Malignant lymphoma presenting as a parotid mass By IVOR A. EMANUEL (New York) Introduction PAROTID swellings are frequently encountered by the head and neck
surgeon. The majority of these are usually of parotid origin. Much less frequently a parotid mass may represent the first clinical manifestation of a system disease process. Three cases are presented in which malignant lymphoma initially manifested itself as an isolated parotid mass, with no other signs or symptoms of systemic disease, either historically or on physical examination. Case report Case 1 First admission A 74-year-old male presented with a painless mass at the left mandibular angle that had slowly increased in size over the past year. There was no change in size with eating. He had no trismus or facial nerve symptoms. His past medical history included longstanding coronary artery disease. Physical examination showed the patient was an elderly thin man in no acute distress. Head and neck examination revealed a firm, non-tender mass 3 x 3 centimeters in diameter at the left angle of the mandible. It was fixed to the underlying tissues, but not to the skin. There was no facial nerve weakness nor any facial sensory deficit. Intraoral examination showed no abnormality either visually and by palpation. Salivary flow was equal bilaterally. There were no other cervical masses or lymphadenopathy. The rest of the ENT exam was normal. On general examination the patient was in mild congestive heart failure as demonstrated by bilateral pitting pedal edema and a gallop rhythm on cardiac auscultation. His abdomen was soft and non-tender with no hepatosplenomegaly. No lymphadenopathy was palpable. On rectal exam, a firm, non-tender 2 + prostatic enlargement was palpated. Laboratory studies revealed a hematocrit of 42 per cent. The white cell count was 7,800 with a normal differential. The remaining blood studies including sedimentation rate were within normal limits. Chest and mandible X-rays were both read as normal. Because of the severity of this congestive heart failure, it was decided to perform incisional biopsy under local anesthesia for diagnostic purposes. The histopathological diagnosis made was that of a mixed tumor.
Ivor A. Emanuel
FIG. I.
Patient on initial presentation.
Because of the significant risk involved with general anesthesia, the patient was discharged to be followed-up by the medical service. Second admission Six weeks later the patient again presented with a markedly enlarged mass at the site of the previous incisional biopsy, associated with some dysphagia. Local examination of the head and neck showed a large non-tender 5 x 5 centimeters in diameter, firm, hard, indurated mass, fixed to the underlying structures but not to skin. There was no facial nerve involvement. Intraoral examination now showed the left tonsil and fauces to be deviated to the right and on palpation a firm mass was palpable in the lateral pharyngeal wall. General examination revealed the patient's cardiac condition to be stable with no signs of cardiac decompensation at this time. The rest of the physical 382
Malignant lymphoma presenting as a parotid mass
FIG. 2. First biospy—Case i. H. & E. stain, low power magnification shows small foci of definite mixed tumor pattern in addition to large cellular areas. Cellularity of this degree is considered atypical, but there is no evidence that the tumor represents a malignancy.
examination was unchanged from his first admission. Laboratory and X-ray findings were also unchanged. The patient was cleared for general anesthesia and was scheduled for definitive surgery at which time the mass was found to infiltrate the sternocleidomastoid muscle and the internal jugular vein with extension to the base of skull and pharynx. Some of the bulk of the mass was removed for tissue diagnosis as the tumor was too extensive to be totally resected. The histopathological diagnosis was that of a poorly differentiated malignant lymphoma with involvement of the acini of the parotid gland. On reviewing the slides from the first surgical procedure, it was now felt that a diagnosis of malignant lymphoma with mixed tumor was the correct one. Further investigation revealed that the bone marrow was infiltrated with 383
Ivor A. Emanuel
FIG.
3.
