Ganglioneuroma complicating pregnancy

L~)s’~ER

$‘r

AL. :

GANGLIOtiEUiXOiVf.4

COMi'LICATING

PREGNANCY

127

He also cited a case, referred by Dr. Jonathan Wainwright, of a patient who had a “suspicious fibroid” removed and in whom there later occurred metastases in the neck, thigh, and abdominal wall. The metastases were removed surgically and the patient was alive twelve years following the hysterectomy. SUMMARY

A desmoid fibroma involving the sacrospinalis and posterior inferior serratus muscles of the back is reported. A supracervical hysterectomy was performed eleven and one-half years before with removal of an anaplastic uterine sarcoma. The patient is alive and well thirteen and one-half years following the hysterectomy, and there is no recurrence from the uterine sarcoma or the desmoid tumor of the back. Clinically t,he desmoid was suspected of bein, v a late metastasis from the uterine tumor. REFERENCES 1. Bellanger, M. H.: Bull. et n&m. Sot. d. chirurgiens de Paris 27: 336, 1935. 2. Donald, J. M., and Caylor, H. D.: Ann. Surg. 89: G31, 1929. 3. Ewing, J.: Neoplaxtic Diseases, Philadelphia, 1940, W. B. Saunders Co., pp. 176, 177, and 228. 4. Marlow, F. W.: Canad. M. A. J. 32: 674, 1935. 5. Mason, J. B.: Ann. Surg. 92: 444, 1930. 6. McFarland, J.: Am. J. Cancer 25: 530, 1935. 7. Nichols, R. W. : Arch. Surg. 7: 227, 1923. 8. Novak, E.: Gynecological and Obstetrical Pathology, Philadelphia, 1940, W. B. Saunders Co., p. 193. 9. Penick, R. M.: Inter. Surg. Dig. 23: 323, 1937. 71). Warren, S., and Sommer, G. N. J. Jr, : Arch. Surg. 33: 425, 1936. 11. Waugh, J. M.: Am. J. Surg. 50: 694, 1940.

GANGLIONEUROMA

COMPLICATING

IJEON FOSTER, M.D., WILLIAM J. ROY COMPTON, M.D., (Prom

the

Booth

Memorial

PREGNANCY BERMAN, M.D., AND

ST. LOUIS, MO.

[Salvation.

Army]

Hospital)

ANGLIONEUROMAS have been located in all parts of the body, but they are most prominent in the retroperitoneal spaces of the G thorax and abdomen. In this report we are chiefly concerned with those tumors found in the abdomen and pelvis. Beneke reported the first case of pelvic retroperitoneal ganglioneuroma in 1898; only 13 have been reported up to 1940 according to Turunen. Pick and Bielschowsky in 1911 revealed the true relationship between ganglioneuromas and the sympathetic nerve elements, and classified true neuromas on an embryonic basis. They named them embryonic sympathetic gangliomas. These tumors are now considered to develop at the site of cell nests that have become displaced during embryonic life and therefore are congenital in origin. They are seldom diagnosed before operation, and at times after operation they are diagnosed as fibromas, liposarcomas, or fibrosarcomas. According to Evans and Francona, the tumor is more frequent in women than in men and more in the left side of the body than the right side. It is more prominent in the first twenty years of life. The

128

AMERICAN

JOURNAI,

OF

W3HTE1’RICS

AND

GYXECOI~OGV

majority of these tumors are benign and cause symptoms because of pressure on the surrounding vital struct.urcs. These tumors are very slow growing and have been known t.o have been present, for years wlt,h no tendency to become malignant. Technical difficulties are usually encountered in t.he delivery of the tumor, since it tends to be firmly adherent to the adjacent structures. Of the 13 cases reported (Turunen) , 8 patients were operated upon and 5 tumors were discovered at post mortem. (:ASF,>

RFI’ORT A

M. B., aged 20 years, white, unmarried, was admitted to the Booth Memorial (Salvation Army) Hospital in June, 1940. Her past history showed that her health had generally been good. She was nervous and had had indigestion for the past four or five years. She had the usual childhood diseases and had had no operations. She was seen some time ago by a physician who told her that she had a tumor of the ovary, and he advised operation. Family history was essentially negative. Her menstrual periods, which began at 15 years of age, occurring every thirty days, were regular, of moderate flow, and there was no dysmenorrhea. The last menstrual period was Jan. 6, 1940. Her general physical examination was essentially negative. Pelvic examination showed a nulliparous vagina, with no evidence of infection. The pelvis was filled with a hard lobular mass rather fixed to the sacrum, about the size of a large grapefruit, which did not seem to be connected to the uterus. The uterus was enlarged to about a five or six months’ gestation. The cervix was pulled up under the symphysis. X-ray of the abdomen showed the fetus outlined rather high in the abdomen, but no definite outline of a tumor mass could be made out. Pelvic measurements were within normal limits. Laboratory findings showed a negative Kahn test, The red blood count was 4,100,OOO and the white blood count was 7,900. Preoperative diagnosis was : a pregnancy of twenty weeks’ duration, and dermoid cyst filling the pelvis, At operation a large retroperitoneal mass was found filling the pelvis. It was fixed to the sacrum and to the iliac vessels and nerves. It was impossible to ligate the entire wide pedicle in one piece, and since the tumor seemed to involve the iliac vessels, most of it was removed, leaving a thin shell of tumor attached to the sacrum. The specimen was examined by Dr. Sam Katz of the Booth Memorial Hospital Pathological Depart,ment. The specimen consisted of a lobulated tumor, pale yellow, firm, and cutting like gristle. The tumor resembled a lipoma except for the firmness. On microscopic section, the connective tissue of the tumor was interposed with numerous cellular structures. Under special stain the larger and smaller groups of large round ganglion cells were seen. In general the ganglion cells had only one nucleus. Occasionally one found a double nucleus. The ganglion cells were well developed, sympathetic ganglion cells. Postoperative diagnosis was a twenty weeks’ gestation, and presacral ganglioneuroma. The postoperative course was smooth and the pregnancy continued uninterrupted. Rectal examination on Aug. 13, 1940, showed a right posterior pelvic mass somewhat irregular and fixed to the pelvic wall,

