Journal of Surgical Oncology 7:493-496 (1975)
Ovarian Cancer Presenting as Umbilical Hernia .......................................................................................... ..........................................................................................
ROGER C. MILLAR, M.D., GLENN W. GEELHOED, M.D., and ALFRED S. KETCHAM, M . D . Six patients are reported whose presenting symptom was umbilical herniation. Upon exploration of the umbilicus with the intent to repair the hernia, ovarian cancer and malignant ascites were encountered in each individual. Cancer at the umbilicus, both metastatic and primary, is briefly discussed. The appearance of an acquired umbilical hernia in an otherwise asymptomatic patient should raise the suspicion of intraabdominal malignancy.
.. .. .. .. .. .. ................................................ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ......................................... ...................................... KEY WORDS: umbilical hernia, ovarian cancer, carcinoma of ovary, cancer-abdominal wall
Herniation at the umbilicus is not an uncommon entity in children, especially in the American Indian and negroid races. During embryologic development, the small bowel develops in the exocoelomic cavity located in the proximal end of the umbilical cord but outside the true coelomic cavity. At two and one-half months of gestation, the small bowel recedes into the abdomen and the exocoelom is obliterated by mesodermal and ectodermal ingrowth of muscle, fascia, and skin (Fig. 1). Occasionally this opening does not close completely and a congenital umbilical hernia results. This umbilical hernia rarely requires surgical correction. Natural body growth processes usually result in closure of the defect by approximately age ten. That the American Indian and negroid races have higher incidence of congenital umbilical hernia is known but not explained. The appearance of an umbilical hernia acquired in the adult, however, is a finding that calls for investigation and explanation. SLXAmerican Indian women from the U.S.P.H.S. facility in Talihina, Oklahoma, each manifested the unusual presentation of umbilical herniation which proved to be a signal of ovarian cancer.
Surgery Branch, National Cancer Institute. Address reprint requests to Dr. Geelhoed, 10N-116, National Cancer Institute, Bethesda, Maryland 20014.
Alan R. Liss, Inc.. 150 F i f t h Avenue, New York. N.Y. 10011
Millar, Geelhoed, and Ketcham
Fig. 1. Embryologic development of the umbilicus, with viscera retreating into the abdomen from their exocoelomic development, makes this site o n the abdominal wall a likely location for lymphatic, hematogenous, or direct extension of visceral malignancy, as well as herniation, when increased abdominal pressures reverse the embryologic migration of the viscera.
CASE REPORTS Patient I A 33-year-old Indian woman stated she had an umbilical hernia as an infant but that it had gradually disappeared without treatment by the age of ten. This umbilical hernia had not reappeared even though she had had four full-term pregnancies. Four months prior to admission, there had been a gradual increase in the size of the umbilicus until a lemon-sized protrusion that was of soft consistency and easily reducible was noted at the time of examination. The mass reduced spontaneously in the supine position. Although the preoperative workup failed to reveal any evidence of other disease, the operation designed to repair the acquired umbilical hernia discovered papillary cystadenocarcinoma of the ovary with ascites and peritoneal and omental metastases. Patient 2 A 47-year-old Indian woman with no previous history of umbilical defect presented with a six-week history of umbilical protrusion. The lesion was easily reducible, and no other evidence of disease was found on physical examination. An operation for umbilical herniorrhaphy established the unexpected diagnosis of papillary cystadenocarcinoma of the ovary with ascites. Patient 3 A 49-year-old operating-room nurse with a history of self-resolving umbilical hernia as an infant was seen two weeks after she first noted the appearance of an umbilical mass. On physical examination, an umbilical hernia was found which was easily reducible in
Ovarian Cancer Presenting as Umbilical Hernia
the supine position; suggestion of an ascitic fluid wave led to paracentesis which was suspicious for tumor cells. At the time of surgical exploration a moderate amount of ascites was noted, and a diagnosis o f papillary cystadenocarcinoma of the ovary was made. Diffuse peritoneal implants were also observed at laparotomy. Patient 4 A 57-year-old female Indian had a pelvic mass detected on routine annual physical examination but gave a history of having noted an umbilical protrusion for the preceding four months. Ascites was not detectable preoperatively, but a preoperative cervical biopsy was read as adenocarcinoma of the endocervix. At the time of surgical exploration, adenocarcinoma of the ovary was found. Ascites was present, although no peritoneal metastases were found. Patient 5 A 51-year-old woman, who recalled having a coin taped to her umbilicus as an infant but had had no apparent evidence of umbilical herniation during her adult years, presented with a four-day history of umbilical protrusion which was easily reducible. She had no other symptoms, but on physical examination a fullness was noted in the pelvis and an endocervical biopsy was read as adenocarcinoma of the cervix. At the time of abdominal exploration, both ovaries were involved with a malignant tumor with fixation to the posterior cul-de-sac and direct extension into the uterine and cervical canal. Patient 6 A 61-year-old woman reported a painless swelling of her umbilicus which had become noticeable two weeks before she sought advice from her physician. She had no prior history of umbilical hernia as a child and had no other symptoms except the impression of a gradual increase in girth. Physical examination revealed a reducible umbilical hernia. When the hernia sac was opened during umbilical herniorrhapy, malignant peritoneal implants were encountered which were diagnosed histologically as undifferentiated ovarian carcinoma. These six patients all had easily reducible umbilical hernias without a discrete mass palpable in the umbilicus. Half of the patients had an antecedent history of infant umbilical herniation which had disappeared without treatment. The average duration of symptoms was seven weeks with a range from four days to 16 weeks. All patients had ascites at the time of surgical exploration, although this finding was not appreciated in all the patients preoperatively. As seen in Table I, the postoperative diagnoses were TABLE 1.
