Epithelioid sarcoma of the sciatic nerve perineural sheath: a mimic of nerve sheath tumor

Agarwal, Manisha; Shatma, M C; Deol, P.S.; Mehta, Vibha; Sarkar, Chitra

doi:10.1007/bf03033727

Cited by 15 publications

(11 citation statements)

References 7 publications

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“…Only two previous case reports of epithelioid sarcoma occurring in relation to nerves have been reported: one in the perineural sheath of the sciatic nerve 12 and the other within the median nerve 13 . Both cases had neurogenic symptoms for at least 2 years; our case had been symptomatic for 4 years.…”

Section: Discussionmentioning

confidence: 52%

“…Enhancement following intravenous gadolinium may be homogenous, patchy or marginal. The margins of the tumour are usually infiltrative.. [11][12][13] Only two previous case reports of epithelioid sarcoma occurring in relation to nerves have been reported: one in the perineural sheath of the sciatic nerve 12 and the other within the median nerve. 13 Both cases had neurogenic symptoms for at least 2 years; our case had been symptomatic for 4 years.…”

Section: Discussionmentioning

confidence: 99%

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Two malignant peripheral nerve lesions of non‐neurogenic origin

et al. 2012

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Section: Discussionmentioning

confidence: 52%

Section: Discussionmentioning

confidence: 99%

Two malignant peripheral nerve lesions of non‐neurogenic origin

et al. 2012

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“…ES around the hand often mimics ganglion cyst [1] or other type of benign tumors like peripheral nerve sheath tumor, both possibly presenting the clinical symptoms compatible with carpal tunnel syndrome [6]. However, involvement and growth around a major peripheral nerve and presentation as a peripheral nerve sheath tumor are extremely rare [5]. In our case, due to the slow growing of the tumor, the patient noticed the mass presentation in his forearm after several months from the occurrence of symptoms mimicking with carpal tunnel syndrome, and MRI eventually revealed the tumor involvement of the median nerve.…”

Section: Discussionmentioning

confidence: 99%

“…It mostly presents as superficial nodules or ulcers; however deep-seated lesions can occur rarely. Although the clinical presentations are varying with a variety of physical-radiological features [4], it is extremely uncommon that ES involves the deep nerves [5, 6]. The initial treatment should be considered in a way of an aggressive wide resection with radical margins, sometimes in association with chemo- and radiotherapy [7].…”

Section: Introductionmentioning

confidence: 99%

Epithelioid Sarcoma of the Forearm Arising from Perineural Sheath of Median Nerve Mimicking Carpal Tunnel Syndrome

Fujii

Honoki

Yajima

et al. 2009

Sarcoma

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We report here a case of epithelioid sarcoma in the forearm of a 33-year-old male presenting with symptoms and signs of carpal tunnel syndrome originating from the direct involvement of the median nerve. Due to the slow growing of the tumor, the patient noticed the presence of tumor mass in his forearm after several months from the initial onset of the symptoms. Magnetic resonance imaging showed an 8 × 4 cm mass involving the median nerve in the middle part of the forearm, and histological analysis of the biopsy specimen revealed the diagnosis of epithelioid sarcoma. Radical surgical resection was performed in conjunction with adjuvant chemotherapy. The function of the flexors were restored by the multiple tendon transfers (EIP → FDS; ECRL → FDP; BrR → FPL; EDM → opponens) with superficial cutaneous branch of radial nerve transfer to the resected median nerve. The function of the affected hand showed excellent with the DASH disability/symptom score of 22.5, and both the grasp power and sensory of the median nerve area has recovered up to 50% of the normal side. The patient returned to his original vocation and alive with continuous disease free at 3.5-year follow-up since initial treatment.