POSTER

ET

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:

GANGLIOtiEUROMA

COMPLICATING

129

PREGNANCY

which seemed to obstruct the inlet. The patient was permitted to go to term at which time a cesarean section was considered. The patient began having contractions on Sept. 17, 1940. On examination it was felt that the pelvic mass previously palpated would not Labor was permitted to cause enough dystocia to obstruct labor. progress and she was delivered of a 6 pound 9 ounce female infant by means of perineal forceps over a right mediolateral episiotomy. No unusual difficulty was encountered in the delivery.

Fig.

l.-Section

from

Fig.

tumor

Z.-High

showing

power

typical

ganglion

of section

from

cells.

tumor

Low

powrr

(X145).

(X630).

The post-partum course was uneventful. On Sept. examination revealed the mass to be smaller. Some be felt on the right side. The patient was last seen which time the pelvis was normal and there was no mass present. She had no symptoms referable to her

22, 1940, a rectal induration could Nov. 1, 1940, at evidence of any pelvis.

This presents an unusual and interesting case. Very few cases of this tumor have been reported in t,hc literature, but as mentioned before, it is probable that many have not been diagnosed and have found their way under the classification of other tumors such as fibromas, lipomas, liposarcomas, and fibrosarcomas. Because of the rarity of the condition a correct preoperative diagnosis is difficult, and the more common conditions are usually considered. The most int,eresting feature of the case was the complete disappearance of the remnants of the tumor after the patient, had delivered. When the pat,ient was last
u. z. allg. Path. 30: I, 1901. E:vans, J. H., md 48: G75, 1940. Fe& E.: Zentralbl. f. Gyn&k, 57: R9, 1933. Ge+eZ: Ibid. 48: 1673, 1924. Kehrer, E.: Arch. f. Gyn;ik. 158: 582, 1934. Law, 4. Robertson: Rurg., Gynec. & Obst. 17: 3, 1913. Sipped, Pa&: Zentralbl. f. Gynlk. 47: 840, 1923. Stoeckel, JO.: Ibid. 47: 33, 1923. Strati: Zentralbl. f. ges. Chir. 39: 482, 1927. Sfra.ssman. P.: Berl. Dtsch. Ges. Gyngk. Halle 15, 2: 486, 1913. Twunm, .,I.: ,1(&t. obst. et g~ynec. &and&v. 20: 25, 1940. Prancona,

Beitr.

z. path.

Anat.

N. T. : Sm. J. Surg.

MONOAMNIOTIC WARREN (From

the

Departmen,f

11. JOSES, M.l)., of

Obstetrics

Medicnl

T

TWIN and

Sas

I’RE(:NANCY FRASCISCO,

Gynecology,

lhivemity

CAI~F. of C'difornict

School)

HE following case seemsof interest because twins so rarely develop in one amniotic sac. A white primipara, aged 2’i years, entered the private service of Dr. Frank W. Lynch in the University of California Hospital on Dec. 1, 1940, in premature labor. Her expected date or’ confinement was Feb. 15, 1941; her last menstrual period May 8, 1940. The prenatal course had been uneventful. The Wassermann test was negative and the pelvis was normal. Twins had been suspected in the early months of pregnancy because of the relatively large size of the uterus, but the rate of growth then slowed down and the possibility was discarded. The family history revealed that, the patient’s mat,ernal great aunt had twins but no other twins occurred in either the patient’s or her husband’s family. On entering the Hospital, the patient stated t.hat she had not felt any fetal movements for several days and that, four hours prior to entry, fleeting uterine contractions had begun, associated with suprapubic pain. These contractions had increased steadily in severity and at the time of examination were severe and occurred every one and onehalf to two minutes. An excessive bloody show occurred as t,he patient, entered the Hospital. Physical examination disclosed: a uterus two fingerbreadths above the umbilicus; no fetal heart could be heard. The uterus was not unusually hard or tender between contra&ions. On rectal examination, the cervix was dilated 4 cm. with the membranes intact and a footling presentation.