Ann Hematol DOI 10.1007/s00277-013-1986-8

LETTER TO THE EDITOR

Asymptomatic gastrosplenic fistula in a patient with marginal zonal lymphoma transformed to diffuse large B cell lymphoma—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 fatal complication of lymphoma which can occur both spontaneously and after chemotherapy [1–4]. Here, we report a patient with diffuse large B cell lymphoma (DLBCL) who was incidentally diagnosed with a GSF on interim PET-CT after three cycles of chemotherapy. A 57-year-old gentleman, who had no significant past history, presented with intermittent low-grade fever and occasional cough of 2 weeks duration. Clinical examination was insignificant except for a single right axillary lymph node (1 cm×1 cm) and palpable spleen. Imaging studies revealed multiple intra-abdominal lymphadenopathy and splenomegaly of 15 cm with two well-defined hypoechoic lesions. He underwent a right axillary lymph node biopsy which was consistent with marginal zone lymphoma (low MIB-1 proliferation index—20 %). He was started on cyclophosphamide and prednisolone, in view of a low-grade lymphoma, as therapy, and was on regular follow up. Two years after the initial diagnosis, he presented with complaints of weight loss and easy fatigability. Clinical evaluation revealed bilateral inguinal lymphadenopathy and hepatosplenomegaly. Repeat imaging of the abdomen showed multiple intra-abdominal lymph nodes and increase in splenomegaly with welldefined heterogeneous lesion measuring 9×8 cm. A new heterogeneous lesion in the left lobe of the liver measuring 8.5×8 cm was noted. High grade transformation of the initial low-grade lymphoma was suspected and an ultrasoundJ. Senapati (*) : A. J. Devasia : A. Viswabandya Department of Clinical Haematology, Christian Medical College and Hospital, Vellore 632004, Tamil Nadu, India e-mail: [email protected] S. Sudhakar Department of Radiology, Christian Medical College and Hospital, Vellore 632004, Tamil Nadu, India

guided biopsy of the para-aortic lymph node revealed DLBCL with a MIB-1 proliferation index of 60 %. He was subsequently started on R-CHOP 21 chemotherapy which he tolerated well. Interim PET-CT done after three cycles to assess the disease status revealed persistent splenomegaly with two focal lesions, of which the larger lesion had eroded the stomach wall forming a GSF with surrounding stomach wall thickening. There was persistence of the hepatic lesion and intraabdominal lymphadenopathy. The PET confirmed the GSF to be metabolically active (Fig. 1). He had no abdominal pain or symptoms suggestive of gastrointestinal bleeding and stool was negative for occult blood. Endoscopy of the upper gastrointestinal (GI) tract revealed a big excavated ulcer with erythematous, elevated, and irregular margins in the gastric fundus with an opening noted at one edge of the ulcer with food particles in it. Biopsy from the margins of the ulcer showed features of Helicobacter pylori associated gastritis with extensive ulceration. The patient was started on triple drug therapy for H. pylori . He remained completely asymptomatic for his incidentally detected GSF. However, in view of an impending bleed, surgery consultation was sought and planned to proceed with a surgical correction, involving a sleeve gastrectomy. However, the patient refused any surgical intervention and was lost to follow up thereafter. GSF has been described in gastric adenocarcinoma [5], Crohn’s disease [6], splenic abscess [7], and trauma. GSF has also been described in connection to both Hodgkin and non-Hodgkin lymphomas [1], developing spontaneously or after chemotherapy. Splenic lymphomas have a high propensity for invasion of adjacent structures. In one series of ten patients with splenic DLBCL [8], nine showed breach of the splenic capsule and seven invading the structures, of which four involved the stomach. Colonosplenic fistulas have also been described in context to lymphoma [9]. Gastric MALTomas resulting in gastrosplenic, gastrocolic, and

Ann Hematol

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 larger superiorly placed lesion with a gastrosplenic fistula (air fluid level) with surrounding stomach wall thickening

gastropancreatic fistulas have also been reported [10]. This, to our knowledge, is the first reported case of a GSF in a case of DLBCL from the Indian subcontinent. Abdominal pain remains the most common symptom [1, 4, 11, 12]; however, massive hematemesis [10, 12, 13] or asymptomatic presentations have also been reported. Constitutional symptoms like fever, weight loss, and increased fatiguability are present commonly, which are probably secondary to the underlying disease process. Upper abdominal tenderness and splenomegaly are the most consistent clinical finding [14]. Occasionally, patients may appear toxic, which could be because of a splenic abscess [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 air fluid level in a plain CT is diagnostic and corresponds to the location of the fistulous tract [12]. An endoscopy of the upper gastrointestinal tract can help to visualize the gastric opening of the fistula, and diagnostic excisional biopsies can be done

