Hemolymphangioma involving bones and bladder detected on 68Ga-NEB PET/CT: A rare case report.

RATIONALE: Hemolymphangioma is a rare developmental defect of combined vasal and lymphatic vasculature. It is very rare that hemolymphangioma affects the bones and bladder simultaneously, and this condition has never been reported in PubMed. PATIENT CONCERNS: A 12-year-old male has a history of hospitalization for recurrent episodes of chyluria of 8 years duration and progressively worsening gross hematuria with right hip pain for 6 months. DIAGNOSIS: Chylous test of urine fluid was positive. There was no organic disease in the kidneys. Ga-NOTA-Evans Blue (NEB) positron emission tomography/computed tomography (PET/CT) images demonstrated clearly several round and strip-shaped low-density shadows with mildly increased radioactive uptake in both bladder wall and pelvis, including sacral, pubic, and ischial bones. Histopathological analysis of biopsy on pubic and ischial bones confirmed the diagnosis of hemolymphangioma. INTERVENTIONS: The patient received treatment with traditional Chinese medicine. OUTCOMES: At the 6-month follow-up visit, the patient's symptoms of chyluria, hematuria, and pain were all mitigated. LESSONS: Hemolymphangioma is a rare benign disease. Ga-NEB PET/CT is a specific method for the lymphatic system, and it might provide more accurate and comprehensive information about the disorder of the lymphatic system compared with CT and magnetic resonance imaging. When patients suffer from suspected lesions of the lymphatic system, Ga-NEB PET/CT might be recommended.

[Urological pathology of lymphatic origin]. / Patología urológica de origen linfático.

OBJECTIVES: Lymphatic disease is a rarely cause of some very unspecific genitourinary manifestations, assuming a diagnostic challenge in most cases. The aim of this paper is to warn about the possible etiology of these urological lymphatic presentations and discuss its management. METHODS: Retrospective review of clinical data in pediatric patients with urological pathology of lymphatic origin between 2008-2014. Three patients, two boys and a girl, were included. The complaints were: redundant prepuce, inguinal scrotal tumor and urinary incontinence. Clinical, diagnosis and treatment protocol is described. RESULTS: The first case is a male with genital lymphedema presented at birth as a redundant prepuce and the subsequent emergence of lower limb lymphedema, treated with compression bandages. The second case, a groin lymphangioma which debuted as a sudden painless inguinal tumor simulating a cord cyst, it was sclerosed and treated with OK- 432. Finally, a preteen who consulted for involuntary leakage of urine, genital lymphedema likely primary cause (lymphodisplasia) or malformation was diagnosed, which improved with two Lipiodol® embolization. CONCLUSIONS: Genitourinary manifestations can be the debut of more complex lymphatic pathology, so it is necessary to consider this cause, uncommon and nonspecific. Its management must be conservative initially by compression bandages, OK-432 or embolization with Lipiodol®.

OBJETIVOS: La patología linfática es infrecuente como causa de manifestaciones genitourinarias inespecíficas, suponiendo un reto diagnóstico en la mayoría de los casos. El objetivo de este trabajo es advertir sobre la posible etiología linfática de estas presentaciones urológicas y discutir su manejo. MATERIAL Y METODOS: Revisión retrospectiva de los datos clínicos de los pacientes pediátricos con patología urológica de origen linfático entre el año 2008-2014. Se incluyeron 3 pacientes, dos niños y una niña. Todos ellos se evaluaron para obtener un diagnóstico fiable. Los motivos de consulta fueron: prepucio redundante, tumoración inguino-escrotal e incontinencia urinaria. Se describe la clínica, el protocolo diagnóstico y el tratamiento de los mismos. CASOS CLINICOS: El primer caso se trata de un lactante varón con un linfedema genital que presentaba un prepucio redundante congénito y la aparición posterior de linfedema en el miembro inferior que es tratado con vendajes compresivos. El siguiente, un linfangioma inguinal que debutó con una tumoración inguinal indolora de aparición súbita simulando un quiste de cordón y tratado mediante esclerosis con OK-432. Por último, una preadolescente que consultó por escapes involuntarios de orina diagnosticada de linfedema genital de probable causa primaria (linfodisplasia) o malformativa que mejoró con dos embolizaciones de Lipiodol®. CONCLUSIONES: Las manifestaciones genitourinarias pueden ser el debut de una patología linfática más compleja, por ello es necesario tener presente esta etiología infrecuente e inespecífica. Su manejo inicialmente debe ser conservador, mediante vendajes compresivos, OK-432 o embolización con Lipiodol®.

