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Rare embryonal rhabdomyosarcoma of external acoustic canal: a case report and literature review
AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y – H EA D A N D N E CK ME D I CI NE AN D SUR G E RY XX ( 2 0 14 ) XXX – XXX
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Case Report
Rare embryonal rhabdomyosarcoma of external acoustic canal: a case report and literature review☆,☆☆ M.S. Eksan, MD a,⁎, 1 , Y. Noorizan, MS, ORLHNS a, 1 , Y.K. Chew, MS, ORLHNS a, 1 , M.D. Noorafidah, MMed, Path b, 1 , A. Asma, MS, ORLHNS c, 1 a b c
ORL Department Hospital Pakar Sultanah Fatimah, Muar, Johor, Malaysia Pathology Department Hospital Pakar Sultanah Fatimah, Muar, Johor, Malaysia ORL Head and Neck Surgery Department, UKM Medical Centre, Cheras, Kuala Lumpur, Malaysia
ARTI CLE I NFO
A BS TRACT
Article history:
Embryonal rhabdomyosarcoma of external ear canal is very rare, even in the pediatric
Received 20 June 2014
population. We report an embryonal rhabdomyosarcoma of external acoustic canal, occurring in a 12-year-old Chinese girl who presented with left ear canal mass. Wide local excision of the mass was done under local anesthesia. Histopathological examination revealed the diagnosis. She then underwent 20 cycles of radiotherapy. CECT scan post treatment showed clearance of tumor cell. Now, patient is disease free for 5 years. We believe that early diagnosis followed by complete resection of the tumor with clear margin and radiotherapy improves the prognosis of the disease. © 2014 Elsevier Inc. All rights reserved.
1.
Introduction
Rhabdomyosarcoma arises from immature mesenchymal cells that are committed to skeletal muscle differentiation. It is commonly classified as embryonal, alveolar, pleomorphic, and mixed types. It is the most common soft tissue sarcoma in pediatrics, accounting for 5%–10% of all childhood malignancies with a relative predilection for the head and neck region with a rate about 40% [1]. Rhabdomyosarcoma of external ear canal is extremely rare, thus making the early diagnosis challenging.
2.
Case report
A 12-year-old Chinese girl presented with left ear canal mass which was increasing in size in 6 months’ duration. Examination showed a lobulated mass at the left external acoustic meatus measuring 2 × 1 cm with a wide base. Wide local excision was successfully done under local anesthesia. Histopathological examination revealed embryonal rhabdomyosarcoma. HRCT Temporal post excision showed no evidence of residual tumor or local infiltration. CT scan, ultrasonography of hepatobiliary system and chest x-ray were done and showed no evidence of
☆
None of the authors has any conflict of interest, financial or otherwise. This manuscript is original and it, or any part of it, has not been previously published; nor is it under consideration for publication elsewhere. ⁎ Corresponding author at: Dept of Otorhinolaryngology, Head and Neck Surgery, Hospital Pakar Sultanah Fatimah, Jalan Salleh, 84000 Muar, Johor, Malaysia. Tel.: + 60 69521901x119, + 60 122356680. E-mail address: [email protected] (M.S. Eksan). 1 Each of the authors has contributed to, read and approved this manuscript.
☆☆
http://dx.doi.org/10.1016/j.amjoto.2014.07.009 0196-0709/© 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Eksan MS, et al, Rare embryonal rhabdomyosarcoma of external acoustic canal: a case report and literature review, Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.07.009
2
AM ER IC AN JOURNAL OF OTOLARYNGO LOGY – H EAD AN D N E CK ME D I CI NE AN D SUR GE RY XX ( 2 0 14 ) XXX– X XX
metastasis or lymph node involvement. She then underwent 20 cycles radiotherapy. Repeat CT scan of temporal bone and neck showed no focal mass at external ear canal. On regular follow up as outpatient for over 5 years after the diagnosis, patient is well and free of recurrence.
3.