Ovarian Malignancy Presenting as Umbilical Hernia ~~~
Infant Umbilical Hernia
Duration of Umbilical Hernia
Present, resolved by age 10 yr. None Present, resolved as a child None Present, resolved by age 2 yr. None
_ _ Papillary cystadenocarcinoma
6 weeks 2 weeks
Papillary cy stadenocarcinoma Papillary cystadenocarcinoma
47/F 49/F 57/F 51iF 6111
16 weeks 4 days
Ovarian adenocarcinoma Ovarian adenocarcinoma
Ovarian carcinoma, type unspecified
Millar, Geelhoed, and Ketcham
cystadenocarcinoma in three, adenocarcinoma in two, and one carcinoma of unspecified type.
DISCUSSION The umbilicus has been reported t o be a site of metastases of intraabdominal cancer (2-8). OLeary and O’Leary ( 5 ) reviewed the literature in 1964 and found 157 cases of umbilical cancer in reporting two more cases they had treated. Steck and Helwig (6) reported a series of patients reviewed at the Armed Forces Institute of Pathology (AFIP) in 1965 consisting of 48 malignancies located in the umbilicus. In summing these two reviews and adding those cases reported since 1964 or those omitted from the previous studies, a total of 235 cases of malignant disease diagnosed at the umbilicus can be cumulatively reviewed. Of these patients, 53 had primary tumors: 32 adenocarcinomas, 13 squamous cell carcinomas, 5 melanomas, 2 basal cell carcinomas, and 1 myosarcoma. One hundred eighty-two patients had metastatic disease at the umbilicus; of these, 57 originated in stomach, 37 were unknown primaries, and 18 were of ovary, 18 intestine and biliary tree, 10 pancreas, 7 uterus, 2 fallopian tube, 2 endometrium, and 1 each of prostate, kidney, cervix, appendix, liver, penis, and breast. All of the metastases were from intraabdominal lesions, except for the last two cases mentioned above. O’Leary and O’Leary ( 5 ) do not state how many patients had umbilical hernia as the presenting sign of their disease. The AFIP series revealed that the presenting symptom in 18 of 40 patients reviewed was an umbilical mass thought to be a hernia. The lymphatic drainage of the umbilicus is directed toward both axillae and groins. It is presumed that metastasis to the umbilicus is by retrograde lymphatic spread of tumor cells originating from outside the abdomen and by orthograde dissemination to the umbilicus of intraabdominal disease. Cases of direct extension of intraabdominal tumors to the umbilicus have also been reported. The six patients in our series did not have metastatic disease at the umbilicus. The umbilical hernia was a manifestation of presumably increased abdominal pressure secondary to ascites. The fact that infant umbilical hernias are prevalent in Indians may indicate that this group has an increased tendency t o develop herniation at this site when intraabdominal pressure is chronically increased in the adult. The appearance of an umbilical hernia in an adult should raise the suspicion of increased intraabdominal pressure, perhaps secondary to malignancy, as a possible etiologic cause for the acquired hernia.
REFERENCES 1. Bailey, H. Demonstrations of Physical Signs in Clinical Surgery, 13th ed. Williams & Wilkins, 1960, pp. 344-356. 2. Falkinburg, L. W., and Savran, J. Adenocarcinoma of the umbilicus secondary t o carcinoma of the breast. Amer. J . Surg. 87:795-797 (1954). 3. Greenberg, E., Gregg, J. A., and McIlrath, D. C. Umbilical hernia as a sign of occult intra-abdominal malignancy. New Physician 15:159-161 (1966). 4. Horn, J. J., Fred, H. L., Lane, M., and Hudgins, P. T. Umbilical metastases. Arch. Intern. Med. 114:799-802 (1964). 5. O’Leary, J. L., and O’Leary, J . A. Carcinoma of the umbilicus. Am. J. Obstet. Gynecol. 89: 136-1 37 (1964). 6. Steck, W. D., and Helwig, E. B. Cutaneous remnants of the omphalomesenteric duct. Arch. Dermatol. 90:463-470 (1964). 7. Steck, W. D. Tumors of the umbilicus. Cancer 18:907-915 (1965). 8. Williams, C. Unusual surgical lesions of t h e umbilicus. Ann. Surg. 124:1108-1124 (1946).