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Lymphomatoid granulomatosis is a lymphoproliferative process affecting multiple organ systems usually including the lungs. Significant gastrointestinal involvement, however, has rarely been reported.
Pathologic examination reveals a vasocentric polymorphous lymphoid infiltrate. A case of lymphomatoid granulomatosis with gastrointestinal manifestations necessitating aggressive surgical intervention
is reported. The clinical presentation, pathologic features, and various aspects of therapy of lymphomatoid granulomatosis involving the gastrointestinal tract are discussed.
Cancer 51:694-700. 1983.
was originally
described by Liebow et al.' as an angiocentric granulomatous process, usually in the lung, characterized by
a polymorphous lymphoid infiltrate with histopathologic similarity to certain lymphomas and the potential
for evolution into lymphoma. Extrapulmonary disease
was noted in 83% of cases reported by Saldana et al.'
This may involve the central and peripheral nervous
systems, skin, kidneys, liver, spleen, adrenals, and
heart. Gastrointestinal tract involvement, however, is
r a ~ - e ' .particularly
~-~
in autopsied cases. Information regarding the pathologic and clinical features of this unusual complication of lymphomatoid granulomatosis is,
therefore, minimal. A patient with lymphomatoid granulomatosis involving multiple systems, including skin,
lungs, and central nervous system is reported. His course
was marked by life-threatening lower intestinal hemorrhage secondary to lymphomatoid granulomatosis involving the colon and small intestine. The clinical presentation, the pathologic features, and an evaluation of
the various modalities of therapy, including aggressive
surgical procedures, are discussed.
YMPHOMATOID GRANULOMATOSIS
Case Report
A 44-year-old white man was admitted to Vanderbilt University Hospital for evaluation of a persistently tender right
lower quadrant mass. He was well until November of 1977
From the Departments of Medicine, Pathology and Surgery Vanderbilt University Hospital, Nashville, Tennessee.
Address for reprints: Mark D. Rattinger, MD,235 Seminole Avenue, Palm Beach, FL 33480.
Accepted for publication December 7, I98 I .
694
No. 4
GASTROINTESTINAL
LYMPHOMATOID GRANULOMATOSIS
Rattinger
121.
695
Pathologic Features
Sections Of skin and subcutaneous tissue ( 1977)
showed a well circumscribed inflammatory infiltrate ex-
696
CANCERFebruary 15 1983
Vol. 5 1
No. 4
GASTROINTESTINAL
LYMPHOMATOID
GRANULOMATOSISRattinger et a/.
The clinical presentation of lymphomatoid granulomatosis depends on the organ systems involved. Symptoms may be nonspecific such as weight loss, fever, and
malaise, or may be manifestations of specific organ sysA common feature is the eventual
tem inv~lvement.'.~,~
FIG.6. Colonic mass. Tumor involves submucosa and muscle (top) in some areas, but
predominantly involves pericolonic adipose
tissue.
697
698
CANCERFebruary 15 1983
VOl. 5 1
FIG. 7. Small intestine showing dense inflammatory infiltrate in submucosa and muscularis propria.
lymphomatoid granulomatosis. no deaths from gastrointestinal disease were noted. In this later series necrotizing
lesions of small intestine and gallbladder were seen in
four patients during life, and gastrointestinal tract disease was noted in 7% of autopsies.3 In the same series
No. 4
GASTROINTESTINAL
LYMPHOMATOID
GRANULOMATOSIS Ratfinger d al.
699
addition of chemotherapy; corticosteroids and chemotherapy; chemotherapy, alone or with later addition of
corticosteroids; and antibiotics or no therapy. They
found no significant difference in mortality among the
groups with from 24 to 3 1% of patients remaining disease-free at the time of the study (Group I, 24%; Group
I1 24%; Group 111, 3 1%; Group IV, 27%).
Although gastrointestinal involvement has been rarely
seen in lymphomatoid granulomatosis, all of the other
clinical and pathologic features in our case are similar
to the original description. More importantly, the vasocentric lymphoproliferative lesions were typical and
were noted at various times in this patient in skin, lung,
and gastrointestinal tract.
Because of multiple organ system involvement surgery has had only a limited role in treatment of lymphomatoid granulomatosis. However, due to the unusual gastrointestinal lesions aggressive abdominal resection was necessary in our patient. Although he had
recurrence in other organ systems, his gstrointestinal and
nutritional complaints were significantly ameliorated by
surgery. Perforation of the bowel may apparently occur
as a complication of lymphomatoid granulomatosis, and
surgery may be necessary.
Conclusion
CANCERFebruary 15 1983
700
has been consistently useful in this disease, and remission is not unusual. The reported case has been instructional because of the widespread nature of the disease,
the life-threatening aspects, and the apparent response
to a combination of surgical and medical therapy.
REFERENCES
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