Discussion
Rhabdomyosarcoma represents the most common head and neck sarcoma in the pediatric population. The localization in external ear canal is very rare. H. Charchi in 2010 reported that only 3.75% of the head and neck rhabdomyosarcomas arise from the ear canal [1]. Diagnosis of auricular rhabdomyosarcoma could be delayed by its clinical resemblance to more common and benign lesion in pediatrics such as chronic otitis with otorrhea and aural polyp [2]. In this case, she presented with progressively enlarging aural mass that was obvious on inspection, without history of otorrhea or infection, which increased our index of suspicion of malignant tumor. In one reported series of 50 patients that presented with ear mass, the most common causes of ear mass were inflammatory polyps (20) followed by cholesteatoma (12), chronic non specific inflammation (7), abscess (1), malignancies; squamous cell carcinoma (7), embryonal rhabdomyosarcoma (1) and other benign lesions; carcinoid tumor (1) and nevus (1) [3]. The histopathological examination was essentially required with the support of immunohistochemical studies to make diagnosis. Histologically, rhabdomyosarcoma was one of small round blue cell in childhood like neuroblastoma, lymphoma and Ewing’s sarcoma, and embryonal rhabdomyosarcoma was characterized by primitive spindle cells, often with a myxoid background [1]. Immunohistochemical studies, including staining for desmin, smooth muscle actin and myogenin, are additional studies that can support the diagnosis of rhabdomyosarcoma [1]. (Figs. 1, 2) Modalities of treatment are predominantly surgery, chemotherapy or radiotherapy. Localized or easily accessible non-orbital non-parameningeal head and neck tumors are most often amenable to surgery with low long-term surgical
Fig. 2 – Immunohistochemical staining: vimentin positive.
morbidity and can also be used in the salvage therapy of those who do not respond to chemotherapy and radiotherapy [1]. Recent trends suggest that radical resection procedures are unnecessary, with response to chemotherapy and radiotherapeutic regimes dramatically improving overall event-free survival [4]. Local and regional tumor with embryonal histology responded significantly to VAC (vincristine, dactinomycin, cyclophospamide), VAI (vincristine, dactinomycin, ifosfamide), or VIE (vincristine, ifosfamide, etoposide) plus surgery or radiotherapy [5]. In our case, wide local excision under local anesthesia was done for diagnostic and therapeutic aim without involving resection of bone. Once the diagnosis was confirmed she then underwent 45 Gy, 20 cycles of radiotherapy to the affected ear. CT scan post radiotherapy was clear of any tumor. She was closely followed up and showed no sign of recurrence and asymptomatic for over 5 years. As conclusion, in view of its rarity, making an early correct diagnosis is a challenge. Early diagnosis with early management brought good outcome to the patient. Based on this case, it’s evidenced that local excision of the tumor followed by radiotherapy, offered good prognosis with less morbidity to the patient.
REFERENCES
Fig. 1 – Immunohistochemical staining: myoglobin positive.
[1] Charchi H, Mansouri N, Harmouch A, et al. Epidemiological, histological and immunohistochemical analysis of head and neck rhabdomyosarcoma in children. J Afr Cancer 2011; 3:222–6. [2] Devaney O, Boschman CR, Willard SC, et al. Tumours of the external ear and temporal bone Kenneth. Lancet Oncol 2005; 6:411–20. [3] Agarwal NM, Popat VC, Traviad C, et al. Clinical and histopathological study of mass in ear: a study of fifty cases. Indian J Otolaryngol Head Neck Surg 2013;65(Suppl 3):S520–5. [4] Durve DV, Kanegaonkar RG, Albert D, et al. Paediatric rhabdomyosarcoma of ear and temporal bone. Clin Otolaryngol 2004;29:32–7. [5] Crist WM, Anderson JR, Meza JL, et al. Intergroup Rhabdomyosarcoma Study—IV: results for patients with nonmetastatic disease. J Clin Oncol 2001;19:3091–102.
