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Abstract: Lymphangioma circumscriptum (LC) is a rare benign condition, with marked dilation of surface lymphatic vessels in the deep and subcutaneous layers. Vulvar LC can become a highly disabling condition with vulvar discomfort, itching, burning and lymph seeping being the dominant symptoms. Biopsy is mandatory for the diagnosis. There is no consensus on the standard treatment for vulvar LC and recurrence is frequent. In complex cases with wide disease location, combination of different treatment options, such as abrasive methods and surgery, may lead to the best clinical and aesthetical result, with extended disease-free periods. We present a patient with a long history of Crohn disease with multiple pelvic surgeries who developed an extensive vulvar LC., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2023, ASCCP.)

Introduction: Lymphangioma, a rare benign tumor of the lymphatic system, is called lymphangiomatosis when it involves >1 organ, which is more rarely complicated with thoracic obstruction, the relationship between them remains unclear. With the development of enteroscopy, clinicians know more about small intestinal lymphangioma and attempt to treat it through enteroscopic injection sclerotherapy(EIS)., Patient Concerns: A 59-year-old male firstly manifested with gastrointestinal bleeding after a gastric perforation, who was diagnosed with lymphangiomatosis by balloon-assisted enteroscopy and abdomen CT showing >1 organ with multiple cysts besides the small intestine. The patient received an EIS, then the melena disappeared. Surprisingly he came back because of refractory ascites confirmed to be chylous by chemical tests 7 months later., Diagnosis: Lymphangiography could not determine the location of lymphatic leakage, Ultrasonography showed stenosis of the left cervical part of the thoracic duct., Intervention: On the condition that medical treatment is ineffective, thoracic duct exploration and lysis of fibrous adhesion were performed., Outcomes: Ascites significantly reduced at last., Lessons: Lymphangiomatosis is the malformation of the lymphatic system involving multiple organs, it has a possibility to be associated with thoracic obstruction. Capsule endoscopy and enteroscopy are effective methods to diagnose small intestinal lymphangioma, and EIS is an effective therapy., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Metastasis of a papillary thyroid microcarcinoma (PTMC) in the lateral neck is characterized primarily by solid lymphadenopathy, although some cases may rarely present with a cervical cystic mass. We report a case of lateral cervical lymph node metastases of PTMC that appeared as a cystic lymphangioma of the lateral neck., Patient Concerns: A 55-year-old man with a painless egg-sized mass in the right side of the neck that had been present for 1 month underwent physical examination, ultrasonography, computed tomography (CT), fine needle aspiration biopsy (FNAB), and intraoperative fast-frozen pathological examination, which indicated that the cystic masses in the neck were benign. However, the final pathology report identified the lateral neck masses as lymph node metastases of thyroid carcinoma., Diagnosis: The patient was diagnosed with PTMC of the right lobe of the thyroid gland with lateral neck metastases., Interventions: The patient underwent right cervical neck dissection together with a right thyroidectomy, followed by levothyroxine therapy and routine follow-up., Outcomes: No postoperative complications were reported, and the thyroid-stimulating hormone inhibition target was <0.1 mmol/L; there was no detectable tumor recurrence on routine clinical follow-up for up to 16 months., Conclusions: This case report emphasizes the need to consider cervical lymph node metastases of thyroid carcinoma in the differential diagnosis for patients with large, multiple, simple cystic neck masses., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Hemolymphangioma is a benign tumor comprised of the newly-formed lymph spaces and blood vessels, which can usually be found in the head and neck of the affected children. There are few reports regarding cases with hemolymphangioma in small intestine, spleen, esophagus, and other organs., Patient Concerns: Herein, a 55-year-old woman was presented in this study, she had complained of discomfort in the right upper abdomen for 2 months, and was discovered with a space-occupying lesion in proximal jejunum on computed tomography (CT). Eventually, the lesions were confirmed through double-balloon enteroscopy (DBE) to be located in the jejunum 60 cm away from the Treitz ligament., Diagnose: Subsequently, the small intestine was partially resected, and postoperative pathology had confirmed the diagnosis of small intestinal hemolymphangioma., Interventions: Excisional surgery of the lesion was planned. On surgery, the lesions were discovered to be about 33 cm to 22 cm when engorged the superficial vessels. No enlarged lymph nodes were seen at the root of the mesentery, and no obvious lesion was observed in the remaining small intestine., Outcomes: Follow-up for 6 months showed no recurrence., Lessons: Hemolymphangioma lacks typical clinical symptoms, and the correct preoperative diagnosis of hemolymphangioma remains challenging. Due to the increasing use of endoscopic diagnostic techniques, it is expected that hemolymphangioma in gastrointestinal tract may be detected and endoluminal located before surgery more feasibly. This case report aimed to highlight the contributions of CT and DBE to an accurate preoperative diagnosis and surgical strategy planning.

