Occipitocervical Hemolymphangioma in an Adult with Neck Pain and Stiffness: Case Report and Literature Review

Introduction Hemolymphangioma is an extremely rare malformation of the lymphatic and blood vessels. A limited number of hemolymphangioma cases occurring in the pancreas, extremities, spleen, and other organs have been reported until September 2017. To the best of our knowledge, no cases of hemolymphangioma in the occipitocervical region have been reported in the literature. Case Presentation We reported the case of a 23-year-old male patient with an occipitocervical lesion presenting atypically as neck pain and stiffness over a period of five months. Although hemolymphangioma has historically demonstrated a female predilection (2.25 : 1 female to male) and presentation in the third to fourth decades of life, this case is an atypical manifestation occurring in a young male patient. The clinical characteristics and management choices of this uncommon case of hemolymphangioma in the occipitocervical region are discussed, and a review based on the available literature is also presented. Conclusion Hemolymphangioma of the occipitocervical region is an uncommon presentation of a rare lesion. Although rare, hemolymphangioma should be considered a differential diagnosis for masses occurring in the occipitocervical region. Complete surgical resection is the treatment of choice and affords a good prognosis.


Introduction
Hemolymphangioma is a malformation of both the lymphatic and vascular systems. Clinically, the onset of hemolymphangioma can vary from a slowly growing cyst over a period of years to an aggressive enlarging tumor, without invasive ability [1]. A limited number of hemolymphangioma cases occurring in the pancreas, extremities, spleen, and other organs have been reported until September 2017 . To the best of our knowledge, the present study describes the rst case of hemolymphangioma in the occipitocervical region, which appears to be a rare site of presentation of a rare neoplasm and reviews the clinical characteristics and management choices of this condition based on the existing available literature. A 23-year-old man presented with a posterior occipitocervical subcutaneous mass that had been present since birth and had been growing for years. He complained of neck pain and sti ness for approximately 5 months, with no symptoms of extremity numbness or pain.

Case Presentation
ere was no history of trauma or weight loss and was no family history of cancer. Physical examination revealed a partially spherical lesion, compressible, with mild tenderness. Laboratory data showed no infectious or in ammatory ndings.
Magnetic resonance imaging (MRI) examination was performed preoperatively, in order to establish the extent of the tumor and de ne its association with the surrounding tissues. e occipitocervical MRI scan ( Figure 1) showed an approximate lesion measuring 11.2 × 3.5 × 6.9 cm with an irregular shape, unclear boundary, and uneven signal intensity on sagittal T2-weighted imaging (WI), dominantly high signal intensity on T2-WI fat suppressed, and markedly heterogeneous enhancement on coronal enhanced scan in the occipitocervical subcutaneous tissue. e patient was admitted for surgery. During the operation, the boundary of the mass was unclear. Macroscopically, the mass spaces between all mass measurements were 10.8 × 3.4 × 6.2 cm. It was polycystic and soft in consistency. Histopathological examination ( Figure 2) revealed abnormal lymphatic and blood vessels with polycystic spaces, thin wall, and dyed red lymph and blood cells within the lumen, which con rmed a diagnosis of hemolymphangioma. e postoperative course of the patient was uneventful, and the patient was discharged 2 weeks following the surgery. At 8 months of follow-up, he had no visible recurrence of the subcutaneous lesion and no evidence of neck pain and sti ness.

Literature Review
In preparation of this case report, a review of the existing medical literature was performed with the PubMed database, using the following keyword: "Hemolymphangioma" to identify all possible studies published up to September 2017. References from these articles were also reviewed. After carefully reviewing and summarizing each published article, we selected 25 original studies with 26 case reports . We nd this tumor typically appearing in adult patients ranging in age from 2 months to 62 years, with an average age of 31.4 years. Nearly 61.5% (16/26) of all cases occurred in patients over the age of 20. Hemolymphangioma is most common in female patients, with the sex distribution of male to female 1 : 2.25. e clinical manifestation is not typical, whereby it can be hidden for a long time.
e reported anatomic location of hemolymphangioma has been 23.1% (6/26) in the pancreas, 15.3% (4/26) in the extremities, 11.5% (3/26) in the spleen, and almost 69.2% (18/26) in internal or visceral locations. To date, no reports documenting recurrence have been reported after short-term follow-up. e clinical characteristics and management choices of all 26 patients with hemolymphangioma are shown in Table 1.

