Background: The incidence of malignant granular cell tumor, an extremely rare Schwann cell-derived tumor with a poor prognosis, is reported to be approximately 0.2% of malignant soft tissue tumors. We report a case of a malignant granular cell tumor originating from the ulnar nerve.
Case presentation: A 71-year-old woman presented with a mass in her right forearm. Magnetic resonance imaging showed a tumor with homogenous intensity of T1 and heterogeneous hyperintensity of T2, continuous with the ulnar nerve. Incisional biopsy revealed a malignant granular cell tumor, and marginal excision of the tumor was performed. Histologically, the tumor size was 9.2 cm and consisted of eosinophilic, granular polygonal to round and spindle-shaped cells, with vesicular and prominent nucleoli, and increased mitosis. Immunohistochemically, the tumor cells were positive for S-100 protein, CD68, H3K27me3, TFE3, and SOX10 and negative for smooth muscle alpha-actin, desmin, cytokeratin AE1/3, epithelial membrane antigen, and synaptophysin. The Ki-67 positivity rate was 12%. These findings were consistent with those of malignant granular cell tumors. In addition, no metastasis or recurrence was observed 15 years after the excision.
Conclusion: Surgical resection is the standard treatment option. In our case, the diagnostic criteria for malignant granular cell tumors were histologically met. Patients with malignant granular cell tumors have a poor prognosis. However, no metastasis or recurrence was observed in this case 15 years after the surgery.
ACKNOWLEDGEMENTS
Sei Morinaga, Norio Yamamoto, Katsuhiro Hayashi, Akihiko Takeuchi, Shinji Miwa, Kentaro Igarashi, Hirotaka Yonezawa, Yohei Asano, Shiro Saito, Takayuki Nojima, and Hiroyuki Tsuchiya determined treatment plan. Sei Morinaga and Norio Yamamoto conducted the follow-up. Sei Morinaga was a major contributor in writing the manuscript. Takayuki Nojima reviewed the histological specimens. Hiroyuki Tsuchiya oversaw the study. All authors read and approved the final manuscript.
CONFLICTS OF INTEREST DISCLOSURE
The authors declare that they have no conflicts of interest.
| [1] |
Rose B, Tamvakopoulos GS, Yeung E, et al. Granular cell tumors: a rare entity in the musculoskeletal system. Sarcoma. 2009; 765927. https://doi.org/10.1155/2009/765927
|
| [2] |
Ordóñez NG. Granular cell tumor: a review and update. Adv Anat Pathol. 1999; 6: 186-203. https://doi.org/10.1097/00125480-199907000-00002
|
| [3] |
Fanburg-Smith JC, Meis-Kindblom JM, Fante R, et al. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998; 22: 779-794. https://doi.org/10.1097/00000478-199807000-00001
|
| [4] |
Meling TR, Fridrich K, Evensen JF. Nedregaard B: Malignant granular cell tumor of the skull base. Skull base. 2008; 18: 59-66. https://doi.org/10.1055/s-2007-992763
|
| [5] |
Aksoy S, Abali H, Kilickap S, et al. Metastatic granular cell tumor: a case report and review of the literature. Acta Oncol. 2006; 45: 91-94. https://doi.org/10.1080/02841860500341132
|
| [6] |
Usui M, Ishii S, Yamawaki S, et al. Malignant granular cell tumor of the radial nerve: an autopsy observation with electron microscopic and tissue culture studies. Cancer. 1977; 39: 1547-1555. https://doi.org/10.1002/1097-0142(197704)39:4＜1547::AID-CNCR2820390428＞3.0.CO;2-O
|
| [7] |
Weisman RA, Konrad HR, Canalis RF. Granular cell myoblastoma involving the recurrent laryngeal nerve. Arch Otolaryngol. 1980; 106: 294-297. https://doi.org/10.1001/archotol.1980.00790290046015
|
| [8] |
Shimamura K, Osamura RY, Ueyama Y, et al. Malignant granular cell tumor of the right sciatic nerve. Report of an autopsy case with electron microscopic, immunohistochemical, and enzyme histochemical studies. Cancer. 1984; 53: 524-529. https://doi.org/10.1002/1097-0142(19840201)53:3＜524::AID-CNCR2820530326＞3.0.CO;2-5
|
| [9] |
Saperstein AL, Lusskin R, Doniguian AE, et al. Malignant granular cell tumor mimicking herniated nucleus pulposus. Clin Orthop Relat Res. 1996; 324: 244-250. https://doi.org/10.1097/00003086-199603000-00030
|
| [10] |
