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Giant cell tumor of soft tissue: A case report for the first time in ear
Human Pathology: Case Reports 10 (2017) 12–14
Contents lists available at ScienceDirect
Human Pathology: Case Reports journal homepage: http://www.humanpathologycasereports.com
Case Report
Giant cell tumor of soft tissue: A case report for the first time in ear Shahd Hafiz a,⁎, Mohammad Shaheen a, Neveen Awadh a, Ghada Esheba b,c a b c
Faculty of Medicine, Umm AlQura University, Saudi Arabia Department of Pathology, Faculty of Medicine, Tanta University, Egypt Department of Pathology, Faculty of Medicine, Umm AlQura University, Saudi Arabia
a r t i c l e
i n f o
Article history: Received 30 December 2016 Received in revised form 14 April 2017 Accepted 26 April 2017 Available online xxxx
a b s t r a c t Giant cell tumor of soft tissue is a rare benign tumor of low malignant potential. It counterparts soft tissue bone tumor. Majority of cases are in extremities and to a lesser extent in other regions but rarely in head and neck. Here we report for the first time giant cell tumor of soft tissue in the ear. A 28 years old female presented with painless right ear mass slowly progressing over 2 months associated with discharge for 2 weeks. The condition was initially diagnosed as chronic otitis media. CT scan and Excisional biopsy was done. Microscopically, examination revealed multiple tumor nodules that diffusely infiltrate the soft tissue and covered partially by unremarkable epithelium. The nodules are composed of bland mononuclear cells, short spindle cells and osteoclast giant cells. The mononuclear and giant cells lack striking atypia. However; the mononuclear cells exhibit brisk mitotic activity. The tumor was completely excised and follow up was scheduled after 1 year. © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Primary Giant Cell Tumor of Soft Tissue (GCT-ST) also called Soft Tissue Giant Cell Tumor of Low Malignant Potential (GCT-ST LMP) [1–3] is a very rare neoplasm which described in almost 70 cases only [1]. Clinical and Histological features resemble giant cell bone tumors. The majority of reported cases were in the extremities, thigh and trunk. Other uncommon anatomical sites have been reported in pelvis, breast and mediastinum [4]. The tumor is extremely rare in head and neck region [1–3]. Most patients are in their 5th decade of life but it can occur at any age group [4–6] with almost similar incidence in both genders [3]. Here, we report a rare case of GCT- ST arising in an area to the best of our knowledge never been reported before; the ear. 2. Case A 28-year-old medically free female complaining of right ear mass for 2 months associated with discharge for 2 weeks. Physical examination revealed right ear mass in the anterior third of the external auditory canal. The mass was reddish in color, non-tender and there was pus discharge. The tympanic membrane was intact but retracted. Other systemic examination was unremarkable. Culture and sensitivity results revealed Pseudomonas Aeruginosa and she was treating accordingly. CT temporal bone showed thickening of the right tympanic membrane,
⁎ Corresponding author. E-mail address: shahd.hafi[email protected] (S. Hafiz).
soft tissue mass lesion 17 × 15 mm obliterating the right external auditory canal (Fig. 1). Excisional biopsy was done. Microscopic examination revealed multiple tumor nodules that diffusely infiltrate the soft tissue and covered partially by unremarkable epithelium. The nodules were composed of bland mononuclear cells, short spindle cells and osteoclast giant cells. The mononuclear and giant cells lack striking atypia. However; the mononuclear cells exhibit brisk mitotic activity. The mitotic count is 6 per 10 high power field (HPF), The tumor margins were positive (Fig. 2). The diagnosis was right ear soft tissue giant cell tumor. The tumor was completely excised. Follow up was scheduled after 1 year. 3. Discussion A Giant Cell tumor of soft tissue (GCT-ST) is a very rare tumor that resembles osseous GCT clinically and histologically [1,4]. It was first described in the literature by Salm and Sissons in 1972 [1,4]. They reported a case series of 10 benign primary soft tissue tumors [4,5]. In the same year, Guccion and Enzinger reported a 32 case series of the same characteristic soft tissue tumors but exhibiting aggressive malignant transformation as atypia, abundant mitotic activity and pleomorphism [1,4,5,7,8]. However, according to literature, GCT ST predominantly exhibits a benign behavior with high recurrence rate if it is inadequately excised and extremely low possibility for metastasis especially to the lungs [1,3–5]. GCT-ST usually but not exclusively affects elderly and has no gender predilection [4]. Majority of the cases occur in the extremities [1,4,5]. Head and neck GCT-ST is rarely diagnosed and accounts only for 7%
http://dx.doi.org/10.1016/j.ehpc.2017.04.004 2214-3300/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
S. Hafiz et al. / Human Pathology: Case Reports 10 (2017) 12–14
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Fig. 1. Temporal Bone CT is showing thickening of the right tympanic membrane. 17*15 mm soft tissue mass lesion seen obliterating the right external auditory canal. Normal right middle ossicles. Normal right Internal Auditory Canal. Normal right cochlea and semicircular canal. No evidence of bone erosion. Normal left Internal and external Auditory Canal. Normal left middle ear ossicles, Normal left cochlea and semicircular canal. Impression of CT scan: soft tissue mass lesion seen obliterating the right External Auditory Canal with thickening of the right tympanic membrane.
