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Giant pancreatic lymphangioma with mesenteric extension – A case report
The Egyptian Journal of Radiology and Nuclear Medicine xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
The Egyptian Journal of Radiology and Nuclear Medicine journal homepage: www.sciencedirect.com/locate/ejrnm
Case Report
Giant pancreatic lymphangioma with mesenteric extension – A case report Mrudang Raval a,⇑, Pradeep Goyal b, Tanay Shah c a
SDMH, Jaipur, India OK Diagnostics SDMH, Jaipur, India c Lokmanya Tilak Municipal General Hospital, Mumbai, India b
a r t i c l e
i n f o
Article history: Received 21 July 2016 Accepted 8 August 2016 Available online xxxx Keywords: Pancreatic lymphangioma Mesentery Multicystic lymphangioma
a b s t r a c t Cystic lymphangioma of the pancreas is uncommon entity. Complete excision is necessary to prevent recurrence. We present huge multicystic pancreatic lymphangioma extending beyond the pancreatic margin into the mesentery causing displacement of bowel loops and scalloping of liver surface. Extension into the mesentery mimicked the other cystic lesions of the mesentery, but the expansion and thinning of pancreatic parenchyma by cystic spaces suggest pancreatic origin. The cystic mass was resected en-block along with pancreatic body and tail. Preoperative diagnosis of this entity is usually difficult. Lymphangioma should be considered in the differentials of huge multicystic lesion involving pancreas and mesentery. Ó 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction
2. Case report
Lymphangioma is an uncommon benign lymphatic origin cystic tumor of pancreas. They are found to occur commonly in head and neck region, but intra-abdominal and pancreatic origin is rarely encountered. Pancreatic lymphangioma has been reported to occur in all age groups, but commonly affects pediatric age group. Lymphangioma is a multiseptated cystic neoplasm of lymphatic malformation leading to lymphangiectasia lined by endothelial cells. The authors present a huge pancreatic lymphangioma extending to mesentery displacing bowel loops and scalloping left lobe liver surface. To the best of our knowledge, this is the largest pancreatic lymphangioma described in the English literature.
A 12 yr old male child presented with complaint of generalized pain in abdomen and abdominal distension since 10 days. On examination the abdomen was soft and nontender. A large rounded mass was palpable in epigastric region. Laboratory tests were unremarkable except for a raised leukocyte count. Ultrasonography showed a large septated cystic mass in epigastric region extending up to umbilical region. Triple phase CT scan of abdomen was performed in arterial, portal and equilibrium phases. CT showed a large multiseptated mass extending from subphrenic region to umbilical fossa scalloping undersurface of liver in caudate and left lobe. The mass intermingled with pancreatic body parenchyma with replacement of parenchyma at places. Pancreatic head, uncinate process and tail were normal. The mass engulfed splenic artery, left gastric artery, proximal common hepatic artery and splenic vein. No solid component was seen within the mass. Provisional
Peer review under responsibility of The Egyptian Society of Radiology and Nuclear Medicine. ⇑ Corresponding author. E-mail address: [email protected] (M. Raval).
http://dx.doi.org/10.1016/j.ejrnm.2016.08.011 0378-603X/Ó 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Raval M et al. Giant pancreatic lymphangioma with mesenteric extension – A case report. Egypt J Radiol Nucl Med (2016), http://dx.doi.org/10.1016/j.ejrnm.2016.08.011
2
M. Raval et al. / The Egyptian Journal of Radiology and Nuclear Medicine xxx (2016) xxx–xxx
diagnosis of pancreatic-mesenteric lymphangioma was given on the basis of CT findings. Operative findings revealed a multilobulated cystic mass arising from distal pancreatic body and tail which encased the splenic vessels and abutted mesocolon, aorta and portal vein. On gross of histopathological examination, the mass was cystic with outer surface congested and attached to tail of pancreas. On sectioning, the cysts were multilocular and filled with brownish serous fluid. Biopsy revealed large cystic spaces lined by single layer of endothelium. The wall showed lymphoid tissue. The diagnosis of pancreatic lymphangioma was confirmed histopathologically (see Images 1–4).
