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Lymphoma of head and neck masquerading as carcinoma
Lymphoma of Head and Neck Masquerading as Carcinoma E. G. ELIAS, MD, FACS, Buffalo, New York A. MIllELMAN, MD, Buffalo, New York
In a recent review of 150 cases of lymphoma, reticulum cell sarcoma, or lymphocytic lymphosarcoma for which major surgical procedures were performed at Roswell Park Institute, twenty-five cases presented in the head and neck region in an unusual fashion. Lymphomas usually appear in this region as enlarged lymph nodes, and occasionally as skin lesions. In these twenty-five cases (Table I), the lymphoma presented in the oronasopharynx as a mass or a malignant ulcer with indurated everted edges, wide base, necrotic center, and regional invasion with induration, grossly simulating carcinoma. (Figure 1.) These ulcers varied in size and shape. They can occur anywhere in the pharynx. They may start as an ulcer or as a hard mass (Figure 2) that enlarges and then ulcerates. Some of these ulcers are painful ; others are not. Another clinical form in which lymphoma may present itself in the head and neck region is as an enlarged thyroid gland (Figure 3), tonsil (Figure 2), submaxillary salivary gland, or parotid gland (Figure 4). This anatomic distribution can simulate carcinoma or mixed tumors. Figure 5 shows a roentgenogram of an antral lesion that was found to be reticulum cell sarcoma. There were palpable cervical lymph nodes in ten patients, but none of the twenty-five cases had any distant enlarged lymph nodes or palpable masses elsewhere at the time of discovery of the disease. Biopsy of these lesions usually results in a clearcut histologic diagnosis. Occasionally, the histologic examination may confuse a reticulum cell sarcoma for an anaplastic carcinoma. Of twentyfive patients in this group, the correct diagnosis was made in sixteen. The other nine cases fell into three different diagnostic classifications. From the General Clinical Research Center, Roswell Park Memorial Institute, Buffalo, New York, 14203. This work was supported by USPHS grant If1 MO1 RROO262-06. Presented at the Tenth International Cancer Congress, Houston, Texas, 1970.
424
Diagnostic
Classification
1. Incorrect Diagnosis. Six patients were included in this category. The biopsies of five were designated as anaplastic carcinoma, and one (infraorbital lesion) was called melanoma. After surgical extirpation or at autopsy, it was definitely established that the lesion was reticulum cell sarcoma. These six cases are presented in Table II. The two patients who presented with enlarged cervical lymph nodes are of special interest and their cases are presented: CASEI. The patient (AF, #110124), a seventy-three year old white woman, presented with a painless mass in the right side of the neck. Biopsy of this mass was interpreted as showing metastatic anaplastic carcinoma in a lymph node, with the primary lesion unknown. Examination failed to identify a primary tumor. The patient was treated with radiotherapy. Seven months later she died of sepsis secondary to pneumonia. Autopsy revealed reticulum cell sarcoma in mesenteric and retroperitoneal lymph nodes.
TABLE I
The Distribution of the Presenting LeslOns Descriptionof Lesion
Number of Cases
Enlarged lymph node; the biopsy specimen is thought to reveal anaplastic carcinoma; primary lesion is unknown
3 2 1 3 2* 5 3 2* 1 1 2 1 25
Ulcerating lesion, floor of mouth Ulcerating lesion, base of tongue Tonsillar lesion Ulcerating lesion of palate Thyroid tumor Parotid tumor Tumor of submaxillary gland lnfraorbital nodule lntraorbital lesion with exophthalmos Tumor of nostril or nasopharynx Antral lesion Total l
Same patient presenting with two lesions.
The American
Journal
of Surgery
Lymphoma
Figure I. Lymphoma a malignant ulcer.
Figure 3. completely
of the roof of the mouth presented
Surgical specimen of thyroid gland. infiltrated by reticulum cell sarcoma.
as
The gland is
CASE II. The patient (KP, $X24338), a forty-one year old white man, presented with a painless mass in the right side of the neck, the biopsy of which was interpreted as showing metastatic anaplastic carcinoma in a lymph node, of unknown primary. Examination failed to reveal a primary tumor. Lymphangiography and exploratory laparotomy were negative. No therapy was initiated. He underwent follow-up study in the clinic. At the end of two years he complained of back pain, anorexia, and loss of weight. Repeat lymphangiography was interpreted as positive for lymphoma. He was re-explored and the mesenteric and retroperitoneal lymph nodes were found to be massively enlarged. Biopsy of these masses revealed reticulum cell sarcoma. He was treated with radiotherapy. He is now living with no clinical and well one year after radiotherapy evidence of disease.
