Mucinous cystadenocarcinoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
Mucinous cystadenocarcinoma of the renal pelvis was first described in 1960 by Hasebe et al. Mucinous cystadenocarcinoma is one of the most aggressive forms of cancer. KRAS mutations are found in mucinous carcinomas. The organs involved in pathogenesis of mucinous cystadenocarcinoma are ovary, appendix, pancreas, colon, rectum, retroperitoneal organs, testes, salivary gland, lung, bladder, and breast. On gross pathology, multiloculated, smooth grey surface, and multilocular mass with thin walls and mucinous material are characteristic findings of mucinous cystadenocarcinoma. On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous cystadenocarcinoma. Mucinous cystadenocarcinoma commonly affects individuals older than forty years of age. Females are more commonly affected with mucinous cystadenocarcinoma of pancreas than males. Common risk factors in the development of mucinous cystadenocarcinoma are obesity and post-menopausal women on hormone replacement therapy. According to the American Joint Committee on Cancer (AJCC), there are 4 stages of mucinous cystadenocarcinoma based on the clinical features and findings on imaging. Each stage is assigned a letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis. Findings on CT suggestive of mucinous cystadenocarcinoma include rounded or ovoid tumor, internal septations, and calcification. The main mode of treatment of mucinous cystadenocarcinoma is chemotherapy and radiation. The most effective treatment for mucinous cystadenocarcinoma is surgical resection.
Historical Perspective
Mucinous adenocarcinoma of the renal pelvis was first described in 1960 by Hasebe et al.[1]
Pathophysiology
Mucinous adenocarcinoma is one of the most aggressive forms of cancer. KRAS mutations are found in mucinous carcinomas. The organs involved in pathogenesis of mucinous cystadenoma are ovary, appendix, pancreas, colon, rectum, retroperitoneal organs, testes, salivary gland, lung, bladder, and breast. On gross pathology, multiloculated, smooth grey surface, and multilocular mass with thin walls and mucinous material are characteristic findings of mucinous adenocarcinoma. On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous adenocarcinoma.
Causes
Mutations in the KRAS gene cause mucinous cystadenocarcinoma.
Differentiating Mucinous Cystadenocarcinoma from other Diseases
Mucinous cystadenocarcinoma must be differentiated from mucinous cystadenoma, serous cystadenoma, and pseudocyst.
Epidemiology and Demographics
Mucinous cystadenocarcinoma commonly affects individuals older than forty years of age. Females are more commonly affected with mucinous cystadenocarcinoma of pancreas than males.
Risk Factors
Common risk factors in the development of mucinous cystadenocarcinoma are obesity and post menopausal women on hormone replacement therapy.
Natural history, Complications and Prognosis
If left untreated, most of the patients with mucinous cystadenocarcinomas may be confined to the organ itself. Common complications of mucinous cystadenocarcinoma include metastasis and inguinal hernia. The presence of metastasis is associated with a particularly poor prognosis among patients with mucinous cystadenocarcinoma.
Staging
According to the American Joint Committee on Cancer (AJCC), there are 4 stages of mucinous cystadenocarcinoma based on the clinical features and findings on imaging. Each stage is assigned a letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis.
History and Symptoms
Symptoms of mucinous cystadenocarcinoma of ovary include mass in the abdomen, increase in abdominal size, bloating, weight loss, shortness of breath, and pain abdomen.
Physical Examination
Patients with mucinous cystadenocarcinoma usually appear normal. Physical examination of patients with mucinous cystadenocarcinoma is usually remarkable for abdominal distention, shifting dullness, a palpable abdominal mass, and coarse crackles upon auscultation of the lung bases.
CT
Findings on CT suggestive of mucinous cystadenocarcinoma include rounded or ovoid tumor, internal septations, and calcification.
MRI
Findings on MRI suggestive of mucinous cystadenocarcinoma include lower signal intensity for loculi with watery mucin on T1-weighted images.
Ultrasound
Ultrasound may be helpful in the diagnosis of mucinous cystadenocarcinoma. Findings on ultrasound suggestive of mucinous cystadenocarcinoma include mural thickening and solid components.
Biopsy
On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous adenocarcinoma.
Medical Therapy
The main mode of treatment of mucinous cystadenocarcinoma is chemotherapy and radiation.
Surgery
The most effective treatment for mucinous cystadenocarcinoma is surgical resection.
References
- ↑ HASEBE M, SERIZAWA S, CHINO S (1960). "[On a case of papillary cystadenocarcinoma following malignant degeneration of a papillary adenoma in the kidney pelvis]". Yokohama Med Bull. 11: 491–500. PMID 13712095.