Simsir A, Osborne BM, Greenebaum E. Malignant granular cell tumor: a case report and review of the recent literature. Hum Pathol. 1996; 27: 853-858. https://doi.org/10.1016/S0046-8177(96)90462-1
|
| [11] |
Sonobe H, Iwata J, Furihata M, et al. Malignant granular cell tumor: report of a case and review of the literature. Pathol Res Pract. 1998; 194: 507-513. https://doi.org/10.1016/S0344-0338(98)80119-2
|
| [12] |
Wieczorek TJ, Krane JF, Domanski HA, et al. Cytologic findings in granular cell tumors, with emphasis on the diagnosis of malignant granular cell tumor by fine-needle aspiration biopsy. Cancer. 2001; 93: 398-408. https://doi.org/10.1002/cncr.10143
|
| [13] |
Hyodo M, Sadamoto A, Murakami S. Malignant granular cell tumour of the cervical sympathetic nerve trunk. J Laryngol Otol. 2001; 115: 833-835. https://doi.org/10.1258/0022215011909107
|
| [14] |
Di Tommaso L, Magrini E, Consales A, et al. Malignant granular cell tumor of the lateral femoral cutaneous nerve: report of a case with cytogenetic analysis. Hum Pathol. 2002; 33: 1237-1240. https://doi.org/10.1053/hupa.2002.129207
|
| [15] |
Papachristou DJ, Palekar A, Surti U, et al. Malignant granular cell tumor of the ulnar nerve with novel cytogenetic and molecular genetic findings. Cancer Genet Cytogenet. 2009; 191: 46-50. https://doi.org/10.1016/j.cancergencyto.2009.01.011
|
| [16] |
Go MH, Kim SH, Cho KH. Brachial plexus tumors in a consecutive series of twenty-one patients. J Korean Neurosurg Soc. 2012; 52: 138-143. https://doi.org/10.3340/jkns.2012.52.2.138
|
| [17] |
De Luca G, Luciano A, Benincasa G, et al. Giant malignant granular cell tumor (GCT) of the posterior mediastinum. J Thorac Oncol. 2013; 8: 1107-1108. https://doi.org/10.1097/JTO.0b013e318299ad62
|
| [18] |
Aviles S, Tay T, Jones WD, et al. Intraneural malignant granular cell tumor—report of an extremely rare tumor in a child. Hand Surg. 2014; 19: 103-107. https://doi.org/10.1142/S0218810414720046
|
| [19] |
Hurrell MA, McLean C, Desmond P, et al. Malignant granular cell tumour of the sciatic nerve. Australas Radiol. 1995; 39: 86-89. https://doi.org/10.1111/j.1440-1673.1995.tb00242.x
|
| [20] |
Imao K, Ogose A, Kawashima H, et al. Two cases treated with chemotherapy for malignant granular cell tumor. J East Jpn Orthop Traumatol. 2009; 21: 577-582.
|
| [21] |
Tsukamoto S, Takeda M, Honoki K, et al. Malignant granular cell tumor of the median nerve:a case report with a literature review of 157 cases. Skeletal Radiol. 2019; 48: 307-316. https://doi.org/10.1007/s00256-018-3017-2
|
| [22] |
Kapur P, Rakheja D, Balani JP, et al. Phosphorylated histone H3, Ki- 67, p21, fatty acid synthase, and cleaved caspase-3 expression in benign and atypical granular cell tumors. Arch. Pathol. Lab Med. 2007; 131: 57-64. https://doi.org/10.5858/2007-131-57-PHHKPF
|
| [23] |
Chamberlain BK, McClain CM, Gonzalez RS, et al. Alveolar soft part sarcoma and granular cell tumor: An immunohistochemical comparison study. Hum Pathol. 2014; 45: 1039-1044. https://doi.org/10.1016/j.humpath.2013.12.021
|
| [24] |
Schoolmeester JK, Lastra RR. Granular cell tumors overexpress TFE3 without corollary gene rearrangement. Hum Pathol. 2015; 46: 1242-1243. https://doi.org/10.1016/j.humpath.2015.04.004
|
| [25] |
Imanishi J, Yazawa Y, Saito T, et al. Atypical and malignant granular cell tumors in Japan: a Japanese musculoskeletal oncology group (JMOG) study. Int J Clin Oncol. 2016; 21: 808-816. https://doi.org/10.1007/s10147-016-0949-1
|
| [26] |
Moten AS, Movva S, von Mehren M, et al. Granular cell tumor experience at a comprehensive cancer center. J Surg Res. 2018; 226: 1-7. https://doi.org/10.1016/j.jss.2018.01.027
|
| [27] |
Moten AS, Zhao H, Wu H, et al. Malignant granular cell tumor: Clinical features and long-term survival. J Surg Oncol. 2018; 118: 891-897. https://doi.org/10.1002/jso.25227
|
| [28] |
Hurrel MA, McLean C, Desmond P, et al. Malignant granular cell tumor of the sciatic nerve. Australas Radiol. 1995; 39: 86-89. https://doi.org/10.1111/j.1440-1673.1995.tb00242.x
|
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