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Non-Hodgkin lymphoma with pancreas involvement, mimicking a pancreas IPMN: A case report

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Non-Hodgkin lymphoma with pancreas involvement, mimicking a pancreas IPMN: A case report

 

abstract

A case of pancreatic involvement by non-Hodgkin's lymphoma is presented. The patient, a 48-year-old woman had a large tumor in the tail of the pancrs with a subcarinal lymph node enlargement. She had the history of polycysic kidney disease and IPMN like cystic mass on pancreas tail. She was planned to elective surgery about pancreas lesion but she was presentd with fever and on CT check and found newly developed subcarinal LNE and increasement of pancreas lesion. So she underwent FNA on subcarinal lesiion and was diagnosed with NHL.

Therefore, distal pancreatecomy were performed, although a definitive preoperative diagnosis was not obtained. This tumor was identified, by histopathology and immunohistochemistry, as diffuse mixed type lymphoma with a B-cell phenotype. Postoperatively, She was planned to  receive chemotherapy. It is important to establish a definitive diagnosis for this disease, to remove the tumor, and to treat the patient with appropriate chemotherapy. CASE REPORT

 

A 48-year-old woman presented with   acute  abdominal pain and nausea. Her past history was unremarkable except for hyperension and polycystic kidney disease of 20 years duration which was followed  with US and routine lab vital check up. Her laboratory tests upon admission were as follows: white blood cell count 13.35  mm-3 (reference range: 4.6 - 10.2mm-3), hemoglobin 13.5 g/dL (reference range: 12.2-16.0  g/dL), lactate dehydrogenase 412.0  U/L (reference range: 240-480 U/L), glucose 102 mg/dL (reference range: 70-110 mg/dL), total protein 6.90 g/dL (reference range: 6.0-8.3), albumin 4.1 g/dL (reference range: 3.3-5.2 g/dL). The tumor marker levels of AFP, CEA, ca15-3 were all within the normal range except CA 125  41U/mL (reference range: 0- 35 U/mL),  CA 19-9  29.6 U/mL (reference range: 0- 27 U/mL ). Abdominal computed tomography (Figure 1) revealed a 3.5cm sized  solid and cystic mass in the pancreatic tail which could be clearly distinguished from the pancreas parenchyma and nearby surrounding soft tissues. The cystic portion could not  be distinguished from  malignacy including necrotic portion . Liver and kidneys showed variable sized cysts. Spleen was enlarged up to 12.cm. About 3.3cm sized soft tissue mass in right posterior mediastium abutting the heart.(Figure2)환자는 특별한 처치없이 통증은 가라앉았고

췌장의 수술을 권유하였으나 개인적인사정으로 1개월뒤에 f/u 하기로 하고 퇴원하였다가 1개월뒤에 발열  복통으로 다시 응급실통하여 입원함

입원당시 피검사 소견은 다음과같았고

f/u한 CT소견은 큰변화가 있어서 FNA EUS MRI PET CHEST CT 등을시행하고 subcarinal lesion에서. VETS

 

 To differentiate the pancreatic mass as an endocrine or an exocrine tumor, serotonin, 3-methoxytiramine, metanephrine, normetanephrine, vanyl mandelic acid, 5 hydroxy indole acetic acid and dopamine tests were performed and were found to be within normal levels. Percutaneous ultrasonography-guided biopsy of the suspicious peripancreatic node confirmed the diagnosis. Immunohistochemical studies of the biopsied tissue demonstrated large atypical lymphoid cells which were positive for CD20 with a Ki-67 index of 30%. The final diagnosis was diffuse large B cell lymphoma. Bone marrow aspiration and biopsy did not show any involvement of disease.

 

---------------------------------

 

 

Outside abdomen CT, 2015.12.12.

Clinical information: Left back pain.

Pancreas tail에 장경 3.5cm 으로 측정되는 비교적 경계가 분명한 solid and cystic mass가 있음. 이전 2015.11.05. outside CT 와 비교하여 크기 변화는 뚜렷하지 않으나 내부에 cystic or necrotic portionprominent 해보임. Massstomachabutting

하고 있음. Malignant lesion의 가능성을 배제해야 하겠음. Main pancreatic duct는 늘어나 있지 않음.

Liver와 양측 kidneymultiple small cyst들이 있으며, 가장 큰 것은 right kidney에 있는 것으로 장경 7.2cm으로 측정됨. 양측 kidneycyst 중에 일부에서는 NECT에서 high attenuation으로 보여 hemorrhagic cyst로 생각됨.

