|Ahead of print publication
Mesenteric cystic lymphangioma – A rare entity with unusual presentation
Merin Jose, Shirish Chandanwale, Madhuri Singh, Akshi Raj, Charusheela Gore
Department of Pathology, Dr. D. Y. Patil Medical College, Pune, Maharashtra, India
|Date of Submission||30-Jul-2020|
|Date of Decision||30-Jul-2020|
|Date of Acceptance||03-Jun-2021|
Tower 21 Flat 404, Blue Ridge Township, Hinjewadi Phase 1, Pune - 411 057 Maharashtra
Source of Support: None, Conflict of Interest: None
Lymphangiomas are a rare benign proliferation of the lymphatic system. Intra-abdominal lymphangioma is rare and accounts for <5% of lymphangioma cases. Cystic mesenteric lymphangioma is extremely rare and accounts for <1% of abdominal lymphangiomas. Abdominal swelling, pain, vomiting, and constipation are common clinical presentations. We report a case of a 5-year-old boy with no significant medical history of illness who presented with acute abdomen. Preoperative accurate diagnosis by radiological modalities is very difficult. Surgical excision is the choice of treatment. Definitive diagnosis requires histopathological examination.
Keywords: Cystic, lymphangioma, mesenteric
|How to cite this URL:|
Jose M, Chandanwale S, Singh M, Raj A, Gore C. Mesenteric cystic lymphangioma – A rare entity with unusual presentation. Med J DY Patil Vidyapeeth [Epub ahead of print] [cited 2021 Dec 6]. Available from: https://www.mjdrdypv.org/preprintarticle.asp?id=321295
| Introduction|| |
Lymphangiomas are a rare benign proliferation of the lymphatic system consisting of multiple cystic spaces lined by endothelium. Although they can occur in adults, they are commonly seen in children in the head, neck, and axilla. Intra-abdominal lymphangioma is very rare, with a reported incidence of 1:100,000 and 1:20,000 admissions in adults and children. Mesenteric lymphangioma is extremely rare and accounts for <1% of abdominal lymphangiomas. Although they can be asymptomatic and detected incidentally, symptoms occur only after enlarging. Common symptoms include palpable abdominal mass, abdominal pain, fever, fatigue, weight loss, and hematuria. They are often confused with other retroperitoneal cystic tumors arising from the kidney, liver, and pancreas.
Although this condition is known for long time, there is still controversy regarding its origin, classification, and pathology. Cystic lymphangioma is important and is a difficult diagnosis in children. Although imaging studies such as ultrasonography (USG), computed tomography, and magnetic resonance imaging (MRI) are used for diagnosis, preoperative diagnosis is often difficult. Definitive diagnosis is often made by histopathological examination of excised specimen. We report one such case of mesenteric lymphangioma in a 5-year-old boy who presented with acute abdominal pain.
| Case Report|| |
A 5-year-old boy presented with complaints of severe pain in abdomen, fever, and vomiting for the past 2 days. There was no history of trauma or any other significant medical or surgical history. He had normal birth and developmental milestones. Physical examination revealed abdominal distension, hyperactive bowel sounds, and tenderness on palpation around paraumbilical region and in the right iliac fossa.
A USG of the abdomen revealed a well-defined thin-walled multiloculated anechoic cystic lesion extending from right suprapubic region crossing the midline up to left paraumbilical region, anterior to left psoas and aorta. It was separate from the bowel loops and showed multiple moving echoes and septations within. No internal vascularity was noted.
MRI of the abdomen scan confirmed the presence of lobulated multi-septate near-fluid intensity lesion measuring 72 mm × 23 mm × 123 mm (TRA × AP × CC) in the etroperitoneum on the right side of the abdomen in proximity to ascending colon [Figure 1]a. A clinical diagnosis of retroperitoneal cystic lesion was made.
|Figure 1: (a) Magnetic resonance imaging of abdomen, (b) gross image of cystic lymphangioma|
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Surgical excised specimen of the cyst along with a small part of the small bowel was received for histopathological examination. External surface was lobulated and showed few congested vessels [Figure 1]b. On cutting the cystic lesion, blood-tinged hemorrhagic fluid came out. Multiple tissue sections were formalin-fixed and paraffin processed. 3–4 thick μsections were cut and stained with hematoxylin and eosin.