Image 1. Coronal CT image of abdomen shows a large cystic lesion involving pancreatic body and tail and encasing the vessels few septas are seen within the lesion.
Image 2. Axial section at the level of origin of celiac trunk shows a multiseptated cystic lesion centered in mesentery and encasing the mesenteric and celiac vessels left kidney is displaced posteriorly.
3. Discussion Etiology of lymphangioma remains unclear. Congenital developmental defects of the lymphatics have been proposed, such as the proliferation and dilatation of blind ended lymphatic sacs which lack proper connections with the venous system [1]. The theory of a congenital etiology may be supported by the fact that most cases present in childhood. Other potential causes include abdominal trauma, localized lymphatic degeneration and lymphatic obstruction [2,3]. In abdomen, the commonest site is mesentery [4,1], but it may also occur in omentum, retroperitoneum and mesocolon [1,5]. Lymphangiomas of pancreas have also been reported [6]. Clinical presentation of pancreatic lymphangioma is non-specific and includes abdominal pain and nausea, vomiting and palpable mass [7]. Lymphangioma may increase in size up to 20 cms in greatest dimension [7]. CT usually shows well circumscribed homogenous cystic masses in or adjacent to pancreas. Multiple fine septations and thin wall may enhance after intravenous contrast injection. Cystic lymphangioma if large enough may exert mass effect on adjacent organs. Rarely phlebolith like calcification may also be seen. Sonography shows anechoic or hypoechoic fluid filled multiseptated mass in pancreatic region [8]. In our case large multiseptated mass was seen on CT scan extending from subphrenic region to umbilical fossa scalloping undersurface of liver in caudate and left lobe. The mass intermingled with pancreatic body parenchyma with partial replacement of parenchyma. The major differential diagnosis was a mesenteric lymphangioma. Mesenteric lymphangiomas are more common entities as compared to pancreatic form. Presenting complaints and the age predominance of both mesenteric and pancreatic lymphangiomas are overlapping. In our case the multiseptated cystic mass was involving or intermingling with the body of pancreas, which points to the fact that the mass lesion was arising from the pancreas or in other words
Image 3. Axial section at the level of pancreatic body shows a cystic lesion involving the pancreatic body and tail, extending into mesentery.
Please cite this article in press as: Raval M et al. Giant pancreatic lymphangioma with mesenteric extension – A case report. Egypt J Radiol Nucl Med (2016), http://dx.doi.org/10.1016/j.ejrnm.2016.08.011
M. Raval et al. / The Egyptian Journal of Radiology and Nuclear Medicine xxx (2016) xxx–xxx
3
Image 4. A and B - Gross specimen showed a multiseptated cystic lesion involving the body and tail of pancreas extending into mesentery. h & e stained section on low power view shows dilated lymphatic vessels adjacent to the pancreatic acini. Vessels contain proteinaceous material.
the epicenter of the lesion was in body of pancreas and not in the mesentery. Although lymphangiomas are known to spread trans-spatially, inability to visualize the mass separately from the body of pancreas and expansion of the body of pancreatic parenchyma by the mass were strong indicators that it was arising from the pancreas. Taking this into consideration, primary diagnosis was kept as pancreatic lymphangioma with mesenteric lymphangioma as a differential diagnosis. En-block resection of the mass lesion was done by our gastrointestinal surgeons along with pancreatic body and tail. The diagnosis was confirmed histopathologically in our case. The histopathological specimen showed cystic spaces lined by endothelium with wall showing lymphoid tissue. Further confirmation of the diagnosis can be done by subjecting the specimen to immunohistochemistry. Pancreatic lymphangioma shows positive staining of factor VIII-R antigen, CD31 and CD34, which are sensitive and specific markers for the identification of lymphatic and capillary endothelium. However, immunohistochemistry was not done in our case. 4. Conclusion Preoperative diagnosis of pancreatic lymphangioma is usually difficult, but a pancreatic lymphangioma must be considered if the cystic multiseptated mass is involving both the pancreatic parenchyma and mesentery.
