Plasmacytoma of middle ear and upper respiratory tract By M. A. NOORANI (Dar es Salaam, Tanzania)
THE extramedullary plasmacytoma on the whole is a very rare disease. A survey of the world literature in English has revealed only 192 cases of plasmacytoma of the head and neck region. Not a single case of extramedullary plasmacytoma of the middle ear has been published so far. The main purpose of this paper is to present the first-ever reported case of extramedullary plasmacytoma of the middle ear, and also to present two more cases of extramedullary plasmacytoma of the upper respiratory tract. All these cases were encountered in the Department of Otolaryngology, Faculty of Medicine, University of Dar es Salaam, Tanzania. Certain problems in the diagnosis and management of extramedullary plasmacytoma of the head and neck region will be discussed and the world literature in English will be reviewed. Case reports
Case 1: Mr. K.D., married, aged fifty years, trader. This patient was admitted to the E.N.T. Department of Muhimbili Hospital, Dar es Salaam, on 28 June 1972. He complained of the presence of a growth in the left ear, deafness, otorrhoea and weakness of the left side of his face. The duration of symptoms was a few months. There was a history of recurrent attacks of blood-stained discharge from the same ear. He also complained of the presence of a mass in the right nostril, nasal obstruction, rhinorrhoea and epistaxis. Clinical examination of the left ear revealed a fairly large friable greyishwhite polyp in the external auditory canal. There was plenty of pus around the polyp and the left side of his face was completely paralysed. There was no swelling or tenderness over the mastoid region. On X-ray examination both mastoids were found to be fully pneumatized. On examination of his nose, a large greyish-white friable polyp was seen protruding from the right nostril (Fig. 1). This was arising from the lateral wall. The sinus X-rays showed only thickening of the lateral wall of right nasal cavity (Fig. 2). On 5 July 1973 the polyp from the left ear was removed with Glegg's snare under general anaesthetic and sent for histopathology. This polyp was arising from the middle-ear cavity and there was subtotal perforation of the eardrum. It was difficult to determine the exact site of origin and extent of the growth due to profuse haemorrhage at the time of operation. The haemorrhage was controlled by packing with ribbon gauze. The nasal polyp was also removed at the same time and sent for histopathology. The nasal polyp was arising from the lateral wall of the nasal cavity. The biopsy of the aural polyp was reported as infected plasmacytoma and that of the nasal polyp as plasmacytoma. 105
M. A. Noorani Following these surprising biopsy results, the patient was investigated to exclude multiple myeloma. The skeletal survey did not show any evidence of multiple myeloma. The plasma proteins were reported as follows: Total protein 6-3 g./ioo ml. Albumen 2-8 g./ioo ml. Globulin 4-5 g./ioo ml. Albumen/globulin ratio o-6:i
FIG. I .
Case 1: The growth is seen protruding from right nostril. He has left facial paralysis as well.
There was no Bence Jones protein in the urine. The other haematological investigations were within normal limits. He was treated by radiotherapy. 1,800 rads were given to the left ear and 3,000 rads to the nose. There was a good response to radiotherapy. So far there is no sign of recurrence either in the middle ear or the nasal cavity. Case 2: Mr. S.P., married, aged thirty-five years, farmer. This patient was first admitted to the E.N.T. Department of Muhimbili Hospital on 20 July 1972. At that time he complained of nasal obstruction, 106
Clinical records foul-smelling discharge and swelling of right side of his nose. The duration of these complaints was one month. There was no history of epistaxis at this time.
J
FIG. 2. Case I: O.M. view of paranasal sinuses showing thickening of lateral wall of right nasa cavity.
