Health Articles

Benign neoplasms of (he mesentery and peritoneum are twice as common as malignant neoplasms; however, they are still rare.
BENIGN NEOPLASMS
The most common benign neoplasms are fibromas and lipomas. Most often found incidentally during routine examination, these tumors frequently grow large before causing symptoms. The most common symptoms are vague abdominal pain and bloating caused by compression or traction of adjacent structures. Intestinal obstruction may occur.
Diagnosis is usually made by x-rays that reveal extrinsic compression of the large or small bowel. Surgical excision is curative.
MALIGNANT NEOPLASMS
Malignant neoplasms of the mesentery and peritoneum, which are rare, usually include mesothelioma, fibrosarcoma, or leiomyosarcoma. Symptoms and signs include vague abdominal pain and bloating caused by traction or compression of adjacent structures and intestinal obstruction. Weight loss, anorexia, and weakness can also occur. Mesothelioma is associated with asbestos exposure.
Diagnosis is made by x-rays, including an upper GI series, barium enema, and CT scan, lhal reveal extrinsic compression or signs of invasion of the small or large bowel and other local structures. Surgery is the only effective treatment for cure or palliation. However, chemotherapy and radiation therapy may improve results for mesothelioma.

Malignant neoplasms of the bile ducts are rare and often difficult to diagnose.
BENIGN NEOPLASMS
Papilloma and adenoma are the most common benign neoplasms of the bile ducts, although fibroadenoma, adenomyoma. leiomyoma, granular cell myoblastoma, neurinoma, and hamartoma also occur. Intermittent jaundice and right upper quadrant pain arc the most common symptoms. The treatment of choice is local excision.
MALIGNANT NEOPLASMS
By far the most common malignant neoplasm is adenocarcinoma. Bile duct cancer is more common in men: the average age at diagnosis is 60 yr. Tumors of the upper portion of the ducts (50% of all lesions) are intimately related to the liver; those of the middle portion, to the portal vein and hepalic artery; and those of the lower portion, to the pancreas and duodenum. Such localization hay both diagnostic and prognostic implications.
Predisposing factors include primary sclerosing cholangitis, Clo-norchis sinensis infestation, and industrial exposure (in automobile and rubber manufacturing plant workers).
Symptoms, Signs, and Diagnosis
Because of their location, malignant tumors usually cause early symploms. Jaundice occurs in almost all patients. Right upper quadrant pain occurs in > 50%. Other associated symploms and signs include weight loss, nausea, vomiting, anorexia, fever, chills, diarrhea, constipation, clay-colored stools, and hepatomegaly. If obstruction occurs below the cystic duel, a distended gallbladder (Courvoisier’s sign) may be palpaled.
Diagnosis*of large tumors is made by ultrasonography and CT scan. However, percutaneous transhepatic cholangiography or endoscopic retrograde cholangiopancreatography frequently is more importanl in making Ihe diagnosis and localizing the tumor. Liver function test re-sulls are consistent with cxlrahepatic obstruction.
Treatment
The prognosis for bile duct cancer is poor with a 5-yr survival rate of only 5%. Tumors in the proximal portion of the bile duct system rarely are operable. When they are, complex liver and duct resections are required, and there are few long-term survivors. Palliation may be accomplished by dilation and slent insertion either cndoscopically or via Iranshepatic cholangiography. Resectable tumors of the central portion of the bile ducts may be treated by local en bloc excision. Bile duct drainage is reestablished by hepaticojejunostomy.
For resectable tumors of the distal duets, radical resection and pancreatoduodenectomy (Whipple’s operation) provide some promising benefit wilh 5-yr survival rales of 20% to .30%. With nonresectable tumors, palliation may be accomplished by hepaticojejunoslomy or by stenl placement followed by internal radiation via endoscopic retrograde cholangiopancreatography or percutaneous catheter insertion into the duels. Chemotherapy, radiation therapy, and liver transplantation are ineffective.

