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Case report: Extramedullary plasmacytoma of the larynx
C’omprr~rmd MedmI Ima,qny ond (iruphrc,. Vol. lb. No. 5. pp. 359-36 Pnnted I” the U.S.A. All rights reserved.
CASE REPORT:
I, 1992 Copynghl
@
0X95-61 I l/9? $5.00 + 00 Pergamon Pres Ltd
I992
EXTRAMEDULLARY PLASMACYTOMA OF THE LARYNX
Richard R. Barbu,*+ Arfa Khan,* Jason L. Port,* Allan Abramson,* and Willa S. Gartenhaus§ Departments of *Radiology, *Otolaryngology and Communicative Disorders, and “Medicine, Long Island Jewish Medical Center, the Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park, NY 11042 (Received
I April 1992)
Abstract-A case of extramedullary plasmacytoma of the larynx is presented. The case is unique for the extramedullary plasmacytoma’s marked extension into the mediastinum. The computed tomography (CT) features are described. Key Words:
Extramedullary plasmacytoma, Larynx, Multiple myeloma. Computed tomography tinum between the trachea and esophagus up to the level just superior to the aortic arch. The mediastinal portion of the mass projected into the posterior part of the trachea causing partial narrowing of the tracheal lumen (Figs. 2A, 2B, and 2C). The barium swallow revealed a large mass extending between the trachea and esophagus from the level of C-7 to T-4. The mass caused compression of the posterior portion of the trachea and the anterior portion of the esophagus (Fig. 3). A bone survey was negative. The clinical evaluation of the patient was negative for systemic disease. The patient was referred for radiotherapy. The patient has remained disease free for 3 yrs following the completion of the radiotherapy.
INTRODUCTION Extramedullary plasmacytoma is a rare tumor representing less than 1% of all head and neck tumors. It involves the upper respiratory tract and oral cavity in 75% to 90% of cases (1, 2). In this report, we describe a case of extramedullary plasmacytoma of the larynx that was studied by computed tomography (CT) scan. The CT features have not been previously described. Case report A 69-yr-old man presented with voice change and difficulty swallowing. His symptoms had worsened over a period of 3 yrs during which time he had been seen by several otolaryngologists. A chest radiograph revealed a large superior mediastinal mass deviating the trachea to the left (Fig. 1). Direct laryngoscopy and esophagoscopy were performed. Swelling of the left lateral wall of the larynx extending from the aryepiglottic fold to the level of the ventricle along the medial wall of the piriform sinus was found. Esophagoscopy was normal. The mass on frozen section was felt to be a small cell tumor. On final section, however, the diagnosis of plasmacytoma of the larynx was made. Further radiological evaluation to assess the extent of local disease and exclude multiple myeloma consisted of a CT scan of the larynx and chest, a barium swallow, and a bone survey. The CT scan of the larynx and chest showed a soft tissue mass involving the left side of the epiglottis, the left aryepiglottic fold, and the left false vocal fold with complete filling of the ventricle below the level of the false vocal cords and obscuration of the true vocal cords. The mass involved the retrocricoid area with posterior displacement of the esophagus. The mass extended inferiorly into the medias’To
DISCUSSION Extramedullary plasmacytoma of the larynx represents between 6% and 18% of all extramedullary plasmacytomas ( 1). Males are affected two to four times as frequently as females. The fifth, sixth, and seventh decades of life account for approximately 75% of cases (1, 2). Patients report complaints of feeling a lump in the throat, dysphagia, hoarseness, and stridor (3). Extramedullary plasmacytoma of the larynx often presents as a polypoid or sessile mass, but may present as a diffuse submucosal swelling and thickening as in the present case (4). The diagnosis is established by biopsy and histologic examination. Microscopic examination typically reveals a monomorphic pattern of broad sheets of plasma cells in a sparse capillary stroma containing no inflammatory cells (5). Amyloid may be present in 1 1 to 38% of cases (1). The differential diagnosis includes plasma cell granuloma, pseudolymphoma, malignant melanoma, undifferentiated carcinoma, metastases, Waldenstrom’s macroglobinemia, and granulocytic sarcoma malignant lymphoma,
whom correspondence should be addressed.