Please cite this article as: Eksan MS, et al, Rare embryonal rhabdomyosarcoma of external acoustic canal: a case report and literature review, Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.07.009
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Case Report
Rare embryonal rhabdomyosarcoma of external acoustic canal: a case report and literature review☆,☆☆ M.S. Eksan, MD a,⁎, 1 , Y. Noorizan, MS, ORLHNS a, 1 , Y.K. Chew, MS, ORLHNS a, 1 , M.D. Noorafidah, MMed, Path b, 1 , A. Asma, MS, ORLHNS c, 1 a b c
ORL Department Hospital Pakar Sultanah Fatimah, Muar, Johor, Malaysia Pathology Department Hospital Pakar Sultanah Fatimah, Muar, Johor, Malaysia ORL Head and Neck Surgery Department, UKM Medical Centre, Cheras, Kuala Lumpur, Malaysia
ARTI CLE I NFO
A BS TRACT
Article history:
Embryonal rhabdomyosarcoma of external ear canal is very rare, even in the pediatric
Received 20 June 2014
population. We report an embryonal rhabdomyosarcoma of external acoustic canal, occurring in a 12-year-old Chinese girl who presented with left ear canal mass. Wide local excision of the mass was done under local anesthesia. Histopathological examination revealed the diagnosis. She then underwent 20 cycles of radiotherapy. CECT scan post treatment showed clearance of tumor cell. Now, patient is disease free for 5 years. We believe that early diagnosis followed by complete resection of the tumor with clear margin and radiotherapy improves the prognosis of the disease. © 2014 Elsevier Inc. All rights reserved.
1.
Introduction
Rhabdomyosarcoma arises from immature mesenchymal cells that are committed to skeletal muscle differentiation. It is commonly classified as embryonal, alveolar, pleomorphic, and mixed types. It is the most common soft tissue sarcoma in pediatrics, accounting for 5%–10% of all childhood malignancies with a relative predilection for the head and neck region with a rate about 40% [1]. Rhabdomyosarcoma of external ear canal is extremely rare, thus making the early diagnosis challenging.
2.
Case report
A 12-year-old Chinese girl presented with left ear canal mass which was increasing in size in 6 months’ duration. Examination showed a lobulated mass at the left external acoustic meatus measuring 2 × 1 cm with a wide base. Wide local excision was successfully done under local anesthesia. Histopathological examination revealed embryonal rhabdomyosarcoma. HRCT Temporal post excision showed no evidence of residual tumor or local infiltration. CT scan, ultrasonography of hepatobiliary system and chest x-ray were done and showed no evidence of
☆
None of the authors has any conflict of interest, financial or otherwise. This manuscript is original and it, or any part of it, has not been previously published; nor is it under consideration for publication elsewhere. ⁎ Corresponding author at: Dept of Otorhinolaryngology, Head and Neck Surgery, Hospital Pakar Sultanah Fatimah, Jalan Salleh, 84000 Muar, Johor, Malaysia. Tel.: + 60 69521901x119, + 60 122356680. E-mail address: [email protected] (M.S. Eksan). 1 Each of the authors has contributed to, read and approved this manuscript.
☆☆
http://dx.doi.org/10.1016/j.amjoto.2014.07.009 0196-0709/© 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Eksan MS, et al, Rare embryonal rhabdomyosarcoma of external acoustic canal: a case report and literature review, Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.07.009
2
AM ER IC AN JOURNAL OF OTOLARYNGO LOGY – H EAD AN D N E CK ME D I CI NE AN D SUR GE RY XX ( 2 0 14 ) XXX– X XX
metastasis or lymph node involvement. She then underwent 20 cycles radiotherapy. Repeat CT scan of temporal bone and neck showed no focal mass at external ear canal. On regular follow up as outpatient for over 5 years after the diagnosis, patient is well and free of recurrence.
3.
