Aesthesioneuroblastoma of Nose Lt Col SC Gupta*, Lt Col AK Das+, Lt Col MD Venkatesh#, Gp Capt RC Kashyap**, Lt Col S Bhattacharya++ MJAFI 2004; 60 : 71-72 Key Words : Aesthesioneuroblastoma; Nasal tumours; Olfactory neuroblastoma
Introduction esthesioneuroblastoma (also called olfactory neuroblastoma) is a rare, slow growing, malignant tumour of neuroectodermal origin that begins in neuroepithelial cells of the olfactory membrane. It was first described by Berger and Luc [1] in 1924, and since then approximately 300 cases have been reported [2]. The tumour appears primarily in the nasal cavity and nasopharynx of adults as a painful swelling and eventually may spread to the sinuses, palate, orbit and brain. These neuroendocrine tumours are rarely associated with hormone excess syndromes such as Cushing syndrome [3] or inappropriate secretion of ADH [4]. These tumours are locally aggressive and have a metastatic potential to regional lymph nodes, and distant sites. Kadish et al [5] developed a staging considering the local extent of the tumour.
A
Case Report A 31 year old male presented with history of right sided nasal obstruction along with mass in right nasal cavity of 6 months duration. There was no history of epistaxis, nasal discharge, frequent sneezing or hyposmia. On examination of nose a single smooth pale looking 2x1.5 cm firm mass was seen in right nasal cavity, arising from upper part of nasal septum. On probing, the mass did not bleed and did not show any white spots on its under surface. Rest of the nasal and paranasal sinus examination was essentially normal. On palpation of neck, no lymph nodes were found enlarged. Systemic examination also did not reveal any abnormality. On investigation, routine blood and urine tests were within normal limits. X-ray paranasal sinuses Water’s view did not reveal any haziness or bony erosion of any of the sinuses. CT scan (axial and coronal) of nose and paranasal sinuses showed a soft tissue mass arising from right side of upper part of septum without any sinus or intracranial extension. Without coming to any conclusive diagnosis it was decided to perform excision biopsy of the mass. The mass was excised in toto along with wide base with electric cautery under local anaesthesia. No excessive bleeding occurred during excision and the post-operative period was uneventful. The tumour mass was subjected to histopathological and immunohistochemical examination and to our surprise the tumour was reported as aesthesioneuroblastoma, one of the rarest *
tumours of nose and paranasal sinuses. Histologically the tumour appeared to have features of a small cell tumour. The tumour cell had a propensity to a perivascular arrangement. The nuclear characteristics did not conform to that of lymphoid cells. Immunohistochemistry was performed to delineate the cell type. The cells were positive for S-100 proteins, synaptophysin and neuron specific enolase. They were negative for lymphocyte common antigen (LCA), vimentin and desmin. Further electron-microscopy was performed wherein the diagnostic membrane bound electron dense cytoplasmic granules were demonstrated (Fig 1). As tumour was already removed in toto with wide base, the patient was subjected to radiotherapy. A total dose of 6000 rads over a period of six weeks was given. At present the patient is under observation without any recurrence.
Discussion Aesthesioneuroblastoma (olfactory neuroblastoma) is an uncommon tumour of the sensory epithelium of the nasal cavity close to the cribriform plate. Histological differentiation is sometimes more difficult because these
Fig. 1 : Electron micrograph of tumour cells showing three nuclei (N) and intervening cytoplasm having membrane bound electron dense granules (G) magnification x 20,000
Classified Specialist (ENT), Military Hospital, Jhansi, +Associate Professor, Department of ENT, **Professor and Head, Department of ENT, ++Associate Professor, Department of Pathology, Armed Forces Medical College, Pune - 411 040, #Classified Specialist (ENT), Command Hospital (Central Command), Lucknow.