Discussion
Hemolymphangioma is thought to originate from the mesenchymal tissue [15] and typically found by palpation or with symptomatic compression of nearby anatomic structures [21]. It may be divided into primary and secondary lymphatic vascular tumors. e primary tumor is considered to be a congenital malformation of the lymphatic vascular system, possibly formed due to obstruction of the venolymphatic communication between dysembryoplastic vascular tissue and the systemic circulation. By contrast, the secondary tumor is caused by poor lymph drainage and lymphatic damage resulting from surgery or trauma [15,20].      Case Reports in Medicine is tumor is a benign hamartoma of blood and lymphatic vessels with a predilection for the pancreas, spleen, and lower extremity and, less commonly, in occipitocervical lesion. In general, hemolymphangiomas are large masses of varying sized cystic cavities with thin walls, having multiple thin septa and containing uid similar to hemorrhagic uid, and rarely of clear lymphatic nature. e tumor size varies due to the anatomical location and relationship to the neighboring tissues [15]. e majority of small tumors remain asymptomatic for a long period of time. As the tumor develops, discomfort occurs, which is mainly caused by surrounding or in ltrating the neighboring tissues or other major structures [15]. In the current case, the main complaints were neck pain and sti ness for approximately ve months, without symptoms of extremity numbness or pain. In clinical examinations, they are usually described as soft and compressible masses, loculated in pattern [20]. e histopathological examination suggests that the tumors often consist of abnormal lymphatic and blood vessels with polycystic spaces, and the thin-walled cystic lesion has connective septa covered by endothelium [7]. However, all cases in the literature had no abnormal laboratory ndings as did our patient. e impossibility to preoperatively de ne the histological type of the tumor explains the di culties to reach a correct di erential diagnosis. Biopsy should not be performed because of the high risk of massive bleeding. It is very important and crucial for radiologists to recognize these lesions and establish an accurate diagnosis so as to avoid a biopsy, which could cause severe hemorrhage [6]. In the present study, an occipitocervical MRI showed a lesion with irregular shape, unclear boundary, and uneven signal intensity on sagittal T2-WI, dominantly high signal intensity on T2-WI fat suppressed, and markedly heterogeneous enhancement on coronal enhanced scan in the occipitocervical subcutaneous tissue. ese observations may indicate the presence of a lower number of tortuous blood vessels and water-based substance in the lesion, which was then con rmed during surgery. Imaging examinations, including ultrasound, computed tomography, and MRI scans, are useful in order to con rm the diagnosis, identify the tumor nature, and observe its extension and association with the surrounding tissues, assisting the selection of the surgical strategy [20]. However, an accurate diagnosis of the tumor cannot be preoperatively established in spite of modern imaging techniques and can be postoperatively based on histological evidence.
Most researchers believe that hemolymphangioma is commonly a benign disease and has no invasive ability [1,2,[5][6][7][8][9][10][11][13][14][15][16][17][18][19][20][21][22][23]. But some studies recently found that it can invade the adjacent structures [3,4,12]. Toyoki et al. discovered that the tumor from pancreas invaded to the duodenum to cause the duodenal bleeding [3]. In 2009, Sun and colleagues reported that the giant tumor originated in pancreas, in ltrated the transverse mesocolon and greater omentum, and tightly adhered to duodenum and superior mesenteric artery [4]. Recently, Zhang et al. found multiple hemolymphangioma of the visceral organs and reported the retroperitoneal tumor extending to the left colon and small bowel mesentery [12]. e optimal treatment strategy for this lesion remains controversial. Surgical resection appears to be the most e ective treatment for hemolymphangioma, especially when the tumor increases in size and creates pressure on the surrounding tissues. To prevent recurrence, during surgery, a thorough radical resection may be necessary. Furthermore, with tumor adhesion or invasion to the surrounding organs, the removal of adjacent organs needs to be considered [3]. Tumor removal may also be associated with complications such as infection, stula, and hemorrhage [4,16]. Beninson et al. successfully treated hemolymphangioma in a neonate using a pressure dressing initially followed by combined compression therapy [9]. Wang and colleagues reported a case of successful treatment of hemolymphangioma of the tongue with a variable-pulse 595 nm pulsed-dye laser [23]. All cases in the existing literature had good clinical outcomes as did our case. e risk of recurrence seems very low, and no documented reports of recurrence have been reported in the literature. In addition, so far, no case of malignant transformation was reported. However, careful follow-up with MRI or ultrasound is recommended.

Conclusion
Hemolymphangioma of the occipitocervical region is an uncommon presentation of a rare lesion. Although rare, hemolymphangioma should be considered a di erential diagnosis for masses occurring in the occipitocervical region. Complete surgical resection is the treatment of choice and a ords a good prognosis.

Consent
Informed consent of the patient was obtained for publication of the case.