Streamlined%lrticles CAROTID
BODY TUMORS
HERMAN CHARACHE, M.D. Brooklyn, New York
N
‘OT many surgeons have operated upon or are acquainted with the cIinicaI picture of carotid body tumors. OnIy 29; cases have been reporte& in the Iiterature up to the present. Because of the rarity of the condition a critica review of the Iiterature was made and an additional case associated with carcinoma of the urinary bIadder reported. The diagnosis of carotid body tumor is seldom made or even entertained. They are symptomIess tumors and of many years’ duration. Fifteen per cent of these tumors are malignant. The majority of them envelop the carotid arteries and juguIar vein. The treatment of choice is surgical removal. The magnitude of the surgica1 procedure is influenced by the necessity of Iigation of one, two or three carotid vesseIs. This procedure, however, increases the mortality and morbidity of the patient. Radiation therapy is recommended for inoperabIe cases. It is hoped that this articIe wilI stimuIate further thought on the subject of carotid body tumors. * * * * In 1929 Bevan and McCarthy’ stated that very few surgeons have operated upon more than one patient, a few have operated upon two patients and no one has operated on more than three. This statement generaIIy stiI1 hoIds true, with certain exceptions. Since that exceIIent articIe was written, Rankin and WeIIbrock2 reported tweIve cases in 1931. HertzIer3 reported seven persona1 cases in 1937. Harrington et aI.” reported nineteen cases in 1941 and Lahey December,
1949
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and Warren> reported eighteen cases in I 947. The aforementioned articIes and that of Keen and FunkeG have formed a very sound foundation for the further study of tumors of the carotid body. Carotid body tumors retain the shape, IobuIation and encapsulation of the norma carotid body as we11 as the histology, but on a Iarger scaIe. They vary in size from a hazeInut to an orange. The Iargest t.umor described by Reid’ weighed 190 Gm. Firm pressure wiI1 diminish its size by forcing the bIood out of the highIy vascuIar mass. Some writers described the tumor grossIy as resembling a kidney whiIe others compare it to the thyroid gland. The tumor is aImost aIways uniIatera1 aIthough eight bilateral cases were reported. Lund* mentions a case in which a second tumor appeared in the opposite side of the neck twenty-nine years after removal of the first one. The right and Ieft sides are equaIIy involved. MaIe and femaIe are aImost equaIIy affected. The oIdest patient reported in the Iiterature was seventy-three and the youngest seven; the average age is between thirty and forty years. Heredity does not inffuence the growth of these tumors although Chase9 reports two members of the same famiIy with carotid body tumors. The tumor is of a firm, rubbery consistence. The surface is smooth aIthough in some instances it may be noduIar. The coIor varies from grey-red to purpIe-red. It is not attached to the skin but is fixed to the underIying structures. It is FreeIy movabIe from side to side but not from above downward. It practicaIIy always
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pulsates but the pulsation is not that full expansion and contraction of an aneurysm but a transmitted puIsation from the carotid vesseIs. In some cases the tumor may buIge into the pharynx whiIe in others it may extend to the base of the skuI1. As the tumor grows in size, it enveIops the carotid vesseIs and juguIar veins in the majority of cases; or it may invoIve or press upon the gIossopharyngea1, hypogIossa1, vagus, phrenic or superior cervical sympathetic. Very few cases of maIignancy of carotid body tumors are reported. Bevan and McCarthy state that unIess tumor ceIIs can be demonstrated invading the tumor capsuIe or vesse1 waIIs with or without regional Iymph node invoIvement, it is very diffIcuIt, if not impossibIe, to make a diagnosis of maIignancy. They state that not more than 15 per cent of the tumors are maIignant. Rankin and WeIIbrock reported a case of biIatera1 carotid body tumor that was maIignant on the Ieft side and benign on the right. Cragg’O reported a case of concurrent tumor of the Ieft carotid body and biIatera1 ZuckerkandI’s bodies. The maIignant tumors rareIy, if ever, resuIt in distant metastasis. Ewing’l states, “ General metastases are not observed.” This opinion is aIso heId by WiIIis.12 LocaI extension with invoIvement of the regiona lymph nodes is not uncommon in maIignancy of the carotid body. Recurrence may take pIace even in benign tumors if they are not compIeteIy removed. The cIinica1 diagnosis of carotid body tumor is seIdom made or even considered as a differentia1 diagnosis in tumors of the neck. It is a symptomIess tumor which occurs in the upper anterior cervica1 triangIe and which may exist for many years. The average duration of the tumor before the patient came to the surgeon compIaining of a sweIIing in the neck was 5.7 years. The Iongest time was thirty-five years. Symptoms mgy be present when the tumor buIges into the pharynx or presses or on the vagus, cervica1 sympathetic recurrent IaryngeaI nerves. Pressure symp-
