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Primary recurrent orbital schwannoma treated with surgical excision and Mitomycin-C.

ABSTRACT: Purpose:Primary orbital schwannoma (POS) is a slow growing, benign encapsulated peripheral nerve sheath tumor that occurs infrequently within the orbit. Recurrence of POS is extremely rare. Previous speculations for reasons of recurrence include incomplete excision and tumor seeding. Observations:We present the fifth case reported in the literature to date of POS that had 2 episodes of recurrences within 8 years after diagnosis, in which rapid and insidious relapses were observed after initial surgical resection. This is also the first reported recurrent POS in which topical Mitomycin-C (MMC) has been employed during surgical excision with an aim to prevent further recurrences. Conclusions:AND IMPORTANCE: Whilst complete surgical excision remained the standard for management for most POS, when there are multiple recurrences and in cases where total excision is not possible, addition of topical MMC may be an option that may bring about tumour regression as demonstrated in our case.

SUBMITTER: Leung KCP

PROVIDER: S-EPMC7315101 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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Primary recurrent orbital schwannoma treated with surgical excision and Mitomycin-C.

Leung Kai Ching Peter KCP Lam Nerissa Kei Yen NKY Chan Edwin E Ko Tak Chuen Simon TCS

American journal of ophthalmology case reports 20200615

<h4>Purpose</h4>Primary orbital schwannoma (POS) is a slow growing, benign encapsulated peripheral nerve sheath tumor that occurs infrequently within the orbit. Recurrence of POS is extremely rare. Previous speculations for reasons of recurrence include incomplete excision and tumor seeding.<h4>Observations</h4>We present the fifth case reported in the literature to date of POS that had 2 episodes of recurrences within 8 years after diagnosis, in which rapid and insidious relapses were observed ...[more]

PMID: 32613139

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Project description:BackgroundThe management of vestibular schwannoma has evolved over the past hundred years. In the last decades, surgery has been gradually replaced by radiation therapy as a primary treatment modality, particularly for small tumors, due to the less invasive nature and the compared reported outcomes in tumor control and hearing preservation. However, irradiation sometimes fails to stop tumor growth. In a long-term follow-up after primary fractionated stereotactic radiotherapy, the rate of treatment failure was reported as 3% and needed surgical salvage. For single-fraction modality, Hasegawa et al. reported salvage treatment after primary Gamma Knife radiosurgery in 8%, where 90% of these underwent surgery and 50% of those who were treated with a second gamma knife surgery required surgical intervention later. An increase in tumor volume by more than 10-20%, tumor growth after three years, and no return to pretreatment volume after transient swelling have been considered as tumor recurrence rather than pseudoprogression, a transient increase in tumor volume after radiotherapy that occurs up to 30% of cases. It has been reported that microsurgery after radiotherapy is more difficult, with most authors reporting a loss of defined arachnoid planes and worse cranial nerve outcomes, especially for hearing and facial nerve function.Case descriptionA 43-year-old female patient was incidentally (asymptomatic) diagnosed on a magnetic resonance imaging (MRI) scan harboring a left vestibular schwannoma, grade T2 (Hannover classification), in 2015. Neurologic examination was unremarkable, and audiometry testing was normal. She was initially treated with observation. Three years later, in 2018, the lesion had enlarged, becoming a grade T3a and reaching the cistern of the cerebellopontine angle. The tumor was then treated with fractionated stereotactic radiosurgery (5 sessions of 5 Gy). MRI scans in 2019 and 2020 showed slight tumor growth. This enlargement was attributed to a pseudoprogression after radiosurgery, and only observation was advocated. In 2022, 4 years later, after radiosurgery, the tumor was still growing, and the patient began to suffer from hearing loss. A failure treatment was considered, and microsurgery was indicated. The patient was counseled about the risk of functional nerve impairment, and surgical consent was obtained. A retro sigmoid approach was planned. A gross total resection was attempted due to the clear subperineural plane during tumor dissection and because it was the only option that would provide a cure for the patient. The adjacent neurovascular structures were firmly adhered to the tumor capsule, which represented a major challenge for microdissection. The tumor was soft, without significant bleeding. A total resection was achieved, and the facial nerve was anatomically preserved. The patient developed facial paresis (House-Brackmann III) in the immediate postoperative period, which improved at the 6-month follow-up. Hearing loss did not improve. Postoperative MRI showed total resection.ConclusionMicrosurgery after radiotherapy for vestibular schwannoma is challenging in terms of indication, when to indicate, resection target, difficulty in dissection due to local changes, and outcome. Gross total resection may be considered, as it is the only treatment that may provide a cure for the patient. However, the patient should be counseled about the risks.

