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Primary adenoid cystic carcinoma of the esophagus
Primary adenoid cystic carcinoma of the esophagus Report of a case and review of the literature A case of primary adenoid cystic carcinoma of the esophagus is reported and a total of 15 cases was reviewed. There were 9 men, although earlier reports indicated the disease was more common in women. The lesion was located in the middle third of the esophagus in 10 cases. The possibility of tumor development from embryonal rests of the tracheohronchial tree in the esophagus is discussed. The prognosis of adenoid cystic carcinomas is not well known, but the treatment and prognosis appear similar to those of epidermoid carcinomas of the esophagus.
A. Pourzand, M.D., L. Freant, M.D., R. Levin, M.D., J. Peabody, M.D., and K. Absolon, M.D., Ph.D., Washington, D. C.
/ Y d e n o i d cystic carcinoma (cylindroma), which is a disease of the salivary glands, upper respiratory tract, palate, maxillary gums, tongue, and the floor of the mouth, is an uncommon tumor of the esophagus. In a large series of adenoid cystic carcinomas, there was not a single case arising in the esophagus.1 In a review of 2,518 cases of carcinoma of the esophagus, there is also no mention of this type of lesion.2, ' Nelms and Lunda' recently reported a case and reviewed 8 additional cases.'" In this article we present a case and review 5 more cases from the literature. 101 Case report A 59-year-old black woman was seen April 9, 1974, in the Emergency Room of the Washington Hospital Center complaining of hematemesis. The evening prior to admission, while eating, she felt nauseated and vomited several "cupfuls" of bright red blood, which later became the color
From the Departments of Surgery and Pathology, Washington Hospital Center, 110 Irving Street, N.W., Washington, D. C. 20010, and George Washington University Medical School, Washington, D. C. Received for publication Nov. 19, 1974.
and consistency of coffee grounds. Past history revealed that she had drunk a pint of whisky a day for the past 20 years. No history of dysphagia, weight loss, melena, or jaundice was present. The physical examination revealed that the patient was in mild distress, the sclerae were slightly pale, and a few spider angiomata were present on the upper chest and face. The abdomen was slightly distended, and 2+ free ascites was noted. The liver's edge was soft and palpable 6 cm. below the right costal margin in the midclavicular line. No nodes were palpable and no splenomegaly was present. The hemoglobin value was 11.7 Gm. per cent and hematocrit 36.1 per cent. Liver function tests showed the following: serum bilirubin 1.3 (direct 0.55, indirect 0.75), serum glutamic oxaloacetic transaminase 90, serum glutamic pyruvic transaminase 48, alkaline phosphatase 2.52 Bodansky units, prothrombin time 15.4 seconds (control 11.1), partial thromboplastin time 43 seconds (control 33). The other biochemical findings revealed a sulfobromophthalein retention of 39 per cent in 45 minutes, and the liver scan showed diffuse hepatocellular disease. On esophagoscopy 20 cm. from the incisors, a bleeding mass was present and no evidence of varices was seen. The following day, the patient passed a tarry stool, and the hemoglobin and hematocit values dropped to 8.6 Gm. per cent and 27.6 per cent. She continued to bleed and required 14 units of blood and coagulation fac-
785
The Journal of
786
Pourzand et al.
Thoracic and Cardiovascular Surgery
Fig. 1. Barium swallow showing a 4 cm. mass located at the level of the carina.
tors in the next 5 days. On April 15, bleeding had completely stopped. An upper gastrointestinal series showed a 4 cm. mass located at the level of the carina posterior and lateral to the left (Fig. 1). The chest x-ray findings were unremarkable. Esophagoscopy was repeated, and multiple biopsies of the tumor were taken. The microscopy report was undifferentiated carcinoma of the esophagus. On April 26, 1974, left thoracotomy and esophagectomy with esophagogastrostomy were performed; no evidence of metastases was present. Morphology of the tumor. The specimen, consisting of the esophagus and esophagogastric junction, measured 14 cm. in length and 4 cm. in circumference. Opening the specimen showed a nodular, soft, lobulated lesion measuring 2 by 2 cm. The mucosa covering the lesion was yellowish pink and thick. On microscopic examination. the lesion was a polypoid carcinoma of the midesophagus with mucosal ulceration and submucosal involvement. The lesion was of a mixed type, with features varying from epidermoid (Fig. 2 ) . to basal cell (Fig. 3), to mucin-producing adenoid cystic carcinoma (Fig. 4 ) . The musculature was not invaded, and periesophageal lymph nodes were free of metastasis. No ectopic gastric mucosa was found.