Patient on second admission six weeks later. The mass showing a dramatic increase in size.
lymphomablasts. Splenic and liver scans were positive and a retroperitoneal lymphangiogram showed tumor involvement above and below the diaphragm. The patient, with widely disseminated disease, was started on a course of chemotherapy using vincristin, cytoxin, prednisone and procarbizine, in weight related doses. Initially, there was massive tumor lysis with shrinkage of the tumor in the neck. Several weeks later the patient's general condition deteriorated rapidly and despite a change in the chemotherapeutic regime, he expired. Permission for post-mortem was not obtained. Case 2
A 55-year-old female presented with a 3 month history of a slowly growing, painless swelling in the left preauricular area. There was no fluctuation in size related to eating nor any previous history of a similar swelling in that region. 384
Malignant lymphoma presenting as a parotid mass
FIG. 4. Second biopsy—Case 1. H. & E. stain, low power magnification shows the poorly differentiated malignant lymphoma with the tissue composed of large sheets of uniform malignant lymphoid cells with large vesicular nuclei, some with prominent nucleoli. The tumor cells were found to be invading the surrounding skeletal muscle and parotid acini.
General history revealed the patient to have well controlled mild hypertension. Physical examination showed a healthy, obese female with a blood pressure of 140/90 in no acute distress. Local examination of the head and neck showed a well denned firm, partially mobile 3 centimeters in diameter mass in the left preauricular area, which appeared to be in the substance of the parotid gland, without any deeper extension. There was no facial nerve involvement or sensory deficit. No other cervical or lymph node enlargement was present. Intraoral exam was negative. General examination showed no abdominal organomegaly or generalized lymphadenopathy. Laboratory investigations showed a hematocrit of 49 per 385
Ivor A. Emanuel
FIG. 5. H. & E. stain under high power magnification shows cells which are larger than normal lymphocytes with nuclei which are both vesicular and angulated in appearance, many having prominent nucleoli, these being characteristic of a poorly differentiated malignant lymphoma.
cent and a white cell count of 4,800 with a normal differential count. There was an elevated sedimentation rate of 60. Chest and mandible X-rays were normal. The patient subsequently underwent superficial parotidectomy under general anesthesia with preservation of the facial nerve. A firm, encapsulated mass involving the superficial lobe of the parotid gland was noted to be present within the gland substance. The histopathological diagnosis was that of a well differentiated malignant lymphoma with diffuse involvement of the gland. On further investigation, lymphangiogram showed positive retroperitoneal node involvement below the diaphragm. Bone marrow aspiration and splenic and liver scans were negative. The patient was referred for radiotherapy, as the disease process was relatively localized to the parotid gland and the retroperitoneal lymph nodes. Case 3 This seventy-five-year old female presented with a six month history of a left preauricular mass which had gradually increased in size. It was painless and its size did not change with meals. 386
Malignant lymphoma presenting as a parotid mass General history was that of severe cardiac disease with past history of cardiac arrhythmias and hypertension for which she was receiving appropriate medication. Physical exam
There was a 2 X 3 centimeters mass in the left preauricular area. It was firm, non-tender and freely mobile and was felt to be in the superficial lobe of the parotid gland. There was no facial nerve weakness and no other cervical involvement. Laboratory findings were a hematocrit of 45 per cent, white cell count of 5,700 with normal differential, and a raised sedimentation rate of 50. X-rays of chest and mandible were normal. On general examination the patient was in no acute distress and normotensive. She had atrial fibrillation, but otherwise her cardiac status was stable. There was no abdominal organomegaly nor any systemic lymph adenopathy. While the patient was in the hospital, she developed severe cardiac arrhythmias which necessitated a pacemaker insertion. Several days later she was taken to surgery where superficial parotidectomy with preservation of the facial nerve. Histopathology report was malignant lymphoma, diffuse undifferentiated in type, with infiltration of the glandular acini by the malignant cells. Discussion There are a number of systemic conditions which may present with a parotid swelling (Table I). Our discussion will be limited to malignant lymphomas. TABLE 1. SYSTEMIC DISEASES THAT MAY PRESENT AS A PAROTID MAS6
a.