Body Tumors toms may be maniiested by headache, dizziness, hoarseness, cough, dysphagia or fainting speIIs. Stokes-Adams syndrome and Horner’s syndrome have been reported in severa cases. The differentia1 diagnoses in order of importance and frequency are metastatic tumors, Iymphomti, tubercuIoma, neurofibroma, branchia1 cysts, aberrant thyroid and aneurysm. Bronchogenic cysts are most IikeIy to resembIe cIinicaIIy tumors of the carotid body. If cIinica1 evaIuation faiIs to estabIish a diagnosis and aneurysm is definiteIy ruIed out, a biopsy wouId not onIy estabIish a diagnosis but wouId aIso dictate the mode of treatment. The treatment of choice of tumors of the carotid body is compIete surgica1 remova without Iigation of the carotid vesseIs if possibIe. The magnitude of the surgical procedure is inffuenced by the necessity of Iigation of one, two or three carotid vesseIs. This in turn depends on the fixation of the vesseIs to or into the tumor substance. In many instances the tumor compIeteIy enveIops the vesseIs and fuses with them making their dissection from the tumor impossibIe. Important nerves of the neck may be simiIarIy incorporated into the tumor substance aIthough not as frequentIy. Ligation of the common carotid artery in aduIts, particuIarIy those who are in the Iatter half of middIe age or beyond, is an extremeIy serious procedure which carries with it a high rate of mortaIity, 30 to 65 per cent (Rankin and WeIIbrock). In their series of tweIve cases it was necessary to Iigate the common carotid artery in three cases. AI1 of the patients died within the first forty-eight hours. Harrington et a1. report a mortality of 20 per cent which they attribute in most part to hemipIegia. The average age of the patients who died foIIowing Iigation was fifty-three whiIe the average age of those who survived Iigation was thirty-one. In Keen and Funke’s twenty-six cases four patients died from pneumonia foIIowing resection or division of the vagus nerve and five died from hemipIegia, aphasia and American Journal of Surgery
Charache-Carotid acute edema of the lungs. In three the voice was affected. Phelps, Case and Snyderl” reviewed 154 cases of which 148 patients were operated upon with a mortality of 24 per cent. The mortality was 30 per cent in the group of patients in which carotid ligation was Seven per cent of those who necessary. recovered foIlowing ligation of the carotid artery had cerebral comphcations. According to Dickenson and Traver in 50 per cent of the reported cases it has been necessary to sacrifice the common carotid artery which has resulted in the death of 30 per cent of the patients. Half of those who survive will have permanent brain damage. Keen comes to the foIlowing conclusion: “The symptoms and inconvenience of the tumor of the carotid body are generahy so sIight that I do not think any patient who is not suffering seriousIy or where the tumor does not exhibit symptoms of malignancy or a rapid growth shouId be subjected to the grave dangers which such operation may involve. Surely no prudent surgeon wouId be wiIIing to cause his patient to face such dangers simply for cosmetic purposes. If, on the other hand, the tumor is growing rapidIy and presents a mahgnant then I beIieve operation is appearance, justified, but not otherwise.” Considering the difficulty in diagnosing carotid body tumors, the clinical uncertainty of their malignant nature and the fact that carotid ligation is not aIways necessary, one wouId rather agree with the conclusion of Lahey and Warren that “al1 IateraIIy Iocated, discrete and movable tumors of the neck shouId be explored.” Only through expIoration and the taking of a biopsy can one determine not only the nature of a primary tumor of the neck but the operability and prognosis. Bevan and McCarthy beIieve that neopIasms of the carotid body should not be removed when it is necessary to Iigate the common carotid artery in order to compIete the operation. They recommend IocaI anesthesia as best for this operation. December,
1949
Body
Tumors
FIG. I. Photomicrograph shows papillary of the urinary bIadder. X 50.
