena totální exstirpace. Ve 4 případech byla kontinuita nervu zachována (3x neurofibrom, Ix schwannom), ve 3 případech musel být nerv přerušen (Ix neurofibrom, Ix plexiformní neurofibrom u Recklinghausenovy choroby, Ix maligní schwannom). Pacienti, u kterých bylo možno zachovat kontinuitu nervu měli dobrý výsledek léčby. Ze 3 pacientů, u kterých byl nerv přerušen, byla v případě benigního neurofibromu provedena transplantace s kompletní reinervací, avšak u pacienta s Recklinghausenovou chorobou a u pacientky s maligním schwannomem zůstává trvalá leze postiženého lervu. Závěrem lze konstatovat, že histologická klasifikace předurčuje možnosti chirurgické léčby tumorů periferních nervů.
The influence of histological classification on the options of the peripheral nerve tumors surgery Peripheral nerve sheath tumors (PNST) constitute significant part of all tumors of peripheral nerves. In 1998-2000 years we have operated 7 such patients - 3 females, 4 males in our department. The definite diagnosis was made by histology. Total removal was performed in all patients. The nerve continuity was preserved in 4 cases (3x neurofibroma, Ix schwannoma) and nerve had to be disconnected in 3 cases (Ix neurobibroma, Ix plexiform neurofibroma as a part of von Recklinghausen disease (VRD), Ix malignant schwannoma). Good neurological outcome was in patients with preserved nerve continuity. Out of 3 patients with the need of nerve disconnection. graft and complete reinervation was performed in case of benign neurofibroma, but in the patient with von Recklinghausen disease and in the patient with malignant schwannoma the permanent laesion of nerve remained. We can conclude that histological classification directly predetermine the options of the peripheral nerve tumors surgery.