Granulomatous lesions 1. Sarcoidosis 2. Tuberculosis 3. Actinomycosis b. Benign Lymphoepithelial Sialadenopathy 1. Sjogrens syndrome 2. Associated with collagen disease e.g. arteritis nodosa, scleroderma, lupus erythematosus
The medical literature contains many studies and reports of malignant lymphomas developing and/or presenting in the region of the head and neck. Malignant lymphomas are second in frequency only to epidermoid carcinoma as isolated tumors occurring in the head and neck (McNelis and Pai, 1969). Cervical lymph node involvement is by far the most common primary presentation of lymphoma in the head and neck (McNelis and Pai, 1969; Peckham, 1974; Catlin, 1967), although a substantial number present as extranodal masses. Lymphoma of the tonsil is by far the most common in this latter group (Catlin, 1967; Freeman et al., 1972). The most exhaustive study of the presenting sites of extranodal lymphoma is found in the collected data of Freeman et al. (1972). In their series of 1,467 cases 387
Ivor A. Emanuel of extranodal lymphoma, 69 presented in the salivary glands. In another series of primary manifestation of malignant lymphomas in the head and neck (McNelis and Pai, 1969), 4 out of 153 cases (2-6 per cent) presented as a primary parotid enlargement, without any other overt clinical evidence of lymphoma. Catlin (1967) showed in his series that out of 229 malignant lymphomas in the head and neck 14 presented as a parotid tumor. In his series there was no sex preponderance and majority of patients were in the 50-80-year-old age group. A study by Bailey and Brindley (1970) of 26 patients referred with otolaryngological complaints, for whom a diagnosis of malignant lymphoma had not been entertained initially, a positive diagnosis of malignant lymphoma in all was subsequently made. Of the 26 patients, two of them presented with an enlarging mass in the parotid region, both of which were proven to be malignant lymphoma following parotidectomy. In the same institution, of 238 patients with malignant lymphoma, 14 (6 per cent) presented with otolarynological complaints only. The number of parotid tumors which were subsequently diagnosed as being malignant lymphomas is very small. This may be seen in Table II which summarizes several series in the literature. TABLE II. THE NUMBER OF MALIGNANT LYMPHOMAS COMPARED TO TOTAL NUMBER OF PAROTID TUMORS BY HISTOLOGICAL DIAGNOSIS
Foote and Frazell (1953) Berdal et al. (1970) Leegaard and Lindeman (1969) Hugo et al. (1973) Ross Everoth (1964)
# of Cases of Neoplasms (Benign
surgeon. The majority of these are usually of parotid origin. Much less frequently a parotid mass may represent the first clinical manifestation of a system disease process. Three cases are presented in which malignant lymphoma initially manifested itself as an isolated parotid mass, with no other signs or symptoms of systemic disease, either historically or on physical examination. Case report Case 1 First admission A 74-year-old male presented with a painless mass at the left mandibular angle that had slowly increased in size over the past year. There was no change in size with eating. He had no trismus or facial nerve symptoms. His past medical history included longstanding coronary artery disease. Physical examination showed the patient was an elderly thin man in no acute distress. Head and neck examination revealed a firm, non-tender mass 3 x 3 centimeters in diameter at the left angle of the mandible. It was fixed to the underlying tissues, but not to the skin. There was no facial nerve weakness nor any facial sensory deficit. Intraoral examination showed no abnormality either visually and by palpation. Salivary flow was equal bilaterally. There were no other cervical masses or lymphadenopathy. The rest of the ENT exam was normal. On general examination the patient was in mild congestive heart failure as demonstrated by bilateral pitting pedal edema and a gallop rhythm on cardiac auscultation. His abdomen was soft and non-tender with no hepatosplenomegaly. No lymphadenopathy was palpable. On rectal exam, a firm, non-tender 2 + prostatic enlargement was palpated. Laboratory studies revealed a hematocrit of 42 per cent. The white cell count was 7,800 with a normal differential. The remaining blood studies including sedimentation rate were within normal limits. Chest and mandible X-rays were both read as normal. Because of the severity of this congestive heart failure, it was decided to perform incisional biopsy under local anesthesia for diagnostic purposes. The histopathological diagnosis made was that of a mixed tumor.
Ivor A. Emanuel
FIG. I.
Patient on initial presentation.
