International Journal of Pediatric Otorhinolaryngology 78 (2014) 1416–1418
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Extragonadal primary yolk sac tumour of temporal bone Vineet Narula * , Ravi Meher, Kanika Rana, Jaffer Husein Sura, Jyotsana Nigam, Swati Tandon, Divya Sharma Department of ENT, BL Taneja block, Maulana Azad Medical College and Associated Lok Nayak, G.B. Pant and G.N.E.C. Hospitals, New Delhi 110002, India
A R T I C L E I N F O
A B S T R A C T
Article history: Received 2 June 2014 Received in revised form 9 June 2014 Accepted 11 June 2014 Available online 20 June 2014
Yolk sac tumour, also known as endodermal sinus tumour (EST) is malignant germ cell tumours usually seen in children less than 3 years of age, involving the testis. We describe a rare case of extragonadal primary yolk sac tumour of the temporal bone in a 2.5 years old child presenting with a left sided post auricular mass and facial nerve palsy. Biopsy was consistent with yolk sac tumour showing expression of alpha fetoprotein. Patient was treated with chemotherapy including cisplatin, etoposide, and bleomycin. Post chemotherapy there was decrease in the swelling with improvement in facial nerve palsy. ã 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Extragonadal Yolk sac tumour Temporal bone
1. Introduction Yolk sac tumour (YST) also known as endodermal sinus tumour (EST), is malignant germ cell tumour usually seen in children less than 3 years of age, involving the testis. It is rarely found primarily in extragonadal sites with no involvement of the gonads. Only 4 cases of temporal bone primary yolk sac tumour have been reported in the literature. We describe this case of primary YST of temporal bone with review of previous cases in the literature and also discuss the management of such cases. 2. Case report A 2.5 year old male child presented with a painful swelling in the post-aural region since birth rapidly increasing in size in last 3 months and extending to the pre-auricular region. Patient also had complaints of left sided foul smelling, blood stained ear discharge for last 1 year and deviation of angle of mouth with incomplete eye closure for last 2 months. On examination the swelling was found to be 10 8 cm in size involving the pre and post-auricular region, firm in consistency. Overlying skin was erythematous, with raised local temperature and tenderness present. The patient also had left sided lower motor neuron type facial nerve palsy, House Brackmann Grade V. Rest of the cranial nerves were normal. Rest of the clinical examination including testicular palpation was normal (Fig. 1).
* Corresponding author. Tel.: +91 9868400007. E-mail address: [email protected] (V. Narula). http://dx.doi.org/10.1016/j.ijporl.2014.06.016 0165-5876/ ã 2014 Elsevier Ireland Ltd. All rights reserved.
Patient had history of excision of mass 7 months back in another hospital with inconclusive histopathology report. Slides were reviewed in our hospital which showed it to be extragonadal primary yolk sac tumour of temporal bone. Patient showed a raised serum alpha fetoprotein levels which further confirmed the histopathological finding. The routine haematology including coagulation profile, liver function test, beta human chorionic gonadotropin and carcinoembryonic antigen levels were within normal limits. The patient underwent a contrast enhanced computed tomographic (CT) scan of temporal bone and brain which revealed a large heterogeneously enhancing soft tissue mass lesion involving external auditory canal, middle ear, mastoid air cells with intracranial extension into left posterior temporal and left cerebellar hemisphere. Ultrasound abdomen examination for any abdominal mass or lymph node was within normal limits. Primary chemotherapy with cisplatin, etoposide and bleomycin was started. Six cycles of chemotherapy were given with a gap of 3 weeks between each cycle and each cycle lasting 3 days. Following 6 cycles of chemotherapy patient showed marked decrease in the size of the swelling with improvement in facial nerve palsy with normal serum alpha fetoprotein levels. The patient has been asymptomatic for 6 months following chemotherapy and is under follow up. 3. Discussion Germ cell tumours have diverse clinical, pathologic, and prognostic features, with a reported incidence of 3% of pediatric malignancies, with EST being the most common type [2,3]. Extragonadal germ cell tumours (EGCTs) of the head and neck
V. Narula et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1416–1418
1417
Fig. 1. (a) Clinical view of left pre auricular and post auricular swelling with left facial nerve palsy. (b) Post chemotherapy reduction in swelling size.
