Symptomatic lymphangioma of the adrenal gland: a case report

Symptomatic lymphangioma of the adrenal gland: a case report Lymphangiomas (LAs) are rare benign tumors of the lymphatic vessels. In total, 95% of all reported LAs are located in the head, neck and the mediastinum. LAs of the adrenal gland are very rare and currently, only ~54 cases have been reported in literature. We present a case of a big left-sided adrenal LA. Abdominal imaging revealed a big cystic lesion in the left upper abdomen of unknown origin. For diagnostic and therapeutic reasons we performed explorative midline laparotomy. The left adrenal gland was found to be the origin of the cystic tumor. Hence, the patient underwent adrenalectomy in order to remove the intact cystic lesion. Diagnosis was then confirmed by histological examination. With adrenal LAs being a very rare entity, diagnosis is challenging and only little evidence exists on treatment options. We discuss diagnostic, therapeutic and surgical approaches concerning such cases and provide an overview of the current literature. INTRODUCTION CASE REPORT Lymphangiomas (LAs) are rare benign tumors of the lymphatic A 32-year-old female patient was referred to our emergency vessels. The etiology of LAs has not yet been clearly established. department complaining of progressive, colicky abdominal They are believed to result from malformations of sequestered pain accompanied by intermittent nausea and vomiting during lymphatic tissue. These lesions can be present at birth or occur at a period of more than 3 weeks. The pain was localized in the any age. The most common sites of occurrence are head, neck epigastric region and radiated through to the back. In addition, and the mediastinum accounting for 95% of all reported LAs [1]. the patient complained of generalized paresthesia as well as The remaining 5% are located in the abdominal cavitiy, involving episodes of hot flashes and palpitations. There was no alter- the mesentery of the small intestine, mesocolon, omentum, pan- ation in stool consistency or frequency. Her past medical his- creas or adrenal gland. LAs of the adrenal gland are very rare and tory revealed no long-term medication or previous abdominal currently, only approximately 54 cases have been reported in lit- operations. erature [2, 3]. These lesions are usually detected as asymptomatic, On physical examination, the patient had normal vital signs. incidental finding in patients undergoing an abdominal imaging Her abdomen was soft, non-tender and non-distended. Laboratory procedure for other reasons. The diagnosis is very challenging investigations showed a slightly elevated white blood cell and to date, the histological tissue analysis remains the only reli- count as well as C-reactive protein level. Beta-HCG test was able tool to confirm the diagnosis of an adrenal LA. negative. Computed tomography scanning of the pulmonary We present a case of a big left-sided adrenal LA causing arteries was performed in order to rule out pulmonary embol- non-specific abdominal pain and symptoms. ism. The simultaneous display of the upper abdomen revealed Received: February 15, 2018. Accepted: May 7, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy106/4999376 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 R. Liechti et al. Figure 1: (A, B) Coronal and sagittal T2 weighted, fat suppressed MR image of the abdomen showing displacement of the spleen as well as the left kidney by the adrenal tumor. (C) Intra-operative finding showing close proximity of the cystic lesion to the left colonic flexure. (D) Surgically excised cystic tumor together with the left adrenal gland. a big cystic lesion, which seemed to be attached to the left kid- At the 3-month follow-up, the patient presented with sleep ney. Biochemical analysis and hormone testing showed no disorder, nausea and weight loss. The ACTH stimulation test signs of a hyper functioning mass. Abdominal MRI revealed a showed a relative adrenocortical insufficiency while DHEA-S maximum diameter of 12.5 cm. The origin of the cystic lesion levels remained normal. Consecutively the patient was treated was suspected either in the mesentery or the omentum majus with hydrocortisone, as needed during stressful situations. The (Fig. 1A and B). Considering the abdominal symptoms as well as further course remained uneventful (Fig. 1). the fact that neither origin nor dignity could be determined in this patient, a total surgical removal followed by histological examination was indicated. DISCUSSION We performed an upper midline laparotomy. To ensure a Among all of the reported cases, no single imaging modality good anatomical overview, mobilization of the left colonic was able to correctly diagnose an adrenal LA. Similar to our flexure was necessary and the omental bursa was entered. case, five adrenal LAs have even been characterized as a non- The distal part of the pancreas showed close proximity to the adrenal lesion prior to the operation [3]. A recent review of the cystic lesion and needed careful separation. After adhesiolysis literature concerning the general management of LAs