On clinical examination the upper part of his external nose was found to be swollen on the right side. There was a large dark-reddish friable growth in the right nostril. This was arising from the lateral wall. There was no swelling or tenderness over the right maxilla. His teeth and hard palate looked normal. The cervical lymph nodes were not palpable. His general health was good. The Hb was 92 per cent. The results of other routine blood and urine examination were within normal limits. The sinus X-rays did not show any involvement of the right maxillary sinus. A biopsy was taken on 1 August 1972. This was reported as plasmacytoma. Therefore, the following investigations were carried out to exclude the presence of multiple myeloma: Total plasma protein 7-6 g./ioo ml. Albumen 2-7 g./ioo ml. Globulin 4-9 g./ioo ml. Albumen/globulin ratio o-6:i 107
M. A. Noorani There was no Bence-Jones protein in the urine and the skeletal survey was negative. The bone marrow studies revealed a slight increase in plasma cells but there was no evidence of multiple myeloma. The electrophoresis was reported as within normal limits. The patient was advised to stay in the hospital for about six weeks more for a course of radiotherapy, but he decided to go home to sort out his personal problems and promised to return to the hospital after one week. Instead he turned up about ten months later. He reported to the E.N.T. department on 30 May 1973. This time he complained of recurrent attacks of bleeding from the right nostril for the past two months. During this period he also developed a swelling over the right maxilla and hard palate. This swelling is clearly visible in Figure 3. The sinus X-rays were repeated and these revealed
FIG. 3. Case 2: Lateral view of the patient. Swelling of right cheek is quite prominent.
opacity of the right antrum and erosion in its medial wall (Fig. 4). The growth was treated by radiotherapy. The total tumour dose was 4,000 rads. This was followed by total maxillectomy on 10 August 1973. So far the patient is symptom-free and there is no sign of recurrence. Case 3 : Mrs. R.S., married, aged twenty-five years, housewife. This patient was first admitted to Muhimbili Hospital on 18 April 1972. She was referred from one of the provincial hospitals in Tanzania where she was admitted for the treatment of severe epistaxis. She complained of the presence of a large growth in the nose, recurrent attacks of epistaxis and nasal obstruction. The duration of complaints was unknown. While she was in this regional hospital she had an attack of severe epistaxis and was given blood transfusion. 108
Clinical records On clinical examination she was found to have a massive growth over the nose (Fig. 5). The left nostril was completely filled with a firm whitish growth. The whole nose was grossly distorted by the tumour. The septum was completely destroyed and the whole of the nasal cavity was filled with the tumour mass. The tip and left nostril were completely destroyed. The growth had eroded through the walls of the left nostril and had spread over the bridge, left cheek and upper Up. The growth had also extended up to the left lower eyelid but the eyelid itself was not involved. Although it was oedematous she complained of epiphora. The upper lip was markedly swollen. The middle part of the growth had ulcerated through the skin. The hard palate and teeth were not involved and there was no involvement of the cervical lymph nodes.
FIG. 4. Case 2: The photograph of sinus X-rays showing opacity of right maxillary sinus.
The Hb was 61 per cent and total WBC 7,600. Routine examination of urine and stools did not reveal any abnormalities. She had severe epistaxis on the following day. This was treated by conservative measures and two pints of blood were transfused. A biopsy from the tumour was taken on 24 April 1972. This was 109
M. A. Noorani reported as plasmacytoma. Hence the following investigations were carried out to exclude the generalized disease: Total plasma protein 6-3 g./ioo ml. Albumen 2-8 g./ioo ml. Globulin 4-5 g./ioo ml. Albumen /globulin ratio o-6:i There was no Bence-Jones protein in the urine, and the skeletal survey was negative. Bone-marrow studies showed a moderate increase in plasma cells but there were no myeloma cells. The electrophoresis was normal. She was given a full course of irradiation with Cobalt 60 but the response was not satisfactory. The tumour was therefore excised on 1 December 1972.
FIG. 5. Case 3: Plasmacytoma of nose infiltrating the surrounding regions.
