LETTER TO THE EDITOR

Asymptomatic gastrosplenic fistula in a patient with marginalzonal lymphoma transformed to diffuse large B celllymphoma—a case report and review of literature

Jayastu Senapati & Anup J. Devasia & Sniya Sudhakar &

Auro Viswabandya

Received: 20 November 2013 /Accepted: 2 December 2013# Springer-Verlag Berlin Heidelberg 2013

Dear Editor,Gastrosplenic fistula (GSF) is a rare and potentially fatalcomplication of lymphoma which can occur both spontane-ously and after chemotherapy [1–4]. Here, we report a patientwith diffuse large B cell lymphoma (DLBCL) who was inci-dentally diagnosed with a GSF on interim PET-CT afterthree cycles of chemotherapy.

A 57-year-old gentleman, who had no significant pasthistory, presented with intermittent low-grade fever and occa-sional cough of 2 weeks duration. Clinical examination wasinsignificant except for a single right axillary lymph node(1 cm×1 cm) and palpable spleen. Imaging studies revealedmultiple intra-abdominal lymphadenopathy and splenomega-ly of 15 cm with two well-defined hypoechoic lesions. Heunderwent a right axillary lymph node biopsy which wasconsistent with marginal zone lymphoma (low MIB-1 prolif-eration index—20 %). He was started on cyclophosphamideand prednisolone, in view of a low-grade lymphoma, astherapy, and was on regular follow up. Two years after theinitial diagnosis, he presented with complaints of weight lossand easy fatigability. Clinical evaluation revealed bilateralinguinal lymphadenopathy and hepatosplenomegaly. Repeatimaging of the abdomen showed multiple intra-abdominallymph nodes and increase in splenomegaly with well-defined heterogeneous lesion measuring 9×8 cm. A newheterogeneous lesion in the left lobe of the liver measuring8.5×8 cm was noted. High grade transformation of the initiallow-grade lymphoma was suspected and an ultrasound-

guided biopsy of the para-aortic lymph node revealed DLBCLwith aMIB-1 proliferation index of 60%. Hewas subsequent-ly started on R-CHOP 21 chemotherapy which he toleratedwell. Interim PET-CT done after three cycles to assess thedisease status revealed persistent splenomegaly with two focallesions, of which the larger lesion had eroded the stomach wallforming a GSF with surrounding stomach wall thickening.There was persistence of the hepatic lesion and intra-abdominal lymphadenopathy. The PET confirmed the GSFto be metabolically active (Fig. 1).

He had no abdominal pain or symptoms suggestive ofgastrointestinal bleeding and stool was negative for occultblood. Endoscopy of the upper gastrointestinal (GI) tractrevealed a big excavated ulcer with erythematous, elevated,and irregular margins in the gastric fundus with an openingnoted at one edge of the ulcer with food particles in it. Biopsyfrom the margins of the ulcer showed features ofHelicobacterpylori associated gastritis with extensive ulceration. The pa-tient was started on triple drug therapy for H. pylori . Heremained completely asymptomatic for his incidentally de-tected GSF. However, in view of an impending bleed, surgeryconsultation was sought and planned to proceed with a surgi-cal correction, involving a sleeve gastrectomy. However, thepatient refused any surgical intervention andwas lost to followup thereafter.

GSF has been described in gastric adenocarcinoma [5],Crohn’s disease [6], splenic abscess [7], and trauma. GSFhas also been described in connection to both Hodgkin andnon-Hodgkin lymphomas [1], developing spontaneously orafter chemotherapy. Splenic lymphomas have a high propen-sity for invasion of adjacent structures. In one series of tenpatients with splenic DLBCL [8], nine showed breach of thesplenic capsule and seven invading the structures, of whichfour involved the stomach. Colonosplenic fistulas have alsobeen described in context to lymphoma [9]. GastricMALTomas resulting in gastrosplenic, gastrocolic, and

J. Senapati (*) :A. J. Devasia :A. ViswabandyaDepartment of Clinical Haematology, Christian Medical College andHospital, Vellore 632004, Tamil Nadu, Indiae-mail: jsalwayswins@gmail.com