account for 5% of all benign and malignant germ cell tumours [1,4]. Teratomas are the most common EGCTs in childhood, which are mostly benign but occasionally have malignant components, like in embryonal carcinoma or ESTs [1]. Endodermal sinus tumours (yolk sac tumours) are rare malignant germ cell neoplasms that represent a selective overgrowth of yolk sac endoderm associated with extraembryonic mesoblast. They usually arise in gonadal sites. There are some rare reports of an extragonadal occurrence of ESTs involving the urinary bladder [5], omentum [6], nasal cavity [7], and head and neck region [7]; the sacrococcygeal [8], retroperitoneum [8], mediastinum [8], and the paranasal sinuses [9]; the parotid gland [10]; the oral cavity [11]; the liver [15]; the brain [15]; and the vagina [2]. There are many histological patterns of YST including microcystic, endodermal sinus, macrocystic, solid, reticular, polyvesicular-vitelline, hepatoid, glandular and pseudopapillary [1]. In the above case the histopathology showed reticular areas formed by a loose meshwork lined by flat and cuboidal cells with pleomorphic nuclei, prominent nucleoli, frequent mitotic figures and vacuolated cytoplasm, with a perivascular organoid arrangement of tumour
cells (Schiller–Duval bodies). Intracytoplasmic and extracellular hyaline globules that stained positively with periodic acid-Schiff were also seen. Immunohistochemistry was strongly positive for AFP with focal expression of pancytokeratin and vimentin, consistent with yolk sac tumour. The reticular form is the most common pattern which was found in the present case [1]. Schiller– Duval bodies are considered pathognomonic for the YST (Figs. 2 and 3). These tumors secrete AFP. Immunofluorescence techniques have been used to localize the site of AFP synthesis in YST samples. The serum levels of this protein may be used as a tumor marker to help in the diagnosis and follow-up of YSTs. The serum AFP was elevated in the present patient and decreased after chemotherapy. Primary yolk sac tumour of temporal bone is very rare. We could find only 4 cases of primary YST of temporal bone in the English literature. In 1987, Stanley et al. [12] presented the first reported case of an EGCT arising from the temporal bone. Their patient was a developmentally delayed patient, and tumour was cured by chemotherapy. In 2000, Frank et al. [13] reported a case of YST of temporal bone in an 18 month old girl presenting with left
Fig. 2. (a) Biopsy showing microcystic pattern of tumor cells (H & E, 20X). Inset: numerous extracytoplasmic hyaline globules (Arrow, H & E, 40X). (b) Immunohistochemistry showing AFP expression in tumour cells (40X).
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4. Conclusion EGCT of temporal bone are rare tumors and high degree of suspicion is required to diagnose them. Biopsy of such tumors must be examined by an expert histopathologist to arrive at a diagnosis along with serum alpha protein level to confirm. The management requires a combined modality of surgery with chemotherapy. Conflict of interest None. Acknowledgement None. References
Fig. 3. Axial section of contrast enhanced computed tomographic scan of temporal bone and brain showing heterogeneously enhancing mass involving the temporal bone with intracranial extension.
facial nerve palsy and mass in left external ear. The patient was treated with chemotherapy, which included cisplatin, etoposide and bleomycin similar to the present case. In 2003 a case of EST arising from temporal bone was reported by Devaney et al. [14]. In 2012, Rozbahany et al. [15] reported a case of yolk sac tumour of post aural region in a 2 year old girl who presented with a postaural swelling, blood stained ear discharge and EAC mass. Computed tomographic scan of the temporal bone showed a large solid mass in the postauricular soft tissue with extension to the mastoid and middle ear and infratemporal fossa. The mass was surgically excised with total parotidectomy with temporalis muscle flap reconstruction. The patient received post operative chemotherapy and was symptom free for 1 year. Out of the 4 cases of primary yolk sac tumour of temporal bone reported in the literature, 3 cases have been treated using chemotherapy while 1 case has been treated surgically with post op chemotherapy. In a series reported by Garnick et al. [16], 15 patients with EGCTs were treated with vinblastine, bleomycin and cisplatin followed by tumor-reductive surgery. In our case since the tumour was not resectable hence chemotherapy was chosen as the preferred option.
