Chapter 34 (page 175)

The Pyloric Sphincteric Cylinder in Health and Disease

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Chapter 34 (page 175)

Zornoza and Dodd (l980) pointed out that the gross appearance and microscopic characteristics of the lesion were identical in the primary gastric and disseminated forms. The only differences between them were the distribution of the disease and the potential for future spread. Disseminated lymphoma was a lethal process that ran a rapid course. Although moderate stiffening of the gastric walls might occur in the intraluminal fungating form, peristaltic waves were not completely absent. The diffusely enlarged and distorted gastric rugae appeared to be more or less fixed.

Craig and Gregson (l98l) reiterated that one of the findings strongly suggesting the diagnosis of lymphoma was mucosal involvement extending across the pylorus into the duodenum. The US National Cancer Institute (l982), in a retrospective study of over 1,000 cases, published a revised and modified classification of non-Hodgkin's lymphoma based on six previous classifications, including that of Rappaport (l966). This has become known as the "Working Formulation for non-Hodgkin's Lymphomas". It is based primarily on clinical correlations, especially survival curves, age, sex, presenting sites and stage of the disease. Other recent classifications have been based, in part, upon modern concepts of the immune system and lymphoid physiology.

According to Sandler (l984), whose views differ from those of Ming (l973), primary gastric lymphomas arise from lymphoid tissue in the lamina propria and extend laterally along the submucosal layer. As the mucosa is not involved in the first place, endoscopy and endoscopic biopsy are unreliable diagnostic modalities. The muscular layer is generally spared till a late stage of the disease. The diffuse infiltration by mature and immature lymphoid cells and histiocytic cells may result in large, rigid folds or the appearance of linitis plastica. The lesions may also be of a polypoid or fungating nature. They do not constrict the lumen or interfere with peristalsis, and pyloric obstruction is unusual.

Because the tumor is predominantly submucosal, the diagnosis of gastric lymphoma by endoscopic biopsy can be difficult, according to Fork et al. (l985); in one report a success rate of only 44 percent was achieved.

Only a few cases of malignant gastric lymphomatous disease have been encountered in our department in many thousands of upper gastrointestinal barium investigations during the past 3 to 4 years. The following are two of the cases:

Case Reports

Case 34.1 G.C., l7 year old male, had a two year history of epigastric pain, vomiting, weight loss and retardation of growth. Physical examination revealed severe iron deficiency anaemia. Radiographic study showed multiple lobulated filling defects in the corpus and sinus of the stomach, constant irregularity of the greater and lesser curvatures and a narrowing at the commencement of the pyloric sphincteric cylinder, in the region of the left pyloric loop (Fig. 34.1). The cylinder was partially contracted throughout the examination, never contracting or relaxing maximally; this was associated with a patent pyloric aperture measuring 4.0 mm in diameter. Gastric emptying of fluid barium was delayed. Endoscopy showed diffuse, nodular infiltration of the entire corpus and "antrum", with contact bleeding. The infiltration surrounded the pyloric orifice, which was patent. The duodenum could not be visualized. Histology, according to the "Working Formulation", revealed a high grade, malignant, non-Hodgkin lymphoma. Bone marrow biopsy was normal.

Fig. 34.1. Case G.C. Irregularities of lesser and greater curvatures extending to commencement of pyloric sphincteric cylinder. Cylinder partially contracted (arrows). Pyloric aperture patent

At operation the entire stomach from the gastro-oesophageal junction to the pylorus was found to be involved by lymphomatous infiltration, and a total gastrectomy with an oesophago-jejunal anastomosis was performed. Macroscopically the resection specimen showed diffuse thickening of the walls with effacement of the normal mucosal pattern. Microscopically tumor cells extended from the mucosa into the muscular layer and in several areas as far as the serosa. Electron microscopically the cells were determined to belong to the lymphoma group, the condition being diagnosed by a combination of light microscopy, electron microscopy and immunocytology as a large cell (B-cell), immunoblastic lymphoma. The distal border of the resection specimen was free of tumor cells and liver biopsy was normal. A few enlarged mesenteric lymph nodes proved to be of reactive type.

Case 34.2 J.J., 43 year old male, presented with intermittent epigastric pain, vomiting, loss of appetite and loss of weight, of one year's duration. Physical examination revealed epigastric tenderness. Radiographic study showed a constant irregularity of the lower part of the lesser curvature, with a permanent, ulcer-like projection; a large, lobulated filling defect was present in the duodenal bulb (Fig. 34.2). The pyloric sphincteric cylinder remained expanded throughout the examination, failing to contract, and the pyloric orifice remained patent. The appearances were ascribed to a mass lesion, probably with ulceration, infiltrating the distal lesser curvature of the stomach and the first part of the duodenum. At endoscopy a deformity of the gastric "antrum", presumably due to external compression was seen, while the pyloric ring appeared normal. A large polypoid, lobulated mass measuring approximately 4.0 cm in diameter, was present in the duodenal bulb; the surrounding duodenal mucosa appeared normal. The biopsy specimen was inadequate but the possibility of malignant lymphoma was mentioned. Abdominal sonography showed no abnormality in the pancreas, liver, gall bladder and kidneys. Chest radiographs and bone marrow trephine biopsies were normal.

Fig. 34.2 Case J.J. Irregularity lower lesser curvature with ulcer-like projection. Pyloric sphincteric cylinder expanded. Pyloric aperture patent (arrow). Large, lobulated filling defect in duodenal bulb