Acknowledgement I would like to thank Dr. Rajesh Bhojwani (Gastrointestinal surgeon) and Dr. Jaimini Patel (Pathologist) for all the guidance they have provided me to complete this article. References [1] Weeda VB, Booij KAC, Aronson DC. Mesenteric cystic lymphangioma: a congenital and an acquired anomaly? Two cases and a review of the literature. J Pediatr Surg 2008;43:1206–8. [2] Losanoff JE, Richman BW, El-Sherif A, Rider KD. Mesenteric cystic lymphangioma. J Am Coll Surg 2003;196(4):598–603. [3] Chen CW, Hsu SD, Cheng MF, Yu JC. Cystic lymphangioma of the jejunal mesentery in an adult: a case report. World J Gastroenterol 2005;11(32):5084–6. [4] Campbell WJ, Irwin ST, Biggart JD. Benign lymphangioma of the jejunal mesentery: an unusual cause of small bowel obstruction. Gut 1991;32:1568. [5] Stein M, Hsu RK, Schneier PD, Ruebner BH, Mina Y. Alcohol ablation of a mesenteric lymphangioma. JVIR 2000;11:247–50. [6] Miliaras S, Trygonis S, Papandoniou A, Kalamaras S, Trygonis C, Kiskinis D. Mesenteric cyst of the descending colon: report of a case. Acta Chir Belg 2006;106:714–6. [7] Paal E, Thompson LD, Heffess CS. A clinicopathologic and immunohistochemical study of ten pancreatic lymphangiomas and a review of the literature. Cancer 1998;82:2150–8. [8] Igarashi A, Maruo Y, Ito T, et al. Huge cystic lymphangioma of the pancreas: report of a case. Surg Today 2001;31:743–6.
Conflict of interest The authors declare that there are no conflict of interests.
Please cite this article in press as: Raval M et al. Giant pancreatic lymphangioma with mesenteric extension – A case report. Egypt J Radiol Nucl Med (2016), http://dx.doi.org/10.1016/j.ejrnm.2016.08.011
Contents lists available at ScienceDirect
The Egyptian Journal of Radiology and Nuclear Medicine journal homepage: www.sciencedirect.com/locate/ejrnm
Case Report
Giant pancreatic lymphangioma with mesenteric extension – A case report Mrudang Raval a,⇑, Pradeep Goyal b, Tanay Shah c a
SDMH, Jaipur, India OK Diagnostics SDMH, Jaipur, India c Lokmanya Tilak Municipal General Hospital, Mumbai, India b
a r t i c l e
i n f o
Article history: Received 21 July 2016 Accepted 8 August 2016 Available online xxxx Keywords: Pancreatic lymphangioma Mesentery Multicystic lymphangioma
a b s t r a c t Cystic lymphangioma of the pancreas is uncommon entity. Complete excision is necessary to prevent recurrence. We present huge multicystic pancreatic lymphangioma extending beyond the pancreatic margin into the mesentery causing displacement of bowel loops and scalloping of liver surface. Extension into the mesentery mimicked the other cystic lesions of the mesentery, but the expansion and thinning of pancreatic parenchyma by cystic spaces suggest pancreatic origin. The cystic mass was resected en-block along with pancreatic body and tail. Preoperative diagnosis of this entity is usually difficult. Lymphangioma should be considered in the differentials of huge multicystic lesion involving pancreas and mesentery. Ó 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction
2. Case report
Lymphangioma is an uncommon benign lymphatic origin cystic tumor of pancreas. They are found to occur commonly in head and neck region, but intra-abdominal and pancreatic origin is rarely encountered. Pancreatic lymphangioma has been reported to occur in all age groups, but commonly affects pediatric age group. Lymphangioma is a multiseptated cystic neoplasm of lymphatic malformation leading to lymphangiectasia lined by endothelial cells. The authors present a huge pancreatic lymphangioma extending to mesentery displacing bowel loops and scalloping left lobe liver surface. To the best of our knowledge, this is the largest pancreatic lymphangioma described in the English literature.
