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Role of radiation therapy in the treatment of olfactory neuroblastoma
In1 J. Radiation Oncology Biol. Phys.. Vol. 6. pp. 349-352 0 Pergamon Press Ltd.. 1980. Printed m the: US A.
0 Brief Communiclation ROLE ‘OF RADIATION THERAPY IN THE TREATMENT OF OLFACTORY NEUROBLASTOMA KHURSHID
AHMAD,
M.D.;
and
D.L.O.t*
JUAN
V.
FAYOS,
M.D.$
University Hospital, Alice Cracker Lloyd, Radiation Therapy Center, Department of Radiology, Ann Arbor, Michigan 48 109 Nine patients with olfactory neuroblastoma were treated at the Radiation Therapy Service of the University of Michigan Medical Center (UMMC); their case histories are presented. There was a slight female predominance and the peak age distribution was between 60-69 years (4 patients). Distant metastases were found in 44.4% of the patients; the commonest site was bone (3 of 4 patients). One patient developed sudden visual loss in one eye and partial loss in the othler resulting from a hematoma at the optic chiasmal region. We have ascribed it to radiation damage. Our results show that this tumor is radiocontrollable; control at primary site occurs in 66.6% of patients. We recommend postoperative irradiation in all tumors that are extensive initially. Olfactory neuroblastoma, Radiation therapy, Radiocontrollable,
INTRODUCTION
Radionecrosis.
for several months and years before the patient seeks medical advice. They are locally invasive but also metastasize either to regional lymph nodes, lung or bones. The main objective of this study was to determine the effectiveness of irradiation in the control of the primary tumor. The survival and complications were also studied.
Olfactory neuroblastomas are extremely rare tumors. The neoplasm was first recorded by Berger and his associates4 in 1924 in the French literature; they named it L’esthesioneuro-epitheliome olfactif. Since then there have been several isolated case reports, small series, and a large review of the literature reported.” These tumors are neuroectodermal in origin; they consist of neurofibrils and neuro-secretary granuIes8,12 arising from the upper part of the nasal cavity. Three morphological variants of these tumors in accordance with their histological appearance have been described, i.e., neuroepitheliomas with true rosettes, neuroblastomas with pseudorosettes and neurocytomas without any true or pseudorosettes. Since there is no correlation between the histologic subtypes and the biological course of the tumor the term “olfactory neuroblastoma”’ has, been adopted as a genera1 term to include: all the three histologic subtypes.‘-’ The majority of cases usually occur in patients who are between the age of 20 and 40 years; there is a slight male predominance. Symptoms are usually representative of nasal disease or of local invasion into surrounding structures e.g. orbit and brain etc. They usually consist of unilateral nasal obstruction, rhinorrhea, epistaxis, anosmia, headaches, diplopia, proptosis and nasal swelling. The tumor usually appears as polypoidal, greyish-red and friable. It grows slowly and the symptoms may be present
METHODS
AND MATERIALS
During the period 1963 to 1978, nine patients (Table 1) with histologically proven olfactory neuroblastomas were seen at the radiation therapy service of the University of Michigan Medical Center (UMMC). There were four males and five females. Two patients were between the ages of 1O-1 9 years, one between 20-29, two between 40-49, and four between 60-69 years. The youngest patient was 13 years and the oldest, 69 years. None of the patients had regional nodal metastasis at the time of diagnosis. Surgery prior to irradiation consisted of gross removal of the tumor in four patients (Nos. 5,6,7,9), incomplete excision in two (Nos. 3,8), apparent gross removal but microscopic disease left behind at edges in two (Nos. 2,4) and biopsy of the tumor only in one (No. 1). All patients were treated with Co6’ teletherapy, tumor doses ranged from 2800 to 7056 rad but the majority (7/9 patients) received more than 6000 rad. The treatment technique involved either two anterior oblique fields or three fields consisting of two lateral and
*Diploma in Laryngology & Otology from Royal College of Physicians and Surgeons, England. tFellow in Radiology. *Present address: Radiation Therapy Division, Department
of Radiotherapy, University of Miami, Miami, FL 33 101. Requests for reprints: K. Ahmad, M.D., Radiation Therapy, University Hospital, Ann Arbor, MI 48109. Accepted for publication 23 October 1979. 349
350
Radiation
Oncology
0 Biology
0 Physics
Table I. Olfactory
Extent of primary
Pt/Age/Sex
Surgical
March
neuroblastoma
treatment
1980,
Volume 6, Number 3
(9 patients) Radiation dose-rad
Initial radiation date: Status after treatment
Survive
5066
4163: Tumor recurred 54 months later. Rhizotomy for pain.