Histopathological examination showed multiple cystic spaces of varying sizes containing eosinophilic lymph separated by fibrous septa containing few congested capillaries and lymphocytes. Cystic spaces were lined by flattened endothelial cells [Figure 2]a and [Figure 2]b. Immunohistochemical (IHC) staining showed reactivity with CD 31 and CD 34 [Figure 2]c and [Figure 2]d which confirmed the nature of endothelial cells. HMB 45 and calretinin did not show any immunoreactivity.
|Figure 2: (a) Cystic spaces separated by thin fibrous septa (H and E, ×100), (b) cystic spaces lined by flattened endothelium and contain lymph (H and E, ×400), (c) immunohistochemistry, endothelial cells (CD31 positive, ×100), (d) immunohistochemistry, endothelial cells (CD34 positive, ×400)|
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| Discussion|| |
Most lymphangiomas represent malformations rather than true neoplasm and are thought to result from failure of the lymphatic system to communicate with venous system. They are believed to originate from the early sequestration of lymphatic vessels during the embryonic period that fail to establish connections with normal draining lymphatics, and therefore, they are considered a congenital rather than acquired. However, there are some reports which suggest that inflammation, abdominal trauma, abdominal surgery, radiation, or lymphatic obstruction may play a role in genesis. Occasionally, they can be discovered in adults at various locations.
Intra-abdominal lymphangioma is rare and accounts for <5% of lymphangioma cases. In abdomen, the most common location includes small bowel mesentery (70%) with 50%–60% located in the ileal mesentery. Other locations include the omentum, retroperitoneum, mesocolon, pancreas, spleen, and adrenal gland.
Clinical manifestations are determined by the size and location of lymphangiomas. There are no pathognomonic signs and symptoms of mesenteric lymphangioma. Many reports suggest abdominal swelling, pain, vomiting, and constipation as common clinical presentations. Very rarely, it presents as acute abdomen. Our case was a 5-year-old boy with no significant medical history of illness who presented with acute abdomen.
Radiological investigations suggested a retroperitoneal cystic lesion. USG findings of cystic lesion with multiple thin septa have been described in mesenteric lymphangioma. These findings are insufficient in acute preoperative diagnosis.
They are categorized into capillary, cavernous, and cystic. Losanoff et al. classified them into pedicled, sessile retroperitoneal extended or multicentric which may help in treatment strategy. Our case was a cystic swelling without pedicle.
Cystic lymphangioma is often needed to be differentiated from cystic mesothelioma and lymphangiomyoma. Histological examination of the cyst in our case showed multiple cystic spaces lined by flattened epithelial cases and separated by fibrous stroma containing few congested capillaries. Some spaces contain lymph. Positive IHC staining of the lymphatic endothelium with CD31 and CD34 confirmed the diagnosis of lymphangioma. Negative immunostaining with calretinin and HMB45 ruled out multicystic mesothelioma and lymphangiomyoma, respectively.
Earlier cystic lymphangioma was considered to be neoplastic in nature with aggressive behavior. Many investigators believe now that they are hamartomas, while others believe that both are neoplastic and hamartomatous in nature. Fibrosis of lymphatic channels following surgeries and infections has been postulated to be the underlying pathogenesis.
Complications include mass effect on adjacent structures, rupture, secondary infection, volvulus, and intestinal obstruction.
Various treatment modalities such as treatment with OK-432, steroids, bleomycin, drainage of lymphangioma, and instillation sclerotherapy have been tried. There is often risk of recurrence and perforation. However, surgical excision is a treatment of choice. The treatment is mainly surgical; it consists of enucleation when feasible; the segmental intestinal resection is achieved when the cyst adheres intimately to the bowel. Prognosis is excellent if cyst resection is complete.
There were no intraoperative and postoperative complications in our patient, and the patient is on clinical follow-up.
| Conclusion|| |
Cystic mesenteric lymphangioma is a benign proliferation of lymph vessels and is extremely rare. Clinical manifestations depend on the size and location of lymphangioma. Presentation with acute abdomen is very rare. Preoperative accurate diagnosis by radiological modalities is very difficult. Surgical excision is the choice of treatment. Definitive diagnosis is made by histopathological examination.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]