28. Ulcerogenous pylorostenosis, Malignant liver tumors & Mediastinal tumors

What is the definition of ULCEROGENOUS PYLOROSTENOSIS?

Ulcerogenous pylorostenosis involves the thickening of the pylorus muscles, blocking food from entering the small intestine. It is a form of Gastric Outlet Obstruction (GOO) caused by cycles of inflammation and repair, leading to scarring, muscle spasm, and oedema at the gastroduodenal junction.

What is the etiology of ULCEROGENOUS PYLOROSTENOSIS?

The most common cause is malignancy, followed by peptic ulcer disease, especially duodenal ulcers. Acute peptic ulcer disease causes obstruction through inflammation and oedema, while chronic disease leads to obstruction via scarring and fibrosis.

What is the epidemiology of ULCEROGENOUS PYLOROSTENOSIS?

Peptic ulcer disease currently accounts for less than 5% of all gastric outlet obstruction cases. It is more commonly associated with duodenal ulcers than gastric ulcers.

What is the clinical presentation of ULCEROGENOUS PYLOROSTENOSIS?

Patients typically present with a history of peptic ulcers and experience gradually increasing abdominal pain over weeks or months. Other signs include anorexia, vomiting, failure of antacids to provide relief, and upper gastrointestinal tenderness.

What are the symptoms of ULCEROGENOUS PYLOROSTENOSIS?

Key symptoms include increasing ulcer pain, anorexia, and vomiting. Patients may also experience weight loss, and the vomitus often contains food ingested hours earlier but lacks bile staining.

What are the diagnostic methods (labs, imaging, physical examination) of ULCEROGENOUS PYLOROSTENOSIS and their findings?

Laboratory findings may include anemia, hypokalemic hypochloremic metabolic alkalosis, hypochloremia, hyponatremia, and increased bicarbonate. Imaging like X-rays may show a large gastric fluid level, and endoscopy is used to rule out neoplasm. Physical examination can reveal upper GI tenderness, signs of dehydration and malnutrition, and a succussion splash from retained gastric contents.

What is the pharmacological treatment of ULCEROGENOUS PYLOROSTENOSIS?

Proton pump inhibitors (PPIs) are indicated for treatment. Additionally, total parenteral nutrition should be started if chronic obstruction has caused severe malnutrition.

What is the surgical treatment/management of ULCEROGENOUS PYLOROSTENOSIS?

Surgical intervention is indicated if 5-7 days of gastric aspiration do not improve the obstruction. Treatment commonly consists of a truncal or parietal vagotomy with a drainage procedure, such as gastrojejunostomy.

What is the definition of MALIGNANT LIVER TUMORS?

Malignant liver tumors can be either primary, originating in the liver, or metastatic, meaning they have spread to the liver from other organs like the gastrointestinal tract, breast, or bronchus. Metastatic liver tumors are generally more common than primary ones.

What is the classification of MALIGNANT LIVER TUMORS and their features?

Malignant liver tumors are classified as primary or metastatic. Primary types include Hepatocellular carcinoma (HCC), the most common primary liver malignancy arising from hepatocytes, which can appear as single or multiple nodules. Another primary type is Cholangiocarcinoma, an adenocarcinoma arising from the biliary tree, which can be extrahepatic or intrahepatic and has a very poor prognosis. Other rare primary types include Angiosarcoma, which originates from endothelial cells, and Hepatoblastoma, an embryonal tumor most common in young childhood. Metastatic neoplasms, which are more common, particularly spread from the gastrointestinal tract, breast, and lungs to the liver.

What is the definition of HEPATOCELLULAR CARCINOMA?

Hepatocellular carcinoma is a malignant tumour frequently associated with chronic liver disease like cirrhosis. It is the most common primary liver malignancy, arising from the hepatocytes.

What is the epidemiology of HEPATOCELLULAR CARCINOMA?

It is more common in males (M>F) and is typically diagnosed in the elderly.

What is the etiology of HEPATOCELLULAR CARCINOMA?

Its etiology includes viral hepatitis (HBV, HCV) and liver cirrhosis from causes like fatty liver or alcohol. Hemochromatosis, steroids, and oral contraceptive pills (OCP) can also contribute.

What are the risk factors of HEPATOCELLULAR CARCINOMA?

Risk factors include cirrhosis, Hepatitis B and C, alcohol, aflatoxins, and chemicals such as Thototrast and vinyl chloride. Liver flukes, schistosomiasis, hemochromatosis, Wilson’s disease, and α1-antitrypsin deficiency are also relevant risk factors.

What is the clinical presentation of HEPATOCELLULAR CARCINOMA?

Patients may present with weight loss, anorexia, right upper quadrant (RUQ) pain, ascites, and fever with jaundice. Hepatomegaly is also a common clinical sign.

What are the symptoms of HEPATOCELLULAR CARCINOMA?