D C neo at 75m
12/67: Primary controlled. Mets to skull at 103 m. No further treatment.
A C mets at lllm
A/D
I
61
F
Diplopia. Paralysis of 3rd & 6th It. cranial nerves. Destruction of bony walls of sphenoid sinuses.
Transnasal biopsy of sphenoid sinus
2
13
F
Nasal cavity unilateral ethmoid, frontal & spenoid sinuses & medial side of orbit.
Excision of tumor two times. Microscopic disease left behind.
3
14
M
Nasal cavity, antrum, ethmoid, sphenoid sinuses, medial & inferior walls of orbit.
Frontal ethmoidectomy, craniotomy & Caldwel-Luc, incomplete excision.
6100
12/70: Primary controlled. Mets to reg. nodes at I2 m. RND* done. Subsequent lung mets & received chemotherapy
D C mets at 28m
4
40
F
Nasal polypi. Para nasal sinuses clear.
Nasal polypectomies several times. Microscopic disease.
7056
6/7 I : Primary controlled
A S neo at 92m
5
66
M
Mass in nasal cavity
Local excision
7020
9/72: Primary controlled. Mets to Ll, L3 at 24 m. Treated with radiation & chemotherapy.
D C mets at 33m
6
46
F
Mass nasal cavity
Gross excision
6998
9/74: Primary controlled. Sudden visual disturbance at 21 m. Exploration & path showed hematoma at optic chiasma.
A S neo at 52m
I
69
F
Mass in sphenoid sinus with destruction of body of sphenoid, dorsum sellae clivus.
Gross removal through trans-sphenoidal approach.
6600
4/75: Primary uncontrolled. Mets to skull & several vertebrae
D C neo at 5m
8
62
M
Mass ethmoid with extension into frontal lobe.
Bifrontal craniotomy Subtotal excision
6840
12/76: Primary trolled.
A S neo at 24m
9
21
M
Large tumor involving rt ethmoid, rt. orbit, nasopharynx with intracranial extension.
Gross total removal through frontal craniotomy and transnasal approach.
2800
6/78: Treatment discontinued because of rapid progression of tumor into nasal cavity, both orbits & meningitis.
*Radical
Neck Dissection,
6200 after second operation
con-
D C neo at 3m
D C= Dead with, A C= Alive with, A S= Alive without, m = months, Pt = Patient.
one anterior field. Wedge filters were used to achieve homogeneous dose distribution with the object to irradiate the volume of the tumor and to protect the adjacent regions. All patients were treated five days a week with daily fractionation of 180-200 rad. Followup information was obtained mainly through examination of the patients, either in Radiation Therapy or other hospital services, but also by the cancer registry of the hospital or through direct contact with the patient or the patient’s physician. Evaluation of the primary tumor was done on each visit by physical examination and appropriate roentgenograms. If a suspicious area was found, it was biopsied. If recurrence appeared at any time
after irradiation this was considered no control of disease. If no disease was evident on followups, provided the patient had been followed for at least one year this was considered control of disease. No effort was made to biopsy the tumor area in the absence of any signs of recurrence. We felt that the cost, inconvenience and reliability of the results did not justify the procedure. Thus, one patient who died 3 months after irradiation was considered to have died of the disease while a patient who was alive at 24 months with no evidence of disease was considered to be controlled. Survival was calculated in months from the time of initiation of radiation therapy.