Project description:Background Recurrence of basal cell carcinoma (BCC) after complete surgical excision is rarely reported. Risk factors for this negative outcome are not well-studied. We present the clinical and histological features of recurrent BCCs in our institution. Methods All patients between January 2016 to December 2020 whose primary BCCs were excised with free surgical margins according to the histopathology report, and represented later with local recurrence were included. The medical files were retrieved to record patient's age, sex, sun exposure, tumor site, size, clinical diagnosis, histopathology variant of primary lesion, least free margin distance of the original lesion, and recurrence time. Results Eighteen patients (11 males and 7 females ranged between 50 and 75 years old) fulfilled the inclusion criteria; all of their lesions were located in head and neck regions. The mean recurrence time was 31.2 months (11–86) and the histological variant was the same of primary in 17/18 patients. Primary tumors showed nodular subtype in 77.8% of patients and 55.6% of the primary tumors were less than 15 mm in diameter. Sun exposure history was given by 77.8% of patients while the rest of patients had non-significant exposure. All recurrent excised lesions were of free margin less than 4 mm. Conclusion We found that the primary tumors of all studied recurrent BCCs were excised with surgical margins less than 4 mm. We recommend follow up for all excised BCCs either those of low or high risk histological variants. Tumor size does not appear a considerable risk factor for local recurrence. Highlights • We report recurrent BCCs in 18 patients, all were located in head and neck region.• Sun exposure history was given by 77.8% of patients while the rest of patients gave a negative history of sun exposure.• Nodular histological subtype was seen in 77.8% of patients.• All primary tumors were excised by general or plastic surgeons with a surgical margin less than 4 mm.

Project description:To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) at the King Khaled Eye Specialist Hospital, Saudi Arabia. Demographics, clinical presentation, and surgical intervention data were collected. Histopathological specimens were studied with hematoxylin-eosin, Alcian blue, and immunohistochemical markers; S-100, CD44, CD117, smooth muscle actin (SMA), neurofilament, and Ki-67. Of the 43 NFs specimens, 20 were primary and 23 recurrent tumors. For primary NF, the ratio of plexiform to the diffuse type was 13:7, however in recurrent tumors was 3:8 after the first recurrence, and 1:5 after multiple recurrences. Of the 17 patients with primary tumors that had paired recurrent tumors, 12/17 (70.6%) primary NFs were plexiform and 5/17 (29.4%) were diffuse. However, when tumors recurred, 13/17 tumors (76.5%) were diffuse and only 4/17 tumors (23.5%) had a plexiform pattern. The odds of a tumor having a diffuse pattern in recurrent NF was significantly higher than the plexiform pattern [OR = 7.8 (95% confidence interval 1.69:36.1) P = 0.008]. Primary plexiform NFs underwent an excision at a significantly younger age than the diffuse type. Recurrent NFs had significantly higher CD44, CD117, and neurofilament labeling (P = 0.02, P = 0.01 and P<0.001 respectively) but had significantly decreased Alcian blue, and S-100 labeling (P = 0.03, and P = 0.02 respectively) compared to primary tumors. SMA and Ki-67 proliferation index were not different between primary and recurrent NFs (P = 0.86, and P = 0.3 respectively). There appears to be a high risk for primary plexiform NFs to develop a diffuse histologic pattern when they recur. Immunohistochemical staining suggests a role of mast cells (CD117) and expression of infiltration makers (CD44) in the transformation of plexiform tumors to the diffuse phenotype.

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Primary recurrent orbital schwannoma treated with surgical excision and Mitomycin-C.

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Primary recurrent orbital schwannoma treated with surgical excision and Mitomycin-C.

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