Review of literature and discussion Fourteen cases of primary adenoid cystic carcinoma of the esophagus were collected
from the literature. The patients' ages ranged from 51 to 72 years. There were 9 men and 6 women, even though earlier reports indicated that the disease is more common in women. Major symptoms included dysphagia of 2 to 60 months' duration and weight loss. No massive gastrointestinal bleeding occurred except in our patient. The lesion was located in the middle third of the esophagus in 10 cases, the lower third in 4, and upper third in 1 case. Twelve patients had a resection, 1 had radiation therapy, 2 were treated with a combination of radiation and resection, and 1 did not receive any treatment. Four patients died within 10 days after the operation because of immediate postoperative complications, and 6 died between 2 and 24 months after initial treatment. Two patients were lost to follow-up, and 3 are alive and free of disease at the time of publication (2, 3, and 15 months after treatment). In a few cases, autopsies were performed. They showed widespread metastases to the mediastinum, lung, liver, cerebellum, bones, or soft tissues. In 1925, Jackson'" reported two tumors with "basal cell characteristics" among 671
Volume 69 Number 5
Adenoid
May, 1975
cystic carcinoma of esophagus
*&&&
Fig. 2. Epidermoid differentiation.
Fig. 3. Basaloid appearance. iv-jf^
*«* v ;*r>
*,-":>■
Fig. 4. Mucin-producing adenoid cystic carcinoma.
7 87
The Journal of
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Pourzand et al.
Thoracic and Cardiovascular Surgery
carcinomas of the esophagus. Geschickter11 in 1935, reviewing 80 malignant esophageal tumors, stated: "Adenocarcinoma and basal cell cancer are rarely found in this location." His Fig. 15 shows an adenocarcinoma of basaloid appearance, with two cell layers lining the ductules, which probably was an adenoid cystic carcinoma."- ■ ' "' Gregg and Stamler"1 reported a similar case in the addendum to their article. Cases described as basal cell tumors of the esophagus often represent undifferentiated epidermoid carcinomas of the transitional cell type.17 This tumor also masquerades under diagnoses such as "undifferentiated carcinoma," "spheroidal cell carcinoma," and "basal cell carcinoma." Very few authors refuse to accept the existence of adenoid cystic carcinoma. ls ' '" Adenoid cystic carcinoma of the esophagus probably arises from submucosal glands. Azzopardi5 suggests that there is no reason why mucus glands of the esophagus and their ducts should not give rise to tumors corresponding to those found in major salivary glands. The adenoid cystic carcinomas of the esophagus reviewed showed all the characteristics seen in other sites. Some tumors show areas with a lack of differentiation, so that examination of such areas alone confuses the true nature of the tumor. Bergman and Charnas 7 reported a case of adenoid cystic carcinoma of the esophagus quite similar to a cylindroma of the tracheobronchial tree and promoted the hypothesis that the tumor had originated from embryonal rests of the tracheobronchial tree in the esophagus. Their patient was a 65-year-old man who had dysphagia of several years' duration. A 57-year-old woman in another report also had dysphagia for 5 years."' The same hypothesis was suggested in 1952 to explain the origin of the primary oat cell carcinomas of the esophagus.20 Benisch and Toker12 state that reported cases of primary adenoid cystic carcinoma of the esophagus are not exactly comparable to morphologically similar tumors occurring in salivary glands. They suggest the term "incomplete adenoid cystic
differentiation" for such esophageal lesions. They also suggest that the lesser degree of undifferentiation in esophageal lesions, in comparison with salivary glands, is responsible for the higher degree of malignancy in esophageal tumors. In regard to prognosis, satisfactory data are not available because of the small number of reported cases. The treatment of primary adenoid cystic carcinomas is similar to that of epidermoid carcinomas. REFERENCES 1 Ahlbom, H. E.: Mucous and Salivary Gland Tumors, Acta Radiol. 23: 1, 1935 (Suppl.). 2 Turnbull, A. D., Rosen, P., Goodner, J. T., et al.: Primary Malignant Tumors of the Esophagus Other Than Typical Epidermoid Carcinoma, Ann. Thorac. Surg. 15: 463, 1973. 3 Lowe, W.: Survival With Carcinoma of the Esophagus, Ann. Intern. Med. 77: 915, 1972. 4 Nelms, D. C , and Lunda, M. A.: Primary Adenoid Cystic Carcinoma of the Esophagus, Cancer 29: 440, 1972. 5 Azzopardi, J., and Menzier, T.: Primary Esophageal Adenocarcinoma, Br. J. Surg. 49: 497, 1962. 6 U. S. Naval Medical School: Color Atlas of Pathology, Philadelphia, 1954, J. B. Lippincott Company, p. 234. 7 Bergman, M., and Charnas, R. M.: Tracheobronchial Rests in the Esophagus: Their Relation to Benign Structures and Certain Types of Cancer of the Esophagus, J. THORAC. SURG.