con- between the splenic surface, the posterior gastric wall as well cluded that treatment options should be individualized as the parietal peritoneum, the left adrenal gland with its depending on size, symptoms and anatomic localization of the associated vein, was found to be the origin of the cystic tumor. tumor [4]. With adrenal LAs being a very rare entity, the utiliza- Hence total adrenalectomy was performed in order to remove tion of treatment guidelines for adrenal incidentalomas seems the intact cystic lesion (Fig. 1C and D). Following a short period to be appropriate. If the cystic adrenal lesion is clinically and of postoperative nausea and vomiting, gradual return to a nor- biologically inactive and its appearance remains unchanged mal diet was possible. The patient was discharged on the sixth over a period of 18 months time, conservative management is postoperative day. justified [5]. There is no evidence of reliable diagnostic value of The histological examination of the specimen revealed an preoperative biopsy or fine-needle aspiration for adrenal neo- adrenal lymphangioma located in a normal structured adrenal plasms [6]. gland. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy106/4999376 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Symptomatic lymphangioma of the adrenal gland 3 Adrenal masses with either suspicious imaging aspects or DISCLAIMER size larger than 4 cm should be considered for resection due to The views expressed in the submitted article are not an official a substantial risk of malignancy [7]. position of the institution or funder. Up until now, only one adrenal LA has been resected using a retroperitoneoscopic approach and only three adrenal LAs have been treated via laparoscopic adrenalectomy [2, 3]. Taking in REFERENCES consideration that both the laparoscopic as well as retroperito- 1. Poncelet V. Retroperitoneal cystic lymphangioma. J Belge neoscopic approach result in reduced blood loss, less post- Radiol 1998;81:245. operative pain, shorter hospital stay and quicker recovery as 2. Kim KH, Lee JI, Bae JM. Significant growth of adrenal lym- compared to open surgery, minimally invasive surgery seems phangioma: a case report and review of the literature. Int J to be suitable for such lesions [8]. However, for adrenal neo- Surg Case Rep 2015;17:48–50. plasms >10 cm in diameter, open adrenalectomy is recom- 3. Michalopoulos N, Laskou S, Karayannopoulou G, Pavlidis L, mended. This is due to the fact that these large adrenal masses Kanellos I. Adrenal gland lymphangiomas. Indian J Surg may be diagnosed as malignant on definitive histologic analysis 2015;77:1334–42. [9]. In our case laparoscopic adrenalectomy was not an option 4. Ha J, Yu YC, Lannigan F. A review of the management of considering the enormous diameter of the cyst. lymphangiomas. Curr Pediatr Rev 2014;10:238–48. In histological examination, two cases of adrenal LA showed 5. Khoda J, Hertzanu Y, Sebbag G, Lantsberg L, Barky Y. cellular atypia with a certain risk of malignant transformation Adrenal cysts: diagnosis and therapeutic approach. Int Surg [3, 10]. However, the available data regarding postoperative sur- 1993;78:239–42. veillance of adrenal LAs showed no signs of recurrence at 24- 6. Mazzaglia PJ, Monchik JM. Limited value of adrenal biopsy month follow-up after surgical resection. Therefore patients in in the evaluation of adrenal neoplasm: a decade of experi- whom the LA has been resected together with the adrenal ence. Arch Surg 2009;144:465–70. gland are considered cured. 7. Grumbach MM, Biller BM, Braunstein GD, Campbell KK, Carney JA, Godley PA, et al. Management of the clinically CONCLUSION inapparent adrenal mass (‘incidentaloma’). Ann Intern Med 2003;138:424–9. Up until today diagnosis of adrenal LAs cannot be established 8. Phitayakorn R, McHenry CR. Laparoscopic and selective non-invasively. In order to diagnose and treat these lesions, open resection for adrenal and extraadrenal neuroendo- adrenalectomy is considered the appropriate method for large crine tumors. Am Surg 2008;74:37–42. (>4 cm in diameter) or symptomatic LAs. Small (<4 cm in diam- 9. Brix D, Allolio B, Fenske W, Agha A, Dralle H, Jurowich C, eter), clinically and biologically inactive adrenal cysts may be et al. German Adrenocortical Carcinoma Registry. Laparoscopic treated conservatively, provided that regular radiological sur- versus open adrenalectomy for adrenocortical carcinoma: sur- veillance can be warranted. Generally long-term follow-up gical and oncologic outcome in 152 patients. Eur Urol 2010;58: showed excellent results and prognosis after resection of 609–15. adrenal LAs. 10. Ellis CL, Banerjee P, Carney E, Sharma R, Netto GJ. Adrenal lymphangioma: clinicopathologic and immunohistochem- CONFLICT OF INTEREST STATEMENT ical characteristics of a rare lesion. Hum Pathol 2011;42: The authors have no conflict of interest. 1013–8. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy106/4999376 by Ed 'DeepDyve' Gillespie user on 21 June 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Symptomatic lymphangioma of the adrenal gland: a case report