[1] P. Kusumakumari, N. Geetha, V.G. Chellam, M.K. Nair, Endodermal sinus tumors in the head and neck region, Med. Pediatr. Oncol. 29 (October(4)) (1997) 303–307. [2] J. Lacy, M. Capra, L. Allen, Endodermal sinus tumor of the infant vagina treated exclusively with chemotherapy, J. Pediatr. Hematol. Oncol. 28 (November(11)) (2006) 768–771. [3] M. Arora, R.K. Shrivastav, M.P. Jaiprakash, A rare germ-cell tumor site: vaginal endodermal sinus tumor, Pediatr. Surg. Int. 18 (September(5–6)) (2002) 521– 523. [4] E.E. Lack, Extragonadal germ cell tumor of the head and neck region: review of 16 cases, Hum. Pathol. 16 (January(1)) (1985) 56–64. [5] W.H. Mui, K.C. Lee, S.C. Chui, et al., Primary yolk sac tumour of the urinary bladder: a case report and review of the literature, Oncol. Lett. 7 (January(1)) (2014) 199–202. [6] Y. Xinghui, H. Jing, L. Mingju, et al., Endodermal sinus tumour of the omentum in a child, Pediatr. Radiol. 34 (December(12)) (2004) 985–987. [7] B.C. Filho, J.B. McHugh, R.L. Carrau, et al., Yolk sac tumor in the nasal cavity, Am. J. Otolaryngol. 29 (July–August(4)) (2008) 250–254. [8] A. De Backer, G.C. Madern, R. Pieters, et al., Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors, Eur. J. Pediatr. Surg. 18 (February(1)) (2008) 1–6. [9] A. Mishra, A.K. El-Naggar, F. DeMonte, et al., Endodermal sinus tumor of the paranasal sinuses, Head Neck 30 (April(4)) (2008) 539–543. [10] E. Viva, F. Zorzi, G. Annibale, et al., Endodermal sinus (yolk sac) tumor of the parotid gland: a case report, Int. J. Pediatr. Otorhinolaryngol. 24 (November(3)) (1992) 269–274. raş, Akman E:, Endodermal sinus (yolk sac) tumor of oral [11] A. Kutluhan, S. Ug cavity originating from gingiva, Auris Nasus Larynx 25 (December(4)) (1998) 459–462. [12] R.J. Stanley, B.W. Scheithauer, E.I. Thompson, et al., Endodermal sinus tumor (yolk sac tumor) of the ear, Arch. Otolaryngol. Head Neck Surg. 113 (February (2)) (1987) 200–203. [13] T.C. Frank, V.K. Anand, C. Subramony, Yolk sac tumor of the temporal bone: report of a case, Ear Nose Throat J. 79 (March(3)) (2000) 183–192. [14] K.O. Devaney, A. Ferlito, A. Rinaldo, Endodermal sinus tumor (yolk sac tumor) of the temporal bone: an exotic disease for otorhinolaryngologists and head and neck surgeons, Acta Otolaryngol. 123 (August(6)) (2003) 747–748. [15] N.A. Rozbahany, M. Hasanzadazar, H. Latifi, et al., Yolk-sac tumor of the postauricular region: case report and review of the literature, J. Oral Maxillofac. Surg. 70 (August(8)) (2012) 1891–1895. [16] M.B. Garnick, G.P. Canellos, J.P. Richie, Treatment and surgical staging of testicular and primary extragonadal germ cell cancer, JAMA 250 (October(13)) (1983) 1733–1741.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Extragonadal primary yolk sac tumour of temporal bone Vineet Narula * , Ravi Meher, Kanika Rana, Jaffer Husein Sura, Jyotsana Nigam, Swati Tandon, Divya Sharma Department of ENT, BL Taneja block, Maulana Azad Medical College and Associated Lok Nayak, G.B. Pant and G.N.E.C. Hospitals, New Delhi 110002, India
A R T I C L E I N F O
A B S T R A C T
Article history: Received 2 June 2014 Received in revised form 9 June 2014 Accepted 11 June 2014 Available online 20 June 2014
Yolk sac tumour, also known as endodermal sinus tumour (EST) is malignant germ cell tumours usually seen in children less than 3 years of age, involving the testis. We describe a rare case of extragonadal primary yolk sac tumour of the temporal bone in a 2.5 years old child presenting with a left sided post auricular mass and facial nerve palsy. Biopsy was consistent with yolk sac tumour showing expression of alpha fetoprotein. Patient was treated with chemotherapy including cisplatin, etoposide, and bleomycin. Post chemotherapy there was decrease in the swelling with improvement in facial nerve palsy. ã 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Extragonadal Yolk sac tumour Temporal bone