Radiation therapy in treatment of olfactory neuroblastoma 0
RESULTS Results were analyzed #according to control of the primary tumor, survival and time to recurrence, complications, and regional and distant metastasis. Control of primary site The primary site was controlled in 6/9 patients (66.6%). These patients had received tumor doses of 6200,6 100,7056, 7020,6998, and 6840 rad respectively. Surgical treatment in these six patients consisted of gross total removal in two (Nos. 5,6), incomplete excision in two (Nos. 3,8) and microscopic disease left behind in two (Nos. 2,4). In two of three patients with uncontrolled disease (Nos. 1,7), surgical treatment consisted of biopsy in No. 1 and gross excision in No. 7. They received 5066 and 6600 rad postoperatively. In the third patient with uncontrolled disease (No. 9), the tumor recurred and progressed rapidly during the course of irradiation. He had gross total removal of tumor before irradiation. However, the treatment was discontinued after 2800 rad. This patient had received 6000 rad 14 years prior to our treatment for extensive recurrent nasopharyngeal angiofibroma. Survival and time of recurrence Three of six patients whose primary site was controlled, were alive at 92, 54 and 24 months after treatment (Nos. 4,6,8); two were dead with metastasis at 28 and 33 months (Nos. 3,5) and one was alive at 111 months with metastasis to parietal and occipital bones of skull (No. 2). Although no biopsies were obtained of the suspected metastatic lesions, the roentgenographic appearance was compatible with metastasis. Additional work-up on the patient revealed no primary lesion. The possibility of being related to radiation damage was excluded since the lesions were outside the volume irradiated. The metastatic lesions could have originated from an occult neoplasm but we iascribe them to the olfactory neuroblastoma for which the patient had a diagnosis. In three patients with uncontrolled primary tumors, death occurred at 75, 5 and 3 months (Nos. 1,7,9) from local extension into brain. One also had distant metastasis. The recurrence in these patients developed at 54 months in one; in the other two, the primary tumor never completely regressed. Complications One patient (No. 6) developed sudden blindness 29 months after irradiation in one eye and partial loss of vision in the other. At surgical exploration she was found to have a small hematoma at optic chiasmal region which was reported pathologically as “hematoma of recent to have resulted origin.” We consider this complication from some vascular phenomenon or radiation damage. The optic chiasmal area had received 6706 rad in 50 days, 34 fractions. No other complications were noted.
K. AHMAD and J. V. FAYOS
351
Regional and distant metastasis Metastases appeared in four of nine patients (44.4%). In three metastases occurred in the bones (skull in No. 2 at 103 months, lumbar vertebrae in No. 5 at 24 months, skull and vertebrae after 4 months in No. 7). The fourth patient developed metastases to the neck nodes and lung (No. 3). In all four patients, except No. 7., the primary site remained under control. DISCUSSION Surgery has been considered the main modality of treatment since the review of the world literature by Skolnick et al” in 1966; they stated that the treatment of choice for the primary tumor was surgery, and irradiation should be reserved for recurrence. It is not uncommon for these patients to seek medical advice months and years after the appearance of the symptoms because the symptoms resemble sinusitis and can be misleading. Though the tumor grows slowly it usually has invaded the surrounding structures by the time of diagnosis. Because of complex-anatomical relationship in this area, it is difficult to remove all of the tumor surgically and even to treat this area with radiation therapy. Kadish et a1.6 and Djalilian et al.5 noted several local recurrences when surgery was