Cutaneous lymphangioma circumscriptum (CLC) is an uncommon congenital lymphatic malformation. Its dermoscopic features have been reported, however, if blood has infiltrated the lacunas, a pink hue overwhelms the lacunar structures, rendering dermoscopic features indistinguishable. In addition, dermoscopy cannot assess the subcutaneous extent of the lesion before surgery. The high-frequency array transducer Sonography with a high-frequency transducer is excellent in its resolution to evaluate skin lesions and is unaffected by infiltration of blood. Here, the authors report the use of ultrasound (color Doppler and pulse wave Doppler) for the diagnosis and management of CLC.An 18-year-old man presented 10 years previously with an asymptomatic 0.5 × 0.7 cm cluster of dark reddish vesicular lesions on his buttock. The lesions had recently increased in size and number. There was associated pachyderma. The lesions were hyperechoic and well defined on grayscale ultrasound and hypervascular on color Doppler ultrasound. Cluster cystics of lymphatic spaces were found throughout the whole dermis, especially in the papillary dermis. The lymphatic channels extended downwards to the reticular dermis and the superficial layer of the subcutaneous tissue. The pathological findings revealed lymphangiomas invading the subcutaneous tissue. The flat endothelial cells were partly positive for D2-40, which is a marker of lymphatic endothelial cells. Ultrasound was able to delineate the margins of the lesions. Diagnosis of CLC using preoperative color Doppler and pulse wave Doppler could reduce unnecessary repeated pre- and postoperative biopsies.

Lymphangiomas are benign hamartomatous tumors which are characterized by abnormal proliferation of lymphatic vessels. They are lymphatic malformations which are commonly localized to head and neck region. They are rare located in the oral cavity and tongue, lips, buccal mucosa, soft palate, floor of the mouth are mostly affected.

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.

We report a case of a 29-year-old woman with a pancreatic lymphangioma who presented clinically as a case of acute pancreatitis. Lymphangiomas are benign tumors of vascular origin with lymphatic differentiation, most commonly found in the head and neck. Pancreatic lymphangiomas are extremely rare, accounting for only 1% of abdominal lymphangiomas, with approximately 60 cases reported in the literature. Although imaging findings are characteristic and can point to the diagnosis, confirmation with fine needle aspiration and histopathologic correlation is necessary. Although these lesions are benign, they can often present a diagnostic dilemma and can be mistaken for other cystic pancreatic lesions, namely, pseudocysts, cysts, cystadenomas, and cystadenocarcinomas.

Rationale: Lymphangiomatous polyps of the palatine tonsils are benign tumors that are rare in both adults and children. Most patients suffering from this disease present with nonspecific symptoms similar to those of chronic tonsillitis., Patient Concern: We report a case of a 21-year-old male miner who presented with a chronic history of a foreign body sensation in the oropharynx and an intermittently sore throat., Diagnosis: The patient was preoperatively diagnosed with the palatine tonsils neoplasm., Interventions: The neoplasm with palatine tonsils was completely resected under general anesthesia. The tissue was sent for histological examination, and the diagnosis was lymphangiomatous polyps of the palatine tonsils., Outcome: The surgical outcome was good, and no surgical site infection was recorded. After 12 months of follow-up, the miner was asymptomatic with no recurrence., Lessons: Tonsillectomy is a curative method to address lymphangiomatous polyps (LAPs) of the tonsils which resulted in no recurrence during the clinical follow-up period. The etiology of this rare disorder and potential pathogenesis should be studied in the future, which would help prevent its occurrence.

