Case Presentation Doron Boltin March, 2015
Case 68 year-old male Previously healthy Medication- vit D Family Hx father CRC (age 70) mother gastric ca (age 70) Screening colonoscopy 02.2011- diminutive polyp (TA-LGD) Referred for gastroscopy (open access) due to epigastric pain 4 weeks Denies weight loss, early satiety, vomiting Physical exam- unremarkable
EGD Normal esophagus and GE junction Suspected corporal atrophy Proximal gastric body and fundus with localized hyperemia and edema with scant erosions and noted absence of gastric folds. Normal antrum and duodenum
CD 20
CD 10
Ki 67
Further Tests PCR (Ig heavy chain rearrangement) monoclonal PET-CT- no pathological FDG uptake Labs- CBC, Biochemistry, LDH- unremarkable Presumptive Diagnosis- Lugano Stage I gastric MALT lymphoma
Working Diagnosis- MALT Lymphoma Pro PCR monoclonality IHC consistent Con No mass No PET uptake
Working Diagnosis- MALT Lymphoma Pro PCR monoclonality IHC consistent Con No mass No PET uptake Referred to hematologist H. pylori eradicated Repeated EGD 2 months later
Clinical course Referred to hematologist H. pylori eradicated Repeated EGD 2 months later
EGD 2 months later Two adjacent polypoid ulcerated masses 20 mm and 30 mm arising in the gastric fundus
Clinical Course Patient rapidly deteriorated CT showed metastatic spread Briefly treated with chemotherapy before succumbing to the disease
Summary 68 yo man, epigastric pain EGD- gastropathy. PCR clonality and IHC suggestive of MALToma Received eradication for H. pylori EGD- fundic mass, histology- poorly diff adenocarcinoma
What s going on? 1. Synchronous MALT lymphoma and carcinoma? 2. Collision/Composite tumor? 3. Carcinoma on background of gastritis?
What s going on? 1. Synchronous MALT lymphoma and carcinoma? 2. Collision/Composite tumor? 3. Carcinoma on background of gastritis?
Synchronous MALT lymphoma and carcinoma Usually seen as 2 discrete masses If our case is indeed synchronous disease, then a. either carcinoma missed at initial EGD, or evolved rapidly thereafter b. MALT lymphoma resolved following eradication Common etiology- H. pylori Largest review, 32 pts (Chan, Am J Gastro 2001) To date, ~70 cases described
Synchronous MALT lymphoma and ~70 cases reported in literature Largest series, N=32 Mean age -64 yrs m:f = 1:2 H. pylori -78% Carcinoma Intestinal = diffuse carcinoma Early -65.6%, Poorly differentiated -40.6% Lymphoma- usually low grade -75%, larger than carcinoma- 81% LN metastasis- usually from lymphoma -62.5% Independent masses -55%, Contiguous -45% Chan, Am J Gastro 2001
What s going on? 1. Synchronous MALT lymphoma and carcinoma? 2. Collision/Composite tumor? 3. Ca on background of gastritis?
Collision/Composite Tumors Definition- tumor composed of more than one type of neoplastic tissue Collision- 2 primary tumors overlapping Composite- 1 de novo tumor with features of two distinct cell origins Cannot usually make the distinction Accounts for ~45% of synchronous gastric MALTomas and carcinomas in literature Appealing, due to common etiologies of MALToma and carcinoma (H. pylori)
What s going on? 1. Synchronous MALT lymphoma and carcinoma? 2. Collision/Composite tumor? 3. Ca on background of gastritis?
Pitfalls in MALToma diagnosis Monoclonality is not pathognomonic of neoplasia Monoclonality of IgH observed in 0-85%% of cases of severe chronic gastritis?
Prevalence of Clonality in Chronic Gastritis Hummel, Gut 2006
Hummel, Gut 2006 How Should We Distinguish MALT Lymphoma from Chronic Gastritis?
Prevalence of Clonality According to Wotherspoon Score Wotherspoon 1-2 Wotherspoon 3-4 Wotherspoon 5 2% Monoclonal 22% Monoclonal 92% Monoclonal Hummel, Gut 2006
Take Home Message Sequence of events not entirely clear in our case Synchronous lymphoma and carcinoma is exceedingly rare Monoclonality may not be diagnostic of malignancy
ראובני אברהם 6730623-3