Volume
122, September
1971
Figure 2. Tumor of left tonsil; vealed reticulum cell sarcoma.
Tumor of right parotid Figure 4. vealed reticulum cell sarcoma.
of Head and
histologic
gland.
Neck
examination
re-
Parotidectomy
re-
.*
Figure 5. Roentgenogram antrum. The biopsy revealed
showing a filling defect reticulum cell sarcoma.
in left
425
Elias and Mittelman 2. Questionable Diagnosis. A diagnosis of anaplastic carcinoma was made in two patients, one from a tumor of the thyroid gland, and the other from the nasopharynx. The thyroid lesion was treated by radical extirpation, and the nasopharyngeal lesion was irradiated. Both patients survived for three years without local recurrence of the disease. At autopsy, however, both patients were found to have reticulum cell sarcoma in the abdominal cavity. It is most likely that the original lesions in these cases were reticulum cell sarcoma, but the possibility of pre-existing occult carcinoma cannot be completely ruled out. 3. Diagnosis Equivocal for Malignancy. Repeated biopsies of the nasopharyngeal mass in one patient showed only hyperplasia of lymphoid tissue. An enlarged cervical lymph node and an abdominal mass later developed in this patient. Exploratory laparotomy revealed reticulum cell sarcoma in lymph nodes of the mesentery.
TABLE
Number of Cases
II
Reticulum
Site of Biopsy
Cell Sarcoma
Primary Histologic Diagnosis
1
Mouth (floor)
Anaplastic carcinoma
1
Tongue (base)
Anaplastic carcinoma
&a (yr)
426
Treatment and Outcome
60 Surgical extirpation: died 7 mo later; cause unknown 53 Surgical extirpation and radiotherapy; 69 lymph nodes were negative
for tumor; died 7 mo
1
Tons5
Anaplastic carcinoma
61
1
lnfraorbltal skin
Melanoma
83
2
Cervical
Anaplastic carcinoma
73 41
Comments It is apparent that lymphoma can be deceiving to the clinician as well as to the pathologist. Ulceration and necrosis are characteristic of caroinoma, but lymphomas may have the same. A biopsy may reveal highly undifferentiated cells, and as a result the lesion may be erroneously labeled as anaplastic carcinoma. This in turn may lead to unnecessary radical surgery and/or high doses of radiotherapy. Our review shows that the misdiagnosed lesions are commonly reticulum cell sarcomas. It may therefore be advisable to rebiopsy a lesion in the oropharynx to include normal and involved tissue for further study of its microscopic relationship to the mucosa. Carcinoma has a transition between the invaded mucosa and the normal mucosa, whereas lymphoma tends to bear no relation to the mucosa. If a biopsy specimen of a cervical lymph node is designated as anaplastic carcinoma and no primary lesion is found, both clinician and pathologist should keep in mind that the lesion may be lymphoma. A search should be made for other palpable lymph nodes and rebiopsy and lymphography may be indicated. In the case of lymphoma in a salivary gland, the lesion originates in the lymph nodes adjacent to the gland and then invades the gland proper.
In Six Cases
lymph nodes
later of myocardlal infarction Surgical extirpation and radiotherapy: 2 posltlve lymph nodes out of 142; living and well 12 yr later with no evidence of disease Wide excision: one year later palpable lymph nodes developed In neck: treated by radlotherapy; died 3 yr later of myocardial infarction See Case I See Case II
Summary The purpose of this paper is to emphasize the following : 1. An ulcerating lesion in the oropharynx can be a lymphomatous lesion. The histologic diagnosis of anaplastic carcinoma, if made, should be questioned and all efforts exerted to rule out lymphoma. 2. A biopsy specimen of a cervical lymph node that is believed to be anaplastic carcinoma, of unknown primary, should be similarly questioned. Reticulum cell sarcoma must be considered in the differential diagnosis. 3. Enlarged salivary glands can be the primary site of lymphoma. 4. Lymphangiography and exploratory laporotomy may be necessary for an accurate histologic diagnosis. Acknowledgment: We wish to thank Dr V Bakamjian of the Department of Head and Neck for his advice and guidance.