The liver is the most common site of metastasis from other cancers. Liver metastases arc found at autopsy in 30% to 50%> of patients with cancer.
Primary malignancy of the liver is rare in the USA and Western Europe but common in Africa and Asia. About 90% of primary adult hepatic cancers originate in the hepatocyle and are called hepatocellular carcinoma or hepatoma; 5% to 10% originate in the bile ducts as chol-angiocarcinoma or are a mixed type, cholangiohepatoma. Other primary liver cancers are exceedingly rare and include cystadenocarcinoma, angiosarcoma, and hepatoblastoma. Hepatic malignancies are more common in men than in women and occur between ages 50 and 70.
Hepatocellular carcinoma may be nodular, massive, or diffuse. The nodular type, which is the most common, consists of multiple discrete nodules. The massive type, which essentially accounts for the rest, develops as a large, often necrotic, hemorrhagic mass. The diffuse type is most often associated with cirrhosis and consislsof minule lesions scattered Ihroughout the liver. Predisposing factors include hepatitis B. hepatitis C, alcohol or tobacco use, cirrhosis, and aflatoxin exposure.
Symptoms and Signs
About 70% of patients present with right upper quadrant or epigastric pain and weight loss. With progressive liver dysfunction, menial status may deteriorate. Pulmonary symptoms and pathologic fractures may result from metastases. A rare lumor rupture with intra-abdominal hemorrhage and symptoms of an acute abdomen is life threatening.
Physical examination may reveal hepatomegaly, a right upper quadrant mass, or ascites. Splenomegaly is less common. Lymphadcnop-athy, especially in the right supraclavicular region, can occur. Gynecomastia is rare.
Paraneoplastic syndromes associaled with hepatocellular carcinoma include erythrocytosis. hypercalcemia, hypoglycemia, hyperlipemia, porphyria cutanea tarda, and dysfibrinoginemia.
Diagnosis
Liver function test results arc usually abnormal. Elevated serum alkaline phosphatase levels are common, but elevated serum aminotransferases and bilirubin levels are less so. Hepatitis B surface antigen and hepatitis C virus markers may be positive. Elevaled levels of α-fetoprotein are noted in 90% of patients with hepatocellular carcinoma.
20%. Metastasis is found in 75% of cases a! the lime of diagnosis. Workers in rubber and automotive plants have a particularly high risk of gallbladder cancer.
Gallstones are associated with the development of gallbladder carcinoma, and preventing such carcinoma may justify early cholecystectomy in patients with cholelithiasis.
Symptoms, Signs, and Diagnosis
Symptoms include intermittent pain and dyspepsia similar to that of chronic cholecystitis. In the late stages, weight loss and jaundice develop. Often a firm, tender mass is palpable in the right upper quadrant. Abdominal ultrasonography and CT scan provide visualization of the tumor. Endoscopic retrograde cholangiopancreatography or percutaneous transhepatic cholangiography is useful for a complete evaluation of the biliary tree.
Treatment
Radical cholecystectomy is the treatment of choice for localized disease. Radiation therapy and chemotherapy are ineffective. The prognosis is dismal; the 5-yr survival rate is only 5%. Biliary stenting by endoscopic refrograde cholangiopancreatography or percutaneous transhepatic cholangiography may provide limited palliation of an obstruction.

Hemangioma, the most common benign neoplasm of the liver, is found in about 5% of adult aulopsies. Other benign tumors include hepatocellular adenoma and local nodular hyperplasia, which are most often associated with oral contraceplives and are not usually seen in elderly persons. Bile duct adenomas and cystadenomas and rare mesenchymal tumors have also been reported.
Symptoms and Signs
Usually, these tumors are asymptomatic and are found incidentally at laparotomy or when a CT scan, ultrasonography, or angiography is performed for unrelated symptoms. Rarely, patients with hemangiomas present with massive hemorrhage from rupture or consumptive coagulopathy (Kasabach-Merritt syndrome).
Diagnosis and Treatment
liver function lest results are normal or slightly abnormal. The diagnosis can be established by routine liver biopsy in nonvascular lesions, although laparoscopy or laparotomy is often necessary- Therapy for nonvascular tumors usually consists of segmental resection.

Benign neoplasms of the liver are usually asymptomatic. By far, the most common form of hepatic tumor is metastatic carcinoma.