Cryotherapy for massive vulvar lymphatic leakage complicated with lymphangiomas following gynecological cancer treatment.

Vulvar lymphatic leakage is a severe complication associated with gynecological cancer treatments. However, standard treatment strategies have not yet been determined. We encountered a rare case of a 76-year-old multiparous woman suffering from massive lymphatic fluid leakage from the entire vulva, and papules developed and were identified as lymphangiomas. A large amount of straw-colored discharge continued from all vulvar papules, which extended over the mons pubis. Nine years ago, the patient had undergone a radical hysterectomy with concurrent chemoradiation for uterine cervical cancer treatment. Her serum albumin level was 1.9 mg/dl, which was attributed to the loss of a large amount of lymph fluid due to leakage from the vulva. Her quality of life gradually decreased because of general fatigue and the need for frequent diaper exchanges every 2 h. The patient received a less-invasive treatment with cryotherapy using liquid nitrogen. She also received a multimodality treatment consisting of the intravenous administration of albumin, massage of the lower limbs and intensive rehabilitation. Cryotherapy was administered once a week for 3 months. Her discharge almost stopped and vulvar lymphangiomas decreased without any major complications. To the best of our knowledge, this is the first case report of massive lymphatic leakage complicated with vulvar lymphangiomas. Additionally, this case may represent the first successful treatment of vulva lymph leakage by cryotherapy without recurrence. Cryotherapy may have the potential to improve the quality of life as a less-invasive treatment for gynecological cancer survivors without serious complications.

Bipolar radiofrequency-induced interstitial thermoablation for oral cavity vascular malformations: Preliminary results in a series of 5 children.

We report our assessment of the effectiveness of bipolar radiofrequency-induced interstitial thermoablation (BRIT) for the treatment of certain oral cavity vascular malformations in 5 children. Two of these patients had lymphangiomatous macroglossia (LM), 1 had lymphangioma circumscriptum (LC), and 2 had a venous malformation (VM). Each patient underwent BRIT at least twice; treatment was delivered at 4- to 8-week intervals according to each patient's circumstances. The 2 patients with LM required three treatment sessions; although their tongue volume decreased after each session, both still required a partial glossectomy to achieve a satisfactory reduction in volume. The patient with LC underwent two BRIT treatments, which reduced the size of the lesion by half; the remainder was excised. The 2 patients with a VM (1 buccal and 1 lingual) responded well to BRIT, and their malformations almost completely disappeared. Our early results with BRIT suggest that it is an effective treatment for oral cavity vascular malformations-more so for patients with venous rather than lymphangiomatous lesions.

Infected giant lymphangioma circumscriptum treated with a combination therapy.

Lymphangioma circumscriptum is an uncommon benign disorder of skin and subcutaneous tissues characterized by dilated lymphatic channels. It is an uncommon vascular tumor and it rarely becomes infected. We report a 20-year-old man who had an infected giant tumor in his left thigh. After an extensive resection and radiofrequency energy therapy, he recovered well with an acceptable cosmetic result. We followed up the patient for 2 years without any recurrence.

Lymphatic malformations: a proposed management algorithm.

OBJECTIVE: The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors' experience in managing these lesions as well as current literature on the subject. STUDY DESIGN AND METHODS: A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998-2008) was performed. DATA COLLECTED: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved. RESULTS: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used. CONCLUSION: Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations.

Treatment of lymphangioma circumscriptum with the intense pulsed light system.

Lymphangioma circumscriptum is a superficial lymphatic malformation presenting with grouped translucent vesicles that often contain blood. In the past, several treatment modalities have been described. Here, we report for the first time on treatment with an intense pulsed light source that led to good cosmetic results.

Tratamiento del linfagioma en niños con fraciones ribosómicas bacterianas / Treatment of lymphangioma in children with bacterial ribosomal fractions

Introducción: el propósito del presente estudio fue demostrar la efectividad del extracto de las fracciones ribosómicas bacterianas como tratamiento del linfangioma en niños. Material y métodos: se realizó un análisis prospectivo de enero de 2003 a enero de 2006 de los pacientes que consultaron con diagnóstico de linfangioma en quienes se administró intracavitariamente extracto de fracciones ribosómicas bacterianas como único régimen terapéutico. Se aplicaron de 1 a 3 infiltraciones seriados con un intervalo de 1 mes entre cada uno...