[1]. The cases that have been reported for head and neck GCT-ST, were in the nasal cavity [9,10], lips [7], parotid, pre-auricular, submandibular region (19) but none has been reported in the ear. The size of the GCTSTs in the head and neck localization were reported as 0.7–5 cm [7]. GCT-ST has been also seen in the skin affecting mostly the dermis, in the abdominal wall and retroperitoneum. No etiological factors have been identified for GCT-ST [4]. However, some studies suggested that chronic inflammation may play a role in the pathogenesis, but it remains unproven till now [4]. The most common presenting symptom is painless mass [4,5] with an average symptoms duration of 6 months to 1 year [4,8]. The mass in our case was not tender, freely mobile and covered by intact skin. Similar findings were reported by Calli et al. [2] and Bandyopadhyay et al. [11]. On the contrary, the tumors reported in nose have a different presentation. Bhati et al. [10] reported that the tumor was pigmented and ulcerated, while it was exophytic as described by Lentini et al. [9]. In our case, microscopic examination showed admixture of mononuclear cells exhibiting brisk mitotic activity admixed with short
spindle cells and osteoclast giant cells that lack striking atypia. These findings are in keeping with previous reports [2,11]. Bhati et al. [10] also reported that the epidermis exhibited hyperkeratosis, parakeratosis and ulceration, in addition to the presence of clusters of foamy histiocytes and hemosiderin laden macrophages. Mild to moderate nuclear atypia in spindle cells was documented by Righi et al. [3]. Immunohistochemically, we test for vimentin, CD68, and smooth muscle actin (SMA), CD34, epithelial membrane antigen, S-100, cytokeratins AE1/AE3, desmin, and LCA (leukocyte common antigen, PTPRC). The Ki-67 labeling index was approximately (M ref). The osteoclast-like giant cells usually exhibit positive CD68 [1–3,7] and negative CD34 immunostaining [3–7]. CT scan in our patient showed obliteration of the right external auditory canal with thickening of the right tympanic membrane. Calli et al. [2] reported heterogeneous, soft tissue mass with enhancement margins, while Trabelsi et al. [1] reported well circumscribed heterogeneous mass in the spinal muscle of the neck.
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S. Hafiz et al. / Human Pathology: Case Reports 10 (2017) 12–14
Fig. 2. (A): Multiple tumor nodules diffusely infiltrate the soft tissue and covered partially by unremarkable epithelium. (B): The nodules are composed of bland mononuclear cells, short spindle cells, and osteoclast giant cells. (C): The mononuclear and giant cells lack striking atypia. (D): The mononuclear cells exhibit brisk mitotic activity (arrow).
In diagnosing GCT-ST, we have to put in mind many differentials because of the disease seldom nature. The More common neoplastic, non-neoplastic, inflammatory and granulomatous diseases must be carefully ruled out first Such as giant cell tumor of tendon sheath, nodular fasciitis, benign fibrous histiocytoma, plexiform fibrohistiocytic tumors, Benign fibrous histiocytoma, mucoepidermoid carcinoma, malignant fibrous histiocytoma, malignant melanoma, osteosarcoma, or others [1,7]. Minority of surgeons advise radical excision with close follow up as recurrence rate is high [4,5]. As for Radiotherapy; there is no current evidence available regarding its usage [3,4] but it can be used when needed [3,4]. 4. Conclusion GCT-ST tumor should be considered in any soft tissue mass and should be differentiated from other giant cells tumor. References [1] A. Trabelsi, F. Hammedi, A. Slama, S.B. Abdelkarim, N. Beïzig, H. Khochtali, et al., Giant cell tumor of soft tissue of neck: a case report, North Am. J. Med. Sci. 1 (2009) 319–320.