Rationale: Hemolymphangioma of the pancreas is an extremely rare benign tumor; only 10 patients with this disease have been reported to date, the majority of whom were women., Patient Concerns: We describe a 28-year-old man who presented with abdominal pain and discomfort. Computed tomography and magnetic resonance imaging data showed a huge heterogeneous solid cystic mass at the retroperitoneal pancreatic head. The maximum cross-sectional lengths of the pancreatic lesion were approximately 12 × 8.5 × 12 cm., Diagnosis: Hemolymphangioma of the pancreas., Interventions: The patient underwent a pylorus-preserving pancreatoduodenectomy; surgical excision is the main treatment for this type of tumor., Outcomes: The patient followed up regularly in the outpatient department for 6 months after surgery, and no sign of recurrence was found., Lessons: Although it is uncommon, clinicians ought to consider the diagnosis of hemolymphangioma of the pancreas upon detection of a pancreatic cystic lesion exhibiting fat or calcification.

Rationale: Hemolymphangioma is a rare, noninvasive benign tumor of mesenchymal origin resulting from malformation of vascular and lymphatic vessels. The incidence of hemolymphangioma in the spinal canal is low., Patient Concerns: This report describes 2 patients with a lesion located in the thoracic spinal canal or spinal epidural space, who were misdiagnosed with suspected meningioma or suspected schwannoma, respectively, based on magnetic resonance imaging (MRI)., Diagnoses: Hemolymphangioma., Interventions: The application of a surgery was designed to treat the 2 patients., Outcomes: 2 patients stated that symptoms were improved after the operation., Lessons: This report should raise awareness among clinicians that careful image analysis and consideration of patient history and pathology is required for accurate differential diagnosis of hemolymphangioma in the spinal canal and spinal epidural space., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Hemolymphangioma is a rare vascular developmental error. It comprises malformed venous and lymphatic component in various proportion. To the best of our knowledge, only a few cases have been reported in the literature so far. Here, we report a case of huge intraperitoneal cystic mass in a 3-year-old boy that was presented to hospital with intractable abdominal pain. On examination, he had fever along with associated symptoms like cough and sputum. Abdomen was distended with no tenderness or rebound tenderness. On computed tomography scan, huge cystic mass was seen and was diagnosed as intraperitoneal benign cystic lesion. Excisional surgery of the lesion was planned. On surgery, lesion was found to be originated from greater omentum and no adhesion was seen in surrounding tissue. Complete excision of the lesion was done. Histopathological specimen after surgery suggested it to be hemolymphangioma. Follow-up for 6 months showed no recurrence.Hemolymphangioma of the greater omentum is benign tumor and accurate diagnosis before surgery is still a challenge. Presentation of disease may vary from simple well-defined cystic lesion to aggressive ill-defined lesion mimicking malignancy. Therefore, further research is needed to help doctor with preoperative radiological diagnosis and avoid unnecessary radical surgery., Competing Interests: The authors have no funding and conflicts of interest to disclose.

Surgical removal of head-neck vascular neoplasms may have severe complications, although surgery remains a very important treatment modality. One of the most important aspects is to avoid the rupture of the tumor's capsule and to minimize functional complications, especially in the excision of benign tumors. The transoral robotic surgery allows removing lesions in challenging anatomical areas. Furthermore, the current robotic instrumentation does not allow a fine dissection of the structures and capsule rupture is a potential event. Here, the authors describe a minimally invasive technique using transoral robotic surgery for removal of vascular tumors in head-neck. Particular attention was given to the description of the diagnostic work up, the preoperative assessment and the surgical steps. No complications were encountered postoperatively. Transoral robotic surgery for vascular tumors appears to be a safe and feasible technique with minimal complications.

Objective: To add a new case of primary vulvar lymphangioma circumscriptum to the literature and to review the current literature., Materials and Methods: We searched the PubMed/MEDLINE databases for previous case reports using the key words "vulvar lymphangioma circumscriptum" and "primary vulvar lymphangioma circumscriptum.", Results: We found 21 cases of primary vulvar lymphangioma circumscriptum reported until 2013. Mean age was 32 years (range = 3-76 y), and 62 % of the cases had been treated surgically., Conclusions: The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy.

Oropharyngeal and tonsillar masses are a very rare cause of dysphagia in children. In this article, we describe a rare cause of dysphagia in children, a lymphangiomatous lesion of the palatine tonsil, and discuss the clinical and histopathologic features of these lesions.

Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.