Resection of an orbital lymphangioma with the aid of an intralesional liquid polymer.

A 28-year-old man with long-standing right proptosis presented with an extensive multilobulated partially cystic orbital mass thought to be a lymphangioma. Because of concern that excision or debulking of the lesion was likely to be complicated by excessive bleeding, the lesion was injected with a mixture of ethiodized oil (Ethiodol) and cyanoacrylate glue under direct observation. The mixture caused the injected lobules to assume a firm, rubbery texture, allowing them to be excised without bleeding.

Temperature mapping of magnetic resonance-guided laser interstitial thermal therapy (LITT) in lymphangiomas of the head and neck.

BACKGROUND AND OBJECTIVE: Lymphangiomas of the tongue and neck are uncommon benign congenital lymphatic tumors. These vascular lesions are difficult to treat, frequently recur, and can cause patients significant morbidity. Treatment may also be complicated by adjacent vital anatomic structures. Magnetic resonance (MR)-controlled laser-induced interstitial thermotherapy (LITT) has been proven to be a noninvasive safe treatment. Real-time monitoring of tissue temperature with thermosensitive sequences allows controlled coagulation necrosis. STUDY DESIGN/MATERIALS AND METHODS: LITT was performed in a lymphangioma specimen ex vivo. In four patients (eight procedures) with lymphangiomas of the tongue and neck, MR-guided LITT was performed with a percutaneous approach in a multiapplicator technique. The laser system consisted of a titanium catheter and a protective catheter. The dome of the fiber end had a diameter of 1.4 mm with an active length of 20 mm. Temperature sensitive sequences were used in a 0.5 T open-configured MR scanner with the proton frequency shift technique to map the spatial and temporal distribution of Nd:YAG laser effects (7 Watts, 30 pulses per second, 10 minutes/location). Postoperative MR follow-up was performed at 1 week and at 3 months. In three patients, partial resection of the tumor was performed 6 months after LITT. RESULTS: In three patients, MR clearly showed a diminished tumor volume. All four patients reported subjective amelioration and in three patients former functional problems, such as speech and swallowing were improved. MR thermometry allowed accurate demarcation of changes by heat and distinction of affected tumor volume (3.0 cm +/- 0.3 cm). The histology of the patients 6 months after LITT showed laser-induced fibrosis of former lymphatic tissue. CONCLUSION: The results suggest that LITT can be performed safely with tissue preserving of vital structures and can be effective in the treatment of deep tumors, such as lymphangiomas. However, given the nature of the lesion, the potential for recurrence exists no matter what modality is chosen.

Percutaneous sclerotherapy of lymphangiomas.

PURPOSE: The authors report their experience with percutaneous image-guided sclerotherapy for treatment of unresectable lymphangiomas. MATERIALS AND METHODS: Five patients with unresectable lymphangiomas of the pelvis (n = 2), neck (n = 1), abdomen (n = 1), or leg (n = 1) were treated at two medical centers with sclerotherapy, with use of doxycycline as the sclerosant. Computed tomography was used to guide the procedures, with supplemental lymphoscintigraphy, ultrasound, and magnetic resonance imaging used as needed. RESULTS: Symptomatic relief of lymphedema, lymphorrhea, or a decrease in size of lymphatic pools was achieved, to varying degrees, in the five patients. Follow-up is ongoing, and further sclerotherapy may be indicated as the clinical course dictates. CONCLUSION: Percutaneous sclerotherapy with doxycycline is safe and appears effective for palliative treatment of unresectable lymphangiomas on the basis of our initial clinical experience.

The lymphatic system. Some surgical considerations.

This article on the lymphatics was undertaken for three reasons: The first is to recount the story of the rediscovery of these vessels in the 17th century and briefly review the subsequent events leading up to our present knowledge of the lymphatic system. The second is to emphasize the role of the lymphatics in maintaining extracellular fluid balance, in the removal of protein, fat, and other substances of large molecular size from the tissue spaces, and in the circulation of the lymphocytes from their germinal centers and storage depots to all parts of the body via lymphaticovenous connections. The third reason is to suggest that the responsibility for maintaining the transport function of the lymphatics properly belongs to the vascular surgeon.