the only modality of treatment. In our series, none of the patients who received 6000 rad or more of tumor dose postoperatively developed local recurrence. The failure to control the primary site was directly related to extensive initial size of tumor in 3 patients, (Nos. 1,7,9) and to failure to debulk the tumor because of its location and relatively lower tumor dose in No. 1. The biological behavior of tumor in No. 9 was different and very aggressive. The tumor recurred and spread extensively despite its gross total removal and even during the course of irradiation. This patient had received 6000 rad fourteen years previously for recurrent locally extensive nasopharyngeal angiofibroma. Our data show that in 6 of 9 patients where radiation therapy was used in cancerocidal doses, control of the primary site resulted. As is true of many other cancers the larger the tumor volume, the higher the dose required for eradication.‘.” Immediate post operative irradiation appears to be more effective since the number of malignant cells is at a minimum, if any are left behind. If a recurrence appears later on, the tumor will be larger with a lesser chance of local control.‘.” Radiation occasionally has been reported as a causative factor for brain necrosis.3,9 The likelihood of its occurrence as a result of tumor itself or surgical procedures, is usually ignored. In a recent review of our material of carcinomas of the paranasal sinuses and nasal cavity’.* (141 patients) we found that no patient developed brain necrosis (82% received more than 6000 rad). One of the patients reported here (No. 6) developed a hematoma at the optic chiasmal region 24 months after post operative irradiation; we have ascribed this compli-
352
Radiation
Oncology
0 Biology 0 Physics
March
1980, Volume 6, Number
3
cation to radiation damage although some vascular phenomenon may.have contributed to the development of the hematoma. The technique of irradiation was similar
this tumor is radiocontrollable, as has been proven by the long term local controls in some patients. The possibilities of serious radiation complications are rare. We recom-
in this patient to that of other 141 patients. From the above data it is reasonable to assume
tumors
mend that
high
dose
irradiation
that are locally
after
surgery
in all initial
extensive.
REFERENCES
4. 5.
6.
I.
Ahmad, K., Cordoba, R., Fayos, J.V.: Treatment of carcinoma of maxillary sinus. Submitted for publication. Ahmad, K., Fayos, J.V.: Malignant tumors of paranasal sinuses and nasal cavity. To be submitted for publication. Baron, S.H.: Brain radiation necrosis following treatment of an esthesioneuroblastoma (olfactory neurocytome). Laryngoscope 89: 214-223, 1979. Berger, L., Luc, R.: L’esthesioneuroepitheliome olfactif. Bull. Assoc. Fr. Etude Cancer 13: 410-421, 1924. Djalilian, M., Zujko, R.D., Weiland, L.H., Devine, K.D.: Olfactory neuroblastoma. Surg. Clin. N. Am. 56(4): 75l162,1977. Kadish, S., Goodman, M., Wang, C.C.: Olfactory neuroblastoma. A clinical analysis of I7 cases. Cancer 37: l5711576, 1976. Moss, W.T., Brand, W.N., Battiforce, H.: Radiation oncology. Rationale, Technique, Results, 4th edition. St. Louis, The C. V. Mosby Company. 1973, pp. 21-24.
8. Osmura, R.Y., Fine, G.: Ultrastructure of esthesioneuroblastoma. Cancer 38( 1): 173-l 79, 1976. 9. Rottenberg, D.A., Chernik, N.L., Deck, M.D., Ellis, F., Posner, J.B.: Cerebral necrosis following radiotherapy of extracranial neoplasms. Ann. Neural. l(4): 339-357, 1977. 10. Shukovsky, L.J., Fletcher, G.H.: Time-dose and humor volume relationships in the irradiation of squamous cell carcinoma of tonsillar fossa. Radiology 107: 621-626, 1973. Il. Skolnick, E.M., Massari, F.S., Tenta, L.T.: Olfactory neuropithelioma. Review of world literature and presentation of two cases. Arch. Otolaryngol. 83: 644-653, 1966. 12. Taxy, J.B., Hidvegi, D.F.: Olfactory neuroblastoma. ultrastructural study. Cancer 39: 13 l-l 38, 1977.