359
360
Computerized Medical Imaging and Graphics
Fig. 1. Chest radiograph shows a superior mediastinal mass (M) deviating trachea (T) to left. Arrows outline the right tracheal wall.
(chloroma). Electron microscopy shows a concentrically arranged lamellar rough endoplasmic reticulum and prominent Golgi complex (6). The establishment of whether the extramedullary plasmacytoma is primary or secondary to multiple myeloma must be undertaken once a diagnosis of plasma cell tumor is made. This is accomplished through a serum and urine protein electrophoresis and immunoelectrophoresis, skeletal survey, bone marrow aspirate, chest radiograph, full blood count, erythrocyte sedimentation rate, peripheral blood smear, and serum calcium and phosphorus (1, 2, 7, 8). Further studies including CT scan may be done to evaluate the extent of disease. Multiple head and neck lesions are found in at least 20% of cases (7). The CT scan appearance of extramedullary plasmacytoma of the larynx, however, has not been described in the literature. In the larynx, the most frequently involved areas are the epiglottis followed by the vocal cords, false vocal cords, and ventricles (2). The CT scan in
Fig. 2A. CT scan of larynx shows a soft tissue mass (M) involving the left false vocal cord.
September-October
1992, Volume 16, Number 5
Fig. 2B. CT scan of larynx shows the soft tissue mass (M) almost completely filling the laryngeal ventricle.
this case revealed all of these areas were involved. The soft tissue mass completely filled the ventricle below the level of the false vocal cords and obscured the true vocal cords. The mass also extended inferiorly into the mediastinum causing tracheal and esophageal separation and partial narrowing of the tracheal lumen. In this case, where there was superior mediastinal extension, lymphoma and carcinoma are the more relevant considerations in the differential diagnosis. Extramedullary plasmacytoma is staged as follows: Stage 1, consisting of disease confined to one site; Stage 2, consisting of tumors with local spread or local lymph node involvement; and Stage 3, where metastatic spread has occurred (5). According to this classification, the present case would be characterized as a Stage 2. The importance of establishing whether the extramedullary plasmacytoma is primary or secondary to a systemic process lies in the fact that treatment and prognosis differ depending on the diagnosis. In cases of localized primary extramedullary plasmacytoma,
Fig. 2C. CT scan of superior mediastinum shows a soft tissue mass (M) extending between the trachea (T) and esophagus (arrowhead). The mass is projecting into the posterior part c . ot tracnea.
Extramedullary
plasmacytoma
of the larynx
??
361
R. R. BARBU ef al.
or secondary prognosis.
to multiple
myeloma
affect treatment
and
REFERENCES of the larynx. J. Otolaryngol. 19: I. Kost, K.M. Plasmacytomas 141-146; 1990. K.; Lundgren, J.; Olofsson, J. Extramedullary 2. Bjelkenkrantz, plasmacytoma of the larynx. J. Otolaryngol. 10:28-34; 1981. plas3. Bush, SE.: Goffinet. D.R.; Bagshaw, M.A. Extramedullary macytoma of the head and neck. Radiology I40:80 l-805; 198 I. of the larynx. Laryn4. Maniglia, A.J.: Xue. J.W. Plasmacytoma goscope 93:741-744; 1983. of extrame5. Waldron, J.: Mitchell. D.B. Unusual presentations dullary plasmacytoma in the head and neck. J. Laryngol. Otol. 102:102-104; 1988. plasma6. Kapadia, S.B.: Desai. U.; Cheng, V.S. Extramedullary cytoma of the head and neck. Medicine 6 1:3 17-329: 1982. E.; Canalis, R.F.; Greenberg, P.; Wortham, D.G.; 7. Abemayor. Rowland. J.P.; Sun. N.C.J. Plasma cell tumors of the head and neck. J. Otolaryngol. 17:376-381; 1988. of 8. Gaffney, C.C.: Dawes. P.J.D.K.; Jackson, D. Plasmacytoma the head and neck. Clin. Radiol. 38~385-388; 1987. plas9 Medini, E.; Rao. Y.: Levitt. S.H. Solitary extramedullary macytoma of the upper respiratory and digestive tracts. Cancer 45:2893-2896; 1980. IO. Booth, J.B.; Cheesman, A.D.; Vincenti, N.H. Extramedullary plasmacytoma of the upper respiratory tract. Ann. Otol. Rhinol. Laryngol. 82:709-7 15; 1973.