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Abstract

Lymphangiomas (LAs) are rare benign tumors of the lymphatic vessels. In total, 95% of all reported LAs are located in the head, neck and the mediastinum. LAs of the adrenal gland are very rare and currently, only ~54 cases have been reported in literature. We present a case of a big left-sided adrenal LA. Abdominal imaging revealed a big cystic lesion in the left upper abdomen of unknown origin. For diagnostic and therapeutic reasons we performed explorative midline laparotomy. The left adrenal gland was found to be the origin of the cystic tumor. Hence, the patient underwent adrenalectomy in order to remove the intact cystic lesion. Diagnosis was then confirmed by histological examination. With adrenal LAs being a very rare entity, diagnosis is challenging and only little evidence exists on treatment options. We discuss diagnostic, therapeutic and surgical approaches concerning such cases and provide an overview of the current literature. INTRODUCTION CASE REPORT Lymphangiomas (LAs) are rare benign tumors of the lymphatic A 32-year-old female patient was referred to our emergency vessels. The etiology of LAs has not yet been clearly established. department complaining of progressive, colicky abdominal They are believed to result from malformations of sequestered pain accompanied by intermittent nausea and vomiting during lymphatic tissue. These lesions can be present at birth or occur at a period of more than 3 weeks. The pain was localized in the any age. The most common sites of occurrence are head, neck epigastric region and radiated through to the back. In addition, and the mediastinum accounting for 95% of all reported LAs [1]. the patient complained of generalized paresthesia as well as The remaining 5% are located in the abdominal cavitiy, involving episodes of hot flashes and palpitations. There was no alter- the mesentery of the small intestine, mesocolon, omentum, pan- ation in stool consistency or frequency. Her past medical his- creas or adrenal gland. LAs of the adrenal gland are very rare and tory revealed no long-term medication or previous abdominal currently, only approximately 54 cases have been reported in lit- operations. erature [2, 3]. These lesions are usually detected as asymptomatic, On physical examination, the patient had normal vital signs. incidental finding in patients undergoing an abdominal imaging Her abdomen was soft, non-tender and non-distended. Laboratory procedure for other reasons. The diagnosis is very challenging investigations showed a slightly elevated white blood cell and to date, the histological tissue analysis remains the only reli- count as well as C-reactive protein level. Beta-HCG test was able tool to confirm the diagnosis of an adrenal LA. negative. Computed tomography scanning of the pulmonary We present a case of a big left-sided adrenal LA causing arteries was performed in order to rule out pulmonary embol- non-specific abdominal pain and symptoms. ism. The simultaneous display of the upper abdomen revealed Received: February 15, 2018. Accepted: May 7, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy106/4999376 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 R. Liechti et al. Figure 1: (A, B) Coronal and sagittal T2 weighted, fat suppressed MR image of the abdomen showing displacement of the spleen as well as the left kidney by the adrenal tumor. (C) Intra-operative finding showing close proximity of the cystic lesion to the left colonic flexure. (D) Surgically excised cystic tumor together with the left adrenal gland. a big cystic lesion, which seemed to be attached to the left kid- At the 3-month follow-up, the patient presented with sleep ney. Biochemical analysis and hormone testing showed no disorder, nausea and weight loss. The ACTH stimulation test signs of a hyper functioning mass. Abdominal MRI revealed a showed a relative adrenocortical insufficiency while DHEA-S maximum diameter of 12.5 cm. The origin of the cystic lesion levels remained normal. Consecutively the patient was treated was suspected either in the mesentery or the omentum majus with hydrocortisone, as needed during stressful situations. The (Fig. 1A and B). Considering the abdominal symptoms as well as further course remained uneventful (Fig. 1). the fact that neither origin nor dignity could be determined in this patient, a total surgical removal followed by histological examination was indicated. DISCUSSION We performed an upper midline laparotomy. To ensure a Among all of the reported cases, no single imaging modality good anatomical overview, mobilization of the left colonic was able to correctly diagnose an adrenal LA. Similar to our flexure was necessary and the omental bursa was entered. case, five adrenal LAs have even been characterized as a non- The distal part of the pancreas showed close proximity to the adrenal lesion prior to the operation [3]. A recent review of the cystic lesion and needed careful separation. After adhesiolysis literature concerning the general management of LAs con- between the splenic surface, the posterior gastric wall as well