A 12 yr old male child presented with complaint of generalized pain in abdomen and abdominal distension since 10 days. On examination the abdomen was soft and nontender. A large rounded mass was palpable in epigastric region. Laboratory tests were unremarkable except for a raised leukocyte count. Ultrasonography showed a large septated cystic mass in epigastric region extending up to umbilical region. Triple phase CT scan of abdomen was performed in arterial, portal and equilibrium phases. CT showed a large multiseptated mass extending from subphrenic region to umbilical fossa scalloping undersurface of liver in caudate and left lobe. The mass intermingled with pancreatic body parenchyma with replacement of parenchyma at places. Pancreatic head, uncinate process and tail were normal. The mass engulfed splenic artery, left gastric artery, proximal common hepatic artery and splenic vein. No solid component was seen within the mass. Provisional
Peer review under responsibility of The Egyptian Society of Radiology and Nuclear Medicine. ⇑ Corresponding author. E-mail address: [email protected] (M. Raval).
http://dx.doi.org/10.1016/j.ejrnm.2016.08.011 0378-603X/Ó 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Raval M et al. Giant pancreatic lymphangioma with mesenteric extension – A case report. Egypt J Radiol Nucl Med (2016), http://dx.doi.org/10.1016/j.ejrnm.2016.08.011
2
M. Raval et al. / The Egyptian Journal of Radiology and Nuclear Medicine xxx (2016) xxx–xxx
diagnosis of pancreatic-mesenteric lymphangioma was given on the basis of CT findings. Operative findings revealed a multilobulated cystic mass arising from distal pancreatic body and tail which encased the splenic vessels and abutted mesocolon, aorta and portal vein. On gross of histopathological examination, the mass was cystic with outer surface congested and attached to tail of pancreas. On sectioning, the cysts were multilocular and filled with brownish serous fluid. Biopsy revealed large cystic spaces lined by single layer of endothelium. The wall showed lymphoid tissue. The diagnosis of pancreatic lymphangioma was confirmed histopathologically (see Images 1–4).
Image 1. Coronal CT image of abdomen shows a large cystic lesion involving pancreatic body and tail and encasing the vessels few septas are seen within the lesion.
Image 2. Axial section at the level of origin of celiac trunk shows a multiseptated cystic lesion centered in mesentery and encasing the mesenteric and celiac vessels left kidney is displaced posteriorly.
3. Discussion Etiology of lymphangioma remains unclear. Congenital developmental defects of the lymphatics have been proposed, such as the proliferation and dilatation of blind ended lymphatic sacs which lack proper connections with the venous system [1]. The theory of a congenital etiology may be supported by the fact that most cases present in childhood. Other potential causes include abdominal trauma, localized lymphatic degeneration and lymphatic obstruction [2,3]. In abdomen, the commonest site is mesentery [4,1], but it may also occur in omentum, retroperitoneum and mesocolon [1,5]. Lymphangiomas of pancreas have also been reported [6]. Clinical presentation of pancreatic lymphangioma is non-specific and includes abdominal pain and nausea, vomiting and palpable mass [7]. Lymphangioma may increase in size up to 20 cms in greatest dimension [7]. CT usually shows well circumscribed homogenous cystic masses in or adjacent to pancreas. Multiple fine septations and thin wall may enhance after intravenous contrast injection. Cystic lymphangioma if large enough may exert mass effect on adjacent organs. Rarely phlebolith like calcification may also be seen. Sonography shows anechoic or hypoechoic fluid filled multiseptated mass in pancreatic region [8]. In our case large multiseptated mass was seen on CT scan extending from subphrenic region to umbilical fossa scalloping undersurface of liver in caudate and left lobe. The mass intermingled with pancreatic body parenchyma with partial replacement of parenchyma. The major differential diagnosis was a mesenteric lymphangioma. Mesenteric lymphangiomas are more common entities as compared to pancreatic form. Presenting complaints and the age predominance of both mesenteric and pancreatic lymphangiomas are overlapping. In our case the multiseptated cystic mass was involving or intermingling with the body of pancreas, which points to the fact that the mass lesion was arising from the pancreas or in other words
Image 3. Axial section at the level of pancreatic body shows a cystic lesion involving the pancreatic body and tail, extending into mesentery.