Spleen12.2cm 으로 borderline size . Retroperitoneumsmall reactive lymphadenopathy가 있음. Uterus에는 myoma들로 추정되는 multiple low density lesion들이 있음. 양측 소량의 pleural effusion 있고, basal lungdependent atelectasis가 있음.

 

[판정]

1. 3.5cm-sized solid and cystic mass in pancreas tail.

-- Prominent cystic or necrotic portion comparison with previous outside CT. --> R/O Malignant lesion. 2. Multiple small cysts in liver and both kidneys.

3. Borderline splenomegaly.

 

Addendum report> About 3.3cm sized soft tissue mass in Rt. posterior mediastinum,abutting the heart (arrow).

-> R/O lymphadenopathy, R/O mediastinal tumor. rec> chest CT.

 

 

 

 

 

 

 

 

 

 

[소화기병센터] EUS(소화기병센터) 실시:2015-12-16 IMG(심찬섭) 판독:2015-12-16 17:17 (Pf)심찬섭/(Pf)심찬섭

[소견]

Midazolam : 3mg EUS에서 pancreas tail에 약 39.8 mm x 31.8 mm 크기의 mixed echogenecity 보이는 mass 보임.

CEUS에서 조영제 투여 후 30초에 조영 증강되었다가 40초부터 서서히 빠지기 시작하였고 1분 후에 거의 다 빠지고 130초 후에

는 조영 변화 보이지 않음.

[판정]

R/O NET R/O SPT

 

 

[영상의학과] CT Chest (+) 실시:2015-12-16 IMG(심찬섭) 판독:2015-12-17 17:07 (Pf)송인영/(Pf)이정근/(R2)강지훈

Clinical information: Fever, back pain, pancreas tail mass, known ADPKD

양측 폐에 국소 병변 없음. Subcarina에 장경 4 x 2.3 cm 가량의 ovoid shapeenhancing LN 있음. Metastasis 가능성 높음.

양측에 소량의 흉수 있음. Cardiomegaly 있음. 포함된 상복부는 최근 시행한 외부 복부 CT 판독을 참고 바람.

[판정]

1. About 4cm subcarinal lymph node. --> Probable metastasis

2. Bilateral small pleural effusion 3. Cardiomegaly

 

 

 

 

 

 

 

 

[소화기병센터] EUS-FNAB(소화기병센터) 실시:2015-12-21 IMG(심찬섭) 판독:2015-12-21 15:26 (Pf)심찬섭/(Pf)심찬섭

[소견]

EUS에서 약 49.2 mm 크기의 subcarinal LN 확인되어 FNA 시행함. 시술 후 합병증 없음.

[판정]

R/O Pancreatic malignancy metastasis rec. check pathology

 

 

 

[병리과] 세포결과[NG1503588] 2015-12-22 09:14 IMG(심찬섭) 한혜승 2015-12-23 16:33

[DIAGNOSIS]

   Subcarinal LN

   Liquid-based body fluid cytology:

   Atypical hematolymphoid cell clusters present

    (see comments)

[H&E : 0 , PAP smear : 2]

[COMMENTS]

   Biopsy is recommended to confirm the nature of this lesion.

   

   [SAMPLE STATUS]

   - Turbidity: transparency

   - Color: red

   - Volume: 2 cc

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

[핵의학과] Torso FDG Fusion PET (두개골기저-대퇴부) 실시:2015-12-23 IMG(심찬섭) 판독:2015-12-23 15:16 (Pf)정현우/(Pf)소영/(Pf)정현우

[소견]

* 핵종 및 용량 : F-18 FDG 6.2 mCi

* 임상 소견 : R/O pancreas cancer, initial w/u * Torso FDG PET/CT 영상 소견 Pancreas tail 부위에 4.5 cm hypermetabolic mass가 관찰됨(SUVmax=10.4). 이 병변은 stomach과도 인접하여 있음. Pancreas head/uncinate process 부위에 1.2 cm의 또 다른 focal hypermetabolic lesion이 관찰됨(SUVmax=8.4).

Subcarinal area4.2 cm hypermetabolic LN가 관찰됨(SUVmax=15.8). 이 병변들은 모두 malignant lesion 가능성을 고려해 보아야 함. Both kidneyslivermultiple cysts가 관찰됨. Lungbonemalignancy를 의심할 뚜렷한 비정상적인 hypermetabolic lesion은 보이지 않음.

그밖의 다른 부위에 malignancy를 의심할 뚜렷한 비정상적인 hypermetabolic lesion은 보이지 않음.

