Abstract
Background
Malignant granular cell tumors (MGCTs) are extremely rare neoplasms with only a limited number of studies published to date. The aim of this study is to elucidate the clinicopathological characteristics and prognostic factors of MGCTs.
Methods
This is a multi-institutional retrospective study of MGCTs with a central pathological review. A total of 18 cases were retrieved. Specimens were blindly reviewed by two pathologists based on the diagnostic criteria by Fanburg-Smith et al. Kaplan–Meier survival probabilities were calculated, and risk factors for poor prognosis were evaluated.
Results
Three and fifteen cases were diagnosed as atypical GCTs (AGCTs) and mGCTs according to the Fanburg-Smith et al. classification, respectively. Four (one atypical and three malignant) cases had metastasis at the first presentation, including lymph node metastasis. Three out of ten cases treated with wide resection developed local recurrence. Although prolonged static disease periods of ≥1 year were observed in four cases receiving chemotherapy, all cases with local recurrence or metastasis, including two atypical cases, eventually died of disease. The 5- and 10-year overall survival rates for localized MGCTs were 69.2 and 34.6 %, respectively. The presence of necrosis was revealed as a risk factor associated with adverse clinical outcomes.
Conclusions
MGCTs have high rates of recurrence and metastasis including lymph node metastasis. As histologically atypical cases also have metastatic potential, close attention should be paid to AGCTs. The combination of histological evaluation and tumor size may lead to more accurate diagnosis of this rare neoplasm.
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References
Fanburg-Smith JC (2013) Malignant granular cell tumour. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (eds) WHO classification of tumours of soft tissue and bone, 4th edn. IARC Press, Lyon, p 190
Fanburg-Smith JC, Meis-Kindblom JM, Fante R et al (1998) Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 22:779–794
Nasser H, Ahmed Y, Szpunar SM et al (2011) Malignant granular cell tumor: a look into the diagnostic criteria. Pathol Res Pract 207:164–168
Kapur P, Rakheja D, Balani JP et al (2007) 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 131:57–64
Tsuchida T, Okada K, Itoi E et al (1997) Intramuscular malignant granular cell tumor. Skeletal Radiol 26:116–121
Saito T, Mitomi H, Torigoe T et al (2012) Malignant granular cell tumor with an unusually long clinical course: an autopsy case with review of literature. J Cancer Sci Ther 4:260–263
Imao K, Ogose A, Kawashima H et al (2009) Two cases treated with chemotherapy for malignant granular cell tumor. J East Jpn Orthop Traumatol 21:577–582
Arai E, Nishida Y, Tsukushi S et al (2010) Intramuscular granular cell tumor in the lower extremities. Clin Orthop Relat Res 468:1384–1389
Sekiya I, Kawaguchi N, Matsumoto S et al (1992) Six cases of granular cell tumor and two cases of malignant granular cell tumor. Clin orthop surg 27:235–240
Coindre JM, Terrier P, Bui NB et al (1996) Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol 14:869–877
Edge S, Byrd D, Compton C et al (2009) American Joint Committee on Cancer Staging Manual. Springer, New York
Enneking WF, Spanier SS, Goodman MA (1980) A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res:106–120
Nakazato Y, Ishizeki J, Takahashi K et al (1982) Immunohistochemical localization of S-100 protein in granular cell myoblastoma. Cancer 49:1624–1628
Nathrath WB, Remberger K (1986) Immunohistochemical study of granular cell tumours. Demonstration of neurone specific enolase, S 100 protein, laminin and alpha-1-antichymotrypsin. Virchows Arch A Pathol Anat Histopathol 408:421–434
Rose B, Tamvakopoulos GS, Yeung E et al (2009) Granular cell tumours: a rare entity in the musculoskeletal system. Sarcoma 2009:765927
Tsukushi S, Nishida Y, Urakawa H et al (2014) Prognostic significance of histological invasion in high grade soft tissue sarcomas. Springerplus 3:544
Rosenthal SA, Livolsi VA, Turrisi AT 3rd (1990) Adjuvant radiotherapy for recurrent granular cell tumor. Cancer 65:897–900
Cruz-Mojarrieta J, Navarro S, Gomez-Cabrera E et al (2001) Malignant granular cell tumor of soft tissues: a study of two new cases. Int J Surg Pathol 9:255–259
Tawfiq N, Sabri S, Saiss K et al (2013) Granular cell tumor: report of a complicated vulvar localization of pulmonary metastases. Cancer Radiother 17:671–674
Khansur T, Balducci L, Tavassoli M (1987) Granular cell tumor. Clinical spectrum of the benign and malignant entity. Cancer 60:220–222
Acknowledgments
The authors thank Dr. Akira Kawai, National Cancer Center Hospital; Dr. Kenji Morii, Kyorin University Hospital; Dr. Yoshihiro Nishida and Dr. Eisuke Arai, Nagoya University Hospital; Dr. Akira Ogose, Niigata University Medical & Dental Hospital; Dr. Yosuke Yabuuchi, Osaka Medical Center for Cancer and Cardiovascular Diseases; Dr. Syunsuke Hamada, Aichi Cancer Center Hospital; Dr. Toshitake Yakushiji, Kumamoto University Hospital; Prof. Kyoji Okada, Akita University Hospital; Dr. Hiroaki Kanda, Cancer Institute Hospital of Japanese Foundation for Cancer Research for data collection in this study, and Dr. Takashi Fujino and two cytologists, Mr. Masaru Nakamura and Mr. Atsushi Seyama, Saitama Medical University International Medical Center for calculating Ki-67 labeling index and supporting the central pathological review. The authors also express their sincere appreciation to Dr. Takafumi Ueda, Director in chief of the Japanese Musculoskeletal Oncology Group (JMOG), for his advice and support.
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An erratum to this article can be found at http://dx.doi.org/10.1007/s10147-016-1051-4.
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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 21, 808–816 (2016). https://doi.org/10.1007/s10147-016-0949-1
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DOI: https://doi.org/10.1007/s10147-016-0949-1