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Primary Appendiceal Malignancy : A Rare Entity in the Differential Diagnosis of Ovarian Cancer
Arshad S Khan*, Girish D Bakhshi**, Prithvipal R Chavan***,
Siddharth Sarangi***, Hemant H Deore***
Abstract
Primary tumours of the appendix are rare and most of them are unrecognized preoperatively, presenting as appendicitis, pelvic masses or with no typical abdominal pain. We report a 46 year old female patient with the suspected diagnosis of ovarian cancer. Upon surgical exploration, there was an ovarian mass with uterine fibroid along with mass in the appendix. Histological examination revealed the diagnosis of primary mucinous adenocarcinoma of the appendix with metastasis in the left ovary. This is an unusual consideration in the differential diagnosis of an ovarian cancer. We discuss the diagnosis and treatment of the cancer of the appendix in relation to ovarian cancer.
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| Fig. 1 : Figure showing large ovarian cyst. |
Fig. 2 : Figure showing enlarged appendix along with ileocaecal region. |
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Introduction
Primary appendiceal malignancy
metastatic to the ovaries is a rare condition that may mimic late stage ovarian cancer. Primary adenocarcinoma of the appendix comprises less than 0.5% of all gastrointestinal neoplasms.1,2 This condition is rarely diagnosed preoperatively. It is important for the gynaecologic oncologist to be aware of the clinicopathological features and surgical management of these malignancies, as the incidence, prognosis, and recommended treatment vary with histological subtype. A case of mucin producing adenocarcinoma of the appendix with metastasis in left ovary, manifesting as a pelvic mass is presented.
Case Report
We present a case of a 46 year old housewife who was admitted with complaints of lump in the abdomen since one year. Her clinical examination revealed a pelvic lump freely mobile and palpable on per vaginal examination. Ultrasonography (USG) showed a large cystic lesion of size 30 cm x 28 cm with multiple irregular septation most likely arising from ovary. CT scan confirmed it to be a cystadenoma arising from the pelvis and involving the uterus and both ovaries most probably neoplastic in origin. Her tumour marker Ca-12-5 was within normal range.
On surgical exploration, there was an enlarged left ovarian cyst (Fig. 1) with a subserosal fibroid of uterus. Besides, this, there was a mass felt in the appendix which was hard and involving the whole appendix till its base in the caecum (Fig. 2). Total hysterectomy with bilateral salpingo-oophorectomy was done. In view of suspected malignancy, tumour in the appendix was managed by right hemicolectomy with ileo-transverse anastomosis. Histopathology report of the specimen revealed primary mucinous adenocarcinoma of the appendix with secondary metastasis of adenocarcinoma in the left ovary. Uterus showed subserosal fibroid whereas, right ovary was normal on histopathology.
Patient had uneventful recovery after surgery. She was given chemotherapy with 5-Fluorouracil. Post-operative follow-up of six months has shown patient to be disease and symptom free.
Discussion
Primary adenocarcinoma of the appendix is common during or after the 5th decade of life though an occasional paediatric case report is seen.3 Only 200 cases have been reported so far. The incidence of primary adenocarcinoma of the appendix is between 0.01 to 0.11 of all appendicectomies. The first case was reported by Berger in 1882. Gilhome and associates reported a series of 10 cases of adenocarcinoma appendix.4,5 Clinically, most of them present as acute appendicitis or appendicular mass.5 Hence it has been suggested that in patients over the age of 50, appendicectomy should be done by laparotomy.
Appendiceal mucinous cystadenocarcinoma is a rare tumour and accounts for less than 0.5% of all intestinal tumours.6 The age of affection of a mucinous cystadenocarcinoma is lower than the typical patient with an adenocarcinoma of the appendix.7 In 15-20% of cases, a second malignancy in the gastrointestinal tract is associated with the disease.7 Malignancies of the appendix are never suspected pre-operatively and seldom post-operatively, the diagnosis being usually made at histopathology of the specimen.4 In our case the underlying pathology proved to be a mucinous cystadenocarcinoma of the appendix on histopathology report.
Patient may present with right lower abdominal pain mimicking appendicitis or can also be asymptomatic. Occasional cases may present with melaena or perforation. Cases presenting as ovarian cystadenocarcinoma is rare.8 Our patient presented as a pelvic mass mimicking ovarian lump.
Preoperative diagnosis, although important for proper surgical management, is difficult due to the absence of specific imaging findings. The presence of a cystic mass in the expected area of the appendix with enhancing wall and nodularity on CT examination suggests the possibility of mucinous cystadenocarcinoma.10 Wall thickness has not been proven to be a reliable differential point between neoplastic and non-neoplastic causes of mucocoele of the appendix.11 Curvilinear nodular or punctate wall calcifications have been reported in both malignant and benign mucocoeles.10,11
Mucinous cystadenocarcinoma is usually a well-differentiated neoplasm, and the gross morphology of this tumour is that of a mucocoele.10 If there is a concomitant ovarian tumour to determine the primary might be difficult. Histological features are not always determinant, but there are some macroscopic findings that may suggest an origin in the appendix. Histologically, adenocarcinoma of the appendix may have colonic or mucinous glands; rarely, cystadenocarcinoma or pseudomyxoma peritonei occurs.8 However, the findings of adenomatous change in the mucosa adjacent to the growth (carcinoma) is the best evidence that the primary site is in the appendix.
Once a diagnosis of a mucinous cystadenocarcinoma has been achieved, either by frozen section examination during surgery or at histopathological examination post-operatively, a right hemicolectomy has been the accepted form of definitive therapy.12 These tumours have a slower growth rate which translates to longer survival than with appendiceal adenocarcinomas.7 Surgical removal of the tumour when detected early is said to offer a cure. The 10 year survival after right hemicolectomy has been found to be 65% in contrast to 37% in patients subjected to appendicectomy alone.7 Patients with disseminated disease are benefited by aggressive debulking, omentectomy and oophorectomy and often require repeated operations for intestinal obstruction or fistulation.7 Radiotherapy and chemotherapy have not been found to be effective in the setting of disseminated disease.7 Prognosis is better in the mucinous type than in the colonic type. Pseudomyxoma peritonei has bad prognosis. The role of adjuvant therapy in the isolated case with the possibility of tumour spillage at surgery, or infiltration of adjacent viscera is a question that might never be answered in view of the rarity of the disease.
References
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12. Lo NS, Sarr MG. Mucinous cystadenocarcinoma of the appendix. The controversy persists: a review. Hepatogastroenterology 2003 Mar-Apr; 50 (50) : 432-7.
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