 

[판정]

Hypermetabolic mass, pancreas tail, with adjacency to the stomach. Hypermetabolic nodule, pancreas head/uncinate process. Hypermetabolic LN, subcarinal area. - R/O malignancy.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

pancreas MRCP 2015-12-24

 

[Reading]

Clinical information: Known pancreas tail mass.

 

Pancreas tail에 장경 4.2cm 으로 측정되는 비교적 경계가 분명한 solid and cystic mass가 있음. Solid portionenhancement가 되어 보이고 diffusion restriction을 보이는 lesion으로 malignant lesion의 가능성이 있음. NETSPT의 가능성이 있으며, pancreatic ductal adenocarcinoma의 가능성은 상대적으로 적음. Massstomachabutting 하고 있음. Main pancreatic duct는 늘어나 있지 않음.

Liver와 양측 kidneymultiple small cyst들이 있으며, 가장 큰 것은 right kidney에 있는 것으로 장경 6.3cm으로 측정됨. 양측 kidneycyst 중에 일부에서는 fluid-fluid level을 보이고 있어 hemorrhagic cyst로 생각됨.

Spleen11.8cm 으로 borderline size .

 

[Conclusion]

1. 4.2cm-sized solid and cystic mass in pancreas tail.

--> R/O Malignant lesion.

DDx. 1) NET.

2) SPT.

3) PDAC, less likely.

2. Multiple small cysts in liver and both kidneys.

 

Addendum report >

pancreas head에도 약 1cm크기의 gradual enhancing nodule이 있고, SMVabutting하고 있으며 diffusion restriction을 보임 (arrow).

2015-11O/S CT, MR상에서는 뚜렷하게 이지 않았던 병변으로, pancreas2개의 병변은 atypicalimaging feature로 보여서 lymphomametastasis도 가능할 것으로 보임.

판독의: 유미혜.

 

 

A Case of Pancreatic LymphomaMichael Piesman, MD; David G. Forcione, MD; David L. Carr-Locke MD, FRCP, FACG, FASGE Series Editor: David L. Carr-Locke, MD, FRCP, FACG, FASGE|Disclosures CASE REPORTA 55-year-old man with a medical history significant for coronary artery disease and obstructive sleep apnea presented to the hospital with a 3-month history of abdominal pain and recent onset of bright red blood per rectum. Approximately 3 months before admission, the patient reported the onset of waxing and waning abdominal pain of increasing severity, which migrated from the right lower to right upper quadrant. Occasionally the pain occurred in the epigastrium and radiated to the back. He reported poor appetite, early satiety, and a 20-pound weight loss over the previous 2 months. He had had nausea, dry heaves, night sweats, and fatigue.One day before admission, the patient noted 2 loose bowel movements that turned the toilet water brownish-red. He has not had any bowel movements since this time. He denied prior hematochezia or melena, but had had small amounts of bright red blood on the toilet paper thought to be related to hemorrhoids. On physical examination, the patient had normal blood pressure and pulse rate; his abdomen was obese, soft, and nondistended. He had mild tenderness in the epigastric region with no peritoneal signs. His stool was guaiac positive. Laboratory analysis revealed a hematocrit drop from 42 % to 27 % over the past 3 months. The white blood cell count was within normal limits and lactate dehydrogenase was elevated to 2 times the normal level. Abdominal computed tomography (CT) scan demonstrated a 7 x 5-cm well-defined mass encasing the celiac axis and involving the head of the pancreas. Multiple enlarged lymph nodes were present adjacent to the mass (Figure 1).(Enlarge Image)Figure 1.The CT scan demonstrates a lobulated 7 x 5-cm mass centered in the region of the pancreatic head and celiac axis.Upper endoscopy revealed an ulcerated friable mass in the second portion of the duodenum with no active bleeding (Figures 2, 3). Endoscopic ultrasound examination revealed an approximately 4-cm well-defined hypoechoic homogeneous mass in the head of the pancreas (Figures 4, 5); with adjacent 2-cm well-defined hypoechoic lymph nodes (Figure 6).(Enlarge Image)Figure 2.Endoscopic view of an ulcerated mass in the second portion of the duodenum.(Enlarge Image)Figure 3.Another view of the ulcerated mass in the second portion of the duodenum.(Enlarge Image)Figure 4.Endoscopic ultrasound image of a 4 x 5-cm hypoechoic homogeneous mass (solid arrow) in the head of the pancreas.(Enlarge Image)Figure 5.Endoscopic ultrasound image demonstrating normal pancreatic parenchyma (solid arrow) adjacent to the hypoechoic mass. The pancreatic duct is 2.7 mm in diameter.(Enlarge Image)Figure 6.Endoscopic ultrasound image of a 2-cm well-defined hypoechoic lymph node adjacent to the pancreas.Fine-needle aspiration of the mass and the lymph node (Figure 7) was performed. Pathology demonstrated the presence of malignant lymphoma dominated by large cells; flow cytometry confirmed the presence of large B-cell lymphoma. The patient was treated with 4 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with complete resolution of the pancreatic mass.(Enlarge Image)Figure 7.Endoscopic ultrasound image demonstrating fine-needle aspiration of a hypoechoic lymph node.