35: 97, 1958. 8 Marcial-Rojas, R. A., and Vallecillo, L. A.: Primary Adenoid Cystic Carcinoma of the Esophagus: Report of One Case and Review of the Literature, Arch. Otolaryngol. 70: 197, 1959. 9 Rapheal, H. A., Ellis, F. A., and Dockerty, M. B.: Primary Adenocarcinoma of the Esophagus, Ann. Surg. 164: 785, 1966. 10 Raven, R. W.: Rare Tumors of Pharynx and Esophagus, Ann. N . Y. Acad. Sci. 114: 1061, 1964. 11 Lortat-Jacob, J.: Primary Adenocarcinoma of the Esophagus: Review of 16 Cases, Surgery 64: 535, 1968. 12 Benisch, B., and Toker, C : Esophageal Carcinomas With Adenoid Cystic Differentiation, Arch. Otolaryngol. 69: 260, 1972. 13 Jackson, C : Carcinoma and Sarcoma of the Esophagus: A Plea for Early Diagnosis, Am. J. Med. Sci. 169: 624, 1925. 14 Geschickter, C. F.: Tumors of the Digestive Tract, Am. J. Cancer 25: 130, 1935.
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May, 1975
15 Evans, R. W.: Histological Appearance of Tumors, Edinburgh, E. & S. Livingstone, Ltd., p. 429. 16 Gregg, J. B., and Stamler, F. W.: Unusual Neoplasms of the Esophagus: Review of the Literature and Report of a Case, Arch. Otolaryngol. 59: 159, 1954. 17 Willis, R. A.: Pathology of Tumors, ed 2, London, Butterworth & Co., Ltd., p. 388.
18 Ackerman, L. V., and del Regato, J. A.: Carcinoma of the Esophagus, in Cancer, St. Louis, 1962, The C. V. Mosby Company. 19 Stout, A. P., and Lattes, R.: Tumors of the Esophagus, in Atlas of Tumor Pathology, fascicle 4, Washington, D. C , 1957, Armed Forces Institute of Pathology, p. 72. 20 McKeown, F.: Oat Cell Carcinoma of the Esophagus, J. Pathol. 64: 889, 1952.
A. Pourzand, M.D., L. Freant, M.D., R. Levin, M.D., J. Peabody, M.D., and K. Absolon, M.D., Ph.D., Washington, D. C.
/ Y d e n o i d cystic carcinoma (cylindroma), which is a disease of the salivary glands, upper respiratory tract, palate, maxillary gums, tongue, and the floor of the mouth, is an uncommon tumor of the esophagus. In a large series of adenoid cystic carcinomas, there was not a single case arising in the esophagus.1 In a review of 2,518 cases of carcinoma of the esophagus, there is also no mention of this type of lesion.2, ' Nelms and Lunda' recently reported a case and reviewed 8 additional cases.'" In this article we present a case and review 5 more cases from the literature. 101 Case report A 59-year-old black woman was seen April 9, 1974, in the Emergency Room of the Washington Hospital Center complaining of hematemesis. The evening prior to admission, while eating, she felt nauseated and vomited several "cupfuls" of bright red blood, which later became the color
From the Departments of Surgery and Pathology, Washington Hospital Center, 110 Irving Street, N.W., Washington, D. C. 20010, and George Washington University Medical School, Washington, D. C. Received for publication Nov. 19, 1974.
and consistency of coffee grounds. Past history revealed that she had drunk a pint of whisky a day for the past 20 years. No history of dysphagia, weight loss, melena, or jaundice was present. The physical examination revealed that the patient was in mild distress, the sclerae were slightly pale, and a few spider angiomata were present on the upper chest and face. The abdomen was slightly distended, and 2+ free ascites was noted. The liver's edge was soft and palpable 6 cm. below the right costal margin in the midclavicular line. No nodes were palpable and no splenomegaly was present. The hemoglobin value was 11.7 Gm. per cent and hematocrit 36.1 per cent. Liver function tests showed the following: serum bilirubin 1.3 (direct 0.55, indirect 0.75), serum glutamic oxaloacetic transaminase 90, serum glutamic pyruvic transaminase 48, alkaline phosphatase 2.52 Bodansky units, prothrombin time 15.4 seconds (control 11.1), partial thromboplastin time 43 seconds (control 33). The other biochemical findings revealed a sulfobromophthalein retention of 39 per cent in 45 minutes, and the liver scan showed diffuse hepatocellular disease. On esophagoscopy 20 cm. from the incisors, a bleeding mass was present and no evidence of varices was seen. The following day, the patient passed a tarry stool, and the hemoglobin and hematocit values dropped to 8.6 Gm. per cent and 27.6 per cent. She continued to bleed and required 14 units of blood and coagulation fac-
785
The Journal of
786
Pourzand et al.