Vulvar lymphangioma circumscriptum (LC) is an unusual benign condition, congenital or acquired, related to interference in the lymph drainage. Acquired cases are usually related to oncological surgical procedures. We report 6 cases of vulvar LC: 3 have been caused by surgery and radiotherapy for gynecologic cancer, whereas the other 3 have originated under benign conditions--Crohn disease, recurring episodes of cellulits, and lower limb lymphedema. In 3 of them, the initial clinical diagnosis was genital wart. Surgical exeresis was performed as the treatment of choice in 5 patients and there were 3 cases of recurrences. Diagnosis of this unusual condition is not always easy even after histopathologic examination. The possibility of vulvar LC should be taken into account as a possible diagnosis in patients with previous oncological surgery or genital warts refractory to the conventional treatment. In these cases, a biopsy of not only the epidermis but also the superficial dermis is recommendable to rule out inflammatory or tumoral processes. Knowledge on the features of this lesion can avoid unnecessary clinical and therapeutic procedures.

Radiation-induced cutaneous vascular neoplasms occur infrequently and comprise benign, so-called atypical vascular lesions (AVL) and angiosarcomas (AS), often being high-grade malignant tumors. Both arise most frequently within previously irradiated skin in breast-conserving-treated mammary cancer patients. Because of the different clinical course and, consequently, different therapeutic approaches, histopathologic distinction of AVL and AS is essential but significant morphologic overlap has been documented. Furthermore, the coexistence of these lesions or progression of AVL into AS has rarely been reported. Whether AVL is a precursor of AS is much debated and unresolved to date. Recent interest has focused on genetic changes and their differences in AS and AVL. MYC amplification and expression of the corresponding protein has been identified in AS in comparison with AVL. Therefore, MYC fluorescent in situ hybridization and anti-MYC immunohistochemical analysis are diagnostically useful in difficult cases. Furthermore, advanced tailored treatment strategies in AS, one of the most aggressive type of sarcoma, rely on identifying genes and proteins involved in malignant angiogenesis.

Lymphangiomas of the ovary are rare and are usually unilateral. We present a 50-yr-old patient who presented with irregular bleeding secondary to multiple uterine leiomyomas who was found to have bilateral ovarian lymphangiomas. There was no evidence of pelvic lymphatic obstruction or of lymphadenopathy, and this appeared to exclude the possibility of acquired lymphangiectasia. The ovarian tumors were associated with a prominent smooth muscle cell component that partly surrounded many of the dilated vascular spaces to the extent that the diagnoses of lymphangioleiomyoma and lymphangioleiomyomatosis were also considered. However, there was no clinical evidence of lymphangioleiomyomatosis in other sites and the smooth muscle cells did not express melanocytic markers immunohistochemically. Lymphangioma and lymphangioleiomyoma should be considered in the differential diagnosis of bilateral, multicystic ovarian neoplasms.

Lymphangioma is a benign, hamartomatous tumor of the lymphatic channels. It is usually found in the head and neck region. Most lymphangiomas are present at birth and are usually diagnosed in infancy or early childhood. When lymphangioma occurs in the mouth, the anterior two thirds of the tongue is the most commonly affected region. A risk of secondary growth is classically described after surgical reduction. In this paper, we present 9 cases of tongue lymphangioma. We provide a literature review of tongue lymphangioma.

Pericardial lymphangiomas are extremely rare benign tumors of lymphatic origin that are usually diagnosed in children. These tumors are often asymptomatic but can cause symptoms secondary to mass effect. We report a case of a giant pericardial lymphangioma that was incidentally discovered in a 58-year-old woman and present imaging, surgical, and pathologic correlations.

Haemolymphangiomas are congenital malformations of the vascular system, benign in origin. They can be either defined at birth or in early childhood, but the recurrence rate is usually high and complete resection is difficult to be achieved. We report two cases of haemolymphangiomas present at the tibias. We used radiographic control, ultrasound and magnetic resonance to detect the nature of the tumour and the anatomical relation with the surrounding tissues providing the appropriate surgical treatment. The diagnosis was confirmed postoperatively by histopathology. The first case presented multiple recurrences because of insufficient draining and immobilization of the leg, whereas the second case had no complications or recurrence postoperatively. Both patients had no complications or recurrence in 24-month follow-up. Haemolymphangiomas are very challenging to be treated as they can grow slowly and remain asymptomatic for many years. Sometimes they can be very aggressive in growth without characteristics of malignancy. The choice of treatment is the complete surgical excision of the tumour, sufficient and long-lasting draining, and immobilization of the extremity to abolish the possibility of recurrence. Other methods of treatment did not show better results than surgical resection.

