Notes by doctor. Claudio Italiano
A young patient, about 35 years old, with no history of pathology from the remote pathological anamnesis, comes to our first aid for intestinal obstruction. A very abdominal picture emerges: a multiple lymphonodes are evident in the omental apron; a sessile polypoid formation, which is exchanged by the radiologist for a lymph node, is entrained inside the intestinal lumen and causes intussusception of the intestine, which causes an alternated symptomatology between the intestinal occlusion and the free alve, open to feces and gas. Once the lymphonode has been performed, for laparoscopy, the diagnosis is made. It is an intestinal lymphoma and the patient is sent to the oncological hematology for the continuation of the specific treatment of the case. The photo is on the left side of the picture, where the villi are ordered and the cells arranged in a precise and functional way, while the space of the lumen is intricately between the villi is represented.
We speak of intestinal lymphoma, when lymphoid cells
are scattered throughout the lamina propria at all levels of the small intestine
and are also present in aggregates in the mucosa and submucosa, which are
particularly prominent in the distal ileum (Peyer's plates). Lymphoma can
originate in the small intestine. This primary intestinal lymphoma can be
localized to a single short intestinal segment with or without involvement of
adjacent mesenteric lymph nodes; it can be multifocal and nodular and affect
different intestinal segments; or it can spread most of the small intestine, as
is the case in our patient. At other times the process affects multiple organs.
It is predominantly non-Hodgkin's lymphoma and frequently of the histiocytic
type, usually located in the small intestine it represents 25% of all tumors of
the small intestine.It is frequently observed in patients with:
- He is celiac
-AIDS
- other states of immunodeficiency
The clinical manifestations of intestinal lymphoma vary depending on the primary
or secondary intestinal localization, or if the lymphoma is widespread or
localized.
The primary lymphoma of the small intestine is located, with the utmost frequency, at a single segment of the small intestine, although in 20 to 25 percent of patients the lesions are multiple. By definition, the liver, spleen, bone marrow and mediastinum should be exempt from a tumor evident at first, if the lymphoma is primary of the intestine. The ileum and fasting are affected with the utmost frequency, while the duodenal involvement is less common. As in secondary lymphoma, primary intestinal lymphocytic lymphomas, histiocytes, and histiocytic-lymphocyte misti are more frequent than primary intestinal Hodgkin's disease. In primary intestinal lymphoma, the neoplasm may arise in the lamina propria or in the lymphatic follicles; it can partially destroy the epithelium causing ulceration.
Isolated or multifocal polypoid tumor masses may develop; rarely, these can obstruct the intestinal lumen if they are large, or induce intussusception. Alternatively, the entire circumference of the intestinal wall can be infiltrated with involvement of all the layers of the wall and aneurysmal dilatation or an annular stenosing lesion can be established. The involvement of the adjacent mesentery and its lymph nodes is frequent. In addition to ulceration, perforation and intussusception in the tumor region can complicate the course. In a syndrome that has distinctive clinical features, including malabsorption, long segments of the small intestine are widely affected by the lymphomatous process. Mucosal involvement is extensive and mesenteric lymph nodes may be enlarged and contain tumor elements, but a gross involvement of other intra-abdominal organs such as the liver and spleen is not evident, although microscopic tumor outbreaks can be seen in any organ at all. 'autopsy.
The mucosa of the affected intestine is often flat, the crypts are elongated and the normal architecture of the villi is lost. Although the architectural appearance of the mucosa may superficially resemble that of the celiac sprue, there are usually differential traits. In diffuse lymphoma, unlike celiac sprue, Lieberkuhn crypts are usually reduced in number. The lamina propria can be infiltrated by a massive number of round cells, some of which appear to be malign according to cytological criteria.The architecture of the lymphatic follicles is usually destroyed and there are no well-defined germinal centers. The presence of primitive lattice cells isolated in the lamina propria is particularly useful, because these cellular elements are normally confined to the germinal centers of the lymphatic follicles. The secondary involvement in Hodgkin's disease is found in only about 5 percent of the cases at the autopsy. Unlike primary intestinal lymphoma, intestinal involvement in patients with disseminated lymphoma is often prominent in the collateral and specific bowel symptoms are often absent from life. In many patients with secondary lymphoma of the small intestine other intra-abdominal viscera, such as the stomach, colon, liver and pancreas are also invaded by the tumor.
The lymphoma of the small intestine is a neoplasm of hematoncologic relevance
and its cause remains unknown, like that of other human neoplasms. It is of
great interest that a high incidence of lymphoma (both intestinal and
extraintestinal) has been described in patients with celiac sprue and that young
adult Arabs and Jews native to the Middle East have a high incidence of primary
diffuse lymphoma of the small intestine . The possible frequent occurrence of
this neoplasm in association with a specific morbid entity and within a
geographically and ethnically defined population may provide clues that may
eventually help to clarify its etiology and pathogenesis.
Primary lymphoma with focal intestinal involvement - Primary lymphoma of the small intestine can develop at any age. In general, the population of affected patients is younger than those affected by other types of gastrointestinal neoplasms. Indeed, about a third of children with malignant lymphoma first appear with symptoms that suggest abdominal involvement. Mostly, the signs and symptoms of primary lymphoma of the small intestine can be attributed to a specific tumor mass. Abdominal pain is common. It is usually crampiform, may be associated with nausea and vomiting and may result in partial obstruction of the intestinal lumen by the tumor mass. Although complete obstruction may occur, it is less common than in patients with other forms of intestinal malignancy, such as carcinomas. Periodic obstruction symptoms may be due to intermittent intussuectivation induced by tumor mass. The abrupt appearance of severe, continuous abdominal pain can signal the perforation of the friable intestinal wall, filled by the tumor mass, which represents an occasional complication. A massive intestinal hemorrhage from an ulcerated tumor mass may sometimes be the onset symptom, but the insidious establishment of anemia caused by occult intestinal bleeding is much more frequent. Non-specific general symptoms such as anorexia, malaise and weight loss may precede localization symptoms. Fever, when persistent and elevated suggests, or extensive lymphomatous involvement, or the presence of complications such as perforation or infection. The most common objective findings are abdominal pain and a palpable abdominal mass. The presence of hepato-splenomegaly and relevant peripheral lymphadenopathy suggests an advanced disseminated disease. Often these organs are not palpable in the primary lymphoma of the small intestine. Ascites when present suggest an extensive mesenteric and / or retroperitoneal tumoral invasion. Complications are reflected in expected findings such as abdominal distension and high-intestinal intestinal noises in intestinal obstruction and intense abdominal pain, contraction and rebound pain in patients with free intestinal perforation. Haematological and chemical routine data are substantially non-specific and reflect complications.Severe anemia and positive guaiac stools indicate intestinal bleeding; a leukocytosis can reflect a complicating infection; and an electrolyte imbalance with hypokalemia and hypochloraemia may be due to pernicious vomiting caused by intestinal obstruction. The barium contrast study of the small intestine is useful for advancing suspicion and for defining the location and extent of primary intestinal lymphoma. Contrast examinations may show infiltration of the intestinal wall with thickening or obliteration of the mucosal folds or, less commonly, a segmental narrowing of the affected intestinal tract. Large masses within the intestinal wall can go into necrosis and droop and their central nucleus can be eliminated in the intestinal lumen. As a consequence of this aneurysmal dilatation of the intestine can be instituted. Otherwise, the tumor may appear as one or more polypoid masses of appreciable size projecting into the lumen. Such polypoid lesions may be associated with intussusception especially in children.