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tumours exhibit little or no differentiation [6] and can often be mistaken for other malignancies such as undifferentiated carcinoma, melanoma, lymphoma, or sarcoma [7,8,9]. Its aggressive biologic behaviour, which is usually reflected in its histologic grade, is characterized by inapparent submucosal spread, local recurrence, atypical distant metastases and poor longterm prognosis. It has a predilection for cervical lymph node metastasis and also has potential for distant metastasis to unusual sites like scalp, face, aorta, spleen, liver, adrenal gland and ovary. In fact one case report discussed a rare case of olfactory neuroblastoma in an adolescent girl with metastatic deposits in the breast [7]. Presenting features are unilateral nasal obstruction, epistaxis, rhinorrhea, sinus pain, or proptosis. Metastasis to neck lymph nodes develop in about 30% of patients. Because of the rarity of this malignancy, no definitive consensus regarding the optimal treatment has been reached and considerable controversy exists over its optimal management. Multimodal therapy has been shown to improve survival in these patients. Surgical resection is the treatment of choice, particularly for locally contained low-grade tumour. Neoadjuvant radiotherapy appears to be helpful. Chemotherapy with cisplatin based regimens is helpful for high-grade malignancies, including in the neoadjuvant setting with
radiotherapy. References 1. Berger L, Luc R. Esthesioneuroepithelioma olfactif. Bulletin de 1" Association Francaise pour 1' Etude du Cancer 1924;13:41021. 2. Jekunen AP, Kairemo KJ, KJA, Lehtonen HP, Kajanti MJ. Treatment of olfactory neuroblastoma : a report of 11 cases. Am J Clin Oncol 1996;19:75-8. 3. Arnesen MA, Scheithauer BW, Freeman S. Cushing syndrome secondary to olfactory neuroblastoma. Ultrastructural Pathology 1994;18:61-8. 4. Al Ahwal M, Jha N, Nabholtz JM, Hugh J, Berchall I, Nguyen GK. Olfactory neuroblastoma : report of a case associated with inappropriate antidiuretic hormone secretion. J Otolaryngol (Canada) 1994;23(6):437-9. 5. Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer 1976;37:1571-6. 6. Mills SE, Frierson HF. Olfactory neuroblastoma - a clinico pathologic study of cases. Am J Surg Patho 1985;9:317-27. 7. Oslen KD, DeSanto LW. Olfactory neuroblasotma : biologic and clinical behaviour. Arch Otolaryngol 1983;109:797-802. 8. Levine PA, McClean C, Cantrell RW. Aesthesioneuroblastoma : The University of Virginia Experience 1960-1985. Laryngoscope 1986;96:742-6. 9. Schwaab G, Micheau C, Le Guillou C et al. Olfactory aesthesioneuroblastoma : a report of 40 cases. Laryngoscope (USA) 1988;98:872-6.
A famous newspaper columnist received a number of manuscripts from a young English writer asking his advice as to the best channel for marketing his writing. He sent the Manuscript back with this note “The one channel I can conscientiously recommend as the greatest outlet for articles of this type is the English Channel.”
MJAFI, Vol. 60, No. 1, 2004
Introduction esthesioneuroblastoma (also called olfactory neuroblastoma) is a rare, slow growing, malignant tumour of neuroectodermal origin that begins in neuroepithelial cells of the olfactory membrane. It was first described by Berger and Luc [1] in 1924, and since then approximately 300 cases have been reported [2]. The tumour appears primarily in the nasal cavity and nasopharynx of adults as a painful swelling and eventually may spread to the sinuses, palate, orbit and brain. These neuroendocrine tumours are rarely associated with hormone excess syndromes such as Cushing syndrome [3] or inappropriate secretion of ADH [4]. These tumours are locally aggressive and have a metastatic potential to regional lymph nodes, and distant sites. Kadish et al [5] developed a staging considering the local extent of the tumour.
A
Case Report A 31 year old male presented with history of right sided nasal obstruction along with mass in right nasal cavity of 6 months duration. There was no history of epistaxis, nasal discharge, frequent sneezing or hyposmia. On examination of nose a single smooth pale looking 2x1.5 cm firm mass was seen in right nasal cavity, arising from upper part of nasal septum. On probing, the mass did not bleed and did not show any white spots on its under surface. Rest of the nasal and paranasal sinus examination was essentially normal. On palpation of neck, no lymph nodes were found enlarged. Systemic examination also did not reveal any abnormality. On investigation, routine blood and urine tests were within normal limits. X-ray paranasal sinuses Water’s view did not reveal any haziness or bony erosion of any of the sinuses. CT scan (axial and coronal) of nose and paranasal sinuses showed a soft tissue mass arising from right side of upper part of septum without any sinus or intracranial extension. Without coming to any conclusive diagnosis it was decided to perform excision biopsy of the mass. The mass was excised in toto along with wide base with electric cautery under local anaesthesia. No excessive bleeding occurred during excision and the post-operative period was uneventful. The tumour mass was subjected to histopathological and immunohistochemical examination and to our surprise the tumour was reported as aesthesioneuroblastoma, one of the rarest *
tumours of nose and paranasal sinuses. Histologically the tumour appeared to have features of a small cell tumour. The tumour cell had a propensity to a perivascular arrangement. The nuclear characteristics did not conform to that of lymphoid cells. Immunohistochemistry was performed to delineate the cell type. The cells were positive for S-100 proteins, synaptophysin and neuron specific enolase. They were negative for lymphocyte common antigen (LCA), vimentin and desmin. Further electron-microscopy was performed wherein the diagnostic membrane bound electron dense cytoplasmic granules were demonstrated (Fig 1). As tumour was already removed in toto with wide base, the patient was subjected to radiotherapy. A total dose of 6000 rads over a period of six weeks was given. At present the patient is under observation without any recurrence.