Please cite this article in press as: Raval M et al. Giant pancreatic lymphangioma with mesenteric extension – A case report. Egypt J Radiol Nucl Med (2016), http://dx.doi.org/10.1016/j.ejrnm.2016.08.011
M. Raval et al. / The Egyptian Journal of Radiology and Nuclear Medicine xxx (2016) xxx–xxx
3
Image 4. A and B - Gross specimen showed a multiseptated cystic lesion involving the body and tail of pancreas extending into mesentery. h & e stained section on low power view shows dilated lymphatic vessels adjacent to the pancreatic acini. Vessels contain proteinaceous material.
the epicenter of the lesion was in body of pancreas and not in the mesentery. Although lymphangiomas are known to spread trans-spatially, inability to visualize the mass separately from the body of pancreas and expansion of the body of pancreatic parenchyma by the mass were strong indicators that it was arising from the pancreas. Taking this into consideration, primary diagnosis was kept as pancreatic lymphangioma with mesenteric lymphangioma as a differential diagnosis. En-block resection of the mass lesion was done by our gastrointestinal surgeons along with pancreatic body and tail. The diagnosis was confirmed histopathologically in our case. The histopathological specimen showed cystic spaces lined by endothelium with wall showing lymphoid tissue. Further confirmation of the diagnosis can be done by subjecting the specimen to immunohistochemistry. Pancreatic lymphangioma shows positive staining of factor VIII-R antigen, CD31 and CD34, which are sensitive and specific markers for the identification of lymphatic and capillary endothelium. However, immunohistochemistry was not done in our case. 4. Conclusion Preoperative diagnosis of pancreatic lymphangioma is usually difficult, but a pancreatic lymphangioma must be considered if the cystic multiseptated mass is involving both the pancreatic parenchyma and mesentery.
Acknowledgement I would like to thank Dr. Rajesh Bhojwani (Gastrointestinal surgeon) and Dr. Jaimini Patel (Pathologist) for all the guidance they have provided me to complete this article. References [1] Weeda VB, Booij KAC, Aronson DC. Mesenteric cystic lymphangioma: a congenital and an acquired anomaly? Two cases and a review of the literature. J Pediatr Surg 2008;43:1206–8. [2] Losanoff JE, Richman BW, El-Sherif A, Rider KD. Mesenteric cystic lymphangioma. J Am Coll Surg 2003;196(4):598–603. [3] Chen CW, Hsu SD, Cheng MF, Yu JC. Cystic lymphangioma of the jejunal mesentery in an adult: a case report. World J Gastroenterol 2005;11(32):5084–6. [4] Campbell WJ, Irwin ST, Biggart JD. Benign lymphangioma of the jejunal mesentery: an unusual cause of small bowel obstruction. Gut 1991;32:1568. [5] Stein M, Hsu RK, Schneier PD, Ruebner BH, Mina Y. Alcohol ablation of a mesenteric lymphangioma. JVIR 2000;11:247–50. [6] Miliaras S, Trygonis S, Papandoniou A, Kalamaras S, Trygonis C, Kiskinis D. Mesenteric cyst of the descending colon: report of a case. Acta Chir Belg 2006;106:714–6. [7] Paal E, Thompson LD, Heffess CS. A clinicopathologic and immunohistochemical study of ten pancreatic lymphangiomas and a review of the literature. Cancer 1998;82:2150–8. [8] Igarashi A, Maruo Y, Ito T, et al. Huge cystic lymphangioma of the pancreas: report of a case. Surg Today 2001;31:743–6.
Conflict of interest The authors declare that there are no conflict of interests.
Please cite this article in press as: Raval M et al. Giant pancreatic lymphangioma with mesenteric extension – A case report. Egypt J Radiol Nucl Med (2016), http://dx.doi.org/10.1016/j.ejrnm.2016.08.011