S. SudhakarDepartment of Radiology, Christian Medical College and Hospital,Vellore 632004, Tamil Nadu, India

Ann HematolDOI 10.1007/s00277-013-1986-8

gastropancreatic fistulas have also been reported [10]. This, toour knowledge, is the first reported case of a GSF in a case ofDLBCL from the Indian subcontinent. Abdominal pain re-mains the most common symptom [1, 4, 11, 12]; however,massive hematemesis [10, 12, 13] or asymptomatic presenta-tions have also been reported. Constitutional symptoms likefever, weight loss, and increased fatiguability are presentcommonly, which are probably secondary to the underlyingdisease process. Upper abdominal tenderness and splenomeg-aly are the most consistent clinical finding [14]. Occasionally,patients may appear toxic, which could be because of a splenicabscess [11]. Contrast enhanced computerized tomography(CECT) is the preferred investigation for GSF [1, 2, 5, 12,13, 15]. The presence of contrast in the spleen on CECT, or airfluid level in a plain CT is diagnostic and corresponds to thelocation of the fistulous tract [12]. An endoscopy of the uppergastrointestinal tract can help to visualize the gastric openingof the fistula, and diagnostic excisional biopsies can be done

[2]. A spontaneous GSF can appear more delineated on followup scans post chemotherapy [15].

Several pathogenic mechanisms have been described in thedevelopment of GSF in lymphoma. Aggressive tumor cellgrowth with gastric wall infiltration and subsequent necrosis,leading to the development of a fistulous tract has beenpostulated [4, 12]. Chemotherapy hastens tumor cell necrosisand increases the chances of GSF formation. The aggressivenature of lymphoma and absence of desmoplastic reaction tosurrounding tissues unlike other malignancies [2, 16] explainstheir increased occurrence in the former.

Treatment is primarily surgical and involves open surgicalremoval of the fistulous tract, along with splenectomy andgastrectomy [1, 3, 12, 13]. Resolution of the GSF with onlychemotherapy has also been described [2]; however, surgeryremains the gold standard. The possibilities of splenic orgastric vessel erosion leading to massive hematemesis orintra-abdominal bleed warrant urgent and aggressive

Fig. 1 A rim of increased FDG uptake (SUV 6.94) is noted in spleen. Corresponding CT images show splenomegaly with two focal lesions, the largersuperiorly placed lesion with a gastrosplenic fistula (air fluid level) with surrounding stomach wall thickening

Ann Hematol

Tab

le1

Major

publications

ofGSF

inlymphom

a

Author

Diagnosis

Gender/

Age

Disease

status

Presentatio

nDiagnostic

modality

Interventio

n/therapy

Outcome

Seib

etal[1]

Hodgkin’slymphom

aStageIIIB

Male/49

Relapsedpost

chem

otherapy

LUQpain

andconstitutional

symptom

sCTabdomen

Partialg

astrectomyandfistulectomy

Diedafter5months

Khanetal[4]

DLBCL

Female/43

Disease

presentatio

nU/A

pain

andconstitutional

symptom

sEndoscopy

ofupperGItract

follo

wed

byCT

Chemotherapy

Aliv

eandin

remission

Palm

owski

etal[2]

DLBCLStageIIBE

Male/56

Post3cycles

ofR-

CHOP2

1chem

otherapy

Feverandsignsof

acute

infection

CTabdomen

Splenectom

ywith

partialg

astric

resection

Finished

6cycles

ofchem

otherapy

Puppala

etal[12]

DLBCL

Female/66

Disease

presentatio

nLUQpain

radiatingto

chest

CTabdomen

oralcontrast

Chemotherapy

Diedafter2monthsof

chem

otherapy

Choietal[15]

SplenicDLBCL

Male/24

Disease

presentatio

nLUQpain

andconstitutionals

symptom

sCTabdomen

follo

wed

byendoscopyof

upperGI

tract/biopsy

Chemotherapy

follo

wed

bysplenectom

y,gastricwedge

resection,anddistal

pancreatectomy

Not

available

Bubenik

etal[16]