[2] A.O. Calli, M. Tunakan, H. Katılmış, S. Kılçıksız, S. Öztürkcan, Soft tissue giant cell tumor of low malignant potential of the neck: a case report and review of the literature/Boyunun Düşük malign Potansiyelli dev Hücreli Tümörü: Olgu Sunumu ve Literatürün Gözden Geçirilmesi, Turkish J. Pathol. 30 (1) (2014) 73–77. [3] S. Righi, P. Boffano, R. Patetta, L. Malvè, D. Pateras, P. De Matteis, et al., Soft tissue giant cell tumor of low malignant potential with 3 localizations: report of a case, Oral Surg. Oral Med. Oral Pathol. Oral Radiol. 118 (5) (2014) e135–e138. [4] P. Saldanha, H.N. Tak, Giant cell tumour of soft tissue–a rare neoplasm, J. Clin. Biomed. Sci. 4 (3) (2014) 326–329. [5] J.X. O'Connell, B.M. Wehrli, G.P. Nielsen, A.E. Rosenberg, Giant cell tumors of soft tissue: a clinicopathologic study of 18 benign and malignant tumors, Am. J. Surg. Pathol. 24 (3) (2000) 386–395. [6] A.M. Oliveira, A.P. Dei Tos, C.D. Fletcher, A.G. Nascimento, Primary giant cell tumor of soft tissues: a study of 22 cases, Am. J. Surg. Pathol. 24 (2) (2000) 248–256. [7] T. Devrim, Soft tissue giant cell tumor of the superior lip: a case report, SDÜ Tıp Fakültesi Dergisi 22 (1) (2015). [8] K. Chand, R. Bhardwaj, T. Rappai, Study of 7 cases of giant cell tumor of soft tissue, Med. J. Armed Forces India 62 (2) (2006) 138–140. [9] M. Lentini, V. Zuccala, C. Fazzari, Polypoid giant cell tumor of the skin, Am. J. Dermatopathol. 32 (1) (2010) 95–98. [10] A. BhAt, V. Geethamani, C. Vijaya, Soft tissue giant cell tumour of low malignant potential: a rare tumour at a rare site, J. Clin. Diagn. Res. 7 (12) (2013) 2960. [11] A. Bandyopadhyay, B. Khandakar, S. Medda, S. Dey, P.C. Paul, Giant cell tumour of soft tissue in neck: an uncommon tumour in an uncommon location, J. Clin. Diagn. Res. 9 (12) (2015) ED19.
Contents lists available at ScienceDirect
Human Pathology: Case Reports journal homepage: http://www.humanpathologycasereports.com
Case Report
Giant cell tumor of soft tissue: A case report for the first time in ear Shahd Hafiz a,⁎, Mohammad Shaheen a, Neveen Awadh a, Ghada Esheba b,c a b c
Faculty of Medicine, Umm AlQura University, Saudi Arabia Department of Pathology, Faculty of Medicine, Tanta University, Egypt Department of Pathology, Faculty of Medicine, Umm AlQura University, Saudi Arabia
a r t i c l e
i n f o
Article history: Received 30 December 2016 Received in revised form 14 April 2017 Accepted 26 April 2017 Available online xxxx
a b s t r a c t Giant cell tumor of soft tissue is a rare benign tumor of low malignant potential. It counterparts soft tissue bone tumor. Majority of cases are in extremities and to a lesser extent in other regions but rarely in head and neck. Here we report for the first time giant cell tumor of soft tissue in the ear. A 28 years old female presented with painless right ear mass slowly progressing over 2 months associated with discharge for 2 weeks. The condition was initially diagnosed as chronic otitis media. CT scan and Excisional biopsy was done. Microscopically, examination revealed multiple tumor nodules that diffusely infiltrate the soft tissue and covered partially by unremarkable epithelium. The nodules are composed of bland mononuclear cells, short spindle cells and osteoclast giant cells. The mononuclear and giant cells lack striking atypia. However; the mononuclear cells exhibit brisk mitotic activity. The tumor was completely excised and follow up was scheduled after 1 year. © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Primary Giant Cell Tumor of Soft Tissue (GCT-ST) also called Soft Tissue Giant Cell Tumor of Low Malignant Potential (GCT-ST LMP) [1–3] is a very rare neoplasm which described in almost 70 cases only [1]. Clinical and Histological features resemble giant cell bone tumors. The majority of reported cases were in the extremities, thigh and trunk. Other uncommon anatomical sites have been reported in pelvis, breast and mediastinum [4]. The tumor is extremely rare in head and neck region [1–3]. Most patients are in their 5th decade of life but it can occur at any age group [4–6] with almost similar incidence in both genders [3]. Here, we report a rare case of GCT- ST arising in an area to the best of our knowledge never been reported before; the ear. 2. Case A 28-year-old medically free female complaining of right ear mass for 2 months associated with discharge for 2 weeks. Physical examination revealed right ear mass in the anterior third of the external auditory canal. The mass was reddish in color, non-tender and there was pus discharge. The tympanic membrane was intact but retracted. Other systemic examination was unremarkable. Culture and sensitivity results revealed Pseudomonas Aeruginosa and she was treating accordingly. CT temporal bone showed thickening of the right tympanic membrane,
⁎ Corresponding author. E-mail address: shahd.hafi[email protected] (S. Hafiz).