Discussion
Primary pancreatic lymphoma is a rare nonepithelial tumor of the pancreas that can mimic pancreatic adenocarcinoma clinically and radiographically. Differentiating between the 2 malignancies is important because pancreatic lymphoma has a high cure rate

Instead of being primary in the gland, pancreatic lymphoma can also represent direct extension from adjacent peripancreatic lymphadenopathy.

Diagnostic criteria of primary pancreatic lymphoma include a pancreatic mass that predominates with gross involvement of only the peripancreatic lymph nodes, no hepatic or splenic involvement, no palpable superficial lymphadenopathy, no enlargement of the mediastinal lymph nodes on chest radiography, and a normal leukocyte count [1].

Although the pancreatic gland is involved secondarily in more than 30% of patients with non-Hodgkin's lymphoma [1]

1. Behrns KE, Sarr MG, Strickler JG. Pancreatic lymphoma: is it a surgical disease? Pancreas 1994; 9:662-667
primary manifestation is rare. Fewer than 2% of extranodal non-Hodgkin's lymphomas arise in the pancreas [2, 3]
2. Teefey SA, Stephens DH, Sheedy PF. CT appearance of primary pancreatic lymphoma. Gastrointest Radiol 1986; 11:41-43
3. Tuchek JMA, De Jong SA, Pickleman J. Diagnosis, surgical intervention, and prognosis of primary pancreatic lymphoma. Am Surg 1993; 59:513-518

This patient fulfilled these three criteria. We believe that this patient had stage 4A disease because of the diffuse involvement of the pancreas with infiltration to the peripancreatic area. No nearby infiltrating adenopathies or tumors of the other organs were noted.

The head of the pancreas is the most common location for primary pancreatic lymphoma, but the entire organ may be affected. The majority of cases of primary pancreatic lymphoma are of the B-cell type, but some cases of T-cell pancreatic lymphoma have been described in a Japanese series [4].

4. Mulkeen AL, Yoo PS, Cha C. Less common neoplasms of the pancreas. World J Gastroenterol 2006; 12:3180-5.




Appearances on CT can be helpful to differentiate the two conditions, but are not definitive[9, 10]
9. Lawler LP, Horton KM, Fishman EK: Peripancreatic masses that simulate pancreatic disease: spectrum of disease and role of CT. Radiographics 2003, 23 (5) :1117–1131

10. Teefey SA, Stephens DH, Sheedy PF 2nd: CT appearance of primary pancreatic lymphoma.Gastrointest Radiol 1986, 11 (1) : 41–43.
Ca19-9 is the most useful tumour marker in pancreatic carcinoma, but can be misleading as it may also be elevated in other malignancies, particularly of the upper gastrointestinal tract, including PPL as described in case one [11]. Without definitive pathologic diagnosis, potentially curable conditions such as PPL; as well as other malignancies with more favourable prognosis, including periampullary, distal common bile duct, duodenal and mucinous cyst adenocarcinomas, may be misdiagnosed[12].
11. Mann DV, Edwards R, Ho S, Lau WY, Glazer G:Elevated tumour marker CA19–9: clinical interpretation and influence of obstructive jaundice. Eur J Surg Oncol 2000, 26 (5) : 474–479.
12. Hart MJ, White TT, Brown PC, Freeny PC: Potentially curable masses in the pancreas. Am J Surg 1987, 154(1) : 134–136.

 

Percutaneous or endoscopic core biopsy should be performed to establish the diagnosis. In early reports, because percutaneous imagingguided biopsy was not considered an accurate diagnostic tool, the correct diagnosis was rarely made [3, 18, 22].