1. Introduction Yolk sac tumour (YST) also known as endodermal sinus tumour (EST), is malignant germ cell tumour usually seen in children less than 3 years of age, involving the testis. It is rarely found primarily in extragonadal sites with no involvement of the gonads. Only 4 cases of temporal bone primary yolk sac tumour have been reported in the literature. We describe this case of primary YST of temporal bone with review of previous cases in the literature and also discuss the management of such cases. 2. Case report A 2.5 year old male child presented with a painful swelling in the post-aural region since birth rapidly increasing in size in last 3 months and extending to the pre-auricular region. Patient also had complaints of left sided foul smelling, blood stained ear discharge for last 1 year and deviation of angle of mouth with incomplete eye closure for last 2 months. On examination the swelling was found to be 10 8 cm in size involving the pre and post-auricular region, firm in consistency. Overlying skin was erythematous, with raised local temperature and tenderness present. The patient also had left sided lower motor neuron type facial nerve palsy, House Brackmann Grade V. Rest of the cranial nerves were normal. Rest of the clinical examination including testicular palpation was normal (Fig. 1).
* Corresponding author. Tel.: +91 9868400007. E-mail address: [email protected] (V. Narula). http://dx.doi.org/10.1016/j.ijporl.2014.06.016 0165-5876/ ã 2014 Elsevier Ireland Ltd. All rights reserved.
Patient had history of excision of mass 7 months back in another hospital with inconclusive histopathology report. Slides were reviewed in our hospital which showed it to be extragonadal primary yolk sac tumour of temporal bone. Patient showed a raised serum alpha fetoprotein levels which further confirmed the histopathological finding. The routine haematology including coagulation profile, liver function test, beta human chorionic gonadotropin and carcinoembryonic antigen levels were within normal limits. The patient underwent a contrast enhanced computed tomographic (CT) scan of temporal bone and brain which revealed a large heterogeneously enhancing soft tissue mass lesion involving external auditory canal, middle ear, mastoid air cells with intracranial extension into left posterior temporal and left cerebellar hemisphere. Ultrasound abdomen examination for any abdominal mass or lymph node was within normal limits. Primary chemotherapy with cisplatin, etoposide and bleomycin was started. Six cycles of chemotherapy were given with a gap of 3 weeks between each cycle and each cycle lasting 3 days. Following 6 cycles of chemotherapy patient showed marked decrease in the size of the swelling with improvement in facial nerve palsy with normal serum alpha fetoprotein levels. The patient has been asymptomatic for 6 months following chemotherapy and is under follow up. 3. Discussion Germ cell tumours have diverse clinical, pathologic, and prognostic features, with a reported incidence of 3% of pediatric malignancies, with EST being the most common type [2,3]. Extragonadal germ cell tumours (EGCTs) of the head and neck
V. Narula et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1416–1418
1417
Fig. 1. (a) Clinical view of left pre auricular and post auricular swelling with left facial nerve palsy. (b) Post chemotherapy reduction in swelling size.