soft tissue mass lesion 17 × 15 mm obliterating the right external auditory canal (Fig. 1). Excisional biopsy was done. Microscopic examination revealed multiple tumor nodules that diffusely infiltrate the soft tissue and covered partially by unremarkable epithelium. The nodules were composed of bland mononuclear cells, short spindle cells and osteoclast giant cells. The mononuclear and giant cells lack striking atypia. However; the mononuclear cells exhibit brisk mitotic activity. The mitotic count is 6 per 10 high power field (HPF), The tumor margins were positive (Fig. 2). The diagnosis was right ear soft tissue giant cell tumor. The tumor was completely excised. Follow up was scheduled after 1 year. 3. Discussion A Giant Cell tumor of soft tissue (GCT-ST) is a very rare tumor that resembles osseous GCT clinically and histologically [1,4]. It was first described in the literature by Salm and Sissons in 1972 [1,4]. They reported a case series of 10 benign primary soft tissue tumors [4,5]. In the same year, Guccion and Enzinger reported a 32 case series of the same characteristic soft tissue tumors but exhibiting aggressive malignant transformation as atypia, abundant mitotic activity and pleomorphism [1,4,5,7,8]. However, according to literature, GCT ST predominantly exhibits a benign behavior with high recurrence rate if it is inadequately excised and extremely low possibility for metastasis especially to the lungs [1,3–5]. GCT-ST usually but not exclusively affects elderly and has no gender predilection [4]. Majority of the cases occur in the extremities [1,4,5]. Head and neck GCT-ST is rarely diagnosed and accounts only for 7%
http://dx.doi.org/10.1016/j.ehpc.2017.04.004 2214-3300/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
S. Hafiz et al. / Human Pathology: Case Reports 10 (2017) 12–14
13
Fig. 1. Temporal Bone CT is showing thickening of the right tympanic membrane. 17*15 mm soft tissue mass lesion seen obliterating the right external auditory canal. Normal right middle ossicles. Normal right Internal Auditory Canal. Normal right cochlea and semicircular canal. No evidence of bone erosion. Normal left Internal and external Auditory Canal. Normal left middle ear ossicles, Normal left cochlea and semicircular canal. Impression of CT scan: soft tissue mass lesion seen obliterating the right External Auditory Canal with thickening of the right tympanic membrane.
[1]. The cases that have been reported for head and neck GCT-ST, were in the nasal cavity [9,10], lips [7], parotid, pre-auricular, submandibular region (19) but none has been reported in the ear. The size of the GCTSTs in the head and neck localization were reported as 0.7–5 cm [7]. GCT-ST has been also seen in the skin affecting mostly the dermis, in the abdominal wall and retroperitoneum. No etiological factors have been identified for GCT-ST [4]. However, some studies suggested that chronic inflammation may play a role in the pathogenesis, but it remains unproven till now [4]. The most common presenting symptom is painless mass [4,5] with an average symptoms duration of 6 months to 1 year [4,8]. The mass in our case was not tender, freely mobile and covered by intact skin. Similar findings were reported by Calli et al. [2] and Bandyopadhyay et al. [11]. On the contrary, the tumors reported in nose have a different presentation. Bhati et al. [10] reported that the tumor was pigmented and ulcerated, while it was exophytic as described by Lentini et al. [9]. In our case, microscopic examination showed admixture of mononuclear cells exhibiting brisk mitotic activity admixed with short
spindle cells and osteoclast giant cells that lack striking atypia. These findings are in keeping with previous reports [2,11]. Bhati et al. [10] also reported that the epidermis exhibited hyperkeratosis, parakeratosis and ulceration, in addition to the presence of clusters of foamy histiocytes and hemosiderin laden macrophages. Mild to moderate nuclear atypia in spindle cells was documented by Righi et al. [3]. Immunohistochemically, we test for vimentin, CD68, and smooth muscle actin (SMA), CD34, epithelial membrane antigen, S-100, cytokeratins AE1/AE3, desmin, and LCA (leukocyte common antigen, PTPRC). The Ki-67 labeling index was approximately (M ref). The osteoclast-like giant cells usually exhibit positive CD68 [1–3,7] and negative CD34 immunostaining [3–7]. CT scan in our patient showed obliteration of the right external auditory canal with thickening of the right tympanic membrane. Calli et al. [2] reported heterogeneous, soft tissue mass with enhancement margins, while Trabelsi et al. [1] reported well circumscribed heterogeneous mass in the spinal muscle of the neck.