Symptoms include weight loss, anorexia, fatigue, nausea, vomiting, and dull RUQ or epigastrium pain. Signs of chronic liver disease such as jaundice, hepatomegaly, splenomegaly, and ascites may also be present.

What are the diagnostic methods (labs, imaging, physical examination) of HEPATOCELLULAR CARCINOMA and their findings?

Laboratory findings may include raised alpha-fetoprotein (AFP), increased AST, ALT, ALP, and GGT. Imaging methods like USG, CT, and MRI are used, showing hypervascularity and possible metastasis. Needle biopsy provides a definitive diagnosis.

What is the pharmacological treatment of HEPATOCELLULAR CARCINOMA?

Anti-angiogenic drugs such as sorafenib are used for non-resectable cases. Other non-surgical treatments for unresectable tumours include transcatheter arterial chemoembolization (TACE), radiofrequency ablation, or selective internal radiation therapy.

What is the surgical treatment/management of HEPATOCELLULAR CARCINOMA?

Surgical resection, which includes hepatectomy, lobectomy, or segmental resection, is a treatment option. Liver transplantation is possible for small tumours (less than 3 nodules, each under 3 cm; or 1 nodule under 5 cm) without metastasis.

What is the definition of CHOLANGIOCARCINOMA?

Cholangiocarcinoma is an adenocarcinoma that arises from the biliary tree, which can be extrahepatic or intrahepatic bile ducts. Most are adenocarcinomas.

What is the epidemiology of CHOLANGIOCARCINOMA?

The mean age of diagnosis for cholangiocarcinoma is in the seventh decade of life.

What is the etiology of CHOLANGIOCARCINOMA?

Its etiology includes primary sclerosing cholangitis (autoimmune), liver parasites (common in Asia), chronic inflammation (cholangitis), choledochal cysts, Caroli's disease, and choledocholithiasis.

What are the risk factors of CHOLANGIOCARCINOMA?

Key risk factors include primary sclerosing cholangitis (PSC), ulcerative colitis (UC), and choledochal cysts.

What is the clinical presentation of CHOLANGIOCARCINOMA?

Clinical features include obstructive jaundice, which may manifest as dark urine, clay-colored stools, and pruritus. Right upper quadrant (RUQ) pain and weight loss are also commonly observed.

What are the symptoms of CHOLANGIOCARCINOMA?

Symptoms include jaundice, often accompanied by pruritus, and weight loss.

What are the diagnostic methods (labs, imaging, physical examination) of CHOLANGIOCARCINOMA and their findings?

Diagnosis involves biopsy, ERCP, USG, and MRCP. Cholangiography, including PTC or ERCP, and tumour markers such as CA19-9 and CEA are also used. ALP may also be elevated.

What is the pharmacological treatment of CHOLANGIOCARCINOMA?

Chemotherapy, specifically cisplatin, is a treatment option for cholangiocarcinoma. Stent placement during PTC or ERCP may be used to relieve biliary obstruction.

What is the surgical treatment/management of CHOLANGIOCARCINOMA?

Resection is a surgical treatment option. Adjuvant radiotherapy or brachytherapy can also be applied. Most patients do not have resectable tumours at the time of diagnosis.

What is the definition of ANGIOSARCOMA?

Angiosarcoma is an extremely rare type of liver cancer that originates from endothelial cells.

What is the epidemiology of ANGIOSARCOMA?

It is characterised by a very long latency period between exposure and disease development.

What is the etiology of ANGIOSARCOMA?

This tumour occurs due to exposure to specific environmental toxins such as vinyl chloride (PVC) and arsenic.

What are the risk factors of ANGIOSARCOMA?

The primary risk factors are exposure to vinyl chloride and arsenic.

What is the clinical presentation of ANGIOSARCOMA?

There is no information about the clinical presentation of Angiosarcoma in the provided sources.

What are the symptoms of ANGIOSARCOMA?

There is no information about the symptoms of Angiosarcoma in the provided sources.

What are the diagnostic methods (labs, imaging, physical examination) of ANGIOSARCOMA and their findings?

There is no information about the diagnostic methods or their findings for Angiosarcoma in the provided sources.

What is the conservative treatment of ANGIOSARCOMA?

There is no information about the conservative treatment of Angiosarcoma in the provided sources.

What is the pharmacological treatment of ANGIOSARCOMA?

There is no information about the pharmacological treatment of Angiosarcoma in the provided sources.

What is the surgical treatment/management of ANGIOSARCOMA?

There is no information about the surgical treatment or management of Angiosarcoma in the provided sources.

What is the definition of HEPATOBLASTOMA?

Hepatoblastoma is an embryonal and rare tumour of the liver.

What is the epidemiology of HEPATOBLASTOMA?

It is the most common liver tumour found in young childhood, typically diagnosed around 15 months of age.