The American Journal of sufwry
In a recent review of 150 cases of lymphoma, reticulum cell sarcoma, or lymphocytic lymphosarcoma for which major surgical procedures were performed at Roswell Park Institute, twenty-five cases presented in the head and neck region in an unusual fashion. Lymphomas usually appear in this region as enlarged lymph nodes, and occasionally as skin lesions. In these twenty-five cases (Table I), the lymphoma presented in the oronasopharynx as a mass or a malignant ulcer with indurated everted edges, wide base, necrotic center, and regional invasion with induration, grossly simulating carcinoma. (Figure 1.) These ulcers varied in size and shape. They can occur anywhere in the pharynx. They may start as an ulcer or as a hard mass (Figure 2) that enlarges and then ulcerates. Some of these ulcers are painful ; others are not. Another clinical form in which lymphoma may present itself in the head and neck region is as an enlarged thyroid gland (Figure 3), tonsil (Figure 2), submaxillary salivary gland, or parotid gland (Figure 4). This anatomic distribution can simulate carcinoma or mixed tumors. Figure 5 shows a roentgenogram of an antral lesion that was found to be reticulum cell sarcoma. There were palpable cervical lymph nodes in ten patients, but none of the twenty-five cases had any distant enlarged lymph nodes or palpable masses elsewhere at the time of discovery of the disease. Biopsy of these lesions usually results in a clearcut histologic diagnosis. Occasionally, the histologic examination may confuse a reticulum cell sarcoma for an anaplastic carcinoma. Of twentyfive patients in this group, the correct diagnosis was made in sixteen. The other nine cases fell into three different diagnostic classifications. From the General Clinical Research Center, Roswell Park Memorial Institute, Buffalo, New York, 14203. This work was supported by USPHS grant If1 MO1 RROO262-06. Presented at the Tenth International Cancer Congress, Houston, Texas, 1970.
424
Diagnostic
Classification
1. Incorrect Diagnosis. Six patients were included in this category. The biopsies of five were designated as anaplastic carcinoma, and one (infraorbital lesion) was called melanoma. After surgical extirpation or at autopsy, it was definitely established that the lesion was reticulum cell sarcoma. These six cases are presented in Table II. The two patients who presented with enlarged cervical lymph nodes are of special interest and their cases are presented: CASEI. The patient (AF, #110124), a seventy-three year old white woman, presented with a painless mass in the right side of the neck. Biopsy of this mass was interpreted as showing metastatic anaplastic carcinoma in a lymph node, with the primary lesion unknown. Examination failed to identify a primary tumor. The patient was treated with radiotherapy. Seven months later she died of sepsis secondary to pneumonia. Autopsy revealed reticulum cell sarcoma in mesenteric and retroperitoneal lymph nodes.
TABLE I
The Distribution of the Presenting LeslOns Descriptionof Lesion
Number of Cases
Enlarged lymph node; the biopsy specimen is thought to reveal anaplastic carcinoma; primary lesion is unknown
3 2 1 3 2* 5 3 2* 1 1 2 1 25
Ulcerating lesion, floor of mouth Ulcerating lesion, base of tongue Tonsillar lesion Ulcerating lesion of palate Thyroid tumor Parotid tumor Tumor of submaxillary gland lnfraorbital nodule lntraorbital lesion with exophthalmos Tumor of nostril or nasopharynx Antral lesion Total l
Same patient presenting with two lesions.
The American
Journal
of Surgery
Lymphoma
Figure I. Lymphoma a malignant ulcer.
Figure 3. completely
of the roof of the mouth presented
Surgical specimen of thyroid gland. infiltrated by reticulum cell sarcoma.
as
The gland is
CASE II. The patient (KP, $X24338), a forty-one year old white man, presented with a painless mass in the right side of the neck, the biopsy of which was interpreted as showing metastatic anaplastic carcinoma in a lymph node, of unknown primary. Examination failed to reveal a primary tumor. Lymphangiography and exploratory laparotomy were negative. No therapy was initiated. He underwent follow-up study in the clinic. At the end of two years he complained of back pain, anorexia, and loss of weight. Repeat lymphangiography was interpreted as positive for lymphoma. He was re-explored and the mesenteric and retroperitoneal lymph nodes were found to be massively enlarged. Biopsy of these masses revealed reticulum cell sarcoma. He was treated with radiotherapy. He is now living with no clinical and well one year after radiotherapy evidence of disease.