Discussion Extramedullary plasmacyto-na is said to be of rare occurrence either as a localized manifestation or as a part of the generalized disease called multiple myeloma. The most common site is the head and neck region. Willis classified plasmacytoma into the following three groups: Group I: multiple myeloma This is a generalized disease involving various bones. The X-ray appearance of the lesions is characteristic. The serum protein is abnormal and Bence-Jones protein is present in urine. The bone marrow findings are also characteristic. Group II: solitary plasmacytoma of bone The lesion is localized to one particular bone only. There is no clinical or laboratory evidence of generalized disease. no
Clinical records Group III: primary plasmacytoma of soft tissue This may be single or multiple. In this paper, I have presented the first case ever reported of plasmacytoma of the middle ear. Incidentally, this patient had plasmacytoma of the nose as well. In addition to this, one case of plasmacytoma of the nasal cavity and one of plasmacytoma of nasal cavity and maxillary sinus have been presented. The survey of the world literature in English has not revealed a single case of plasmacytoma of the middle ear. All these three patients were adults. Two were male and one was female. Poole and Marchetta (1968) reviewed 189 cases and reported that 78 per cent of the cases were male and 22 per cent were female. The incidence is highest between fifty and seventy years of age. But it has been reported in patients as young as five and as old as eighty-nine years of age (Touma, 1971). The same author has reported that 90 per cent of extramedullary plasmacytoma are found in the upper respiratory tract and oral cavity. About 60 per cent of the cases are found in the region of the nose, nasopharynx and paranasal sinuses. A review of 192 cases of plasmacytoma of the head and neck showed the following sites of origin: nose, 40; nasopharynx, 41; paranasal sinuses, 34; tonsil and pharynx, 26; palate, gum and maxilla, 12; oropharynx, 10; larynx, 9; combined, 6; floor of the mouth and tongue, 5; others or site unknown, 9. The aetiology of plasmacytoma is not known. It may manifest itself as a solitary bone or extramedullary soft tissue lesion or as a generalized bone disease. A certain number of patients suffering from a solitary lesion may ultimately progress to generalized disease. Others remain symptom-free for a long time following successful treatment by either radiation alone or surgery alone or combined treatment. Castro, Lewis and Strong (1973) reviewed 772 patients with tumours of the nasal cavity and paranasal sinuses seen over a period of thirtyfive years and reported only twenty-three cases of plasmacytoma. The overall incidence of plasmacytoma was only 3 per cent. This shows that it is a comparatively rare disease. The usual symptoms of plasmacytoma of the nasal cavity are rhinorrhoea, nasal obstruction and epistaxis. The epistaxis is quite often profuse, requiring blood transfusions. All of my three patients had epistaxis. One of them was a very advanced case. This patient suffered from recurrent attacks of profuse nose bleeds and as a result she required frequent blood transfusions. In our part of the world, a large number of patients first report to the doctors when the disease has reached a fairly advanced stage. This is what happened to two of my cases. The third case (Mr. S.P.) was referred to the E.N.T. department when the disease was in a comparatively early stage. When he first reported to me the lesion was confined to the nasal cavity only. The biopsy was taken and this was reported as plasmacytoma. He was therefore advised to stay in the hospital for a course of radiotherapy, but as he had urgent personal business to attend to he decided to go home for one week. Instead he turned up about one year later. By this time the disease had invaded the maxillary sinus as well. Though there has been considerable progress in diagnostic techniques, still it is quite often not possible to determine the exact site of origin and extent of the in
M. A. Noorani disease. This is due to the peculiar anatomical features of this region. The presence of generalized disease is excluded by doing the following investigations: examination of the urine for Bence-Jones protein; serum protein estimation; skeletal survey; bone-marrow studies; and serum protein electrophoresis. These tumours are highly radio-sensitive and therefore initially they should be treated by radio-therapy alone. In my first case only i,8oo rads were required for the middle-ear tumour and 3,000 rads for the tumour in the nose. Only excision biopsy was carried out in this case. Surgery is indicated when response to radiotherapy is not satisfactory. This usually happens in advanced cases as seen in two of my patients. But on the whole the cases of extramedullary plasmacytoma are so few and far between that it is not possible for anybody to advocate the exact line of treatment. Each case should be considered on its own merits. But on the whole the combined treatment appears to give better results. The local recurrences can be treated by surgical excision, electrocoagulation or cryosurgery. The five-year survival rate of extramedullary plasmacytoma is about 50 per cent, while the majority of patients suffering from multiple myeloma die within two years. The prognosis of extramedullary plasmacytoma is unpredictable. All of my three patients have been regularly followed up for the last one-and-a-half years. So far there is no local recurrence and there is no evidence of generalized disease. Many cases of extramedullary plasmacytoma may ultimately progress to multiple myeloma. In rare cases, extramedullary plasmacytoma may present as a solitary bone lesion many years after successful treatment with radiotherapy. Chan and Pam (1969) reported a case of extramedullary plasmacytoma of the nasal cavity presenting as a solitary lesion of the humerous eight years after successful treatment with radiotherapy.