cluded that treatment options should be individualized as the parietal peritoneum, the left adrenal gland with its depending on size, symptoms and anatomic localization of the associated vein, was found to be the origin of the cystic tumor. tumor [4]. With adrenal LAs being a very rare entity, the utiliza- Hence total adrenalectomy was performed in order to remove tion of treatment guidelines for adrenal incidentalomas seems the intact cystic lesion (Fig. 1C and D). Following a short period to be appropriate. If the cystic adrenal lesion is clinically and of postoperative nausea and vomiting, gradual return to a nor- biologically inactive and its appearance remains unchanged mal diet was possible. The patient was discharged on the sixth over a period of 18 months time, conservative management is postoperative day. justified [5]. There is no evidence of reliable diagnostic value of The histological examination of the specimen revealed an preoperative biopsy or fine-needle aspiration for adrenal neo- adrenal lymphangioma located in a normal structured adrenal plasms [6]. gland. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy106/4999376 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Symptomatic lymphangioma of the adrenal gland 3 Adrenal masses with either suspicious imaging aspects or DISCLAIMER size larger than 4 cm should be considered for resection due to The views expressed in the submitted article are not an official a substantial risk of malignancy [7]. position of the institution or funder. Up until now, only one adrenal LA has been resected using a retroperitoneoscopic approach and only three adrenal LAs have been treated via laparoscopic adrenalectomy [2, 3]. Taking in REFERENCES consideration that both the laparoscopic as well as retroperito- 1. Poncelet V. Retroperitoneal cystic lymphangioma. J Belge neoscopic approach result in reduced blood loss, less post- Radiol 1998;81:245. operative pain, shorter hospital stay and quicker recovery as 2. Kim KH, Lee JI, Bae JM. Significant growth of adrenal lym- compared to open surgery, minimally invasive surgery seems phangioma: a case report and review of the literature. Int J to be suitable for such lesions [8]. However, for adrenal neo- Surg Case Rep 2015;17:48–50. plasms >10 cm in diameter, open adrenalectomy is recom- 3. Michalopoulos N, Laskou S, Karayannopoulou G, Pavlidis L, mended. This is due to the fact that these large adrenal masses Kanellos I. Adrenal gland lymphangiomas. Indian J Surg may be diagnosed as malignant on definitive histologic analysis 2015;77:1334–42. [9]. In our case laparoscopic adrenalectomy was not an option 4. Ha J, Yu YC, Lannigan F. A review of the management of considering the enormous diameter of the cyst. lymphangiomas. Curr Pediatr Rev 2014;10:238–48. In histological examination, two cases of adrenal LA showed 5. Khoda J, Hertzanu Y, Sebbag G, Lantsberg L, Barky Y. cellular atypia with a certain risk of malignant transformation Adrenal cysts: diagnosis and therapeutic approach. Int Surg [3, 10]. However, the available data regarding postoperative sur- 1993;78:239–42. veillance of adrenal LAs showed no signs of recurrence at 24- 6. Mazzaglia PJ, Monchik JM. Limited value of adrenal biopsy month follow-up after surgical resection. Therefore patients in in the evaluation of adrenal neoplasm: a decade of experi- whom the LA has been resected together with the adrenal ence. Arch Surg 2009;144:465–70. gland are considered cured. 7. Grumbach MM, Biller BM, Braunstein GD, Campbell KK, Carney JA, Godley PA, et al. Management of the clinically CONCLUSION inapparent adrenal mass (‘incidentaloma’). Ann Intern Med 2003;138:424–9. Up until today diagnosis of adrenal LAs cannot be established 8. Phitayakorn R, McHenry CR. Laparoscopic and selective non-invasively. In order to diagnose and treat these lesions, open resection for adrenal and extraadrenal neuroendo- adrenalectomy is considered the appropriate method for large crine tumors. Am Surg 2008;74:37–42. (>4 cm in diameter) or symptomatic LAs. Small (<4 cm in diam- 9. Brix D, Allolio B, Fenske W, Agha A, Dralle H, Jurowich C, eter), clinically and biologically inactive adrenal cysts may be et al. German Adrenocortical Carcinoma Registry. Laparoscopic treated conservatively, provided that regular radiological sur- versus open adrenalectomy for adrenocortical carcinoma: sur- veillance can be warranted. Generally long-term follow-up gical and oncologic outcome in 152 patients. Eur Urol 2010;58: showed excellent results and prognosis after resection of 609–15. adrenal LAs. 10. Ellis CL, Banerjee P, Carney E, Sharma R, Netto GJ. Adrenal lymphangioma: clinicopathologic and immunohistochem- CONFLICT OF INTEREST STATEMENT ical characteristics of a rare lesion. Hum Pathol 2011;42: The authors have no conflict of interest. 1013–8. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy106/4999376 by Ed 'DeepDyve' Gillespie user on 21 June 2018

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Journal of Surgical Case ReportsOxford University Press

Published: May 18, 2018

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