About the Author-RICHARD
R. BARBU attended the Union CollegeAlbany Medical College Six-Year Medical Education Program. He received the B.S. degree summa cum laude in Biology and History in 1987 and the M.D. degree in 1989. Dr. Barbu then completed an internship in Internal Medicine at Staten Island University Hospital in Staten Island. New York in 1990. He is currently Junior Chief Resident in Diagnostic Radiology at Long Island Jewish Medical Center, clinical campus of Albert Einstein College of Medicine. in New Hyde Park. New York. About the Author-ARFA KHAN graduated from Medical College, Kashmir in 1964. She then completed a Radiology residency at the Queens Hospital Center in 1970. Presently. Dr. Khan is chief of the Division of Thoracic Radiology at the Long Island Jewish Medical Center and Associate Professor of Radiology at the Albert Einstein College of Medicine. Fig. 3. Barium
swallow
shows
that
a small
amount
of barium
has been aspirated into the trachea (T). The soft tissue mass (M) separates the trachea (T) and esophagus (E) and causes extrinsic compression of their lumens.
surgery and radiotherapy, alone or in combination, have been advocated. The present case responded to local radiotherapy and has been disease free for 3 yrs. Chemotherapy is used in advanced, recurrent, or disseminated disease (1, 6, 9, IO). The 5-yr survival for patients with primary extramedullary plasmacytoma is 33% to 75%, while it is only 18% for patients with multiple myeloma (4,6). In 35% to 50% of cases, however, dissemination occurs (6). Patients must therefore be followed closely.
About the Author-JASON LEE PORT attended Columbia University where he completed post-baccalaureate work in 1984. He received the M.D. degree from Mount Sinai School of Medicine in New York in 1988. After completing an internship in Internal Medicine in 1989 at Winthrop University Hospital in Long Island. New York, Dr. Port entered residency training in Diagnostic Radiology at Long Island Jewish Medical Center, Albert Einstein College of Medicine in New Hyde Park, New York. Following completion of his residency in 1993, Dr. Port will begin fellowship training in Neuroradiology at the Cleveland Clinic Foundation in Cleveland, Ohio. About the Author-ALLAN LEWIS ABRAMSON received a B.A. degree from Colgate University in 1962 and the M.D. degree from the State University of New York, Downstate Medical Center in 1967. Dr. Abramson completed a residency in Otolaryngology at Long Island Jewish Hospital and Mount Sinai Hospital in 1972. He is presently Chairman of the Department of Otolaryngology and Communicative Disorders at Long Island Jewish Medical Center.
About the Author-WluA
SUMMARY A case of extramedullary plasmacytoma of the larynx is reported. CT was instrumental in evaluating the tumor’s marked extension into the mediastinum. The extent of disease and whether the tumor is primary
S. GARTENHAUS received a B.A. degree from Brooklyn College in 1967 and then attended the State University of New York, Upstate Medical Center, where she graduated cum laude in 1973. Dr. Gartenhaus completed an internship and residency in Internal Medicine at Long Island Jewish Medical Center in 1976. She did fellowship training at Montefiore Hospital, Mount Sinai Medical Center, and Long Island Jewish Medical Center in Hematology and Oncology. She is presently a staff physician at Long Island Jewish Medical Center.
