a 60yearold lady with no past medical history was evaluated for a 3month history of solidfood dysphagia . she was otherwise healthy , and she denied any symptoms of fevers , chills , or night sweats . the daughter of this sister ( her niece ) was diagnosed with breast cancer at age 38 . her brother 's daughter ( another niece ) was also diagnosed with breast cancer at age 36 . genetic testing was performed on her daughter which confirmed a brca mutation ( brca1 q563x ) which is a truncated mutation . laboratory tests showed no evidence of anemia and mild hypoalbuminemia , albumin 3.3  g / dl . an upper endoscopy performed revealed a large circumferential , ulcerated , multilobar esophageal mass at the gastroesophageal junction and a several large multilobar ulcerative lesions were seen in the gastric fundus on retroflexion ( fig .  1 ) . there was also a large 2.5  cm cleanbased gastric ulcer on the lesser curvature of the stomach near adjacent masses . biopsy results showed an aggressive , highgrade bcell lymphoma , ki67 index was high at 100% . the expression for cd10 , bcl6 , and mum1 in neoplastic cells was consistent with germinal center bcell like ( gcb ) subtype of diffuse large bcell lymphoma ( dlbcl ) without any evidence of ebv infection in the tumor ( fig .  2 ) . further fish studies were positive for bcl2 gene rearrangement in 31% neoplastic cells and negative for bcl6 and myc thus negative for  double hit dhls are defined as a chromosome breakpoint affecting , affecting the myc locus in combination with another recurrent breakpoint , usually bcl2 , although bcl6/mycpositive dhls or bcl2/bcl6/myc triple hit lymphomas ( thls ) may also be observed 1 . most of the studies focused on dhls and thls have concluded that treatment with conventional chemotherapy consisting of rituximab plus cyclophosphamide , doxorubicin , vincristine , and prednisone ( rchop ) is not the best therapeutic option in these cases 2 . hematoxylin and eosin stained histologic section of esophageal / gastric biopsy demonstrates sheets of large lymphoid cells ( panel  a ) . immunohistochemical stain using cd20 antibody shows bright expression of cd20 by large lymphoid cells ( panel  b ) supportive of the diagnosis of diffuse large bcell lymphoma . after review of the results of her biopsy , she was referred to an oncology specialist for evaluation and treatment of dlbcl . as a part of staging , computed tomography ( ct ) of the abdomen and pelvis with intravenous contrast obtained demonstrated not only thickening of the gastroesophageal junction and proximal stomach with large volume abdominal adenopathy and a 5.6  cm splenic mass but also two cystic and solid pelvic masses , one measuring 8.5    7.0  cm and the other 3.7    3.2  cm ( fig . scan revealed a markedly hypermetabolic gastroesophageal junction and gastric wall thickening with thoracic and bulky abdominal hypermetabolic lymphadenopathy and splenic mass . patient at this time was staged to be stage iv dlbcl based on ann arbor staging . ( a ) sagittal view showing extensive abdominal lymphadenopathy . ( b ) coronal view showing solid pelvic masses . given the concerning findings of bilateral rim enhancing lesions initially the thought was , this patient could have a primary ovarian malignancy in addition to dlbcl . however , she was not a good candidate to undergo intubation needed for laparotomy because of bulky thoracic adenopathy and associated high surgical mortality risks . so a decision was made to proceed with chemotherapy for dlbcl and see whether adenopathy improves and postpone the pelvic surgery for the time being . we proceeded with curative intent intense chemotherapy with rchop every 21  days ( rituximab 375  mg / m , cyclophosphamide 750  mg / m , adriamycin 50  mg / m , vincristine 1.4  mg / m , and prednisone 100  mg    4  days ) based on nccn recommendations for stage iv dlbcl 3 . after two cycles of rchop patient underwent a repeat pet scan that showed global improvement including improvement in the adnexal masses , after discussion on tumor board it was decided to continue only chemotherapy as adnexal masses were deemed to be primary lymphoma surrounding ovarian cysts ( fig . patient is currently undergoing chemotherapy for dlbcl and responding well to treatment both radiographically and clinically . after review of the results of her biopsy , she was referred to an oncology specialist for evaluation and treatment of dlbcl . as a part of staging , computed tomography ( ct ) of the abdomen and pelvis with intravenous contrast obtained demonstrated not only thickening of the gastroesophageal junction and proximal stomach with large volume abdominal adenopathy and a 5.6  cm splenic mass but also two cystic and solid pelvic masses , one measuring 8.5    7.0  cm and the other 3.7    3.2  cm ( fig .  