account for 5% of all benign and malignant germ cell tumours [1,4]. Teratomas are the most common EGCTs in childhood, which are mostly benign but occasionally have malignant components, like in embryonal carcinoma or ESTs [1]. Endodermal sinus tumours (yolk sac tumours) are rare malignant germ cell neoplasms that represent a selective overgrowth of yolk sac endoderm associated with extraembryonic mesoblast. They usually arise in gonadal sites. There are some rare reports of an extragonadal occurrence of ESTs involving the urinary bladder [5], omentum [6], nasal cavity [7], and head and neck region [7]; the sacrococcygeal [8], retroperitoneum [8], mediastinum [8], and the paranasal sinuses [9]; the parotid gland [10]; the oral cavity [11]; the liver [15]; the brain [15]; and the vagina [2]. There are many histological patterns of YST including microcystic, endodermal sinus, macrocystic, solid, reticular, polyvesicular-vitelline, hepatoid, glandular and pseudopapillary [1]. In the above case the histopathology showed reticular areas formed by a loose meshwork lined by flat and cuboidal cells with pleomorphic nuclei, prominent nucleoli, frequent mitotic figures and vacuolated cytoplasm, with a perivascular organoid arrangement of tumour
cells (Schiller–Duval bodies). Intracytoplasmic and extracellular hyaline globules that stained positively with periodic acid-Schiff were also seen. Immunohistochemistry was strongly positive for AFP with focal expression of pancytokeratin and vimentin, consistent with yolk sac tumour. The reticular form is the most common pattern which was found in the present case [1]. Schiller– Duval bodies are considered pathognomonic for the YST (Figs. 2 and 3). These tumors secrete AFP. Immunofluorescence techniques have been used to localize the site of AFP synthesis in YST samples. The serum levels of this protein may be used as a tumor marker to help in the diagnosis and follow-up of YSTs. The serum AFP was elevated in the present patient and decreased after chemotherapy. Primary yolk sac tumour of temporal bone is very rare. We could find only 4 cases of primary YST of temporal bone in the English literature. In 1987, Stanley et al. [12] presented the first reported case of an EGCT arising from the temporal bone. Their patient was a developmentally delayed patient, and tumour was cured by chemotherapy. In 2000, Frank et al. [13] reported a case of YST of temporal bone in an 18 month old girl presenting with left
Fig. 2. (a) Biopsy showing microcystic pattern of tumor cells (H & E, 20X). Inset: numerous extracytoplasmic hyaline globules (Arrow, H & E, 40X). (b) Immunohistochemistry showing AFP expression in tumour cells (40X).
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V. Narula et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1416–1418
4. Conclusion EGCT of temporal bone are rare tumors and high degree of suspicion is required to diagnose them. Biopsy of such tumors must be examined by an expert histopathologist to arrive at a diagnosis along with serum alpha protein level to confirm. The management requires a combined modality of surgery with chemotherapy. Conflict of interest None. Acknowledgement None. References
Fig. 3. Axial section of contrast enhanced computed tomographic scan of temporal bone and brain showing heterogeneously enhancing mass involving the temporal bone with intracranial extension.
facial nerve palsy and mass in left external ear. The patient was treated with chemotherapy, which included cisplatin, etoposide and bleomycin similar to the present case. In 2003 a case of EST arising from temporal bone was reported by Devaney et al. [14]. In 2012, Rozbahany et al. [15] reported a case of yolk sac tumour of post aural region in a 2 year old girl who presented with a postaural swelling, blood stained ear discharge and EAC mass. Computed tomographic scan of the temporal bone showed a large solid mass in the postauricular soft tissue with extension to the mastoid and middle ear and infratemporal fossa. The mass was surgically excised with total parotidectomy with temporalis muscle flap reconstruction. The patient received post operative chemotherapy and was symptom free for 1 year. Out of the 4 cases of primary yolk sac tumour of temporal bone reported in the literature, 3 cases have been treated using chemotherapy while 1 case has been treated surgically with post op chemotherapy. In a series reported by Garnick et al. [16], 15 patients with EGCTs were treated with vinblastine, bleomycin and cisplatin followed by tumor-reductive surgery. In our case since the tumour was not resectable hence chemotherapy was chosen as the preferred option.