CASE REPORT:
I, 1992 Copynghl
@
0X95-61 I l/9? $5.00 + 00 Pergamon Pres Ltd
I992
EXTRAMEDULLARY PLASMACYTOMA OF THE LARYNX
Richard R. Barbu,*+ Arfa Khan,* Jason L. Port,* Allan Abramson,* and Willa S. Gartenhaus§ Departments of *Radiology, *Otolaryngology and Communicative Disorders, and “Medicine, Long Island Jewish Medical Center, the Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park, NY 11042 (Received
I April 1992)
Abstract-A case of extramedullary plasmacytoma of the larynx is presented. The case is unique for the extramedullary plasmacytoma’s marked extension into the mediastinum. The computed tomography (CT) features are described. Key Words:
Extramedullary plasmacytoma, Larynx, Multiple myeloma. Computed tomography tinum between the trachea and esophagus up to the level just superior to the aortic arch. The mediastinal portion of the mass projected into the posterior part of the trachea causing partial narrowing of the tracheal lumen (Figs. 2A, 2B, and 2C). The barium swallow revealed a large mass extending between the trachea and esophagus from the level of C-7 to T-4. The mass caused compression of the posterior portion of the trachea and the anterior portion of the esophagus (Fig. 3). A bone survey was negative. The clinical evaluation of the patient was negative for systemic disease. The patient was referred for radiotherapy. The patient has remained disease free for 3 yrs following the completion of the radiotherapy.
INTRODUCTION Extramedullary plasmacytoma is a rare tumor representing less than 1% of all head and neck tumors. It involves the upper respiratory tract and oral cavity in 75% to 90% of cases (1, 2). In this report, we describe a case of extramedullary plasmacytoma of the larynx that was studied by computed tomography (CT) scan. The CT features have not been previously described. Case report A 69-yr-old man presented with voice change and difficulty swallowing. His symptoms had worsened over a period of 3 yrs during which time he had been seen by several otolaryngologists. A chest radiograph revealed a large superior mediastinal mass deviating the trachea to the left (Fig. 1). Direct laryngoscopy and esophagoscopy were performed. Swelling of the left lateral wall of the larynx extending from the aryepiglottic fold to the level of the ventricle along the medial wall of the piriform sinus was found. Esophagoscopy was normal. The mass on frozen section was felt to be a small cell tumor. On final section, however, the diagnosis of plasmacytoma of the larynx was made. Further radiological evaluation to assess the extent of local disease and exclude multiple myeloma consisted of a CT scan of the larynx and chest, a barium swallow, and a bone survey. The CT scan of the larynx and chest showed a soft tissue mass involving the left side of the epiglottis, the left aryepiglottic fold, and the left false vocal fold with complete filling of the ventricle below the level of the false vocal cords and obscuration of the true vocal cords. The mass involved the retrocricoid area with posterior displacement of the esophagus. The mass extended inferiorly into the medias’To
DISCUSSION Extramedullary plasmacytoma of the larynx represents between 6% and 18% of all extramedullary plasmacytomas ( 1). Males are affected two to four times as frequently as females. The fifth, sixth, and seventh decades of life account for approximately 75% of cases (1, 2). Patients report complaints of feeling a lump in the throat, dysphagia, hoarseness, and stridor (3). Extramedullary plasmacytoma of the larynx often presents as a polypoid or sessile mass, but may present as a diffuse submucosal swelling and thickening as in the present case (4). The diagnosis is established by biopsy and histologic examination. Microscopic examination typically reveals a monomorphic pattern of broad sheets of plasma cells in a sparse capillary stroma containing no inflammatory cells (5). Amyloid may be present in 1 1 to 38% of cases (1). The differential diagnosis includes plasma cell granuloma, pseudolymphoma, malignant melanoma, undifferentiated carcinoma, metastases, Waldenstrom’s macroglobinemia, and granulocytic sarcoma malignant lymphoma,
whom correspondence should be addressed.