3 ) . a subsequent positron emission tomography ( pet ) scan revealed a markedly hypermetabolic gastroesophageal junction and gastric wall thickening with thoracic and bulky abdominal hypermetabolic lymphadenopathy and splenic mass . patient at this time was staged to be stage iv dlbcl based on ann arbor staging . given the concerning findings of bilateral rim enhancing lesions initially the thought was , this patient could have a primary ovarian malignancy in addition to dlbcl . however , she was not a good candidate to undergo intubation needed for laparotomy because of bulky thoracic adenopathy and associated high surgical mortality risks . so a decision was made to proceed with chemotherapy for dlbcl and see whether adenopathy improves and postpone the pelvic surgery for the time being . we proceeded with curative intent intense chemotherapy with rchop every 21  days ( rituximab 375  mg / m , cyclophosphamide 750  mg / m , adriamycin 50  mg / m , vincristine 1.4  mg / m , and prednisone 100  mg    4  days ) based on nccn recommendations for stage iv dlbcl 3 . after two cycles of rchop patient underwent a repeat pet scan that showed global improvement including improvement in the adnexal masses , after discussion on tumor board it was decided to continue only chemotherapy as adnexal masses were deemed to be primary lymphoma surrounding ovarian cysts ( fig . patient is currently undergoing chemotherapy for dlbcl and responding well to treatment both radiographically and clinically . this patient had initial symptoms of weight loss and solidfood dysphagia that were evaluated with upper endoscopy , computerized tomography ( ct ) , and pet scans that identified an aggressive bcell lymphoma with esophageal , gastric , and adnexal involvement . although this patient was diagnosed in an atypical fashion on endoscopy , the pet scan showed diffuse abdominal , thoracic adenopathy in addition to splenic involvement . extranodal involvement of the gi tract is more common in nonhodgkin 's as compared to hodgkin 's lymphoma and occurs in approximately 1030% patients 4 . stomach is the most frequently identified primary site ( 6575% ) , and primary esophageal involvement is extremely rare and almost always secondary from the stomach or mediastinal lymph nodes as probably was the case in this patient 5 , 6 . dawson et  al . proposed the following criteria for diagnosis of primary dlbcl of gi tract : ( i ) absence of peripheral lymphadenopathy at the time of presentation ; ( ii ) absence of mediastinal lymphadenopathy ; ( iii ) normal total white blood cell count ; ( iv ) predominance of bowel lesion at the time of laparotomy with only lymph nodes obviously affected in the immediate vicinity ; and ( v ) no lymphomatous involvement of liver and spleen 7 . in addition to the uncommon presentation of dysphagia in this patient , we also observed bilateral pelvic partly cystic masses with rim enhancing solid component . with her family history of brca positivity , there was also a concern for a primary ovarian malignancy in addition to dlbcl . a patient in this situation would normally have undergone an open tissue biopsy but her bulky lymphadenopathy precluded intubation . this decision was fortunate in this case as after two cycles of chemotherapy for dlbcl her pelvic masses greatly improved . in fact , the 6week followup pet scan showed a significant reduction in size of her cystic adnexal masses ( fig . the splenic involvement on the pet scan along with diffuse mediastinal adenopathy suggests that the likely primary site was lymphoid in nature with a very uncommon pattern of spread that included esophagus , stomach , and bilateral adnexa . the primary treatment for lymphoma is chemotherapy , and luckily , this patient because of her diffuse adenopathy was treated with chemotherapy and spared a morbid procedure like laparotomy . dq : guided the authors in writing the manuscript and proof read the final manuscript .
key clinical messagediffuse large bcell lymphoma ( dlbcl ) can involve the esophagus from local spread , distant metastasis and very rarely can also be the primary site . 
 once dlbcl is diagnosed , caution should be exercised in further evaluation for local treatments of sites like adnexal masses as was seen in this case ; sometimes it is dlbcl at atypical sites .