Kndocrine tumors arise from the neuroendocrine cells of the pancreas predominantly in the islets of Ihe pancreatic body and tail. However, enterochromaffin cells and other neuroendocrine cells can arise anywhere in Ihe GI tract. Endocrine tumors are rare in older persons.
These tumors are indistinguishable microscopically without special immunochemical staining. Localization of tumors can be aided by CT scan, portal venous sampling, and intraoperative ultrasonography. They may be cither nonfunctioning or functioning (ie, hormone secreting).
Nonfunctioning tumors may cause obstruction of the biliary tract or duodenum, bleeding into the GI tract, or an abdominal mass. Functioning lumors produce various syndromes.
Insulinomas, which may be single or multiple, produce insulin and arc identified by episodes of hypoglycemia, which may progress to coma in severe cases. Insulinomas are rarely malignant. Removal of all tumor tissue results in complete cure.
Gastrinomas produce the Zollinger-RIHson syndrome, an extremely virulent ulcer diathesis with excessive gastric acid secretion and diarrhea. The lumors can occur near the ampulla of Vater or the antrum as well as in the pancreas and are malignant in about 90% of cases. If metastasis does not occur, complete excision of Ihe lumor is curative. If metastasis does occur, various therapeutic modalities may be used, including administration of large doses of Hb-receptor blockers or omeprazole and, if medical therapy fails, gastric surgery to reduce acid secretion.
Glucagonomas secrete glucagon and have a high potential for malignancy. They lead to mild diabetes mellilus and a severe dermatitis involving portions of the lower half of the body. Although complete removal may not be possible, debtilking the tumor may help relieve symptoms. Streplozocin can help treat Ihe residual (umor.
Vipomas produce vasoactive intestinal polypeptide, pancreatic polypeptide, and perhaps other hormones. Recause the primary characteristics are watery diarrhea, hypokalemia, and achlorhydria, the disease is also known as the WDHA syndrome. Half of the tumors are malignant. As much tumor as possible should be resected, and the residual tumor should be treated with streplozocin.
Somatostatinomas are rare tumors that secrete somaloslatin. Clinical findings include diabetes, steatorrhea, and achlorhydria.
These clinical conditions can sometimes present as multiple endocrine neoplasia syndromes, in which tumors or hyperplasia occurs in two or more endocrine glands. Usually, these conditions are associated with tumors or hyperplasia of the parathyroid, pituitary, thyroid, or adrenal glands.

The only significant benign exocrine pancrealic neoplasm is cystade-nqma, which usually occurs in the body and tail of the pancreas in middle-aged and elderly women. Surgical resection may be needed for diagnosis and relief of symptoms from a large mass.
Pancrealic cancer is the second most common dl cancer in the USA with about 28,000 new cases diagnosed annually. The incidence increases with age and is 10 times greater in men > 75 yr than in the general population. Risk factors include cigarelle smoking, diabetes mellitus, and a diel high in animal fat and alcohol.
Ductal cell adenocarcinoma accounts for 75% to %% of all cancers arising from the pancreas. Others include giant cell carcinoma, adeno-squamous carcinoma, cysladenocarcinoma, and lymphoma. Giant cell carcinoma, also called carcinosarcoma, is a highly malignant lesion with distant metastases occurring early. Adenosquamous carcinoma occurs predominantly in men. more often in patienls wilh a history of radiation therapy. Cystadenocarcinoma, a low-grade malignancy, has the best prognosis because only 20% have metastasized by the time of surgery. Lymphoma of the pancreas accounts for 2% of all non-Hodgkin’s lymphoma and may be of B-cell or T-cell origin.
Symptoms and Signs
The clinical features of pancreatic cancer often depend on the location of the lesions; 80% occur in the head of Ihe pancreas, and 20% in Ihe body and tail. Patients with lesions of the pancreatic head often present with painless jaundice and acholic stools from common duct obstruction or with nausea and vomiting from gastric outlet obstruction. Itching may accompany jaundice. The onset of symptoms in those with lesions of the body and tail is more insidious, amounting to little more than weight loss and vague abdominal or back pain. Symptoms precede diagnosis by about 3 to 6 mo. In 90% of patients with ductal eel I adenocarcinoma, metastases are present at diagnosis, so death Often occurs within 6 mo.
Findings associated with pancreatic cancer include depression, thromboembolic phenomena associated wilh Trousseau’s syndrome, GI bleeding from gastric varices secondary to splenic vein thrombosis, polyarthritis, diarrhea caused by exocrine pancreatic insufficiency, superior vena cava syndrome caused by mediastinal metastases, and Homer’s syndrome caused by thoracic outlet metastasis. The onset of diabetes mellilus or a worsening of preexisting diabetes warrants an evaluation for pancreatic cancer.
Early in the disease the physical examination is negative. Later, an epigastric mass, supraclavicular lymphadenopathy, hepatomegaly from biliary stasis or metastasis, or a large, palpable gallbladder may be noted. Painless jaundice and a palpable gallbladder (Courvoisier’s sign) combined with acholic stools are diagnostic. Silver-colored stools may be noted in cancer of the ampulla of Vater because of the combination of mild bleeding and acholic stools.
Diagnosis
F.arly diagnosis when the tumor is still resectable is rarely possible. It is possible only wiih cancer of the pancreatic head associated with jaundice and with cancer of the ampulla of Vatcr. which may cause early i;iundiee. Diagnosis may be suggested by upper abdominal sonography, although a CT scan of the abdomen can better visualize a pancreaiu muss. In up to 90% of cases, endoscopic retrograde cholangiopancreatography can detect the tumor with the characteristic findings of dm till irregularity and cutoff. Although tissue for diagnosis can usually be obtained by needle biopsy, exploratory laparotomy is often necessary Serologic tumor markers including CEA, CA19-9, and galaetosyltransfeiase isoenzyme II (Gl-ll) may be elevated in some cases but are rarel> useful clinically.
Treatment
Patients who have nonmetastatic, resectable lesions in the pancreatic head may be candidates for pancreatoduodenectomy (Whipple’s operation). Only 10% of patients with ductal cell carcinoma have localized tumors. In most patients, the only procedure that can be performed is a palliative bypass (eg, cholecystojejunostomy for distal hile duct obstruction or gastrojejunostomy for gastric outlet obstruction); however, surgery is usually not warranted, and obstructive jaundice can be managed with an endoscopically placed stent. In older persons with nonrescctable tumors, forgoing all attempts at cure is often besl.
Chemotherapy produces little response and no long-term benefit in patients with adenocarcinoma. Radiation therapy offers minimal benefit, except for palliation of retroperitoneal pain. Palliative treatment wilh a biliary stent (placed endoscopically or radiological]y using transhepatic cholangiography) may reduce jaundice and itching.
Generally, abdominal pain is treated with analgesics and oral narcotics. However, a celiac axis nerve block maybe needed for severe, unrelenting pain. Pruritus from jaundice may be relieved wilh antihistamines or cholestyramine 4 gin orally I to 4 times daily. Pancreatic insufficiency can be managed with pancreatic enzymes (lipase, protease, and amylase).
The overall prognosis is dismal for adenocarcinoma; the I-yr survival rate is < 10%, and the 5-yr survival rate is only 2%. Cystadenocarcinoma. which has a low incidence of metastasis at diagnosis, has a 5-yr survival rate of 65% with aggressive surgery. Lymphoma also has a good prognosis.