Diffuse

histiocytic

lymphom

aMale/58

Postchem

oradiotherapy

NonspecificLUQdiscom

fort

CTabdomen

follo

wed

byendoscopyof

upperGI

tract(fiberoptic)

Splenectom

y,gastricgreater

curvatureresection,distal

pancreatectomy

Uneventfulp

ost-op

period

Ariba etal[11]

DLBCL

Male/25

Postchem

otherapy

Splenomegaly,abdominalpain,

andconstitutionalsym

ptom

s(splenicabscess)

CTcystography

Splenectom

y,fistulectomy,and

gastricwedge

resection

Diedafter2months

Al-Ashgar

etal[17]

Hodgkin’slymphom

a-(nodular

sclerosis)-

IIIS

Female/16

Disease

presentatio

nLUQpain,constitu

tional

symptom

s,lymphadenopathy

splenomegaly,leftpleural

effusion

Endoscopy

ofupperGItract,

barium

swallow,C

Tabdomen

Laparoscopicsurgicalrepairfollo

wed

by7cycles

ABVDchem

otherapy

Aliv

eandin

remission

after

1year

Moran etal[18]

BcellNHL

Male/35

Disease

presentatio

nLUQpain

andconstitutional

symptom

sCTabdomen

follo

wed

byendoscopyof

upperGItract

Abscess

drainage;splenectomy,total

gastrectom

y,Roux-n-y

esophagojejunostom

yfollowed

byCHOPchem

otherapy

Receivedchem

oafter

surgery.Nofurther

details

available.

Dellaportas

etal[13]

SplenicDLBCL

Male/68

Disease

presentatio

nHem

atem

esis

Endoscopy

ofupperGItract

follo

wed

byCTabdomen

Surgicalen

bloc

resectionfollo

wed

bychem

otherapy

Postchem

oon

follo

wup.

Nodetails

available

Dingetal[14]

DLBCL

Male/62

Disease

presentatio

nLUQpain

with

constitutional

symptom

sand

splenomegaly

CTabdomen

follo

wed

byendoscopyof

upperGItract

Splenectom

y,gastricwedge

resection,

anddistalpancreatectomy

follo

wed

byChemo-RT

Wellatfollowup.N

ofurtherdetails

available

Kerem etal[3]

DLBCL

Male/57

Disease

presentatio

nAbdom

inalpain

radiatingto

back

with

epigastric

tenderness

CTabdomen

follo

wed

byPE

T-CTandendoscopyof

upperGItract

Splenectom

y,proxim

algastrectom

y,esophagojejunostom

y,proxim

alpyroloplasty

followed

byCHOP

chem

otherapy

Uneventfulp

ost-op

period;received

chem

otherapy.N

ofurtherdetails

available

LUQ

Leftu

pperquadrant;D

LBCLDiffuse

largeBcelllymphom

a;NHL-N

on-H

odgkin’slymphom

a;CTCom

putedtomography;

PETPo

sitron

emission

tomography;

CHOPC

Cyclophospham

ide,H

Adriamycin,O

Vincristin

e,PPrednisolone;A

BVDA-A

driamycin,B

-Bleom

ycin,V

-Vinblastin

e,D-D

acrabazine

Ann Hematol

management of the fistulous tract. In all the 12 major publi-cations of GSF in lymphoma we have reviewed (Table 1), CTabdomen clinched the diagnosis in all, and surgery remainedthe mainstay of treatment in ten.

GSF can be asymptomatic and yet a dreaded complicationof abdominal lymphoma. A high index of suspicion should bethere in patients with abdominal lymphoma for sudden onsetabdominal pain or gastro intestinal bleeding. However, theymay be evasive when asymptomatic and are often incidentallydiagnosed as in our patient. Open gastrectomy and splenecto-my remains the treatment of choice, and early managementcan prevent catastrophic complications.

Conflict of interest The authors declare that they have no conflict ofinterest.

Informed consent Informed consent was obtained from the patient foruse of clinical data and images in scientific work.

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