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Hemangioendothelioma of the salivary gland
HEMANGIOENDOTHELIOMA
OF THE SALIVARY
GLAND*
HAROLD B. HALEY, M.D. AND ARNOLD S. JACKSON, M.D. Madison,
H
Wisconsin
EMANGIOENDOTHELIOMA
arising in the saGvary gIands is a rare condition. A case of such a tumor in a young infant is presented. The most common IateraI neck tumor in the young infant is a congenita1 cystic hygroma. HemangioendotheIioma in such a Iocation is unusua1. Smith2 says, “A hemangioendothelioma of this region has never been observed at the Children’s Hospita1 (Boston), nor has it been diagnosed prior to the present case in any of the 40,000 chiIdren born at the Boston Lying-in HospitaI.” CASE REPORT T. L., a male infant, was first seen on May 31, 1947, at the age of six weeks with no abnormalities noted. On June 20, 1947, the parents noted swelling of the Ieft cheek. Three days later this was seen by a pediatrician who believed that the infant had a parotitis. A course of penicillin was given and the mass became somewhat smaller. It soon became large again and a course of sulfonamides was given with practically no effect. On July 14, 1947, the patient was seen in surgica1 consultation at which time the mass seemed to arise posterior and inferior to the left parotid gland, and the possible diagnosis of cystic hygroma was considered. The mass was followed for six weeks and grew steadily until it was about 8 cm. in diameter. It felt cystic and did not transihuminate light. The mass seemed to lie in the subcutaneous tissues of the neck. (Figs. I and 2.) On August I 9, I 947, an operation for remova of the tumor was performed by one of the authors (A. S. J.) under ether-oxygen endotracheal anesthesia. There was a large, wine* From the Department
colored tumor about 6 by 5 by 4 cm. involving the left side of the neck from the transverse processes of the sixth cervica1 Vertebra, the carotid and jugular vessels and the anterior surface of the sternocleidomastoid muscte to the skin. The tumor lay adjacent to the submaxihary and parotid glands but grossly seemed to be distinct from them. Many blood vessels extended from the tumor into surrounding tissues. Two lymph nodes were found in the area of the tumor. The tumor was excised intact. (Figs. 3 and 4.) The postoperative course was uneventful, except for development of a partial, transient facial paraIysis. (Fig. 5.) The following pathoIogic report by Dr. EtheIdred L. Schafer gives the gross and microscopic appearance of the specimen removed at surgery : “The gross specimen was a soft but not fIuctuant, well encapsulated, dark red ovoid mass, measuring 5.5 by 4.5 by 3.5 cm. The external surface was faintly bosselated. The capsule was thin and transparent. Cut surfaces were coarsely lobulated, dark red, and slightly bulging. A small amount of blood exuded from the surface. Attached at one pole was a separate ovoid mass, measuring 1.5 cm. in maximum diameter, which had pinkish gray cut surfaces and resembled a lymph node. “Numerous sections from the mass showed a vascular tumor involving salivary gland tissue. Some of the gland was visible at the margins of the tumor, and isolated glands and ducts occurred throughout the tumor. Delicate fibrous connective tissue septa divided the tumor into lobules reminiscent of the normal pattern of the salivary gland. In some regions, variable-sized blood-containing spaces with onIy thin walIs separating them were numerous. These spaces were lined by endothelial cells which in some instances were pIump. An
of Surgery, Jackson Clinic, Madison, 725
Wis.
726 A mcrican ~~~~~~~ or~urgery
MAY, 1948
HaIey, Jackson-HemangioendotheIioma
1
2
FIGS.
3 FIG. 3. Tumor demonstrated
I and 2. Preoperative
at operation.
occasional mitotic figure was noted among these cells. In other foci, compact masses of small ceIIs occurred between the blood spaces. These ceIIs had indistinct cell outhnes. Their nuclei were round or irregulady ova1 and fairly uniform in size. The chromatin material was irregmarly dispersed throughout the nucleus; none contained a nucleolus. Mitotic figures were more numerous in these compact foci, numbering as many as 4 to 5 per high power field. An occasiona multinucIeated cell was
photographs
of infant.
4 FIG. 4. Specimen removed at operation.
noted. The Iymph node at the periphery of the tumor was normaI. (Figs. 6 and 7.) “The tumor beIongs in that group of tumors specihcally to the known as angiomata, hemangiomata. The fohowing must be considered in making a diagnosis: hemangioma (capillary type), hemangioendotheIioma, hemangiopericytoma, and hemangioendotheliobIastoma. The exact nature of the tumor cells and their reIationship to bIood vesseIs could not be demonstrated without special stains.
