Occipitocervical Hemolymphangioma in an Adult with Neck Pain and Stiffness: Case Report and Literature Review.
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 . A limited number of hemolymphangioma cases occurring in the pancreas, extremities, spleen, and other organs have been reported until September 2017 [1-25]. To the best of our knowledge, the present study describes the first 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.
2. Case Presentation
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 stiffness for approximately 5 months, with no symptoms of extremity numbness or pain. There 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 inflammatory findings.
Magnetic resonance imaging (MRI) examination was performed preoperatively, in order to establish the extent of the tumor and define its association with the surrounding tissues. The occipitocervical MRI scan (Figure 1) showed an approximate lesion measuring 11.2 x 3.5 x 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.
The 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 x 3.4 x 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 confirmed a diagnosis of hemolymphangioma. The 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 stiffness.
3. 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 [1-25]. We find 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. The clinical manifestation is not typical, whereby it can be hidden for a long time. The 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. The clinical characteristics and management choices of all 26 patients with hemolymphangioma are shown in Table 1.
Hemolymphangioma is thought to originate from the mesenchymal tissue  and typically found by palpation or with symptomatic compression of nearby anatomic structures . It may be divided into primary and secondary lymphatic vascular tumors. The 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].
This 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 fluid similar to hemorrhagic fluid, and rarely of clear lymphatic nature. The tumor size varies due to the anatomical location and relationship to the neighboring tissues . The 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 infiltrating the neighboring tissues or other major structures . In the current case, the main complaints were neck pain and stiffness for approximately five months, without symptoms of extremity numbness or pain. In clinical examinations, they are usually described as soft and compressible masses, loculated in pattern . The 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 . However, all cases in the literature had no abnormal laboratory findings as did our patient.
The impossibility to preoperatively define the histological type of the tumor explains the difficulties to reach a correct differential 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 . 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. These observations may indicate the presence of a lower number of tortuous blood vessels and water-based substance in the lesion, which was then confirmed during surgery. Imaging examinations, including ultrasound, computed tomography, and MRI scans, are useful in order to confirm the diagnosis, identify the tumor nature, and observe its extension and association with the surrounding tissues, assisting the selection of the surgical strategy . 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, 5-11, 13-23]. 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 . In 2009, Sun and colleagues reported that the giant tumor originated in pancreas, infiltrated the transverse mesocolon and greater omentum, and tightly adhered to duodenum and superior mesenteric artery . 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 .
The optimal treatment strategy for this lesion remains controversial. Surgical resection appears to be the most effective 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 maybe necessary. Furthermore, with tumor adhesion or invasion to the surrounding organs, the removal of adjacent organs needs to be considered . Tumor removal may also be associated with complications such as infection, fistula, and hemorrhage [4, 16]. Beninson et al. successfully treated hemolymphangioma in a neonate using a pressure dressing initially followed by combined compression therapy . Wang and colleagues reported a case of successful treatment of hemolymphangioma of the tongue with a variable-pulse 595 nm pulsed-dye laser . All cases in the existing literature had good clinical outcomes as did our case. The 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.
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.
Informed consent of the patient was obtained for publication of the case.
Conflicts of Interest
The authors declare that there are no conflicts of interest regarding the publication of this paper.
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Yongchao Li, (1,2) Xing Zhang, (1) Xiaodong Pang, (2) Liang Yang, (2) and Baogan Peng (2)
(1) Department of Orthopedics, Gaoyou People's Hospital Affiliated to Soochow University, Yangzhou, Jiangsu 225600, China (2) Department of Spinal Surgery, General Hospital of Armed Police Force, Beijing '00039, China
Correspondence should be addressed to Baogan Peng; firstname.lastname@example.org
Received 1 September 2017; Accepted 18 October 2017; Published 7 December 2017
Academic Editor: Mark E. Shaffrey
Caption: FIGURE 1: MRI demonstrated a mass (arrows) in the occipitocervical subcutaneous tissue with uneven signal intensity on (Figure 1(a)) sagittal T2-weighted, (Figure 1(b)) T2-weighted fat suppressed, and (Figure 1(c)) coronal enhanced images.
Caption: FIGURE 2: Histological analysis of specimen (hematoxylin and eosin stain; magnification x100) showed abnormal lymphatic (black arrowheads) and blood vessels (black stars) with polycystic spaces.