359
360
Computerized Medical Imaging and Graphics
Fig. 1. Chest radiograph shows a superior mediastinal mass (M) deviating trachea (T) to left. Arrows outline the right tracheal wall.
(chloroma). Electron microscopy shows a concentrically arranged lamellar rough endoplasmic reticulum and prominent Golgi complex (6). The establishment of whether the extramedullary plasmacytoma is primary or secondary to multiple myeloma must be undertaken once a diagnosis of plasma cell tumor is made. This is accomplished through a serum and urine protein electrophoresis and immunoelectrophoresis, skeletal survey, bone marrow aspirate, chest radiograph, full blood count, erythrocyte sedimentation rate, peripheral blood smear, and serum calcium and phosphorus (1, 2, 7, 8). Further studies including CT scan may be done to evaluate the extent of disease. Multiple head and neck lesions are found in at least 20% of cases (7). The CT scan appearance of extramedullary plasmacytoma of the larynx, however, has not been described in the literature. In the larynx, the most frequently involved areas are the epiglottis followed by the vocal cords, false vocal cords, and ventricles (2). The CT scan in
Fig. 2A. CT scan of larynx shows a soft tissue mass (M) involving the left false vocal cord.
September-October
1992, Volume 16, Number 5
Fig. 2B. CT scan of larynx shows the soft tissue mass (M) almost completely filling the laryngeal ventricle.
this case revealed all of these areas were involved. The soft tissue mass completely filled the ventricle below the level of the false vocal cords and obscured the true vocal cords. The mass also extended inferiorly into the mediastinum causing tracheal and esophageal separation and partial narrowing of the tracheal lumen. In this case, where there was superior mediastinal extension, lymphoma and carcinoma are the more relevant considerations in the differential diagnosis. Extramedullary plasmacytoma is staged as follows: Stage 1, consisting of disease confined to one site; Stage 2, consisting of tumors with local spread or local lymph node involvement; and Stage 3, where metastatic spread has occurred (5). According to this classification, the present case would be characterized as a Stage 2. The importance of establishing whether the extramedullary plasmacytoma is primary or secondary to a systemic process lies in the fact that treatment and prognosis differ depending on the diagnosis. In cases of localized primary extramedullary plasmacytoma,
Fig. 2C. CT scan of superior mediastinum shows a soft tissue mass (M) extending between the trachea (T) and esophagus (arrowhead). The mass is projecting into the posterior part c . ot tracnea.
Extramedullary
plasmacytoma
of the larynx
??
361
R. R. BARBU ef al.
or secondary prognosis.
to multiple
myeloma
affect treatment
and
REFERENCES of the larynx. J. Otolaryngol. 19: I. Kost, K.M. Plasmacytomas 141-146; 1990. K.; Lundgren, J.; Olofsson, J. Extramedullary 2. Bjelkenkrantz, plasmacytoma of the larynx. J. Otolaryngol. 10:28-34; 1981. plas3. Bush, SE.: Goffinet. D.R.; Bagshaw, M.A. Extramedullary macytoma of the head and neck. Radiology I40:80 l-805; 198 I. of the larynx. Laryn4. Maniglia, A.J.: Xue. J.W. Plasmacytoma goscope 93:741-744; 1983. of extrame5. Waldron, J.: Mitchell. D.B. Unusual presentations dullary plasmacytoma in the head and neck. J. Laryngol. Otol. 102:102-104; 1988. plasma6. Kapadia, S.B.: Desai. U.; Cheng, V.S. Extramedullary cytoma of the head and neck. Medicine 6 1:3 17-329: 1982. E.; Canalis, R.F.; Greenberg, P.; Wortham, D.G.; 7. Abemayor. Rowland. J.P.; Sun. N.C.J. Plasma cell tumors of the head and neck. J. Otolaryngol. 17:376-381; 1988. of 8. Gaffney, C.C.: Dawes. P.J.D.K.; Jackson, D. Plasmacytoma the head and neck. Clin. Radiol. 38~385-388; 1987. plas9 Medini, E.; Rao. Y.: Levitt. S.H. Solitary extramedullary macytoma of the upper respiratory and digestive tracts. Cancer 45:2893-2896; 1980. IO. Booth, J.B.; Cheesman, A.D.; Vincenti, N.H. Extramedullary plasmacytoma of the upper respiratory tract. Ann. Otol. Rhinol. Laryngol. 82:709-7 15; 1973.