3. Tuchek JMA, De Jong SA, Pickleman J. Diagnosis, surgical intervention, and prognosis of primary pancreatic lymphoma. Am Surg 1993; 59:513-518
8. Muller MF, Meyenberger C, Bertschinger P, Schaer R, Marincek B. Pancreatic tumors: evaluation with endoscopic US, CT and MR imaging. Radiology 1994; 190:745-751
12. Ezzat A, Jamshed A, Khafaga Y, et al. Primary pancreatic non-Hodgkin's lymphomas. J Clin Gastroenterol 1996; 23:109-112

 

To our knowledge, MR imaging findings in patients with primary pancreatic lymphoma are not reported in the literature

 

In general, bile and pancreatic ductal dilatation can be easily assessed with MR imaging using MR cholangiopancreatography [24]. Only mild pancreatic ductal dilatation is visible on the MR imaging cases presented. Furthermore, MR imaging is equivalent to CT regarding information about the peripancreatic vessels and enlarged lymph nodes. Additional information is obtained when MR angiography is used [24].
24. Trede M, Rumstadt B, Wendl K, et al. Ultrafast magnetic resonance imaging improves the staging of pancreatic tumors. Ann Surg 1997; 226:393-405

 

 

Figure 1. Abdominal computed tomography shows a mass of the pancreas invading the nearby surrounding soft tissues



 



 




 

A 50-year-old man was reported to have an elevated serum lactate dehydrogenase (LDH) level (269 IU/L) by medical checkup in July 2007. He consulted with a physician in the following month. Serum LDH level continued to increase (313 IU/L). Abdominal ultrasonography was scheduled but he did not show up. On October 9, 2007, he presented with jaundice and was referred to our hospital on the next day. The patient denied any other symptoms such as recurrent fever, night sweat, and loss of body weight. His past history was unremarkable excluding a history of nephritis in his childhood. He had smoked 30 cigarettes per day, and was not alcoholic. His height was 173 cm and body weight 73 kg, blood pressure 124/79 mmHg and pulse rate 68 beats/min. No superficial lymphadenopathy was palpable. Computed tomography (CT) revealed a low-density mass, 10 cm in diameter, in the retroperitoneal space behind the pancreas head (Figure (Figure1),1), due to which the distal bile duct and the PV were obstructed.

Figure 1
Preoperative enhanced computed tomography. A low density mass (arrow), 10 cm in diameter, can be seen in the retroperitoneal space behind the pancreas head.

On October 22, 2008, a pylorus-preserving pancreaticoduodenectomy combined with a PV resection was performed. The PV was reconstructed using an autologous right internal jugular vein graft. The resected specimen showed endoluminal invasion of both the bile duct and the PV (Figure (Figure2).2). Histological examination showed the mass consisting of diffuse sheets of large malignant lymphoid cells (Figure (Figure3A).3A). Immunohistochemical studies revealed that these cells were positive for CD20 (B-cell associated antigen, Figure Figure3B)3B) and CD79a (B-cell associated antigen), and partially positive for CD10 (B-cell associated antigen), and negative for CD3 (T-cell associated antigen), CD4 (T-cell associated antigen) CD5 (T-cell associated antigen), CD8 (T-cell associated antigen) and CD30 (a universal feature of anaplastic large cell lymphoma). The pathologic diagnosis was diffuse large B-cell type non-Hodgkin lymphoma. The patient was transferred to the Department of Hematology and Oncology.

Figure 2
A photograph of the resected specimen. Endoluminal invasion of both the bile duct (white arrow) and the portal vein (black arrow) can be observed.
Figure 3Photomicrographs of the tumor. A: The tumor consists of diffuse sheets of large malignant lymphoid cells (HE, × 20); B: Immunostaining of the tumor. The tumor cells are positive for CD20 ( immunostaining, × 20

 

 

discussion


 

introduction

 

Primary pancreatic lymphoma arising from lymphoid elements in the pancreas itself is extremely rare, and it is defined by following clinical criteria [1]:

1. No evidence of palpable superficial lymphadenopathy,

2. No enlargement of mediastinal nodes,

3. Normal leukocyte count,

4. At surgery, the pancreatic mass predominates, with involved lymph nodes confined to the peripancreatic region,

5. No hepatic or splenic involvement.

 

Extranodal lymphoma is classified as secondary if there is involvement of lymph nodes except for those of an adjacent primary organ or with more than one extra nodal site.

Lymphomatous involvement of the pancreas is usually of the non-Hodgkin’s or B-cell type.[0]

 

Extranodal lymphoma is classified as secondary if there is involvement of lymph nodes except for those of an adjacent primary organ or with more than one extra nodal site. Lymphomatous involvement of the pancreas is usually of the non-Hodgkin’s or B-cell type.

 

Non-Hodgkin and Hodgkin lymphomas frequently involve many structures in the abdomen and pelvis. Extranodal disease is more

common with Non-Hodgkin’s lymphoma than with Hodgkin’s lymphoma. Though it may be part of a systemic lymphoma, single

onset of nodal lymphoma is not rare.