Thoracic and Cardiovascular Surgery
Fig. 1. Barium swallow showing a 4 cm. mass located at the level of the carina.
tors in the next 5 days. On April 15, bleeding had completely stopped. An upper gastrointestinal series showed a 4 cm. mass located at the level of the carina posterior and lateral to the left (Fig. 1). The chest x-ray findings were unremarkable. Esophagoscopy was repeated, and multiple biopsies of the tumor were taken. The microscopy report was undifferentiated carcinoma of the esophagus. On April 26, 1974, left thoracotomy and esophagectomy with esophagogastrostomy were performed; no evidence of metastases was present. Morphology of the tumor. The specimen, consisting of the esophagus and esophagogastric junction, measured 14 cm. in length and 4 cm. in circumference. Opening the specimen showed a nodular, soft, lobulated lesion measuring 2 by 2 cm. The mucosa covering the lesion was yellowish pink and thick. On microscopic examination. the lesion was a polypoid carcinoma of the midesophagus with mucosal ulceration and submucosal involvement. The lesion was of a mixed type, with features varying from epidermoid (Fig. 2 ) . to basal cell (Fig. 3), to mucin-producing adenoid cystic carcinoma (Fig. 4 ) . The musculature was not invaded, and periesophageal lymph nodes were free of metastasis. No ectopic gastric mucosa was found.
Review of literature and discussion Fourteen cases of primary adenoid cystic carcinoma of the esophagus were collected
from the literature. The patients' ages ranged from 51 to 72 years. There were 9 men and 6 women, even though earlier reports indicated that the disease is more common in women. Major symptoms included dysphagia of 2 to 60 months' duration and weight loss. No massive gastrointestinal bleeding occurred except in our patient. The lesion was located in the middle third of the esophagus in 10 cases, the lower third in 4, and upper third in 1 case. Twelve patients had a resection, 1 had radiation therapy, 2 were treated with a combination of radiation and resection, and 1 did not receive any treatment. Four patients died within 10 days after the operation because of immediate postoperative complications, and 6 died between 2 and 24 months after initial treatment. Two patients were lost to follow-up, and 3 are alive and free of disease at the time of publication (2, 3, and 15 months after treatment). In a few cases, autopsies were performed. They showed widespread metastases to the mediastinum, lung, liver, cerebellum, bones, or soft tissues. In 1925, Jackson'" reported two tumors with "basal cell characteristics" among 671
Volume 69 Number 5
Adenoid
May, 1975
cystic carcinoma of esophagus
*&&&
Fig. 2. Epidermoid differentiation.
Fig. 3. Basaloid appearance. iv-jf^
*«* v ;*r>
*,-":>■
Fig. 4. Mucin-producing adenoid cystic carcinoma.
7 87
The Journal of
7 88
Pourzand et al.
