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Answer
Diffuse, large B-cell lymphoma (DLBCL): Needle
biopsy of the RLQ mass revealed a DLBCL involving
the intestinal wall. Bone marrow aspiration
revealed normocellular marrow negative for
lymphoma. After diagnosis, the patient received a
round of CHOP chemotherapy, which consists of
vincristine, doxorubicin, prednisone, and
cyclophosphamide (nitrogen mustard), along with
dexamethasone (Decadron) and granulocyte colony-stimulating factor (G-CSF).
Prophylactic surgery to remove the RLQ mass was
scheduled because of the relatively high risk of
ileocecal bowel perforation during and after
chemotherapy because of the lymphoma's extensive
infiltration of the entire bowel wall, as seen on
CT. The preoperative diagnosis was non-Hodgkin
lymphoma (NHL), ie, DLBCL of the small bowel.
After surgery, lymphoma to the terminal ileum,
right colon, and mesentery of the small bowel
were confirmed. The terminal ileum and proximal
right colon were resected, and an ileocolic anastomosis was made.
Lymphomas are categorized as Hodgkin lymphoma or
NHL. Hodgkin lymphomas are most often localized
to a single axial group of nodes, they spread
contiguously, and they rarely cause extranodal
involvement. In contrast, NHL most frequently
involves several peripheral nodes, they have
noncontiguous or disseminated spread, and they
commonly result in extranodal involvement. NHL
can be further categorized into B-cell and T-cell
lymphomas. DLBCL is a malignancy of mature
B-cells originating from the germinal center or
marginal-zone B cells. It is the most common
histologic subtype of B-cell NHL, accounting for
20% of all NHLs and 60-70% of aggressive lymphoid
neoplasms. On histologic evaluation,
DLBCL-involved lymph nodes show a diffuse pattern
of involvement with loss of normal structures,
such as sinuses and lymphoid follicles.
The median age when DLBCL occurs is in the 60s,
and the patient often presents with a rapidly
enlarging, symptomatic mass, typically in the
neck or abdomen. As many as 40% of patients
present with extranodal involvement. The ileum is
the most common site of extranodal lymphoma,
which accounts for 5% of all lymphomas. As in
this case, mass effect can lead to small SBO.
Depending on the extranodal location of the
lymphoma, other presentations due to mass effect
include superior vena cava (SVC) syndrome,
tracheobronchial compression leading to
respiratory distress, and spinal-cord compression
related to destruction of bone in the vertebral
column. Detection of tumor in the bone marrow is
associated with spread to the CNS in 10-20% of patients.
Constitutional symptoms include fever, weight
loss, and drenching night sweats and occur in 30%
of patients. More than 50% have elevated serum
LDH levels. The International Non-Hodgkin's
Lymphoma Prognostic Factors Project reports a
5-year survival rate of 26-73%; the exact rate
depends on the number of risk factors and the
histologic type. Risk factors for increased
mortality and relapse include age older than 60
years, increased serum LDH level, Ann Arbor stage
III or IV, and more than 1 extranodal disease
site. The mean long-term disease-free survival
rate is about 40%. Relapse is most common in the
first 2-3 years after diagnosis, with relapse
relatively uncommon after 4 years.
For more information on DLBCL, see the eMedicine
articles
<http://knowledge.emedicine.com//splash/shared/pub/xrotw/%5Ehttp://www.emedicine.com/med/topic1358.htm%5Etarget=%5E_blank%5E>Lymphoma,
B-Cell and
<http://knowledge.emedicine.com//splash/shared/pub/xrotw/%5Ehttp://www.emedicine.com/med/topic1360.htm%5Etarget=%5E_blank%5E>Lymphoma,
Diffuse Large Cell (within the Internal Medicine specialty).
References
* Freeman HJ. Free perforation due to
intestinal lymphoma in biopsy-defined or
suspected celiac disease. J Clin Gastroenterol 2003 Oct;37(4):299-302.