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“…7,8 A third case of sciatic nerve involvement was probably an EMS due to its S-100 positivity. 9 Indeed, like EES, EMS and EMPNST stain positively for vimentin and epithelial markers (cytokeratin or EMA), but they also show S-100 positivity. 6,[10][11][12] EES should be distinguished from other epithelioid malignant neoplasms of nerves (EMS and EMPNST) showing a loss of INI-1, and its core feature is the absence of both INI-1 and S-100.…”

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confidence: 97%

“Malignant” foot drop: Enzinger epithelioid sarcoma of the common fibular nerve

et al. 2016

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Enzinger first characterized a peculiar sarcoma with epithelioid features, arising from tendons and aponeuroses. 1 Enzinger epithelioid sarcoma (EES) has an indolent and unpredictable behavior. Its prognosis is difficult to establish, distant metastases may occur, and long-term follow-up is necessary (overall 5-year survival is 45%). 2 EES is a very rare tumor of still unknown histogenesis. On immunohistochemical staining, it is positive for vimentin, cytokeratin, and epithelial membrane antigen (EMA), whereas staining is negative with the neural derived S-100 protein and the nuclear antigen INI-1. The latter is maintained in most epithelioid tumors that mimic EES, such as epithelioid vascular tumors, metastatic carcinomas, and melanomas. 3-5 On the other hand, epithelioid malignant peripheral nerve sheath tumors (EMPNSTs) and epithelioid malignant schwannomas (EMSs) frequently lose INI-1, but they always stain positively with S-100. 5,6 The possible origin of EES from nerve is still controversial. We report a unique observation of a well-characterized EES rising from the common fibular nerve.A 50-year-old, healthy man came to our attention due to a 3-month history of left foot drop with a positive Tinel sign at the fibular head. Knee MRI showed a nodular lesion with inhomogeneous contrast enhancement involving the common fibular nerve. On surgical exploration a malignant infiltrating mass was found inside the nerve. Careful microscopic dissection allowed complete removal of the lesion, sparing about 30% of nerve fascicles and achieving an end-to-side neurorrhaphy of the amputated fascicles. Histologic examination revealed a malignant, infiltrating lesion sparing regional and inguinal lymph nodes. Immunohistochemical stains were positive for vimentin, cytokeratin, EMA, and CD34, whereas INI-1, S-100, endothelial markers (CD31, FLI-1), podoplanin, desmin, and glial fibrillary acidic protein were absent. Such an immunophenotype was consistent with a diagnosis of EES. A total body computed tomography (CT) scan did not reveal any other localization. One year later, follow-up CT scan revealed tumor recurrence in lumboaortic lymph nodes, confirmed by biopsy. The patient underwent 5 courses of epirubicin (105 mg/m 2 ) and ifosfamide (9,000 mg/m 2 ) therapy, combined with radiotherapy (60 Gy), resulting in complete regression of the tumor. At last follow-up, 5 years after diagnosis, the patient had a stable foot drop (MRC 2-3/5) and no clinical or radiological sign of disease recurrence.Two previous reports described possible EES involving nerves (sciatic and median), but they lacked complete characterization. 7,8 A third case of sciatic nerve involvement was probably an EMS due to its S-100 positivity. 9 Indeed, like EES, EMS and EMPNST stain positively for vimentin and epithelial markers (cytokeratin or EMA), but they also show S-100 positivity. 6,10-12 EES should be distinguished from other epithelioid malignant neoplasms of nerves (EMS and EMPNST) showing a loss of INI-1, and its core feature is the absence of both I...

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Epithelioid sarcoma of the sciatic nerve perineural sheath: a mimic of nerve sheath tumor

Cited by 15 publications

References 7 publications

Two malignant peripheral nerve lesions of non‐neurogenic origin

Two malignant peripheral nerve lesions of non‐neurogenic origin

Epithelioid Sarcoma of the Forearm Arising from Perineural Sheath of Median Nerve Mimicking Carpal Tunnel Syndrome

“Malignant” foot drop: Enzinger epithelioid sarcoma of the common fibular nerve

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