[2]. A spontaneous GSF can appear more delineated on follow up scans post chemotherapy [15]. Several pathogenic mechanisms have been described in the development of GSF in lymphoma. Aggressive tumor cell growth with gastric wall infiltration and subsequent necrosis, leading to the development of a fistulous tract has been postulated [4, 12]. Chemotherapy hastens tumor cell necrosis and increases the chances of GSF formation. The aggressive nature of lymphoma and absence of desmoplastic reaction to surrounding tissues unlike other malignancies [2, 16] explains their increased occurrence in the former. Treatment is primarily surgical and involves open surgical removal of the fistulous tract, along with splenectomy and gastrectomy [1, 3, 12, 13]. Resolution of the GSF with only chemotherapy has also been described [2]; however, surgery remains the gold standard. The possibilities of splenic or gastric vessel erosion leading to massive hematemesis or intra-abdominal bleed warrant urgent and aggressive

Hodgkin’s lymphoma Stage III B DLBCL

Seib et al [1]

DLBCL

Splenic DLBCL

Diffuse histiocytic lymphoma

DLBCL

Hodgkin’s lymphoma(nodular sclerosis)IIIS

B cell NHL

Splenic DLBCL

DLBCL

DLBCL

Puppala et al [12]

Choi et al [15]

Bubenik et al [16]

Ariba et al [11]

Al-Ashgar et al [17]

Moran et al [18]

Dellaportas et al [13]

Ding et al [14]

Kerem et al [3]

Male/57

Male/62

Male/68

Disease presentation

Disease presentation

Disease presentation

Disease presentation

Disease presentation

Female/16

Male/35

Post chemotherapy

Post chemoradiotherapy

Disease presentation

Post 3 cycles of RCHOP21 chemotherapy Disease presentation

Relapsed post chemotherapy Disease presentation

Disease status

Male/25

Male/58

Male/24

Female/66

Male/56

Female/43

Male /49

Gender/ Age

LUQ pain with constitutional symptoms and splenomegaly Abdominal pain radiating to back with epigastric tenderness

Hematemesis

Splenomegaly, abdominal pain, and constitutional symptoms (splenic abscess) LUQ pain, constitutional symptoms, lymphadenopathy splenomegaly, left pleural effusion LUQ pain and constitutional symptoms

Nonspecific LUQ discomfort

LUQ pain and constitutionals symptoms

LUQ pain radiating to chest

LUQ pain and constitutional symptoms U/A pain and constitutional symptoms Fever and signs of acute infection

Presentation

Laparoscopic surgical repair followed by 7 cycles ABVD chemotherapy

Splenectomy, gastric wedge resection, and distal pancreatectomy followed by Chemo-RT Splenectomy, proximal gastrectomy, esophagojejunostomy, proximal pyroloplasty followed by CHOP chemotherapy

CT abdomen followed by endoscopy of upper GI tract CT abdomen followed by PETCT and endoscopy of upper GI tract

Endoscopy of upper GI tract followed by CT abdomen

Abscess drainage; splenectomy, total gastrectomy, Roux-n-y esophagojejunostomy followed by CHOP chemotherapy Surgical en bloc resection followed by chemotherapy

CT abdomen followed by endoscopy of upper GI tract

Uneventful post-op period; received chemotherapy. No further details available

Well at follow up. No further details available

Post chemo on follow up. No details available

Received chemo after surgery. No further details available.

Alive and in remission after 1 year

Died after 2 months

Uneventful post-op period

Endoscopy of upper GI tract, barium swallow, CT abdomen

CT abdomen followed by endoscopy of upper GI tract (fiber optic) CT cystography

Not available

Chemotherapy followed by splenectomy, gastric wedge resection, and distal pancreatectomy Splenectomy, gastric greater curvature resection, distal pancreatectomy Splenectomy, fistulectomy, and gastric wedge resection

CT abdomen followed by endoscopy of upper GI tract /biopsy

Finished 6 cycles of chemotherapy

Splenectomy with partial gastric resection

Died after 2 months of chemotherapy

Alive and in remission

Died after 5 months

Outcome

Chemotherapy

Partial gastrectomy and fistulectomy

Intervention/therapy

Chemotherapy

CT abdomen oral contrast

Endoscopy of upper GI tract followed by CT CT abdomen

CT abdomen

Diagnostic modality

LUQ Left upper quadrant; DLBCL Diffuse large B cell lymphoma; NHL-Non-Hodgkin’s lymphoma; CT Computed tomography; PET Positron emission tomography; CHOP C Cyclophosphamide, H Adriamycin, O Vincristine, P Prednisolone; ABVD A-Adriamycin, B-Bleomycin, V-Vinblastine, D-Dacrabazine