 

Overall, secondary involvement of the pancreas with lymphoma is more common than primary pancreatic lymphoma, which accounts for less than 1 % of pancreatic tumours [14] and less than 2 % of extra-nodal lymphoma [5, 6].

 

1. Behrns KE, Sarr MG, Strickler JG. Pancreatic lymphoma: is it a surgical disease? Pancreas. 1994;9(5):662–667. doi: 10.1097/00006676-199409000-00019. [PubMed] [Cross Ref]

2. Weingrad DN, Decosse JJ, Sherlock P, Straus D, Lieberman PH, Filippa DA. Primary gastrointestinal lymphoma: a 30-year review. Cancer. 1982;49(6):1258–1265. doi: 10.1002/1097-0142(19820315)49:6<1258::AID-CNCR2820490630>3.0.CO;2-4. [PubMed] [Cross Ref]

3. Tanaka T, Matsugu Y, Koide K, et al. Malignant lymphoma of the pancreas. Dig Dis Sci. 1996;41(2):402–404. doi: 10.1007/BF02093835. [PubMed] [Cross Ref]

4. Isaacson PG. Gastrointestinal lymphomas of T- and B-cell types. Mod Pathol. 1999;12(2):151–158. [PubMed]

5. Teefey SA, Stephens DH, Sheedy PF., 2nd CT appearance of primary pancreatic lymphoma. Gastrointest Radiol. 1986;11(1):41–43. doi: 10.1007/BF02035029. [PubMed] [Cross Ref]

6. Tuchek JM, De Jong SA, Pickleman J. Diagnosis, surgical intervention, and prognosis of primary pancreatic lymphoma. Am Surg. 1993;59(8):513–518. [PubMed]

 

However, predominant pancreatic involvement is rare.

 

Diffuse large B-cell is the most common type of non-Hodgkin’s lymphoma (NHL), and more than 50 % of patients have some extra-nodal lesions, comprising less than 0.6–2.1 % of pancreatic NHL [7, 8].

 

7. Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29(1):252–260. doi: 10.1002/1097-0142(197201)29:1<252::AID-CNCR2820290138>3.0.CO;2-#. [PubMed] [Cross Ref]

8. Takahashi H, Tomita N, Yokoyama M, et al. Prognostic impact of extranodal involvement in diffuse large B-cell lymphoma in the rituximab era. Cancer. 2012;118(17):4166–4172. doi: 10.1002/cncr.27381. [PubMed] [Cross Ref]

 

Secondary malignant lymphomas were found in 3 % of autopsy cases with pancreatic secondary tumours [9], usually appearing as large and bulky masses.

 

9. Nakamura E, Shimizu M, Itoh T, Manabe T. Secondary tumors of the pancreas: clinicopathological study of 103 autopsy cases of Japanese patients. Pathol Int. 2001;51(9):686–690. doi: 10.1046/j.1440-1827.2001.01258.x. [PubMed] [Cross Ref]

Diagnosis

MRI is an excellent modality for detecting and diagnosing pancreatic lesions as well as assessing the extent of involvement, since the tissue contrast is far superior compared with CT  since the tissue contrast is far superior compared with CT

 

CT and MR features of pancreatic lymphoma

 

In previous reports, radiologic findings of secondary lymphoma with predominant pancreatic involvement were similar to those of primary pancreatic lymphoma including nodular, diffuse and multi-nodular types [14, 27, 28].

14. Saif MW. Primary pancreatic lymphomas. JOP. 2006;7(3):262–273. [PubMed]

27. Cappell MS, Yao F, Cho KC, Axiotis CA. Lymphoma predominantly involving the pancreas. Dig Dis Sci. 1989;34(6):942–947. doi: 10.1007/BF01540283. [PubMed] [Cross Ref]

28. Ishigami K, Tajima T, Nishie A, et al. MRI findings of pancreatic lymphoma and autoimmune pancreatitis: a comparative study. Eur J Radiol. 2010;74(3):e22–e28. doi: 10.1016/j.ejrad.2009.03.022. [PubMed] [Cross Ref]

 

On CT imaging with intravenous contrast, most lesions are shown as well defined, sometimes bulky and infiltrating, homogeneous low-attenuation masses relative to the enhancing pancreatic parenchyma with only mild enhancement [13]. However, lesions that are less well circumscribed may wrongly be misdiagnosed as pancreatic cancer and result in surgical intervention.