* Gajra A. Lymphoma, B-Cell. eMedicine
Journal [serial online]. November 3, 2005.
Available at:
<http://knowledge.emedicine.com//splash/shared/pub/xrotw/%5Ehttp://www.emedicine.com/med/topic1358.htm%5Etarget=%5E_blank%5E>http://www.emedicine.com/med/topic1358.htm.
* Law M, Williams S, Wong J. Role of surgery
in the management of primary lymphoma of the
gastrointestinal tract. J Surg Oncol 1996;61:199-204.
* Randall J, Obeid ML, Blackledge GR.
Haemorrhage and perforation of gastrointestinal
neoplasms during chemotherapy. Ann R Coll Surg Engl 1986 Sep;68(5):286-9.
* ReMine SG, Braasch JW. Gastric and small
bowel lymphoma. Surg Clin North Am 1986 Aug;66(4):713-22.
* Sakakura C, Hagiwara A, Nakanishis M, et
al. Bowel perforation during chemotherapy for
non-Hodgkin's lymphoma. Hepatogastroenterology 1999 Nov-Dec;46(30):3175-7.
* Wada M, Onda M, Tokunaga A, et al.
Spontaneous gastrointestinal perforation in
patients with lymphoma receiving chemotherapy and
steroids. J Nippon Med Sch 1999;66(1):37-40.
BACKGROUND
A 57-year-old man presents to a local emergency
department with severe abdominal pain after being
evacuated from a cruise ship. The pain, which is
most severe in the right lower portion of his
abdomen, started soon after he boarded the ship 2
days ago. Since then, the pain has worsened, and
the patient has noticed his abdomen becoming
progressively "bloated." The pain is associated
with nausea and vomiting. He has not been able to
have a bowel movement. On further questioning,
the patient reports having night sweats,
low-grade fevers, intermittent abdominal
discomfort with constipation, and a 30-lb weight
loss over the last 2-3 months. He has no
significant medical history and is not taking any
medications. He does have a significant family
history of colon cancer, soft tissue sarcoma,
pancreatic cancer, chronic myeloid leukemia (CML), and prostate cancer.
On physical examination, the patient is alert and
oriented. His temperature is 98.8°F, his pulse is
65 beats per minute, his respiratory rate is 18
breaths per minute, and his blood pressure is
104/67 mm Hg. Abdominal examination reveals
localized tenderness to palpation in the right
lower quadrant (RLQ), with a palpable mass. He
has generalized abdominal distension but no
guarding, rebound, or percussion tenderness.
Rectal examination reveals guaiac-positive, brown
stool. Findings from the respiratory and
neurologic portions of the physical examination are unremarkable.
Laboratory investigations are ordered and reveal
a hemoglobin value of 9.4 g/dL, with a
corresponding hematocrit of 30.8%. His WBC count
is 6.2 X 109/L, and his lactate dehydrogenase
(LDH) level is elevated at 285 U/L. Results of an
electrolyte panel, liver function tests, and
renal function tests are within normal limits.
Abdominal CT is performed, which demonstrates a
large right lower quadrant mass (see Image). The
mass causes a small-bowel obstruction, and
several enlarged retroperitoneal and mesenteric
nodes are noted (not pictured). What is the
likely etiology of this mass, and what is its treatment?
Hint
Note the patient's strong family history of cancer.
Author: Anusuya Mokashi, Medical Student, New York Medical College, Valhalla
Janis A. Pastena, MD, FACS, FACEP, Associate
Professor of Clinical Surgery, New York Medical College
eMedicine Editor: Eugene Lin, MD, Department of
Radiology, Virginia Mason Medical Center,
Seattle, WA, Assistant Clinical Professor of
Radiology University of Washington Medical Center, Seattle, WA
Rick G. Kulkarni, MD, Assistant Professor, Yale
School of Medicine, Section of Emergency
Medicine, Department of Surgery, Attending
Physician, Medical Director, Department of
Emergency Services, Yale-New Haven Hospital
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