Discussion
Rhabdomyosarcoma represents the most common head and neck sarcoma in the pediatric population. The localization in external ear canal is very rare. H. Charchi in 2010 reported that only 3.75% of the head and neck rhabdomyosarcomas arise from the ear canal [1]. Diagnosis of auricular rhabdomyosarcoma could be delayed by its clinical resemblance to more common and benign lesion in pediatrics such as chronic otitis with otorrhea and aural polyp [2]. In this case, she presented with progressively enlarging aural mass that was obvious on inspection, without history of otorrhea or infection, which increased our index of suspicion of malignant tumor. In one reported series of 50 patients that presented with ear mass, the most common causes of ear mass were inflammatory polyps (20) followed by cholesteatoma (12), chronic non specific inflammation (7), abscess (1), malignancies; squamous cell carcinoma (7), embryonal rhabdomyosarcoma (1) and other benign lesions; carcinoid tumor (1) and nevus (1) [3]. The histopathological examination was essentially required with the support of immunohistochemical studies to make diagnosis. Histologically, rhabdomyosarcoma was one of small round blue cell in childhood like neuroblastoma, lymphoma and Ewing’s sarcoma, and embryonal rhabdomyosarcoma was characterized by primitive spindle cells, often with a myxoid background [1]. Immunohistochemical studies, including staining for desmin, smooth muscle actin and myogenin, are additional studies that can support the diagnosis of rhabdomyosarcoma [1]. (Figs. 1, 2) Modalities of treatment are predominantly surgery, chemotherapy or radiotherapy. Localized or easily accessible non-orbital non-parameningeal head and neck tumors are most often amenable to surgery with low long-term surgical
Fig. 2 – Immunohistochemical staining: vimentin positive.
morbidity and can also be used in the salvage therapy of those who do not respond to chemotherapy and radiotherapy [1]. Recent trends suggest that radical resection procedures are unnecessary, with response to chemotherapy and radiotherapeutic regimes dramatically improving overall event-free survival [4]. Local and regional tumor with embryonal histology responded significantly to VAC (vincristine, dactinomycin, cyclophospamide), VAI (vincristine, dactinomycin, ifosfamide), or VIE (vincristine, ifosfamide, etoposide) plus surgery or radiotherapy [5]. In our case, wide local excision under local anesthesia was done for diagnostic and therapeutic aim without involving resection of bone. Once the diagnosis was confirmed she then underwent 45 Gy, 20 cycles of radiotherapy to the affected ear. CT scan post radiotherapy was clear of any tumor. She was closely followed up and showed no sign of recurrence and asymptomatic for over 5 years. As conclusion, in view of its rarity, making an early correct diagnosis is a challenge. Early diagnosis with early management brought good outcome to the patient. Based on this case, it’s evidenced that local excision of the tumor followed by radiotherapy, offered good prognosis with less morbidity to the patient.
REFERENCES
Fig. 1 – Immunohistochemical staining: myoglobin positive.
[1] Charchi H, Mansouri N, Harmouch A, et al. Epidemiological, histological and immunohistochemical analysis of head and neck rhabdomyosarcoma in children. J Afr Cancer 2011; 3:222–6. [2] Devaney O, Boschman CR, Willard SC, et al. Tumours of the external ear and temporal bone Kenneth. Lancet Oncol 2005; 6:411–20. [3] Agarwal NM, Popat VC, Traviad C, et al. Clinical and histopathological study of mass in ear: a study of fifty cases. Indian J Otolaryngol Head Neck Surg 2013;65(Suppl 3):S520–5. [4] Durve DV, Kanegaonkar RG, Albert D, et al. Paediatric rhabdomyosarcoma of ear and temporal bone. Clin Otolaryngol 2004;29:32–7. [5] Crist WM, Anderson JR, Meza JL, et al. Intergroup Rhabdomyosarcoma Study—IV: results for patients with nonmetastatic disease. J Clin Oncol 2001;19:3091–102.
Please cite this article as: Eksan MS, et al, Rare embryonal rhabdomyosarcoma of external acoustic canal: a case report and literature review, Am J Otolaryngol–Head and Neck Med and Surg (2014), http://dx.doi.org/10.1016/j.amjoto.2014.07.009