About the Author-RICHARD
R. BARBU attended the Union CollegeAlbany Medical College Six-Year Medical Education Program. He received the B.S. degree summa cum laude in Biology and History in 1987 and the M.D. degree in 1989. Dr. Barbu then completed an internship in Internal Medicine at Staten Island University Hospital in Staten Island. New York in 1990. He is currently Junior Chief Resident in Diagnostic Radiology at Long Island Jewish Medical Center, clinical campus of Albert Einstein College of Medicine. in New Hyde Park. New York. About the Author-ARFA KHAN graduated from Medical College, Kashmir in 1964. She then completed a Radiology residency at the Queens Hospital Center in 1970. Presently. Dr. Khan is chief of the Division of Thoracic Radiology at the Long Island Jewish Medical Center and Associate Professor of Radiology at the Albert Einstein College of Medicine. Fig. 3. Barium
swallow
shows
that
a small
amount
of barium
has been aspirated into the trachea (T). The soft tissue mass (M) separates the trachea (T) and esophagus (E) and causes extrinsic compression of their lumens.
surgery and radiotherapy, alone or in combination, have been advocated. The present case responded to local radiotherapy and has been disease free for 3 yrs. Chemotherapy is used in advanced, recurrent, or disseminated disease (1, 6, 9, IO). The 5-yr survival for patients with primary extramedullary plasmacytoma is 33% to 75%, while it is only 18% for patients with multiple myeloma (4,6). In 35% to 50% of cases, however, dissemination occurs (6). Patients must therefore be followed closely.
About the Author-JASON LEE PORT attended Columbia University where he completed post-baccalaureate work in 1984. He received the M.D. degree from Mount Sinai School of Medicine in New York in 1988. After completing an internship in Internal Medicine in 1989 at Winthrop University Hospital in Long Island. New York, Dr. Port entered residency training in Diagnostic Radiology at Long Island Jewish Medical Center, Albert Einstein College of Medicine in New Hyde Park, New York. Following completion of his residency in 1993, Dr. Port will begin fellowship training in Neuroradiology at the Cleveland Clinic Foundation in Cleveland, Ohio. About the Author-ALLAN LEWIS ABRAMSON received a B.A. degree from Colgate University in 1962 and the M.D. degree from the State University of New York, Downstate Medical Center in 1967. Dr. Abramson completed a residency in Otolaryngology at Long Island Jewish Hospital and Mount Sinai Hospital in 1972. He is presently Chairman of the Department of Otolaryngology and Communicative Disorders at Long Island Jewish Medical Center.
About the Author-WluA
SUMMARY A case of extramedullary plasmacytoma of the larynx is reported. CT was instrumental in evaluating the tumor’s marked extension into the mediastinum. The extent of disease and whether the tumor is primary
S. GARTENHAUS received a B.A. degree from Brooklyn College in 1967 and then attended the State University of New York, Upstate Medical Center, where she graduated cum laude in 1973. Dr. Gartenhaus completed an internship and residency in Internal Medicine at Long Island Jewish Medical Center in 1976. She did fellowship training at Montefiore Hospital, Mount Sinai Medical Center, and Long Island Jewish Medical Center in Hematology and Oncology. She is presently a staff physician at Long Island Jewish Medical Center.