Thoracic and Cardiovascular Surgery
carcinomas of the esophagus. Geschickter11 in 1935, reviewing 80 malignant esophageal tumors, stated: "Adenocarcinoma and basal cell cancer are rarely found in this location." His Fig. 15 shows an adenocarcinoma of basaloid appearance, with two cell layers lining the ductules, which probably was an adenoid cystic carcinoma."- ■ ' "' Gregg and Stamler"1 reported a similar case in the addendum to their article. Cases described as basal cell tumors of the esophagus often represent undifferentiated epidermoid carcinomas of the transitional cell type.17 This tumor also masquerades under diagnoses such as "undifferentiated carcinoma," "spheroidal cell carcinoma," and "basal cell carcinoma." Very few authors refuse to accept the existence of adenoid cystic carcinoma. ls ' '" Adenoid cystic carcinoma of the esophagus probably arises from submucosal glands. Azzopardi5 suggests that there is no reason why mucus glands of the esophagus and their ducts should not give rise to tumors corresponding to those found in major salivary glands. The adenoid cystic carcinomas of the esophagus reviewed showed all the characteristics seen in other sites. Some tumors show areas with a lack of differentiation, so that examination of such areas alone confuses the true nature of the tumor. Bergman and Charnas 7 reported a case of adenoid cystic carcinoma of the esophagus quite similar to a cylindroma of the tracheobronchial tree and promoted the hypothesis that the tumor had originated from embryonal rests of the tracheobronchial tree in the esophagus. Their patient was a 65-year-old man who had dysphagia of several years' duration. A 57-year-old woman in another report also had dysphagia for 5 years."' The same hypothesis was suggested in 1952 to explain the origin of the primary oat cell carcinomas of the esophagus.20 Benisch and Toker12 state that reported cases of primary adenoid cystic carcinoma of the esophagus are not exactly comparable to morphologically similar tumors occurring in salivary glands. They suggest the term "incomplete adenoid cystic
differentiation" for such esophageal lesions. They also suggest that the lesser degree of undifferentiation in esophageal lesions, in comparison with salivary glands, is responsible for the higher degree of malignancy in esophageal tumors. In regard to prognosis, satisfactory data are not available because of the small number of reported cases. The treatment of primary adenoid cystic carcinomas is similar to that of epidermoid carcinomas. REFERENCES 1 Ahlbom, H. E.: Mucous and Salivary Gland Tumors, Acta Radiol. 23: 1, 1935 (Suppl.). 2 Turnbull, A. D., Rosen, P., Goodner, J. T., et al.: Primary Malignant Tumors of the Esophagus Other Than Typical Epidermoid Carcinoma, Ann. Thorac. Surg. 15: 463, 1973. 3 Lowe, W.: Survival With Carcinoma of the Esophagus, Ann. Intern. Med. 77: 915, 1972. 4 Nelms, D. C , and Lunda, M. A.: Primary Adenoid Cystic Carcinoma of the Esophagus, Cancer 29: 440, 1972. 5 Azzopardi, J., and Menzier, T.: Primary Esophageal Adenocarcinoma, Br. J. Surg. 49: 497, 1962. 6 U. S. Naval Medical School: Color Atlas of Pathology, Philadelphia, 1954, J. B. Lippincott Company, p. 234. 7 Bergman, M., and Charnas, R. M.: Tracheobronchial Rests in the Esophagus: Their Relation to Benign Structures and Certain Types of Cancer of the Esophagus, J. THORAC. SURG.
35: 97, 1958. 8 Marcial-Rojas, R. A., and Vallecillo, L. A.: Primary Adenoid Cystic Carcinoma of the Esophagus: Report of One Case and Review of the Literature, Arch. Otolaryngol. 70: 197, 1959. 9 Rapheal, H. A., Ellis, F. A., and Dockerty, M. B.: Primary Adenocarcinoma of the Esophagus, Ann. Surg. 164: 785, 1966. 10 Raven, R. W.: Rare Tumors of Pharynx and Esophagus, Ann. N . Y. Acad. Sci. 114: 1061, 1964. 11 Lortat-Jacob, J.: Primary Adenocarcinoma of the Esophagus: Review of 16 Cases, Surgery 64: 535, 1968. 12 Benisch, B., and Toker, C : Esophageal Carcinomas With Adenoid Cystic Differentiation, Arch. Otolaryngol. 69: 260, 1972. 13 Jackson, C : Carcinoma and Sarcoma of the Esophagus: A Plea for Early Diagnosis, Am. J. Med. Sci. 169: 624, 1925. 14 Geschickter, C. F.: Tumors of the Digestive Tract, Am. J. Cancer 25: 130, 1935.
Volume 69
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Adenoid cystic carcinoma of esophagus
7 89
May, 1975
15 Evans, R. W.: Histological Appearance of Tumors, Edinburgh, E. & S. Livingstone, Ltd., p. 429. 16 Gregg, J. B., and Stamler, F. W.: Unusual Neoplasms of the Esophagus: Review of the Literature and Report of a Case, Arch. Otolaryngol. 59: 159, 1954. 17 Willis, R. A.: Pathology of Tumors, ed 2, London, Butterworth & Co., Ltd., p. 388.
18 Ackerman, L. V., and del Regato, J. A.: Carcinoma of the Esophagus, in Cancer, St. Louis, 1962, The C. V. Mosby Company. 19 Stout, A. P., and Lattes, R.: Tumors of the Esophagus, in Atlas of Tumor Pathology, fascicle 4, Washington, D. C , 1957, Armed Forces Institute of Pathology, p. 72. 20 McKeown, F.: Oat Cell Carcinoma of the Esophagus, J. Pathol. 64: 889, 1952.