Neoplasms of the pancreas include both exocrine and endocrine tumors.

The most common tumor of !he appendix is a carcinoid tumor, which is found in about 1 in 1000 resected appendixes. Rarely malignant, carcinoid tumors are treated by appendectomy. If Ihey are > 2 cm in diameter or show evidence of lymphatic or lymph node involvement, a right colectomy should be performed.
Primary adenocarcinoma of the appendix is rare, even though the appendix is lined with colonic mucosa. This tumor may block the lumen, leading to acute appendicitis and perforation.
Mucoceles produce appendiceal distention and outlet obstruction because of the intraluminal accumulation of mucus. The symptoms suggest early appendicitis. A mucocele may be demonstrated by CT scan. Although mucoceles are usually benign and treatable by appendectomy, some are associated with low-grade adenocarcinoma; if these mucoceles perforate, a form of carcinomatosis known as pseudomyxoma peritonei results. Palients who have this indolent tumor present with increased abdominal girth and a doughy abdomen. They can survive for several years, but repeated accumulation of intraperitoneal mucus or intestinal obstruction occurs. Palliative surgery often prolongs life and makes the patient more comfortable.

Cancer may develop in the perianal skin, the anal canal, or Ihe lower rectum. Epidermoid carcinoma accounts for 2% of colorectal cancers and 90% of anal cancers. The histologic types of anal carcinoma include squamous cell, basal cell, basaloid squamous, and cloacogenic carcinomas. Other malignant neoplasms include Bowen’s disease (intraepithelial squamous cell carcinoma in situ), ex-tramammary Paget’s disease, carcinoid tumor, and malignant melanoma. Cloacogenic carcinoma is most prevalent in patients ages 60 to 70. Factors predisposing persons to anorectal cancer include infections from human papillomavirus types 16 and 18 or human immunodeficiency virus, leukoplakia, lymphogranuloma venereum, chronic fistula formation, irradiation of the anal skin, and organ transplantation.
Symptoms, Signs, and Diagnosis
Bleeding is the mosl common symptom. Other frequent complaints are anal discomfort, constipation, and diminished stool caliber. The presenting feature may be a mass on digital rectal examination, inguinal adenopathy, or perianal dermatitis. Cancer should be considered wilh all nonhealing ulcers or fistulas. Biopsy of all suspicious lesions is essential.
Treatment
The treatment of choice is local surgical excision preceded by a course of radiation therapy and chemotherapy to debul k a large tumor mass. Such radiation therapy and chemotherapy may obviate abdominoperineal resection in many patients. Kulguration with electrocautery and laser photocoagulation are palliative measures in selected cases.