VOL. ILXSV.
No.
5
Haley,
Jackson-Hemangioendothelioma
A nlcricutl Jotjrnd oI’Surscry
727
considered a hemangioendothelioma. Stout’ who saw the sections and made the Laidlaw stains concurred in this diagnosis. Several other pathologists who viewed the sections variousIy diagnosed the tumor as a simple hemangioma or a hemangioendothelioma. The extreme cellularity of the tumor, the mitotic figures, and the multinucleated forms within the neoplastic cells remove this tumor from the group of simple capillary hemangioma. The presence of mitoses is thought to be consistent with a period of rapid growth of the tumor in an infant and not to indicate a malignant neoplasm. “The diagnosis is a hemangioendothelioma arising in the salivary gland.”
FIG. 5. Infant one month postoperatively.
Lateral tumors of the neck are diagnostic and therapeutic probIems met occasionally by head and neck surgeons. AIong with cystic hygroma, other conditions to be considered in the young infant are infectious parotitis, tuberculous adenitis, acute
Sections stained for reticulin by the Laidlaw method revealed anastomosing bIood vessel channels with neopIastic cells largely conlined within their reticulin sheaths. Thus, they are neoplastic endothehal cehs, and the tumor is
FIG. 6. Photomicrograph of a portion of the tumor to show presence of salivary gland ducts. Note the irregularly shaped blood-containing spaces separated by masses of neoplastic cells ( X 150).
FIG. 7, Photomicrograph showing a portion of Figure 6 under higher magnification. Details of the neoplastic cells are more cIearly shown. Note the multinucleated cell at A and the mitotic figure at n ( X 450).
728
orsurgery HaIey, A merican~~~~~~~
Jackson-HemangibendotheIioma
Ieukemia and branchiogenic cysts which rareIy occur at this age. In a review of American Iiterature from 1916 no mention of this type of tumor arising in the saIivary gIands was found. Other tumors in this region are aIso rare. The sections from this case have been reviewed by a number of pathologists, al1 of whom concurred in the opinion that this was a benign vascuIar tumor; however, it was variousIy designated as a hemangioma (simpIe type) and a hemangioendotheIioma. Three of the above pathoIogists mentioned had seen simiIar tumors, apparentIy primary, in a salivary gIand. No reports have appeared in the Iiterature. Reading on this subject is diffIcuIt because the criteria for the classification of bIood vesse1 tumors has not been cIearIy defined. Each author who has written on the subject has his own cIassification and terminoIogy. Ewing,3 Geschickter, Watson and McCarthy4 and Stout5 are among those who have cIassified these tumors. From a surgeon’s standpoint the pathoIogic interpretation of the tumor is important primariIy from a prognostic aspect. Diagnosis in this case rests upon specific criteria set up by Stout5 which are: “No tumor shouId be considered an hemangioendotheIioma unIess the folIowing two features are present: first, the formation of atypica1, endotheIia1 cells in greater numbers than are required to line the vesseIs with a simpIe endotheIia1 membrane; and, secondIy, the formation of vascuIar tubes with a deIicate framework of reticuIin
MAY, 1948
fibers and a marked tendency for their Iumens to anastomose.” Stout considers this a maIignant tumor except in infants when it is usuaIIy benign. HemangioendotheIiomas in the neck of infants have been reported by Smith,’ Watson and McCarthy4 and S\?reitzer and Winer.6 None of the three cases reported arose in saIivary tissue. The tumors in the case reports of Watson and McCarthy and of Sweitzer and Winer were cutaneous and red. SUMMARY
A case of benign hemangioendothelioma of the salivary gIands is presented with a discussion of the gross and microscopic features invoIved and the possibie benignancy of the tumor. A critica review of the Iiterature reveaIs that these tumors are reIativeIy infrequent. The authors wish to express their appreciation for the assistance of Dr. Ethelred L. Schafer,
pathoIogist
of the Jackson
Clinic.
REFERENCES
I. STOUT, A. P. Personal communication. z. SMITH, C. A. Massive cervica1 hemangioendotheIioma in a newIy born infant. Am. J. Dis. Child., 55: 124-127, 1938. 3. EWING, JAMES. NeopIastic Diseases. PhiIadeIphia, 1940. W. B. Saunders. 4. WATSON, W. L. and MCCARTHY, W. D. BIood and lymph vesse1 tumors. Surg., Gynec. w Obst., 71: 569-588, 5. STOUT, A.
‘940.
Ann. Swg., 118: 445-464, 1943. 6. SWEITZER. S. E. and WINER, L. H. Hemangioendothekoma. Arch. Dermat. & Sypb., 34: 9971007, 1936. P. Hemangioendothelioma.