DLBCL Stage IIBE

Palmowski et al [2]

Khan et al [4]

Diagnosis

Author

Table 1 Major publications of GSF in lymphoma

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Ann Hematol

management of the fistulous tract. In all the 12 major publications of GSF in lymphoma we have reviewed (Table 1), CT abdomen clinched the diagnosis in all, and surgery remained the mainstay of treatment in ten. GSF can be asymptomatic and yet a dreaded complication of abdominal lymphoma. A high index of suspicion should be there in patients with abdominal lymphoma for sudden onset abdominal pain or gastro intestinal bleeding. However, they may be evasive when asymptomatic and are often incidentally diagnosed as in our patient. Open gastrectomy and splenectomy remains the treatment of choice, and early management can prevent catastrophic complications. Conflict of interest The authors declare that they have no conflict of interest. Informed consent Informed consent was obtained from the patient for use of clinical data and images in scientific work.

References 1. Seib CD, Rocha FG, Hwang DG, Shoji BT (2009) Gastrosplenic fistula from Hodgkin’s lymphoma. J Clin Oncol 27:e15–e17 2. Palmowski M, Zechmann C, Satzl S, Bartling S, Hallscheidt P (2007) Large gastrosplenic fistula after effective treatment of abdominal diffuse large-B-cell lymphoma. Ann Hematol 87:337–338 3. Kerem M et al (2006) Spontaneous gastrosplenic fistula in primary gastric lymphoma: surgical management. Asian J Surg 29:287–290 4. Khan F, Vessal S, McKimm E, D’Souza R (2010) Spontaneous gastrosplenic fistula secondary to primary splenic lymphoma. Case Rep. 2010, bcr0420102932–bcr0420102932 5. Krause R, Larsen CR, Scholz FJ (1990) Gastrosplenic fistula: complication of adenocarcinoma of stomach. Comput Med Imaging Graph 14:273–276

6. CARY ER, TREMAINE WJ, BANKS PM, NAGORNEY DM (1989) Isolated Crohn’s disease of the stomach. in. Mayo Clin Proc 64:776–779 7. Ballas K et al (2005) Gastrosplenic fistula: a rare complication of splenic abscess. Surg Pract 9:153–155 8. Harris NL, Aisenberg AC, Meyer JE, Ellman L, Elman A (1984) Diffuse large cell (histiocytic) lymphoma of the spleen: clinical and pathologic characteristics of ten cases. Cancer 54: 2460–2467 9. Naschitz J et al (1986) Spontaneous colosplenic fistula complicating immunoblastic lymphoma. Dis Colon Rectum 29:521–523 10. Fallahian F, Rahimi F, Khedmat H, Alizadeh A (2009) A case of gastric mucosa-associated lymphoid tissue lymphoma (maltoma) with multiple gastric fistulas and gastrointestinal bleeding. Shiraz E Med J 10:147–154 11. Aribacs BK et al (2008) Gastrosplenic fistula due to splenic large cell lymphoma diagnosed by percutaneous drainage before surgical treatment. Turk J Gastroenterol 19:69–70 12. Puppala S, Williams R, Harvey J, Crane MD (2005) Spontaneous gastrosplenic fistula in primary gastric lymphoma: case report and review of literature. Clin Radiol Extra 60:20–22 13. Dellaportas D et al (2012) Gastrosplenic fistula secondary to lymphoma, manifesting as upper gastrointestinal bleeding. Endoscopy 43, E395–E395 14. Ding Y-L, Wang S-Y (2012) Gastrosplenic fistula due to splenic large B-cell lymphoma. J Res Med Sci 17 15. Choi JE, Chung HJ, Lee HG (2002) Spontaneous gastrosplenic fistula: a rare complication of splenic diffuse large cell lymphoma. Abdom Imaging 27:728–730 16. Bubenik O, Lopez MJ, Greco AO, Kraybill WG, Cherwitz DL (1983) Gastrosplenic fistula following successful chemotherapy for disseminated histiocytic lymphoma. Cancer 52:994– 996 17. Al-Ashgar HI, Khan MQ, Ghamdi AM, Bamehriz FY, Maghfoor I (2007) Gastrosplenic fistula in Hodgkin’s lymphoma treated successfully by laparoscopic surgery and chemotherapy. Saudi Med J 28: 1898 18. Moran M et al (2009) Spontaneous gastrosplenic fistula resulting from primary gastric lymphoma: case report and review of the literature. Med J Trak Univ. doi:10.5174/tutfd.2009.02645.1

Asymptomatic gastrosplenic fistula in a patient with marginal zonal lymphoma transformed to diffuse large B cell lymphoma--a case report and review of literature.

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