An
13. Tyler, T.C., Chandler, J.R., Wetlie, C., Moffit, B.M.: Olfactory neuroblastoma. South. Med. J. 67: 640-643, 1974.
0 Brief Communiclation ROLE ‘OF RADIATION THERAPY IN THE TREATMENT OF OLFACTORY NEUROBLASTOMA KHURSHID
AHMAD,
M.D.;
and
D.L.O.t*
JUAN
V.
FAYOS,
M.D.$
University Hospital, Alice Cracker Lloyd, Radiation Therapy Center, Department of Radiology, Ann Arbor, Michigan 48 109 Nine patients with olfactory neuroblastoma were treated at the Radiation Therapy Service of the University of Michigan Medical Center (UMMC); their case histories are presented. There was a slight female predominance and the peak age distribution was between 60-69 years (4 patients). Distant metastases were found in 44.4% of the patients; the commonest site was bone (3 of 4 patients). One patient developed sudden visual loss in one eye and partial loss in the othler resulting from a hematoma at the optic chiasmal region. We have ascribed it to radiation damage. Our results show that this tumor is radiocontrollable; control at primary site occurs in 66.6% of patients. We recommend postoperative irradiation in all tumors that are extensive initially. Olfactory neuroblastoma, Radiation therapy, Radiocontrollable,
INTRODUCTION
Radionecrosis.
for several months and years before the patient seeks medical advice. They are locally invasive but also metastasize either to regional lymph nodes, lung or bones. The main objective of this study was to determine the effectiveness of irradiation in the control of the primary tumor. The survival and complications were also studied.
Olfactory neuroblastomas are extremely rare tumors. The neoplasm was first recorded by Berger and his associates4 in 1924 in the French literature; they named it L’esthesioneuro-epitheliome olfactif. Since then there have been several isolated case reports, small series, and a large review of the literature reported.” These tumors are neuroectodermal in origin; they consist of neurofibrils and neuro-secretary granuIes8,12 arising from the upper part of the nasal cavity. Three morphological variants of these tumors in accordance with their histological appearance have been described, i.e., neuroepitheliomas with true rosettes, neuroblastomas with pseudorosettes and neurocytomas without any true or pseudorosettes. Since there is no correlation between the histologic subtypes and the biological course of the tumor the term “olfactory neuroblastoma”’ has, been adopted as a genera1 term to include: all the three histologic subtypes.‘-’ The majority of cases usually occur in patients who are between the age of 20 and 40 years; there is a slight male predominance. Symptoms are usually representative of nasal disease or of local invasion into surrounding structures e.g. orbit and brain etc. They usually consist of unilateral nasal obstruction, rhinorrhea, epistaxis, anosmia, headaches, diplopia, proptosis and nasal swelling. The tumor usually appears as polypoidal, greyish-red and friable. It grows slowly and the symptoms may be present
METHODS
AND MATERIALS
During the period 1963 to 1978, nine patients (Table 1) with histologically proven olfactory neuroblastomas were seen at the radiation therapy service of the University of Michigan Medical Center (UMMC). There were four males and five females. Two patients were between the ages of 1O-1 9 years, one between 20-29, two between 40-49, and four between 60-69 years. The youngest patient was 13 years and the oldest, 69 years. None of the patients had regional nodal metastasis at the time of diagnosis. Surgery prior to irradiation consisted of gross removal of the tumor in four patients (Nos. 5,6,7,9), incomplete excision in two (Nos. 3,8), apparent gross removal but microscopic disease left behind at edges in two (Nos. 2,4) and biopsy of the tumor only in one (No. 1). All patients were treated with Co6’ teletherapy, tumor doses ranged from 2800 to 7056 rad but the majority (7/9 patients) received more than 6000 rad. The treatment technique involved either two anterior oblique fields or three fields consisting of two lateral and
*Diploma in Laryngology & Otology from Royal College of Physicians and Surgeons, England. tFellow in Radiology. *Present address: Radiation Therapy Division, Department
of Radiotherapy, University of Miami, Miami, FL 33 101. Requests for reprints: K. Ahmad, M.D., Radiation Therapy, University Hospital, Ann Arbor, MI 48109. Accepted for publication 23 October 1979. 349
350
Radiation
Oncology
0 Biology
0 Physics
Table I. Olfactory
Extent of primary
Pt/Age/Sex
Surgical
March
neuroblastoma
treatment
1980,
Volume 6, Number 3
(9 patients) Radiation dose-rad
Initial radiation date: Status after treatment
Survive
5066
4163: Tumor recurred 54 months later. Rhizotomy for pain.
