Mucinous cystadenocarcinoma overview

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Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Mucinous Cystadenocarcinoma from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2], Ammu Susheela, M.D. [3]

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.

Pathophysiology

Mucinous adenocarcinoma is one of the most aggressive forms of cancer. KRAS mutations are found in mucinous cystadenocarcinomas. 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.

Diagnosis

Diagnostic study of choice

According to the American Joint Committee on Cancer (AJCC) there are 4 stages of mucinous cystadenocarcinoma based on the clinical features (pattern recongnition) 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.

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.

Electrocardiogram

Electrocardiogram findings in patients with mucinous cystadenocarcinoma are within normal limits.

X-Ray

X-ray is not used in the diagnosis of mucinous cystadenocarcinoma. Instead, ultrasound, CT scan and MRI are used in the diagnosis and staging of the tumor.

CT scan

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 cystadenocarcinoma.

Other imaging findings

There are no other imaging findings associated with mucinous cystadenocarcinoma.

Other diagnostic studies

There are no other diagnostic studies associated with mucinous cystadenocarcinoma.

Treatment

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.

Prevention

Primary prevention

There are no established methods for primary prevention of mucinous cystadenocarcinoma.

References

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