Less commonly, pancreatic lymphoma may present as diffuse enlargement of the gland with an infiltrative tumour that could mimic acute pancreatitis. It is important to note that typical features of acute pancreatitis, including peripancreatic fat stranding and peripancreatic inflammation, are usually minimal if even present. Peripancreatic fluid collections and fat necrosis are typically absent in infiltrating pancreatic lymphoma. To our knowledge, pancreatic ductal disconnection and disruption seen with severe acute pancreatitis has not been reported in lymphoma. Vascular encasement or invasion is rarely seen with pancreatic lymphoma [13].

 

13. Merkle EM, Bender GN, Brambs HJ. Imaging findings in pancreatic lymphoma: differential aspects. AJR Am J Roentgenol. 2000;174(3):671–675. doi: 10.2214/ajr.174.3.1740671. [PubMed] [Cross Ref]

Pancreas tail에 장경 4.2cm 으로 측정되는 비교적 경계가 분명한 solid and cystic mass가 있음. Solid portion은 enhancement가 되어 보이고 diffusion restriction을 보이는 lesion으로 malignant lesion의 가능성이 있음. NET나 SPT의 가능성이 있으며, pancreatic ductal adenocarcinoma의 가능성은 상대적으로 적음. Mass는 stomach과 abutting 하고 있음. Main pancreatic duct는 늘어나 있지 않음.

Liver와 양측 kidney에 multiple small cyst들이 있으며, 가장 큰 것은 right kidney에 있는 것으로 장경 6.3cm으로 측정됨. 양측 kidney의 cyst 중에 일부에서는 fluid-fluid level을 보이고 있어 hemorrhagic cyst로 생각됨.

Spleen은 11.8cm 으로 borderline size 임.

[Conclusion]

1. 4.2cm-sized solid and cystic mass in pancreas tail.

--> R/O Malignant lesion.

DDx. 1) NET.

2) SPT.

3) PDAC, less likely.

2. Multiple small cysts in liver and both kidneys.

 

MRI features

MRI is an excellent modality for detecting and diagnosing pancreatic lesions as well as assessing the extent of involvement, since the tissue contrast is far superior compared with CT.

MRI is an excellent modality for detecting and diagnosing pancreatic lesions as well as assessing the extent of involvement, since the tissue contrast is far superior compared with CT. In general, pancreatic lymphomas appear as homogeneous, low-signal-intensity, focal nodular areas on T1WI, with variable, low or high signal intensity on T2WI, and a generally circumscribed, less-enhanced area relative to surrounding parenchyma on DCE-MRI [13, 28].

 

13. Merkle EM, Bender GN, Brambs HJ. Imaging findings in pancreatic lymphoma: differential aspects. AJR Am J Roentgenol. 2000;174(3):671–675. doi: 10.2214/ajr.174.3.1740671. [PubMed] [Cross Ref]

28. Ishigami K, Tajima T, Nishie A, et al. MRI findings of pancreatic lymphoma and autoimmune pancreatitis: a comparative study. Eur J Radiol. 2010;74(3):e22–e28. doi: 10.1016/j.ejrad.2009.03.022. [PubMed] [Cross Ref]

 

 

MRI findings of primary and secondary lymphoma may at times not be sufficiently specific to render a diagnosis, especially when pancreatic involvement is the first or only finding. Rather, other parameters must be considered, including clinical presentation and relevant imaging studies.

Radiologists should, however, be well versed in these varied imaging manifestations, as well as the characteristic findings of pancreatic lymphoma and its mimickers, to avoid misdiagnosis and unnecessary surgery. In a few cases, the findings of pancreatic lymphoma may be confusing and endoscopic biopsy should be suggested to arrive at a definitive diagnosis.[15]

 

Yasunari Fujinaga, Chandana Lall, Aashish Patel, Tsuyoshi Matsushita, Rupan Sanyal, Masumi Kadoya
Insights Imaging. 2013 June; 4(3): 321–329. Published online 2013 March 20. doi: 10.1007/s13244-013-0242-z
 

treatments

 

Chemotherapy is the mainstay of treatment for non-Hodgkin lymphoma[,]. Surgical intervention delayed the initiation of chemotherapy in the present case, which could miss an opportunity to cure the disease.  

6. Fisher RI, Gaynor ER, Dahlberg S, Oken MM, Grogan TM, Mize EM, Glick JH, Coltman CA Jr, Miller TP. Comparison of a standard regimen (CHOP) with three intensive chemotherapy regimens for advanced non-Hodgkin’s lymphoma. N Engl J Med. 1993;328:1002–1006. [PubMed]
7. Coiffier B, Lepage E, Briere J, Herbrecht R, Tilly H, Bouabdallah R, Morel P, Van Den Neste E, Salles G, Gaulard P, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med. 2002;346:235–242. [PubMed]

 

pancreas FNA limitations

 

Fine needle aspiration (FNA) with percutaneous or endoscopic ultrasound (EUS) guidance should precede the operation in the present case. FNA with percutaneous or EUS guidance permits both morphologic and cytologic analysis of lesions at various locations such as within or adjacent to the gastrointestinal tract, and intra-abdominal and retroperitoneal masses[]. 