[1] P. Kusumakumari, N. Geetha, V.G. Chellam, M.K. Nair, Endodermal sinus tumors in the head and neck region, Med. Pediatr. Oncol. 29 (October(4)) (1997) 303–307. [2] J. Lacy, M. Capra, L. Allen, Endodermal sinus tumor of the infant vagina treated exclusively with chemotherapy, J. Pediatr. Hematol. Oncol. 28 (November(11)) (2006) 768–771. [3] M. Arora, R.K. Shrivastav, M.P. Jaiprakash, A rare germ-cell tumor site: vaginal endodermal sinus tumor, Pediatr. Surg. Int. 18 (September(5–6)) (2002) 521– 523. [4] E.E. Lack, Extragonadal germ cell tumor of the head and neck region: review of 16 cases, Hum. Pathol. 16 (January(1)) (1985) 56–64. [5] W.H. Mui, K.C. Lee, S.C. Chui, et al., Primary yolk sac tumour of the urinary bladder: a case report and review of the literature, Oncol. Lett. 7 (January(1)) (2014) 199–202. [6] Y. Xinghui, H. Jing, L. Mingju, et al., Endodermal sinus tumour of the omentum in a child, Pediatr. Radiol. 34 (December(12)) (2004) 985–987. [7] B.C. Filho, J.B. McHugh, R.L. Carrau, et al., Yolk sac tumor in the nasal cavity, Am. J. Otolaryngol. 29 (July–August(4)) (2008) 250–254. [8] A. De Backer, G.C. Madern, R. Pieters, et al., Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors, Eur. J. Pediatr. Surg. 18 (February(1)) (2008) 1–6. [9] A. Mishra, A.K. El-Naggar, F. DeMonte, et al., Endodermal sinus tumor of the paranasal sinuses, Head Neck 30 (April(4)) (2008) 539–543. [10] E. Viva, F. Zorzi, G. Annibale, et al., Endodermal sinus (yolk sac) tumor of the parotid gland: a case report, Int. J. Pediatr. Otorhinolaryngol. 24 (November(3)) (1992) 269–274. raş, Akman E:, Endodermal sinus (yolk sac) tumor of oral [11] A. Kutluhan, S. Ug cavity originating from gingiva, Auris Nasus Larynx 25 (December(4)) (1998) 459–462. [12] R.J. Stanley, B.W. Scheithauer, E.I. Thompson, et al., Endodermal sinus tumor (yolk sac tumor) of the ear, Arch. Otolaryngol. Head Neck Surg. 113 (February (2)) (1987) 200–203. [13] T.C. Frank, V.K. Anand, C. Subramony, Yolk sac tumor of the temporal bone: report of a case, Ear Nose Throat J. 79 (March(3)) (2000) 183–192. [14] K.O. Devaney, A. Ferlito, A. Rinaldo, Endodermal sinus tumor (yolk sac tumor) of the temporal bone: an exotic disease for otorhinolaryngologists and head and neck surgeons, Acta Otolaryngol. 123 (August(6)) (2003) 747–748. [15] N.A. Rozbahany, M. Hasanzadazar, H. Latifi, et al., Yolk-sac tumor of the postauricular region: case report and review of the literature, J. Oral Maxillofac. Surg. 70 (August(8)) (2012) 1891–1895. [16] M.B. Garnick, G.P. Canellos, J.P. Richie, Treatment and surgical staging of testicular and primary extragonadal germ cell cancer, JAMA 250 (October(13)) (1983) 1733–1741.