Discussion Aesthesioneuroblastoma (olfactory neuroblastoma) is an uncommon tumour of the sensory epithelium of the nasal cavity close to the cribriform plate. Histological differentiation is sometimes more difficult because these
Fig. 1 : Electron micrograph of tumour cells showing three nuclei (N) and intervening cytoplasm having membrane bound electron dense granules (G) magnification x 20,000
Classified Specialist (ENT), Military Hospital, Jhansi, +Associate Professor, Department of ENT, **Professor and Head, Department of ENT, ++Associate Professor, Department of Pathology, Armed Forces Medical College, Pune - 411 040, #Classified Specialist (ENT), Command Hospital (Central Command), Lucknow.
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Gupta et al
tumours exhibit little or no differentiation [6] and can often be mistaken for other malignancies such as undifferentiated carcinoma, melanoma, lymphoma, or sarcoma [7,8,9]. Its aggressive biologic behaviour, which is usually reflected in its histologic grade, is characterized by inapparent submucosal spread, local recurrence, atypical distant metastases and poor longterm prognosis. It has a predilection for cervical lymph node metastasis and also has potential for distant metastasis to unusual sites like scalp, face, aorta, spleen, liver, adrenal gland and ovary. In fact one case report discussed a rare case of olfactory neuroblastoma in an adolescent girl with metastatic deposits in the breast [7]. Presenting features are unilateral nasal obstruction, epistaxis, rhinorrhea, sinus pain, or proptosis. Metastasis to neck lymph nodes develop in about 30% of patients. Because of the rarity of this malignancy, no definitive consensus regarding the optimal treatment has been reached and considerable controversy exists over its optimal management. Multimodal therapy has been shown to improve survival in these patients. Surgical resection is the treatment of choice, particularly for locally contained low-grade tumour. Neoadjuvant radiotherapy appears to be helpful. Chemotherapy with cisplatin based regimens is helpful for high-grade malignancies, including in the neoadjuvant setting with
radiotherapy. References 1. Berger L, Luc R. Esthesioneuroepithelioma olfactif. Bulletin de 1" Association Francaise pour 1' Etude du Cancer 1924;13:41021. 2. Jekunen AP, Kairemo KJ, KJA, Lehtonen HP, Kajanti MJ. Treatment of olfactory neuroblastoma : a report of 11 cases. Am J Clin Oncol 1996;19:75-8. 3. Arnesen MA, Scheithauer BW, Freeman S. Cushing syndrome secondary to olfactory neuroblastoma. Ultrastructural Pathology 1994;18:61-8. 4. Al Ahwal M, Jha N, Nabholtz JM, Hugh J, Berchall I, Nguyen GK. Olfactory neuroblastoma : report of a case associated with inappropriate antidiuretic hormone secretion. J Otolaryngol (Canada) 1994;23(6):437-9. 5. Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer 1976;37:1571-6. 6. Mills SE, Frierson HF. Olfactory neuroblastoma - a clinico pathologic study of cases. Am J Surg Patho 1985;9:317-27. 7. Oslen KD, DeSanto LW. Olfactory neuroblasotma : biologic and clinical behaviour. Arch Otolaryngol 1983;109:797-802. 8. Levine PA, McClean C, Cantrell RW. Aesthesioneuroblastoma : The University of Virginia Experience 1960-1985. Laryngoscope 1986;96:742-6. 9. Schwaab G, Micheau C, Le Guillou C et al. Olfactory aesthesioneuroblastoma : a report of 40 cases. Laryngoscope (USA) 1988;98:872-6.
A famous newspaper columnist received a number of manuscripts from a young English writer asking his advice as to the best channel for marketing his writing. He sent the Manuscript back with this note “The one channel I can conscientiously recommend as the greatest outlet for articles of this type is the English Channel.”
MJAFI, Vol. 60, No. 1, 2004