Because of the significant risk involved with general anesthesia, the patient was discharged to be followed-up by the medical service. Second admission Six weeks later the patient again presented with a markedly enlarged mass at the site of the previous incisional biopsy, associated with some dysphagia. Local examination of the head and neck showed a large non-tender 5 x 5 centimeters in diameter, firm, hard, indurated mass, fixed to the underlying structures but not to skin. There was no facial nerve involvement. Intraoral examination now showed the left tonsil and fauces to be deviated to the right and on palpation a firm mass was palpable in the lateral pharyngeal wall. General examination revealed the patient's cardiac condition to be stable with no signs of cardiac decompensation at this time. The rest of the physical 382
Malignant lymphoma presenting as a parotid mass
FIG. 2. First biospy—Case i. H. & E. stain, low power magnification shows small foci of definite mixed tumor pattern in addition to large cellular areas. Cellularity of this degree is considered atypical, but there is no evidence that the tumor represents a malignancy.
examination was unchanged from his first admission. Laboratory and X-ray findings were also unchanged. The patient was cleared for general anesthesia and was scheduled for definitive surgery at which time the mass was found to infiltrate the sternocleidomastoid muscle and the internal jugular vein with extension to the base of skull and pharynx. Some of the bulk of the mass was removed for tissue diagnosis as the tumor was too extensive to be totally resected. The histopathological diagnosis was that of a poorly differentiated malignant lymphoma with involvement of the acini of the parotid gland. On reviewing the slides from the first surgical procedure, it was now felt that a diagnosis of malignant lymphoma with mixed tumor was the correct one. Further investigation revealed that the bone marrow was infiltrated with 383
Ivor A. Emanuel
FIG.
3.
Patient on second admission six weeks later. The mass showing a dramatic increase in size.
lymphomablasts. Splenic and liver scans were positive and a retroperitoneal lymphangiogram showed tumor involvement above and below the diaphragm. The patient, with widely disseminated disease, was started on a course of chemotherapy using vincristin, cytoxin, prednisone and procarbizine, in weight related doses. Initially, there was massive tumor lysis with shrinkage of the tumor in the neck. Several weeks later the patient's general condition deteriorated rapidly and despite a change in the chemotherapeutic regime, he expired. Permission for post-mortem was not obtained. Case 2
A 55-year-old female presented with a 3 month history of a slowly growing, painless swelling in the left preauricular area. There was no fluctuation in size related to eating nor any previous history of a similar swelling in that region. 384
Malignant lymphoma presenting as a parotid mass
FIG. 4. Second biopsy—Case 1. H. & E. stain, low power magnification shows the poorly differentiated malignant lymphoma with the tissue composed of large sheets of uniform malignant lymphoid cells with large vesicular nuclei, some with prominent nucleoli. The tumor cells were found to be invading the surrounding skeletal muscle and parotid acini.
General history revealed the patient to have well controlled mild hypertension. Physical examination showed a healthy, obese female with a blood pressure of 140/90 in no acute distress. Local examination of the head and neck showed a well denned firm, partially mobile 3 centimeters in diameter mass in the left preauricular area, which appeared to be in the substance of the parotid gland, without any deeper extension. There was no facial nerve involvement or sensory deficit. No other cervical or lymph node enlargement was present. Intraoral exam was negative. General examination showed no abdominal organomegaly or generalized lymphadenopathy. Laboratory investigations showed a hematocrit of 49 per 385
Ivor A. Emanuel
FIG. 5. H. & E. stain under high power magnification shows cells which are larger than normal lymphocytes with nuclei which are both vesicular and angulated in appearance, many having prominent nucleoli, these being characteristic of a poorly differentiated malignant lymphoma.