D C neo at 75m
12/67: Primary controlled. Mets to skull at 103 m. No further treatment.
A C mets at lllm
A/D
I
61
F
Diplopia. Paralysis of 3rd & 6th It. cranial nerves. Destruction of bony walls of sphenoid sinuses.
Transnasal biopsy of sphenoid sinus
2
13
F
Nasal cavity unilateral ethmoid, frontal & spenoid sinuses & medial side of orbit.
Excision of tumor two times. Microscopic disease left behind.
3
14
M
Nasal cavity, antrum, ethmoid, sphenoid sinuses, medial & inferior walls of orbit.
Frontal ethmoidectomy, craniotomy & Caldwel-Luc, incomplete excision.
6100
12/70: Primary controlled. Mets to reg. nodes at I2 m. RND* done. Subsequent lung mets & received chemotherapy
D C mets at 28m
4
40
F
Nasal polypi. Para nasal sinuses clear.
Nasal polypectomies several times. Microscopic disease.
7056
6/7 I : Primary controlled
A S neo at 92m
5
66
M
Mass in nasal cavity
Local excision
7020
9/72: Primary controlled. Mets to Ll, L3 at 24 m. Treated with radiation & chemotherapy.
D C mets at 33m
6
46
F
Mass nasal cavity
Gross excision
6998
9/74: Primary controlled. Sudden visual disturbance at 21 m. Exploration & path showed hematoma at optic chiasma.
A S neo at 52m
I
69
F
Mass in sphenoid sinus with destruction of body of sphenoid, dorsum sellae clivus.
Gross removal through trans-sphenoidal approach.
6600
4/75: Primary uncontrolled. Mets to skull & several vertebrae
D C neo at 5m
8
62
M
Mass ethmoid with extension into frontal lobe.
Bifrontal craniotomy Subtotal excision
6840
12/76: Primary trolled.
A S neo at 24m
9
21
M
Large tumor involving rt ethmoid, rt. orbit, nasopharynx with intracranial extension.
Gross total removal through frontal craniotomy and transnasal approach.
2800
6/78: Treatment discontinued because of rapid progression of tumor into nasal cavity, both orbits & meningitis.
*Radical
Neck Dissection,
6200 after second operation
con-
D C neo at 3m
D C= Dead with, A C= Alive with, A S= Alive without, m = months, Pt = Patient.
one anterior field. Wedge filters were used to achieve homogeneous dose distribution with the object to irradiate the volume of the tumor and to protect the adjacent regions. All patients were treated five days a week with daily fractionation of 180-200 rad. Followup information was obtained mainly through examination of the patients, either in Radiation Therapy or other hospital services, but also by the cancer registry of the hospital or through direct contact with the patient or the patient’s physician. Evaluation of the primary tumor was done on each visit by physical examination and appropriate roentgenograms. If a suspicious area was found, it was biopsied. If recurrence appeared at any time
after irradiation this was considered no control of disease. If no disease was evident on followups, provided the patient had been followed for at least one year this was considered control of disease. No effort was made to biopsy the tumor area in the absence of any signs of recurrence. We felt that the cost, inconvenience and reliability of the results did not justify the procedure. Thus, one patient who died 3 months after irradiation was considered to have died of the disease while a patient who was alive at 24 months with no evidence of disease was considered to be controlled. Survival was calculated in months from the time of initiation of radiation therapy.