Volume
122, September
1971
Figure 2. Tumor of left tonsil; vealed reticulum cell sarcoma.
Tumor of right parotid Figure 4. vealed reticulum cell sarcoma.
of Head and
histologic
gland.
Neck
examination
re-
Parotidectomy
re-
.*
Figure 5. Roentgenogram antrum. The biopsy revealed
showing a filling defect reticulum cell sarcoma.
in left
425
Elias and Mittelman 2. Questionable Diagnosis. A diagnosis of anaplastic carcinoma was made in two patients, one from a tumor of the thyroid gland, and the other from the nasopharynx. The thyroid lesion was treated by radical extirpation, and the nasopharyngeal lesion was irradiated. Both patients survived for three years without local recurrence of the disease. At autopsy, however, both patients were found to have reticulum cell sarcoma in the abdominal cavity. It is most likely that the original lesions in these cases were reticulum cell sarcoma, but the possibility of pre-existing occult carcinoma cannot be completely ruled out. 3. Diagnosis Equivocal for Malignancy. Repeated biopsies of the nasopharyngeal mass in one patient showed only hyperplasia of lymphoid tissue. An enlarged cervical lymph node and an abdominal mass later developed in this patient. Exploratory laparotomy revealed reticulum cell sarcoma in lymph nodes of the mesentery.
TABLE
Number of Cases
II
Reticulum
Site of Biopsy
Cell Sarcoma
Primary Histologic Diagnosis
1
Mouth (floor)
Anaplastic carcinoma
1
Tongue (base)
Anaplastic carcinoma
&a (yr)
426
Treatment and Outcome
60 Surgical extirpation: died 7 mo later; cause unknown 53 Surgical extirpation and radiotherapy; 69 lymph nodes were negative
for tumor; died 7 mo
1
Tons5
Anaplastic carcinoma
61
1
lnfraorbltal skin
Melanoma
83
2
Cervical
Anaplastic carcinoma
73 41
Comments It is apparent that lymphoma can be deceiving to the clinician as well as to the pathologist. Ulceration and necrosis are characteristic of caroinoma, but lymphomas may have the same. A biopsy may reveal highly undifferentiated cells, and as a result the lesion may be erroneously labeled as anaplastic carcinoma. This in turn may lead to unnecessary radical surgery and/or high doses of radiotherapy. Our review shows that the misdiagnosed lesions are commonly reticulum cell sarcomas. It may therefore be advisable to rebiopsy a lesion in the oropharynx to include normal and involved tissue for further study of its microscopic relationship to the mucosa. Carcinoma has a transition between the invaded mucosa and the normal mucosa, whereas lymphoma tends to bear no relation to the mucosa. If a biopsy specimen of a cervical lymph node is designated as anaplastic carcinoma and no primary lesion is found, both clinician and pathologist should keep in mind that the lesion may be lymphoma. A search should be made for other palpable lymph nodes and rebiopsy and lymphography may be indicated. In the case of lymphoma in a salivary gland, the lesion originates in the lymph nodes adjacent to the gland and then invades the gland proper.
In Six Cases
lymph nodes
later of myocardlal infarction Surgical extirpation and radiotherapy: 2 posltlve lymph nodes out of 142; living and well 12 yr later with no evidence of disease Wide excision: one year later palpable lymph nodes developed In neck: treated by radlotherapy; died 3 yr later of myocardial infarction See Case I See Case II
Summary The purpose of this paper is to emphasize the following : 1. An ulcerating lesion in the oropharynx can be a lymphomatous lesion. The histologic diagnosis of anaplastic carcinoma, if made, should be questioned and all efforts exerted to rule out lymphoma. 2. A biopsy specimen of a cervical lymph node that is believed to be anaplastic carcinoma, of unknown primary, should be similarly questioned. Reticulum cell sarcoma must be considered in the differential diagnosis. 3. Enlarged salivary glands can be the primary site of lymphoma. 4. Lymphangiography and exploratory laporotomy may be necessary for an accurate histologic diagnosis. Acknowledgment: We wish to thank Dr V Bakamjian of the Department of Head and Neck for his advice and guidance.
The American Journal of sufwry