Summary The first case of extramedullary plasmacytoma of middle ear is reported. One case of plasmacytoma of the nasal cavity alone and another of the nasal cavity and maxillary sinus have also been described. Some of the problems in diagnosis and management have been discussed. Acknowledgements I wish to thank Dr. James Shaba, Dr. Axel Ruffmann and Dr. Jacob Schneider of the Department of Pathology and Professor K. J. Lindquist of Department of Microbiology for their help in carrying out the laboratory investigations. The photographs were provided by the Medical Illustration Department of the Faculty of Medicine. BIBLIOGRAPHY CASTRO, E L . B., LEWIS, J. S., and STRONG, E. W. (1973) Archives of Otolaryngology,
97, 326. CHAN, K. P., and TAM, C. S. (1969) Cancer, 23, 694. POOLE, A. G., and MARCHETTA, C. (1968) Cancer, 22, 14.
112
Clinical records TOUMA, Y. B. (1971) Journal of Laryngology and Otology, 85, 125. WILLIS,
R. A. (i960) Pathology of Tumours, 3rd ed. (London), p. 791.
Department of Otolaryngology, Faculty of Medicine, University of Dar es Salaam, P.O. Box 4365, Dar es Salaam, Tanzania.
113
THE extramedullary plasmacytoma on the whole is a very rare disease. A survey of the world literature in English has revealed only 192 cases of plasmacytoma of the head and neck region. Not a single case of extramedullary plasmacytoma of the middle ear has been published so far. The main purpose of this paper is to present the first-ever reported case of extramedullary plasmacytoma of the middle ear, and also to present two more cases of extramedullary plasmacytoma of the upper respiratory tract. All these cases were encountered in the Department of Otolaryngology, Faculty of Medicine, University of Dar es Salaam, Tanzania. Certain problems in the diagnosis and management of extramedullary plasmacytoma of the head and neck region will be discussed and the world literature in English will be reviewed. Case reports
Case 1: Mr. K.D., married, aged fifty years, trader. This patient was admitted to the E.N.T. Department of Muhimbili Hospital, Dar es Salaam, on 28 June 1972. He complained of the presence of a growth in the left ear, deafness, otorrhoea and weakness of the left side of his face. The duration of symptoms was a few months. There was a history of recurrent attacks of blood-stained discharge from the same ear. He also complained of the presence of a mass in the right nostril, nasal obstruction, rhinorrhoea and epistaxis. Clinical examination of the left ear revealed a fairly large friable greyishwhite polyp in the external auditory canal. There was plenty of pus around the polyp and the left side of his face was completely paralysed. There was no swelling or tenderness over the mastoid region. On X-ray examination both mastoids were found to be fully pneumatized. On examination of his nose, a large greyish-white friable polyp was seen protruding from the right nostril (Fig. 1). This was arising from the lateral wall. The sinus X-rays showed only thickening of the lateral wall of right nasal cavity (Fig. 2). On 5 July 1973 the polyp from the left ear was removed with Glegg's snare under general anaesthetic and sent for histopathology. This polyp was arising from the middle-ear cavity and there was subtotal perforation of the eardrum. It was difficult to determine the exact site of origin and extent of the growth due to profuse haemorrhage at the time of operation. The haemorrhage was controlled by packing with ribbon gauze. The nasal polyp was also removed at the same time and sent for histopathology. The nasal polyp was arising from the lateral wall of the nasal cavity. The biopsy of the aural polyp was reported as infected plasmacytoma and that of the nasal polyp as plasmacytoma. 105
M. A. Noorani Following these surprising biopsy results, the patient was investigated to exclude multiple myeloma. The skeletal survey did not show any evidence of multiple myeloma. The plasma proteins were reported as follows: Total protein 6-3 g./ioo ml. Albumen 2-8 g./ioo ml. Globulin 4-5 g./ioo ml. Albumen/globulin ratio o-6:i
FIG. I .