carcinoma
Harrington et al. recommend preoperative systemic compression of the common artery against the transverse process of the sixth cervica1 vertebra several times a day for a few weeks, with the hope of deveIoping coIIatera1 circulation in the brain on the affected side. Compression shouId be carried out for gradually increasing periods unti1 the patient can tolerate complete compression of the vessels for a long period without experiencing faintness or Ioss of consciousness. Lahey and Warren state that the common carotid artery should be graduaIIy compressed until the patient can tolerate complete compression for a period of ten minutes three times a day without any symptoms. They also state that if the common, interna and external carotids are Iigated, it should be accompanied by ligation of the interna jugular vein. Radiation therapy in inoperabIe carotid body tumors has not been given a sufficient trial to form a concIusion as to itsappIication. Bevan and McCarthy report good results in one case of carotid body tumor that was explored but not removed. Lahey and Warren report three cases in which patients were treated with radiation therapy resuIting in sIight reduction in the size of the tumor in each instance. Phelps, Case and Snyder, on the other hand, report seven proved cases of carotid body tumors treated by radiation without appreciable
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FIG. 2. Gross specimen of the carotid body tumor shows capsuIe (A) with its septi dividing it into IobuIes (B) and terminating at the hiIus in a pedicle, the ligament of Mayer, (c).
rest&s. One may if a carotid body found inoperabre, be given a trial
therefore con&de tumor is explored radiation therapy
CASE
that and may
REPORT
The
patient was lirst seen on October I I, in the elevator as he went up to be admitted for carcinoma of the urinary bladder. When questioned about the swelling on the left side of his neck, he replied unconcernedly, “Oh, that! That is nothing. I have had it more than two years and it does not bother me.” The patient was a white male, aged seventytwo, weighing I 19 pounds. He was in fairly His blood pressure was good condition. 1943,
170/1oo. He was transferred to the Brooklyn Cancer Institute from another hospital with a diagnosis of carcinoma of the urinary bladder. No mention was made on the transcript about a tumor of the neck. His past history and
family history were essentially negative except for a dry, non-productive cough of two years’ duration. The tumor on the left side of his neck was about the size of an orange, extending from the angle of the mandible to the upper border of the clavicIe and partially covered by the sternocleidomastoid muscle. It was of a soft, rubbery consistency, somewhat nodular, partially fixed to the underlying structures but not to the skin. It was somewhat movable from side to side but not from above downward. The skin appeared intact. A transmitted pulsation was present but no bruit. Slight traction on the tumor produced a cough similar to the one he had had for the past two years. Roentgen and
Body
Tumors
FIG. 3. Photomicrograph shows the histoIogic structure of the tumor. X 140.