OF THE SALIVARY
GLAND*
HAROLD B. HALEY, M.D. AND ARNOLD S. JACKSON, M.D. Madison,
H
Wisconsin
EMANGIOENDOTHELIOMA
arising in the saGvary gIands is a rare condition. A case of such a tumor in a young infant is presented. The most common IateraI neck tumor in the young infant is a congenita1 cystic hygroma. HemangioendotheIioma in such a Iocation is unusua1. Smith2 says, “A hemangioendothelioma of this region has never been observed at the Children’s Hospita1 (Boston), nor has it been diagnosed prior to the present case in any of the 40,000 chiIdren born at the Boston Lying-in HospitaI.” CASE REPORT T. L., a male infant, was first seen on May 31, 1947, at the age of six weeks with no abnormalities noted. On June 20, 1947, the parents noted swelling of the Ieft cheek. Three days later this was seen by a pediatrician who believed that the infant had a parotitis. A course of penicillin was given and the mass became somewhat smaller. It soon became large again and a course of sulfonamides was given with practically no effect. On July 14, 1947, the patient was seen in surgica1 consultation at which time the mass seemed to arise posterior and inferior to the left parotid gland, and the possible diagnosis of cystic hygroma was considered. The mass was followed for six weeks and grew steadily until it was about 8 cm. in diameter. It felt cystic and did not transihuminate light. The mass seemed to lie in the subcutaneous tissues of the neck. (Figs. I and 2.) On August I 9, I 947, an operation for remova of the tumor was performed by one of the authors (A. S. J.) under ether-oxygen endotracheal anesthesia. There was a large, wine* From the Department
colored tumor about 6 by 5 by 4 cm. involving the left side of the neck from the transverse processes of the sixth cervica1 Vertebra, the carotid and jugular vessels and the anterior surface of the sternocleidomastoid muscte to the skin. The tumor lay adjacent to the submaxihary and parotid glands but grossly seemed to be distinct from them. Many blood vessels extended from the tumor into surrounding tissues. Two lymph nodes were found in the area of the tumor. The tumor was excised intact. (Figs. 3 and 4.) The postoperative course was uneventful, except for development of a partial, transient facial paraIysis. (Fig. 5.) The following pathoIogic report by Dr. EtheIdred L. Schafer gives the gross and microscopic appearance of the specimen removed at surgery : “The gross specimen was a soft but not fIuctuant, well encapsulated, dark red ovoid mass, measuring 5.5 by 4.5 by 3.5 cm. The external surface was faintly bosselated. The capsule was thin and transparent. Cut surfaces were coarsely lobulated, dark red, and slightly bulging. A small amount of blood exuded from the surface. Attached at one pole was a separate ovoid mass, measuring 1.5 cm. in maximum diameter, which had pinkish gray cut surfaces and resembled a lymph node. “Numerous sections from the mass showed a vascular tumor involving salivary gland tissue. Some of the gland was visible at the margins of the tumor, and isolated glands and ducts occurred throughout the tumor. Delicate fibrous connective tissue septa divided the tumor into lobules reminiscent of the normal pattern of the salivary gland. In some regions, variable-sized blood-containing spaces with onIy thin walIs separating them were numerous. These spaces were lined by endothelial cells which in some instances were pIump. An
of Surgery, Jackson Clinic, Madison, 725
Wis.
726 A mcrican ~~~~~~~ or~urgery
MAY, 1948
HaIey, Jackson-HemangioendotheIioma
1
2
FIGS.
3 FIG. 3. Tumor demonstrated
I and 2. Preoperative
at operation.
occasional mitotic figure was noted among these cells. In other foci, compact masses of small ceIIs occurred between the blood spaces. These ceIIs had indistinct cell outhnes. Their nuclei were round or irregulady ova1 and fairly uniform in size. The chromatin material was irregmarly dispersed throughout the nucleus; none contained a nucleolus. Mitotic figures were more numerous in these compact foci, numbering as many as 4 to 5 per high power field. An occasiona multinucIeated cell was
photographs
of infant.
4 FIG. 4. Specimen removed at operation.
noted. The Iymph node at the periphery of the tumor was normaI. (Figs. 6 and 7.) “The tumor beIongs in that group of tumors specihcally to the known as angiomata, hemangiomata. The fohowing must be considered in making a diagnosis: hemangioma (capillary type), hemangioendotheIioma, hemangiopericytoma, and hemangioendotheliobIastoma. The exact nature of the tumor cells and their reIationship to bIood vesseIs could not be demonstrated without special stains.
VOL. ILXSV.
No.