Case 1: The growth is seen protruding from right nostril. He has left facial paralysis as well.
There was no Bence Jones protein in the urine. The other haematological investigations were within normal limits. He was treated by radiotherapy. 1,800 rads were given to the left ear and 3,000 rads to the nose. There was a good response to radiotherapy. So far there is no sign of recurrence either in the middle ear or the nasal cavity. Case 2: Mr. S.P., married, aged thirty-five years, farmer. This patient was first admitted to the E.N.T. Department of Muhimbili Hospital on 20 July 1972. At that time he complained of nasal obstruction, 106
Clinical records foul-smelling discharge and swelling of right side of his nose. The duration of these complaints was one month. There was no history of epistaxis at this time.
J
FIG. 2. Case I: O.M. view of paranasal sinuses showing thickening of lateral wall of right nasa cavity.
On clinical examination the upper part of his external nose was found to be swollen on the right side. There was a large dark-reddish friable growth in the right nostril. This was arising from the lateral wall. There was no swelling or tenderness over the right maxilla. His teeth and hard palate looked normal. The cervical lymph nodes were not palpable. His general health was good. The Hb was 92 per cent. The results of other routine blood and urine examination were within normal limits. The sinus X-rays did not show any involvement of the right maxillary sinus. A biopsy was taken on 1 August 1972. This was reported as plasmacytoma. Therefore, the following investigations were carried out to exclude the presence of multiple myeloma: Total plasma protein 7-6 g./ioo ml. Albumen 2-7 g./ioo ml. Globulin 4-9 g./ioo ml. Albumen/globulin ratio o-6:i 107
M. A. Noorani There was no Bence-Jones protein in the urine and the skeletal survey was negative. The bone marrow studies revealed a slight increase in plasma cells but there was no evidence of multiple myeloma. The electrophoresis was reported as within normal limits. The patient was advised to stay in the hospital for about six weeks more for a course of radiotherapy, but he decided to go home to sort out his personal problems and promised to return to the hospital after one week. Instead he turned up about ten months later. He reported to the E.N.T. department on 30 May 1973. This time he complained of recurrent attacks of bleeding from the right nostril for the past two months. During this period he also developed a swelling over the right maxilla and hard palate. This swelling is clearly visible in Figure 3. The sinus X-rays were repeated and these revealed
FIG. 3. Case 2: Lateral view of the patient. Swelling of right cheek is quite prominent.
opacity of the right antrum and erosion in its medial wall (Fig. 4). The growth was treated by radiotherapy. The total tumour dose was 4,000 rads. This was followed by total maxillectomy on 10 August 1973. So far the patient is symptom-free and there is no sign of recurrence. Case 3 : Mrs. R.S., married, aged twenty-five years, housewife. This patient was first admitted to Muhimbili Hospital on 18 April 1972. She was referred from one of the provincial hospitals in Tanzania where she was admitted for the treatment of severe epistaxis. She complained of the presence of a large growth in the nose, recurrent attacks of epistaxis and nasal obstruction. The duration of complaints was unknown. While she was in this regional hospital she had an attack of severe epistaxis and was given blood transfusion. 108
Clinical records On clinical examination she was found to have a massive growth over the nose (Fig. 5). The left nostril was completely filled with a firm whitish growth. The whole nose was grossly distorted by the tumour. The septum was completely destroyed and the whole of the nasal cavity was filled with the tumour mass. The tip and left nostril were completely destroyed. The growth had eroded through the walls of the left nostril and had spread over the bridge, left cheek and upper Up. The growth had also extended up to the left lower eyelid but the eyelid itself was not involved. Although it was oedematous she complained of epiphora. The upper lip was markedly swollen. The middle part of the growth had ulcerated through the skin. The hard palate and teeth were not involved and there was no involvement of the cervical lymph nodes.
