Jm. Fowler et al., PERITONEAL ADENOCARCINOMA (SEROUS) OF MULLERIAN TYPE - A SUBGROUP OF WOMEN PRESENTING WITH PERITONEAL CARCINOMATOSIS, International journal of gynecological cancer, 4(1), 1994, pp. 43-51
Peritoneal adenocarcinoma (serous or other subtype) of Mullerian type
(PAMT) is frequently misclassified as another primary tumor. Peritonea
l carcinomatosis in women without evidence of a primary site may occur
secondary to a number of processes. Confusion regarding the nomenclat
ure has made it difficult to determine the incidence and natural histo
ry of this unique malignancy. Other terms used for this tumor include
mesothelioma, peritoneal papillary serous carcinoma, extra-ovarian ser
ous carcinoma, and normal-sized ovarian carcinoma syndrome. Thirty-fou
r patients (33 serous and one endometrioid) were identified with PAMT
during 1976 through 1988. One hundred and thirty-seven patients underw
ent primary cytoreductive surgery for a preoperative diagnosis consist
ent with ovarian cancer. Twenty-nine (21.2%) were classified as PAMT (
5 of the 34 had their initial surgery at other institutions). The mean
age was 61.4 years. The primary symptoms and signs were abdominal pai
n (68%) and ascites (52%). Twenty-five (73%) had a preoperative diagno
sis of ovarian cancer while the postoperative diagnosis was unknown (4
4%), PAMT (29%), and ovarian cancer (27%). Univariate and multivariate
survival analysis were performed. Survival was independent of age, re
sidual disease, grade, ascites, type of chemotherapy, and second-look
results. In patients with residual disease <1.5 cm, extended survival
was found in those with ascites <1000 ml, residual disease in pelvis o
nly, and small residual volume but statistical significance was not ob
tained. Twenty-eight patients received greater-than-or-equal-to 4 cour
ses of chemotherapy after primary surgery. Twelve of 21 patients (57%)
who received cisplatin (CDDP) survived between 23 and 92 months, whil
e no patient receiving other chemotherapeutic regimens survived more t
han 25 months. The 2 and 3 year survival rate for CDDP was 47% and 33%
vs. 14% and 0% for other regimens. Optimal cytoreductive surgery was
not an independent prognostic factor as found in ovarian cancer, proba
bly secondary to unresectable peritoneal carcinomatosis. PAMT is sensi
tive chemotherapy but only the use of. CDDP was associated with long t
erm survival. Based on these results, women with peritoneal carcinomat
osis consistent with PAMT should receive a CDDP-based regimen after pr
imary surgery.