What is the clinical presentation of HEPATOBLASTOMA?

It is characterised by an elevation of alpha-fetoprotein.

What are the diagnostic methods (labs, imaging, physical examination) of HEPATOBLASTOMA and their findings?

Diagnosis is characterised by an elevation of alpha-fetoprotein.

What is the pharmacological treatment of HEPATOBLASTOMA?

Neoadjuvant chemotherapy is part of the treatment regimen.

What is the surgical treatment/management of HEPATOBLASTOMA?

Resection is a treatment option for hepatoblastoma.

What is the definition of METASTATIC NEOPLASMS?

Metastatic neoplasms in the liver are tumours that have spread to the liver from other primary sites, particularly from the gastrointestinal tract, breast, and bronchus. These are generally more common than primary liver malignancies.

What is the epidemiology of METASTATIC NEOPLASMS?

Metastatic liver neoplasms are more common than primary liver malignancies.

What is the clinical presentation of METASTATIC NEOPLASMS?

Patients are often asymptomatic until the disease becomes advanced and the liver begins to fail.

What are the symptoms of METASTATIC NEOPLASMS?

Symptoms are typically absent until the disease is advanced and the liver starts to fail.

What are the diagnostic methods (labs, imaging, physical examination) of METASTATIC NEOPLASMS and their findings?

Diagnosis involves detecting increased levels of ALP, GGT, LDH, AST, and ALT in laboratory tests. Imaging methods include contrast CT and USG, and physical palpation is considered the best diagnostic method.

What is the surgical treatment/management of METASTATIC NEOPLASMS?

Treatment options include surgical resection, if feasible, and radiofrequency ablation.

What is the definition of mediastinum?

The mediastinum is the central space of the thoracic cavity located behind the sternum and between the two lungs and their respective pleura. It is bound by the thoracic inlet, diaphragm, sternum, vertebral bodies, and pleura.

What are the compartments of mediastinum?

The mediastinum is divided into three compartments: anterior, middle, and posterior. The anterior compartment extends from the sternum to the anterior border of the pericardium.

What is the etiology of MEDIASTINAL TUMORS in each compartment?

In the anterior compartment, common etiologies include thymoma, lymphoma, lipoma, pericardial cysts, and germ cell tumours like teratoma. The middle compartment can involve pericardial cysts, bronchogenic cysts or tumors, lymphoma, lymph node enlargement, and aortic aneurysms. In the posterior compartment, neurogenic tumors, enteric cysts, lymphomas, esophageal tumors, and diaphragmatic hernias are common.

What is the clinical manifestation (mass + systemic effects) of MEDIASTINAL TUMORS?

Approximately 50% of mediastinal tumors are asymptomatic, typically when they are benign. When symptomatic, about 50% are malignant and can cause mass effects such as cough, dyspnea, hoarseness, or superior vena cava syndrome. Systemic effects include paraneoplastic syndromes like myasthenia gravis with thymomas, or fever, night sweats, and weight loss in cases of lymphoma.

What are the symptoms of MEDIASTINAL TUMORS?

Symptoms include chest pain, cough, dyspnea, recurrent respiratory infections, and hoarseness. Other symptoms are dysphagia, Horner syndrome, and facial or upper extremity edema due to SVC compression.

What are the diagnostic methods (labs, imaging, physical examination) of MEDIASTINAL TUMORS and their findings?

Diagnosis involves history and physical examination, laboratory studies, and imaging. Lab findings include elevated alpha-fetoprotein in malignant germ cell tumors and beta-hCG in seminoma or nonseminomatous germ cell tumors. Imaging uses CXR, CT with contrast for location and characteristics, MRI for distinguishing compression versus invasion, and USG for structures near the heart. A definitive diagnosis generally requires a tissue sample obtained via biopsy, such as mediastinoscopy or percutaneous needle aspiration.

What are the treatment methods of MEDIASTINAL TUMORS?

Most isolated benign masses should be excised, sometimes via minimally invasive video-assisted procedures for localized tumors like bronchogenic cysts. For masses likely to be lymphoma, germ cell tumors, or unresectable malignancies, a diagnostic biopsy is preferred over a major operation. Post-operative radiotherapy or chemotherapy is used if the tumor is malignant.

What is the pharmacological treatment of MEDIASTINAL TUMORS?

Pharmacological treatment includes chemotherapy which is administered post-operatively if the tumor is malignant.

What is the surgical treatment/management of MEDIASTINAL TUMORS?

Surgical management involves the excision of most isolated benign masses, or needle aspiration for suspected benign cystic lesions. Resection can be performed via minimally invasive video-assisted procedures for localized tumors, with exploration via sternotomy or thoracotomy used when necessary. A diagnostic biopsy is often preferred over major surgery if the mass is likely to be lymphoma, a germ cell tumor, or an unresectable malignancy.