FIG. 4. Case 2: The photograph of sinus X-rays showing opacity of right maxillary sinus.
The Hb was 61 per cent and total WBC 7,600. Routine examination of urine and stools did not reveal any abnormalities. She had severe epistaxis on the following day. This was treated by conservative measures and two pints of blood were transfused. A biopsy from the tumour was taken on 24 April 1972. This was 109
M. A. Noorani reported as plasmacytoma. Hence the following investigations were carried out to exclude the generalized disease: Total plasma protein 6-3 g./ioo ml. Albumen 2-8 g./ioo ml. Globulin 4-5 g./ioo ml. Albumen /globulin ratio o-6:i There was no Bence-Jones protein in the urine, and the skeletal survey was negative. Bone-marrow studies showed a moderate increase in plasma cells but there were no myeloma cells. The electrophoresis was normal. She was given a full course of irradiation with Cobalt 60 but the response was not satisfactory. The tumour was therefore excised on 1 December 1972.
FIG. 5. Case 3: Plasmacytoma of nose infiltrating the surrounding regions.
Discussion Extramedullary plasmacyto-na is said to be of rare occurrence either as a localized manifestation or as a part of the generalized disease called multiple myeloma. The most common site is the head and neck region. Willis classified plasmacytoma into the following three groups: Group I: multiple myeloma This is a generalized disease involving various bones. The X-ray appearance of the lesions is characteristic. The serum protein is abnormal and Bence-Jones protein is present in urine. The bone marrow findings are also characteristic. Group II: solitary plasmacytoma of bone The lesion is localized to one particular bone only. There is no clinical or laboratory evidence of generalized disease. no
Clinical records Group III: primary plasmacytoma of soft tissue This may be single or multiple. In this paper, I have presented the first case ever reported of plasmacytoma of the middle ear. Incidentally, this patient had plasmacytoma of the nose as well. In addition to this, one case of plasmacytoma of the nasal cavity and one of plasmacytoma of nasal cavity and maxillary sinus have been presented. The survey of the world literature in English has not revealed a single case of plasmacytoma of the middle ear. All these three patients were adults. Two were male and one was female. Poole and Marchetta (1968) reviewed 189 cases and reported that 78 per cent of the cases were male and 22 per cent were female. The incidence is highest between fifty and seventy years of age. But it has been reported in patients as young as five and as old as eighty-nine years of age (Touma, 1971). The same author has reported that 90 per cent of extramedullary plasmacytoma are found in the upper respiratory tract and oral cavity. About 60 per cent of the cases are found in the region of the nose, nasopharynx and paranasal sinuses. A review of 192 cases of plasmacytoma of the head and neck showed the following sites of origin: nose, 40; nasopharynx, 41; paranasal sinuses, 34; tonsil and pharynx, 26; palate, gum and maxilla, 12; oropharynx, 10; larynx, 9; combined, 6; floor of the mouth and tongue, 5; others or site unknown, 9. The aetiology of plasmacytoma is not known. It may manifest itself as a solitary bone or extramedullary soft tissue lesion or as a generalized bone disease. A certain number of patients suffering from a solitary lesion may ultimately progress to generalized disease. Others remain symptom-free for a long time following successful treatment by either radiation alone or surgery alone or combined treatment. Castro, Lewis and Strong (1973) reviewed 772 patients with tumours of the nasal cavity and paranasal sinuses seen over a period of thirtyfive years and reported only twenty-three cases of plasmacytoma. The overall incidence of plasmacytoma was only 3 per cent. This shows that it is a comparatively rare disease. The usual symptoms of plasmacytoma of the nasal cavity are rhinorrhoea, nasal obstruction and epistaxis. The epistaxis is quite often profuse, requiring blood transfusions. All of my