cent and a white cell count of 4,800 with a normal differential count. There was an elevated sedimentation rate of 60. Chest and mandible X-rays were normal. The patient subsequently underwent superficial parotidectomy under general anesthesia with preservation of the facial nerve. A firm, encapsulated mass involving the superficial lobe of the parotid gland was noted to be present within the gland substance. The histopathological diagnosis was that of a well differentiated malignant lymphoma with diffuse involvement of the gland. On further investigation, lymphangiogram showed positive retroperitoneal node involvement below the diaphragm. Bone marrow aspiration and splenic and liver scans were negative. The patient was referred for radiotherapy, as the disease process was relatively localized to the parotid gland and the retroperitoneal lymph nodes. Case 3 This seventy-five-year old female presented with a six month history of a left preauricular mass which had gradually increased in size. It was painless and its size did not change with meals. 386
Malignant lymphoma presenting as a parotid mass General history was that of severe cardiac disease with past history of cardiac arrhythmias and hypertension for which she was receiving appropriate medication. Physical exam
There was a 2 X 3 centimeters mass in the left preauricular area. It was firm, non-tender and freely mobile and was felt to be in the superficial lobe of the parotid gland. There was no facial nerve weakness and no other cervical involvement. Laboratory findings were a hematocrit of 45 per cent, white cell count of 5,700 with normal differential, and a raised sedimentation rate of 50. X-rays of chest and mandible were normal. On general examination the patient was in no acute distress and normotensive. She had atrial fibrillation, but otherwise her cardiac status was stable. There was no abdominal organomegaly nor any systemic lymph adenopathy. While the patient was in the hospital, she developed severe cardiac arrhythmias which necessitated a pacemaker insertion. Several days later she was taken to surgery where superficial parotidectomy with preservation of the facial nerve. Histopathology report was malignant lymphoma, diffuse undifferentiated in type, with infiltration of the glandular acini by the malignant cells. Discussion There are a number of systemic conditions which may present with a parotid swelling (Table I). Our discussion will be limited to malignant lymphomas. TABLE 1. SYSTEMIC DISEASES THAT MAY PRESENT AS A PAROTID MAS6
a.
Granulomatous lesions 1. Sarcoidosis 2. Tuberculosis 3. Actinomycosis b. Benign Lymphoepithelial Sialadenopathy 1. Sjogrens syndrome 2. Associated with collagen disease e.g. arteritis nodosa, scleroderma, lupus erythematosus
The medical literature contains many studies and reports of malignant lymphomas developing and/or presenting in the region of the head and neck. Malignant lymphomas are second in frequency only to epidermoid carcinoma as isolated tumors occurring in the head and neck (McNelis and Pai, 1969). Cervical lymph node involvement is by far the most common primary presentation of lymphoma in the head and neck (McNelis and Pai, 1969; Peckham, 1974; Catlin, 1967), although a substantial number present as extranodal masses. Lymphoma of the tonsil is by far the most common in this latter group (Catlin, 1967; Freeman et al., 1972). The most exhaustive study of the presenting sites of extranodal lymphoma is found in the collected data of Freeman et al. (1972). In their series of 1,467 cases 387
Ivor A. Emanuel of extranodal lymphoma, 69 presented in the salivary glands. In another series of primary manifestation of malignant lymphomas in the head and neck (McNelis and Pai, 1969), 4 out of 153 cases (2-6 per cent) presented as a primary parotid enlargement, without any other overt clinical evidence of lymphoma. Catlin (1967) showed in his series that out of 229 malignant lymphomas in the head and neck 14 presented as a parotid tumor. In his series there was no sex preponderance and majority of patients were in the 50-80-year-old age group. A study by Bailey and Brindley (1970) of 26 patients referred with otolaryngological complaints, for whom a diagnosis of malignant lymphoma had not been entertained initially, a positive diagnosis of malignant lymphoma in all was subsequently made. Of the 26 patients, two of them presented with an enlarging mass in the parotid region, both of which were proven to be malignant lymphoma following parotidectomy. In the same institution, of 238 patients with malignant lymphoma, 14 (6 per cent) presented with otolarynological complaints only. The number of parotid tumors which were subsequently diagnosed as being malignant lymphomas is very small. This may be seen in Table II which summarizes several series in the literature. TABLE II. THE NUMBER OF MALIGNANT LYMPHOMAS COMPARED TO TOTAL NUMBER OF PAROTID TUMORS BY HISTOLOGICAL DIAGNOSIS
Foote and Frazell (1953) Berdal et al. (1970) Leegaard and Lindeman (1969) Hugo et al. (1973) Ross Everoth (1964)
# of Cases of Neoplasms (Benign