ffuoroscopic examination of the neck only corroborated the diagnosis of a soft tissue tumor. The chest x-ray was negative. The biopsy of the tumor in the urinary bladder was reported as papillary carcinoma. (Fig. I.) The cardiogram showed myocardial damage with occasional premature contraction. with 72 per cent The red count was 3,380,ooo hemoglobin; the white count was 14,000 with 17 lympho80 per cent polymorphonuclears, cytes and 3 per cent monocytes. The blood urea 24 mg., creatinin I .9, sugar I 00, cholesterol I 70, serum acid phosphatase 3 and serum alkaline phosphatase 6.8; the blood Wassermann test was negative. On October 20, 1943, the patient was taken to the operating room. Under local anesthesia of I per cent novocain an incision was made along the anterior border of the sternocleidomastoid from the angle of the mandible to the upper border of the clavicle. A soft, encapsulated nodular tumor was found extending from the angle of the mandible to 2 cm. beneath the clavicle. The capsule appeared to fuse with the carotid sheath and was surrounded by dilated veins and fine nerve libers. It was only partially adherent to the carotid vessels. When the carotid sheath was opened, the tumor was found to be attached by a pedicIe to the crotch of the bifurcation of the common carotid artery. The tumor with its pedicle simulated a kidney with its ureter emerging from the pelvis. (Fig. 2c.) The tumor was carefully freed from the surrounding structures by dull and sharp dissection. The tumor pedicle was first loosely clamped and the patient’s reaction observed.
American
Journal
of Surgery
Charache-Carotid It was then doubly clamped, cut and ligated. The tumor was then removed in its entirety. CompIete hemostasis was estabIished and the wound was closed. The histoIogic report confirmed the diagnosis of carotid body tumor. (Fig. 3.) The patient made an uneventful recovery. Ten days later the urologist fulgurated the bladder carcinoma and inserted radon seeds. In August, 1944, the patient deveIoped a moderate amount of hoarseness and Iaryngoscopic examination revealed a partial paralysis of the right vocal cord. The patient was then observed in the Outpatient Department at frequent intervals. In August, 1945, he was admitted to another hospita1 where he died from uremia. There was no recurrence of the tumor of the neck. REFERENCES I. BEVAN, A. D. and MCCARTHY, E. R. Tumors of carotid body. Surg., Gynec. CTObst., 49: 764, 1929. 2. RANKIV, F. W. and WELLBROCK, W. L. Tumors of carotid body, report of 12 cases inctuding one of bilateral tumor. Ann. Surg., 93: 801, 1931.
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I 949
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3. HERTZLER, A. E. Surgical Pathology of Diseases of the Neck. P. 90. PhiIadeIphia, 1937. J. B. Lippincott Co. 4. HARRINGTON,S. W., CLAGETT, 0. T. and DOCKERTY, M. B. Tumors of carotid body, chnical and pathoIogica1 considerations of 20 tumors affecting 19 patients (one biIatera1). Ann. Surg., I r4: 820, 194!. 5. LAHEI., F. H. and WARREN, K. W. Tumors of carotid body. Surg., Gynec. o Obst., 85: 28 I, ,947. 6. KIXN, W. W. and FUI\‘KE.J. Tumors of the carotid gland. J. A. M. A., 47-469, rgo6. 7. REID, hl. R. Adenomata of carotid gland. Johns Hopkins Hosp. Bull., 31: 177, 1020. 8. L.UND, F. R. A case of biIatera1 tumor of carotid body. Boston M. IY S. J., 176: Gzr, 1017. 9. CHASE, W. H. FamiIiaI and biIatera1 tumors of carotid body. J. Path. CYBact., 36: I, 1933. IO. CRAG<;, R. W. Concurrent tumors of the Ieft carotid body and both ZuckerkandI bodies. Arch. Path., 18: 35, 1934. I I. EWING, J. NeopIastic Diseases. 4th ed., p. 384. Philadelohia. 1040. . ,_ W. B. Saunders Co. I2. WILLIS, R. A. The Spread of Tumors in the Human Body. P. 130. London, 1934. J. & A. Churchill. 13. PHELPS, F. W., CASE, S. W. and SXYDER, G. A. C. Primary tumors of carotid body; review of 139 histologically verified cases: report of case. West. J. Surg., 45: 42, ,037.