Case of malignant transformation of vagus nerve schwannoma to angiosarcoma

Ogawa, Tetsuya; Kato, Takashige; Ikeda, Atsuhiko; Nishimura, Kunihiro; Tsuchiya, Yoshimasa; Okamoto, Hiroki; Takahashi, Emiko; Yokoi, Toyoharu

doi:10.1002/hed.23390

Cited by 10 publications

(3 citation statements)

References 11 publications

(13 reference statements)

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“…Recently, a new case of malignant transformation to angiosarcoma is very interestingly discussed. [ 7 ] The accumulation of more cases in the literature will help physicians decide on future diagnostic and treatment strategies.…”

Section: Discussionmentioning

confidence: 99%

Malignant transformation of vagal nerve schwannoma in to angiosarcoma: A rare event

Agarwal

Munjal

Rai

et al. 2015

J Surg Tech Case Report

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Schwannomas are benign, rare peripheral nerve sheath tumors that occur in the head and neck region. Some physicians opt to closely observe cases of schwannoma of the neck on an outpatient basis rather than to perform radical surgery. However, there is a possibility, albeit rare, of malignant transformation of a benign schwannoma. Here, we are reporting the first case from the Indian subcontinent which was transformed into the angiosarcoma from benign vagal schwannoma over a long period. A 47-year-old male patient complaining of left sided neck swelling since last 12 years, swelling was insidious in onset, gradually progressive very slowly. In last 2 months, the size of the swelling was suddenly increased. On examination, there was an approximately 6 cm × 6 cm of size, firm, nodular, well-defined, nontender swelling in the left lateral part of the neck. Fine-needle aspiration cytology (FNAC) revealed paraganglioma and magnetic resonance imaging demonstrated very clearly a tumor, its morphology, and its relation to the surrounding structures, the tumor was thought to be a vagal schwannoma. Surgery was done, and the whole of the tumor was removed in toto. On final histopathological diagnosis, the tumor was proved to be angiosarcoma developed from vagal schwannoma. Postoperative chemotherapy was given but due to distant metastasis, the patient died. Long standing neck masses can convert into malignancy as in our case, therefore, work up of the patient should be done properly. Multiple FNAC should be done because single FNAC can give the false negative result as in our case. This was our diagnostic drawback not to do multiple computed tomography guided FNAC.

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Section: Discussionmentioning

confidence: 99%

Malignant transformation of vagal nerve schwannoma in to angiosarcoma: A rare event

Agarwal

Munjal

Rai

et al. 2015

J Surg Tech Case Report

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“…1 Schwannomas with angiosarcomatous changes are particularly rare, with only 22 cases previously reported in the literature. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] These hybrid tumors are challenging to diagnose and have a significant impact on patient treatment as well as subsequent outcomes. We present a 70-year-old female patient with an angiosarcoma arising within a schwannoma found in the right sciatic notch, highlighting the difficulties of establishing a definitive diagnosis.…”

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confidence: 99%

Angiosarcoma arising in a schwannoma of the peripheral nervous system: illustrative case

Lakomkin

Torres‐Mora

Dozois

et al. 2022

Journal of Neurosurgery: Case Lessons

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BACKGROUND Schwannomas of the peripheral nerves are benign tumors that can very rarely undergo malignant transformation. These lesions are particularly challenging to diagnose via noninvasive techniques but can have significant implications for treatment. OBSERVATIONS This is a case of a 70-year-old female with a prior history of a right sciatic notch tumor that was diagnosed as a conventional schwannoma via histology from an initial biopsy and subsequent surgical debulking. Unfortunately, she experienced significant worsening of her motor deficit, whereby her postoperative foot weakness progressed to complete foot drop in less than 2 years. In addition, she demonstrated significant radiological progression, with more than 1 to 2 cm of growth in each dimension at her subsequent evaluation, along with intractable right leg pain. An additional operation was performed to completely remove the 7 × 8 cm tumor, and histology demonstrated angiosarcoma within a schwannoma. There was no evidence of recurrence at 15 months, and the patient had significant improvement in her pain. LESSONS Rapidly worsening function and radiological progression are not typically seen with conventional benign nerve sheath tumors and should prompt consideration of other lesions. Angiosarcoma within schwannoma is a rare pathology and optimal therapies for these tumors in terms of surgical timing and adjuvant therapy are still unknown.

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“…6,9,10 While a conservative approach may seem prudent at the time of diagnosis, malignant transformation of vagal schwannomas has been known to occur, and the risks of surgical resection at a time when the tumor has grown to a much larger size may be far greater than when the tumor is small, particularly when considering the proximity of these tumors to great vessels. 4,18,19 In addition, recent studies have demonstrated minimal postoperative neural deficits with the use of intraoperative nerve-sparing techniques, including intracapsular enucleation, endoscopic approach, and microsurgical dissection with the aid of electromyographic monitoring of the recurrent laryngeal nerve. [20][21][22][23] Nonetheless, tumor characteristics may preclude nerve-sparing techniques, and unilateral sacrifice of the vagus may be required for complete tumor excision.…”

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confidence: 99%

Favorable Swallowing Outcomes following Vagus Nerve Sacrifice for Vagal Schwannoma Resection

Patel

Eytan

Bishop

et al. 2016

Otolaryngol.--head neck surg.

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Objective To determine the impact of unilateral vagal sacrifice for vagal schwannoma on postoperative swallowing function. Study Design Case series, chart review. Setting Academic medical institution. Subjects and Methods Ten patients underwent vagus nerve sacrifice for vagal schwannoma resection. Archived pathology records dating from 1985 through 2012 at our institution were retrospectively queried for cases of vagal schwannoma with vagus nerve sacrifice. Medical records were abstracted for demographic and disease information as well as cranial nerve and swallowing function. Preoperative and postoperative cranial nerve function, subjective and objective measures of swallowing function, Functional Oral Intake Scale (FOIS) level, and need for vocal fold medialization were variables collected. Data were analyzed with summary statistics. Results The patients who underwent vagal sacrifice for vagal schwannoma at our institution had a mean age of 42.3 years (median, 44 years; range, 15-63 years) and follow-up of 35.6 months (median, 9 months; range, 1-115 months). Most presented with no preoperative cranial nerve deficit or difficulty swallowing. Immediately postoperatively, 90% had a vagus nerve deficit, but 50% had no subjective difficulty swallowing, and 70% had a FOIS level of 7 at postoperative hospital discharge. Within 1 month after surgery, 70% had normal swallowing function according to a modified barium swallow study. A full diet was tolerated by mouth within an average of 2.7 days (median, 2 days; range, 1-6 days) after surgery in this cohort. Seventy percent required vocal fold medialization postoperatively for incomplete glottic closure. Conclusion Vagal nerve sacrifice during resection of vagal schwannoma can be performed with normal postoperative swallowing function.

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Case of malignant transformation of vagus nerve schwannoma to angiosarcoma

Abstract: Schwannomas sometimes undergo malignant transformation; therefore, surgery should be recommended. If follow-up observation is chosen, MRI and FNA should be regular and patients should sign a statement acknowledging awareness of the potential for malignant transformation.

Cited by 10 publications

References 11 publications

Malignant transformation of vagal nerve schwannoma in to angiosarcoma: A rare event

Malignant transformation of vagal nerve schwannoma in to angiosarcoma: A rare event

Angiosarcoma arising in a schwannoma of the peripheral nervous system: illustrative case

Favorable Swallowing Outcomes following Vagus Nerve Sacrifice for Vagal Schwannoma Resection

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