5
Haley,
Jackson-Hemangioendothelioma
A nlcricutl Jotjrnd oI’Surscry
727
considered a hemangioendothelioma. Stout’ who saw the sections and made the Laidlaw stains concurred in this diagnosis. Several other pathologists who viewed the sections variousIy diagnosed the tumor as a simple hemangioma or a hemangioendothelioma. The extreme cellularity of the tumor, the mitotic figures, and the multinucleated forms within the neoplastic cells remove this tumor from the group of simple capillary hemangioma. The presence of mitoses is thought to be consistent with a period of rapid growth of the tumor in an infant and not to indicate a malignant neoplasm. “The diagnosis is a hemangioendothelioma arising in the salivary gland.”
FIG. 5. Infant one month postoperatively.
Lateral tumors of the neck are diagnostic and therapeutic probIems met occasionally by head and neck surgeons. AIong with cystic hygroma, other conditions to be considered in the young infant are infectious parotitis, tuberculous adenitis, acute
Sections stained for reticulin by the Laidlaw method revealed anastomosing bIood vessel channels with neopIastic cells largely conlined within their reticulin sheaths. Thus, they are neoplastic endothehal cehs, and the tumor is
FIG. 6. Photomicrograph of a portion of the tumor to show presence of salivary gland ducts. Note the irregularly shaped blood-containing spaces separated by masses of neoplastic cells ( X 150).
FIG. 7, Photomicrograph showing a portion of Figure 6 under higher magnification. Details of the neoplastic cells are more cIearly shown. Note the multinucleated cell at A and the mitotic figure at n ( X 450).
728
orsurgery HaIey, A merican~~~~~~~
Jackson-HemangibendotheIioma
Ieukemia and branchiogenic cysts which rareIy occur at this age. In a review of American Iiterature from 1916 no mention of this type of tumor arising in the saIivary gIands was found. Other tumors in this region are aIso rare. The sections from this case have been reviewed by a number of pathologists, al1 of whom concurred in the opinion that this was a benign vascuIar tumor; however, it was variousIy designated as a hemangioma (simpIe type) and a hemangioendotheIioma. Three of the above pathoIogists mentioned had seen simiIar tumors, apparentIy primary, in a salivary gIand. No reports have appeared in the Iiterature. Reading on this subject is diffIcuIt because the criteria for the classification of bIood vesse1 tumors has not been cIearIy defined. Each author who has written on the subject has his own cIassification and terminoIogy. Ewing,3 Geschickter, Watson and McCarthy4 and Stout5 are among those who have cIassified these tumors. From a surgeon’s standpoint the pathoIogic interpretation of the tumor is important primariIy from a prognostic aspect. Diagnosis in this case rests upon specific criteria set up by Stout5 which are: “No tumor shouId be considered an hemangioendotheIioma unIess the folIowing two features are present: first, the formation of atypica1, endotheIia1 cells in greater numbers than are required to line the vesseIs with a simpIe endotheIia1 membrane; and, secondIy, the formation of vascuIar tubes with a deIicate framework of reticuIin
MAY, 1948
fibers and a marked tendency for their Iumens to anastomose.” Stout considers this a maIignant tumor except in infants when it is usuaIIy benign. HemangioendotheIiomas in the neck of infants have been reported by Smith,’ Watson and McCarthy4 and S\?reitzer and Winer.6 None of the three cases reported arose in saIivary tissue. The tumors in the case reports of Watson and McCarthy and of Sweitzer and Winer were cutaneous and red. SUMMARY
A case of benign hemangioendothelioma of the salivary gIands is presented with a discussion of the gross and microscopic features invoIved and the possibie benignancy of the tumor. A critica review of the Iiterature reveaIs that these tumors are reIativeIy infrequent. The authors wish to express their appreciation for the assistance of Dr. Ethelred L. Schafer,
pathoIogist
of the Jackson
Clinic.
REFERENCES
I. STOUT, A. P. Personal communication. z. SMITH, C. A. Massive cervica1 hemangioendotheIioma in a newIy born infant. Am. J. Dis. Child., 55: 124-127, 1938. 3. EWING, JAMES. NeopIastic Diseases. PhiIadeIphia, 1940. W. B. Saunders. 4. WATSON, W. L. and MCCARTHY, W. D. BIood and lymph vesse1 tumors. Surg., Gynec. w Obst., 71: 569-588, 5. STOUT, A.
‘940.
Ann. Swg., 118: 445-464, 1943. 6. SWEITZER. S. E. and WINER, L. H. Hemangioendothekoma. Arch. Dermat. & Sypb., 34: 9971007, 1936. P. Hemangioendothelioma.