Radiation therapy in treatment of olfactory neuroblastoma 0
RESULTS Results were analyzed #according to control of the primary tumor, survival and time to recurrence, complications, and regional and distant metastasis. Control of primary site The primary site was controlled in 6/9 patients (66.6%). These patients had received tumor doses of 6200,6 100,7056, 7020,6998, and 6840 rad respectively. Surgical treatment in these six patients consisted of gross total removal in two (Nos. 5,6), incomplete excision in two (Nos. 3,8) and microscopic disease left behind in two (Nos. 2,4). In two of three patients with uncontrolled disease (Nos. 1,7), surgical treatment consisted of biopsy in No. 1 and gross excision in No. 7. They received 5066 and 6600 rad postoperatively. In the third patient with uncontrolled disease (No. 9), the tumor recurred and progressed rapidly during the course of irradiation. He had gross total removal of tumor before irradiation. However, the treatment was discontinued after 2800 rad. This patient had received 6000 rad 14 years prior to our treatment for extensive recurrent nasopharyngeal angiofibroma. Survival and time of recurrence Three of six patients whose primary site was controlled, were alive at 92, 54 and 24 months after treatment (Nos. 4,6,8); two were dead with metastasis at 28 and 33 months (Nos. 3,5) and one was alive at 111 months with metastasis to parietal and occipital bones of skull (No. 2). Although no biopsies were obtained of the suspected metastatic lesions, the roentgenographic appearance was compatible with metastasis. Additional work-up on the patient revealed no primary lesion. The possibility of being related to radiation damage was excluded since the lesions were outside the volume irradiated. The metastatic lesions could have originated from an occult neoplasm but we iascribe them to the olfactory neuroblastoma for which the patient had a diagnosis. In three patients with uncontrolled primary tumors, death occurred at 75, 5 and 3 months (Nos. 1,7,9) from local extension into brain. One also had distant metastasis. The recurrence in these patients developed at 54 months in one; in the other two, the primary tumor never completely regressed. Complications One patient (No. 6) developed sudden blindness 29 months after irradiation in one eye and partial loss of vision in the other. At surgical exploration she was found to have a small hematoma at optic chiasmal region which was reported pathologically as “hematoma of recent to have resulted origin.” We consider this complication from some vascular phenomenon or radiation damage. The optic chiasmal area had received 6706 rad in 50 days, 34 fractions. No other complications were noted.
K. AHMAD and J. V. FAYOS
351
Regional and distant metastasis Metastases appeared in four of nine patients (44.4%). In three metastases occurred in the bones (skull in No. 2 at 103 months, lumbar vertebrae in No. 5 at 24 months, skull and vertebrae after 4 months in No. 7). The fourth patient developed metastases to the neck nodes and lung (No. 3). In all four patients, except No. 7., the primary site remained under control. DISCUSSION Surgery has been considered the main modality of treatment since the review of the world literature by Skolnick et al” in 1966; they stated that the treatment of choice for the primary tumor was surgery, and irradiation should be reserved for recurrence. It is not uncommon for these patients to seek medical advice months and years after the appearance of the symptoms because the symptoms resemble sinusitis and can be misleading. Though the tumor grows slowly it usually has invaded the surrounding structures by the time of diagnosis. Because of complex-anatomical relationship in this area, it is difficult to remove all of the tumor surgically and even to treat this area with radiation therapy. Kadish et a1.6 and Djalilian et al.5 noted several local recurrences when surgery was the only modality of treatment. In our series, none of the