TABLE 1: The clinical characteristics and management choices of 26 patients with hemolymphangioma [1-25]. Case Publication Age Localization Size (cm) year (y)/sex 1 1979 20/F Lower extremity NA 2  1988 1.5/F Lower extremity 4.0 x 5.0 3  1993 NA/M Abdomen 13.0 4  1996 2/F Esophagus 9.5 x 2.5 x 2.0 5  2003 53/F Pancreas 4.0 x 3.0 6  2005 15/F Tongue NA 7  2008 53/M Pancreas NA 8  2009 20/F Pancreas 18.0 x 16.0 x 12.5 9  2010 0.16/F Lower extremity 4.5 10  2010 5/M Lower extremity 2.1 x 1.8 x 0.5 11  2012 57/F Chest wall 9.0 x 9.0 x 5.0 12  2012 57/F Small intestine 5.0 x 4.0 13  2013 53/F Stomach 1.5 14  2013 37/M Rectum 20.0 x 8.0 x 8.0 15  2013 39/F Pancreas 10.0 x 7.0 16  2014 24/F Duodenum 4.0 x 1.5 17  2014 52/F Pancreas 8.0 x 6.5 x 6.0 18  2014 12/M Spleen 15.7 x 8.5 19  2015 57/F Pancreas 8.0 x 6.0 x 4.5 20  2015 62/F Spleen 11.0 x 6.0 x 3.0 21  2015 17/M Waist 12.0 x 6.0 x 6.0 22  2015 25/F Spleen, 28.0 x 24.0 x 15.0 retroperitoneum 23  2015 15/F Para spinous NA 24  2016 48/F Posterior 3.1x2.4 mediastinum 25  2016 3/M Greater omentum 20 x 15 x 6 26  2017 57/M Rectum, sigmoid 25 Case Chief complaint Treatment 1 Painful swelling, chest Urokinase for a short pain, and pulmonary embolism period; prolonged maintenance therapy was with a low dose of heparin 2  Not reported Pressure dressing and combined compression therapy 3  Not reported Autopsy 4  Acute dyspnea Endoscopically 5  Abdominal pain and weight Pancreatoduodenectomy loss 6  Pain and bleeding Pulsed-dye laser 7  Severe anemia due to Pylorous-preserving gastrointestinal bleeding pancreatoduodenectomy 8  Epigastric discomfort Pancreatoduodenectomy 9  Asymptomatic Surgical excision 10  Asymptomatic Surgical excision 11  Chest tightness, shortness Thoracotomy of breath, cough, and expectoration 12  Recurrent melena Partial resection of the small intestine 13  Bloody vomiting and Endoscopic therapy epigastric pain 14  Rectal bleeding and tenesmus Low anterior resection of the rectosigmoid colon with handsewn transanal coloanal anastomosis 15  Abdominal pain Pancreatic body-tail resection combined with splenectomy 16  Severe anemia Surgical excision 17  Abdominal pain and Pylorus preserving epigastric discomfort pancreatoduodenectomy 18  Abdominal pain Laparoscopic partial splenectomy 19  Epigastric discomfort Surgical excision 20  Abdominal pain Total splenectomy 21  Back pain Surgical excision 22  Progressive splenomegaly Splenectomy 23  Scoliosis Posterior spinal fusion 24  Shortness of breath and Thoracic surgery chest tightness 25  Abdominal pain Surgical excision 26  Rectorrhagia Surgical excision Case Follow-up Recurrence Evolution (months) 1 NA NA Favourable 2  9 Not reported Favourable 3  NA NA Stillborn 4  7 Not reported Favourable 5  NA NA Favourable 6  10 Not reported Favourable 7  NA Not reported Favourable 8  26 Not reported Favourable 9  12 Not reported Favourable 10  12 Not reported Favourable 11  3 Not reported Favourable 12  12 Not reported Favourable 13  NA NA Favourable 14  12 Not reported Favourable 15  NA Not reported Favourable 16  NA Not reported Favourable 17  NA NA Died 18  12 Not reported Favourable 19  2 Not reported Favourable 20  12 Not reported Favourable 21  7 Not reported Favourable 22  NA NA Favourable 23  12 Not reported Favourable 24  12 Not reported Favourable 25  6 Not reported Favourable 26  6 Not reported Favourable NA, the data were not available.
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|Title Annotation:||Case Report|
|Author:||Li, Yongchao; Zhang, Xing; Pang, Xiaodong; Yang, Liang; Peng, Baogan|
|Publication:||Case Reports in Medicine|
|Article Type:||Case study|
|Date:||Jan 1, 2017|
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