9. Erickson RA, Tretjak Z. Clinical utility of endoscopic ultrasound and endscopic ultrasound-guided fine needle aspiration in retroperitoneal neoplasms. Am J Gastroenterol. 2000;95:1188–1194. [PubMed]
10. Eloubeidi MA, Varadarajulu S, Desai S, Shirley R, Heslin MJ, Mehra M, Arnoletti JP, Eltoum I, Wilcox CM, Vickers SM. A prospective evaluation of an algorithm incorporating routine preoperative endoscopic ultrasound-guided fine needle aspiration in suspected pancreatic cancer. J Gastrointest Surg. 2007;11:813–819. [PubMed]
11. Guo Z, Kurtycz DF, De Las Casas LE, Hoerl HD. Radiologically guided percutaneous fine-needle aspiration biopsy of pelvic and retroperitoneal masses: a retrospective study of 68 cases. Diagn Cytopathol. 2001;25:43–49. [PubMed]

Erickson and colleagues studied 18 patients with retroperitoneal lesions who underwent EUS and EUS-guided FNA[]. EUS-guided FNA was done in 15 (83%) of the 18 patients, among whom four were diagnosed as having lymphomas and avoided surgical intervention[], whereas, one patient ultimately had to have exploratory surgery for biopsy because neither EUS-guided FNA nor CT-guided percutaneous biopsy could provide enough tissues for definitive classification of a follicular centroblastic centrocytic lymphoma[].  

9. Erickson RA, Tretjak Z. Clinical utility of endoscopic ultrasound and endscopic ultrasound-guided fine needle aspiration in retroperitoneal neoplasms. Am J Gastroenterol. 2000;95:1188–1194. [PubMed]

 

One disadvantage of FNA with percutaneous or EUS guidance is a risk of complications. The possible complications associated with FNA included infection, intracystic hemorrhage, retroperitoneal hematoma, and pancreatitis[,]. Eloubeidi et al studied 547 patients who underwent EUS-guided FNA in the preoperative evaluation of suspected pancreatic cancer[]. In their report, 11 (2%) patients developed a major complication, and one patient required surgical debridement for necrosis[]. Guo et al reported that one (1.5%) of 68 patients had a minor complication (hematoma) from the radiologically guided percutaneous FNA biopsy of pelvic and retroperitoneal masses[].

10. Eloubeidi MA, Varadarajulu S, Desai S, Shirley R, Heslin MJ, Mehra M, Arnoletti JP, Eltoum I, Wilcox CM, Vickers SM. A prospective evaluation of an algorithm incorporating routine preoperative endoscopic ultrasound-guided fine needle aspiration in suspected pancreatic cancer. J Gastrointest Surg. 2007;11:813–819. [PubMed]
11. Guo Z, Kurtycz DF, De Las Casas LE, Hoerl HD. Radiologically guided percutaneous fine-needle aspiration biopsy of pelvic and retroperitoneal masses: a retrospective study of 68 cases. Diagn Cytopathol. 2001;25:43–49. [PubMed]

Another disadvantage of FNA with percutaneous or EUS guidance is a risk of tumor seeding along a needle tract[,]. Although the EUS-guided FNA is considered to have a lower risk of peritoneal carcinomatosis compared with percutaneous one[], a case of tumor seeding of a pancreatic adenocarcinoma because of EUS-guided FNA has been reported[]. Therefore, FNA with percutaneous or EUS guidance should be indicated only when additional information accessible by the procedure significantly affects the subsequent management of patients.

 

12. Micames C, Jowell PS, White R, Paulson E, Nelson R, Morse M, Hurwitz H, Pappas T, Tyler D, McGrath K. Lower frequency of peritoneal carcinomatosis in patients with pancreatic cancer diagnosed by EUS-guided FNA vs. percutaneous FNA. Gastrointest Endosc. 2003;58:690–695. [PubMed]
13. Paquin SC, Gariepy G, Lepanto L, Bourdages R, Raymond G, Sahai AV. A first report of tumor seeding because of EUS-guided FNA of a pancreatic adenocarcinoma. Gastrointest Endosc. 2005;61:610–611. [PubMed] 

 

 


 

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