patients who received 6000 rad or more of tumor dose postoperatively developed local recurrence. The failure to control the primary site was directly related to extensive initial size of tumor in 3 patients, (Nos. 1,7,9) and to failure to debulk the tumor because of its location and relatively lower tumor dose in No. 1. The biological behavior of tumor in No. 9 was different and very aggressive. The tumor recurred and spread extensively despite its gross total removal and even during the course of irradiation. This patient had received 6000 rad fourteen years previously for recurrent locally extensive nasopharyngeal angiofibroma. Our data show that in 6 of 9 patients where radiation therapy was used in cancerocidal doses, control of the primary site resulted. As is true of many other cancers the larger the tumor volume, the higher the dose required for eradication.‘.” Immediate post operative irradiation appears to be more effective since the number of malignant cells is at a minimum, if any are left behind. If a recurrence appears later on, the tumor will be larger with a lesser chance of local control.‘.” Radiation occasionally has been reported as a causative factor for brain necrosis.3,9 The likelihood of its occurrence as a result of tumor itself or surgical procedures, is usually ignored. In a recent review of our material of carcinomas of the paranasal sinuses and nasal cavity’.* (141 patients) we found that no patient developed brain necrosis (82% received more than 6000 rad). One of the patients reported here (No. 6) developed a hematoma at the optic chiasmal region 24 months after post operative irradiation; we have ascribed this compli-
352
Radiation
Oncology
0 Biology 0 Physics
March
1980, Volume 6, Number
3
cation to radiation damage although some vascular phenomenon may.have contributed to the development of the hematoma. The technique of irradiation was similar
this tumor is radiocontrollable, as has been proven by the long term local controls in some patients. The possibilities of serious radiation complications are rare. We recom-
in this patient to that of other 141 patients. From the above data it is reasonable to assume
tumors
mend that
high
dose
irradiation
that are locally
after
surgery
in all initial
extensive.
REFERENCES
4. 5.
6.
I.
Ahmad, K., Cordoba, R., Fayos, J.V.: Treatment of carcinoma of maxillary sinus. Submitted for publication. Ahmad, K., Fayos, J.V.: Malignant tumors of paranasal sinuses and nasal cavity. To be submitted for publication. Baron, S.H.: Brain radiation necrosis following treatment of an esthesioneuroblastoma (olfactory neurocytome). Laryngoscope 89: 214-223, 1979. Berger, L., Luc, R.: L’esthesioneuroepitheliome olfactif. Bull. Assoc. Fr. Etude Cancer 13: 410-421, 1924. Djalilian, M., Zujko, R.D., Weiland, L.H., Devine, K.D.: Olfactory neuroblastoma. Surg. Clin. N. Am. 56(4): 75l162,1977. Kadish, S., Goodman, M., Wang, C.C.: Olfactory neuroblastoma. A clinical analysis of I7 cases. Cancer 37: l5711576, 1976. Moss, W.T., Brand, W.N., Battiforce, H.: Radiation oncology. Rationale, Technique, Results, 4th edition. St. Louis, The C. V. Mosby Company. 1973, pp. 21-24.
8. Osmura, R.Y., Fine, G.: Ultrastructure of esthesioneuroblastoma. Cancer 38( 1): 173-l 79, 1976. 9. Rottenberg, D.A., Chernik, N.L., Deck, M.D., Ellis, F., Posner, J.B.: Cerebral necrosis following radiotherapy of extracranial neoplasms. Ann. Neural. l(4): 339-357, 1977. 10. Shukovsky, L.J., Fletcher, G.H.: Time-dose and humor volume relationships in the irradiation of squamous cell carcinoma of tonsillar fossa. Radiology 107: 621-626, 1973. Il. Skolnick, E.M., Massari, F.S., Tenta, L.T.: Olfactory neuropithelioma. Review of world literature and presentation of two cases. Arch. Otolaryngol. 83: 644-653, 1966. 12. Taxy, J.B., Hidvegi, D.F.: Olfactory neuroblastoma. ultrastructural study. Cancer 39: 13 l-l 38, 1977.
An
13. Tyler, T.C., Chandler, J.R., Wetlie, C., Moffit, B.M.: Olfactory neuroblastoma. South. Med. J. 67: 640-643, 1974.