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S. Hafiz et al. / Human Pathology: Case Reports 10 (2017) 12–14
Fig. 2. (A): Multiple tumor nodules diffusely infiltrate the soft tissue and covered partially by unremarkable epithelium. (B): The nodules are composed of bland mononuclear cells, short spindle cells, and osteoclast giant cells. (C): The mononuclear and giant cells lack striking atypia. (D): The mononuclear cells exhibit brisk mitotic activity (arrow).
In diagnosing GCT-ST, we have to put in mind many differentials because of the disease seldom nature. The More common neoplastic, non-neoplastic, inflammatory and granulomatous diseases must be carefully ruled out first Such as giant cell tumor of tendon sheath, nodular fasciitis, benign fibrous histiocytoma, plexiform fibrohistiocytic tumors, Benign fibrous histiocytoma, mucoepidermoid carcinoma, malignant fibrous histiocytoma, malignant melanoma, osteosarcoma, or others [1,7]. Minority of surgeons advise radical excision with close follow up as recurrence rate is high [4,5]. As for Radiotherapy; there is no current evidence available regarding its usage [3,4] but it can be used when needed [3,4]. 4. Conclusion GCT-ST tumor should be considered in any soft tissue mass and should be differentiated from other giant cells tumor. References [1] A. Trabelsi, F. Hammedi, A. Slama, S.B. Abdelkarim, N. Beïzig, H. Khochtali, et al., Giant cell tumor of soft tissue of neck: a case report, North Am. J. Med. Sci. 1 (2009) 319–320.
[2] A.O. Calli, M. Tunakan, H. Katılmış, S. Kılçıksız, S. Öztürkcan, Soft tissue giant cell tumor of low malignant potential of the neck: a case report and review of the literature/Boyunun Düşük malign Potansiyelli dev Hücreli Tümörü: Olgu Sunumu ve Literatürün Gözden Geçirilmesi, Turkish J. Pathol. 30 (1) (2014) 73–77. [3] S. Righi, P. Boffano, R. Patetta, L. Malvè, D. Pateras, P. De Matteis, et al., Soft tissue giant cell tumor of low malignant potential with 3 localizations: report of a case, Oral Surg. Oral Med. Oral Pathol. Oral Radiol. 118 (5) (2014) e135–e138. [4] P. Saldanha, H.N. Tak, Giant cell tumour of soft tissue–a rare neoplasm, J. Clin. Biomed. Sci. 4 (3) (2014) 326–329. [5] J.X. O'Connell, B.M. Wehrli, G.P. Nielsen, A.E. Rosenberg, Giant cell tumors of soft tissue: a clinicopathologic study of 18 benign and malignant tumors, Am. J. Surg. Pathol. 24 (3) (2000) 386–395. [6] A.M. Oliveira, A.P. Dei Tos, C.D. Fletcher, A.G. Nascimento, Primary giant cell tumor of soft tissues: a study of 22 cases, Am. J. Surg. Pathol. 24 (2) (2000) 248–256. [7] T. Devrim, Soft tissue giant cell tumor of the superior lip: a case report, SDÜ Tıp Fakültesi Dergisi 22 (1) (2015). [8] K. Chand, R. Bhardwaj, T. Rappai, Study of 7 cases of giant cell tumor of soft tissue, Med. J. Armed Forces India 62 (2) (2006) 138–140. [9] M. Lentini, V. Zuccala, C. Fazzari, Polypoid giant cell tumor of the skin, Am. J. Dermatopathol. 32 (1) (2010) 95–98. [10] A. BhAt, V. Geethamani, C. Vijaya, Soft tissue giant cell tumour of low malignant potential: a rare tumour at a rare site, J. Clin. Diagn. Res. 7 (12) (2013) 2960. [11] A. Bandyopadhyay, B. Khandakar, S. Medda, S. Dey, P.C. Paul, Giant cell tumour of soft tissue in neck: an uncommon tumour in an uncommon location, J. Clin. Diagn. Res. 9 (12) (2015) ED19.