three patients had epistaxis. One of them was a very advanced case. This patient suffered from recurrent attacks of profuse nose bleeds and as a result she required frequent blood transfusions. In our part of the world, a large number of patients first report to the doctors when the disease has reached a fairly advanced stage. This is what happened to two of my cases. The third case (Mr. S.P.) was referred to the E.N.T. department when the disease was in a comparatively early stage. When he first reported to me the lesion was confined to the nasal cavity only. The biopsy was taken and this was reported as plasmacytoma. He was therefore advised to stay in the hospital for a course of radiotherapy, but as he had urgent personal business to attend to he decided to go home for one week. Instead he turned up about one year later. By this time the disease had invaded the maxillary sinus as well. Though there has been considerable progress in diagnostic techniques, still it is quite often not possible to determine the exact site of origin and extent of the in
M. A. Noorani disease. This is due to the peculiar anatomical features of this region. The presence of generalized disease is excluded by doing the following investigations: examination of the urine for Bence-Jones protein; serum protein estimation; skeletal survey; bone-marrow studies; and serum protein electrophoresis. These tumours are highly radio-sensitive and therefore initially they should be treated by radio-therapy alone. In my first case only i,8oo rads were required for the middle-ear tumour and 3,000 rads for the tumour in the nose. Only excision biopsy was carried out in this case. Surgery is indicated when response to radiotherapy is not satisfactory. This usually happens in advanced cases as seen in two of my patients. But on the whole the cases of extramedullary plasmacytoma are so few and far between that it is not possible for anybody to advocate the exact line of treatment. Each case should be considered on its own merits. But on the whole the combined treatment appears to give better results. The local recurrences can be treated by surgical excision, electrocoagulation or cryosurgery. The five-year survival rate of extramedullary plasmacytoma is about 50 per cent, while the majority of patients suffering from multiple myeloma die within two years. The prognosis of extramedullary plasmacytoma is unpredictable. All of my three patients have been regularly followed up for the last one-and-a-half years. So far there is no local recurrence and there is no evidence of generalized disease. Many cases of extramedullary plasmacytoma may ultimately progress to multiple myeloma. In rare cases, extramedullary plasmacytoma may present as a solitary bone lesion many years after successful treatment with radiotherapy. Chan and Pam (1969) reported a case of extramedullary plasmacytoma of the nasal cavity presenting as a solitary lesion of the humerous eight years after successful treatment with radiotherapy.
Summary The first case of extramedullary plasmacytoma of middle ear is reported. One case of plasmacytoma of the nasal cavity alone and another of the nasal cavity and maxillary sinus have also been described. Some of the problems in diagnosis and management have been discussed. Acknowledgements I wish to thank Dr. James Shaba, Dr. Axel Ruffmann and Dr. Jacob Schneider of the Department of Pathology and Professor K. J. Lindquist of Department of Microbiology for their help in carrying out the laboratory investigations. The photographs were provided by the Medical Illustration Department of the Faculty of Medicine. BIBLIOGRAPHY CASTRO, E L . B., LEWIS, J. S., and STRONG, E. W. (1973) Archives of Otolaryngology,
97, 326. CHAN, K. P., and TAM, C. S. (1969) Cancer, 23, 694. POOLE, A. G., and MARCHETTA, C. (1968) Cancer, 22, 14.
112
Clinical records TOUMA, Y. B. (1971) Journal of Laryngology and Otology, 85, 125. WILLIS,
R. A. (i960) Pathology of Tumours, 3rd ed. (London), p. 791.
Department of Otolaryngology, Faculty of Medicine, University of Dar es Salaam, P.O. Box 4365, Dar es Salaam, Tanzania.
113