Vulvar lymphangioma circumscriptum is a rare, benign proliferation of the lymphatic system that presents a diagnostic and management challenge. It may be confused with condyloma acuminata, molluscum contagiosum, or other vulvar disorders. Treatment options include observation, surgical excision, laser ablation, or sclerosing therapy. We report 3 cases, 2 associated with vulvar squamous cell carcinoma and 1 with hidradenitis suppurativa.

Objective: To describe the clinical course, diagnostic features, and treatment of a case of Gorham-Stout syndrome involving the petrous apex and causing chronic cerebrospinal fluid (CSF) leak., Study Design: Clinical capsule report., Setting: Academic pediatric hospital., Patient: A 12-year-old boy presented with a destructive lesion of the right petrous apex and a 1-month history of headache, nausea, and vomiting., Interventions: Computed tomographic and magnetic resonance imaging revealed a nonenhancing lytic lesion of the right petrous apex and mandibular condyle. The lesions were bright on T2-weighted imaging with high signal intensity extending into the surrounding soft tissues. Leptomeningeal enhancement and an opening pressure of 0 cm of water on lumbar puncture suggested chronic CSF hypotension. Initial surgical exploration revealed diffuse infiltration of CSF into the soft tissue lateral to the temporal bone. Subsequent middle ear and mastoid obliteration was performed to definitely repair the CSF leak., Results: The clinical presentation, physical, laboratory, radiologic, and operative findings are consistent with a diagnosis of Gorham-Stout syndrome. In this case, lymphangiomatosis led to massive osteolysis of the petrous apex with CSF fistula into the surrounding soft tissues and middle ear with chronic intracranial hypotension., Conclusion: This is the second report of chronic CSF leak resulting from lymphangiomatosis of the cranial base (Gorham-Stout syndrome).

D2-40 is a commercially available monoclonal antibody directed against human podoplanin, a transmembrane mucoprotein that is expressed in lymphatic endothelial cells. Since its introduction, D2-40 immunoexpression has been described in a variety of lymphovascular neoplasms including lymphangioma, Kaposi sarcoma, and hemangioendothelioma, as well as nonvascular neoplasms such as epithelioid mesothelioma, seminoma, and hemangioblastoma. More recently, D2-40 immunoexpression has been reported in primary adrenal cortical tumors, schwannomas, and adnexal tumors of the skin. This brief review provides an update on the ever-expanding proposed applications of D2-40 immunohistochemistry in surgical pathology.

Pinocchio or Cyrano nasal tip deformity is a rare situation that develops secondary to the soft tissue tumors underneath. In literature, there is only one case reported with Pinocchio nasal deformity secondary to cavernous lymphangioma. In this study, we present a Pinocchio or Cyrano nasal deformity with skin involvement secondary to lymphangioma circumscriptum.

Background: Lymphangioma circumscriptum (LC) is a benign dilation of lymph channels localized to the skin and subcutaneous tissues. Primary vulvar involvement is very rare, with only 34 cases reported., Case: A 17-year-old girl presented with pain and an 18-cm mass of the right labium majus that started 4 years earlier. She underwent wide local excision, and histological examination showed multiple dilated vascular channels in papillary dermis with a diagnosis of vulvar LC., Conclusion: Vulvar lymphangioma may mimic other vulvar tumors. Excision may be the best treatment of extensive vulvar LC.

Study Design: A case report., Objectives: To report and discuss a case of lymphangioma in the epidural space of the cervicothoracic spine., Summary of Background Data: Lymphangioma of the soft tissue is not uncommon, but lymphangiomas presenting as a spinal soft tissue tumor in the epidural space are extremely rare., Methods: A 61-year-old woman with pain and numbness in the left arm. Magnetic resonance imaging demonstrated a half ring-shaped soft tissue mass in the left epidural space from the C6 to T1 level. The patient underwent osteoplastic laminectomy from C6 to T1. Histopathologic diagnosis of the resected tumor was cavernous lymphangioma., Results: One year after the operation, the patient reported mild pain in left upper limb, but there was no evidence of local recurrence on magnetic resonance imaging., Conclusion: To the authors' knowledge, this is an extremely rare reported case of lymphangioma presenting as a spinal soft tissue tumor in the cervicothoracic epidural space. Further observation is recommended because of the possibility of local recurrence.

The CT manifestation of lymphangiohemangioma in a 17-year-old boy is reported. A mass composed of the central malformed, dilated venous channels and peripheral unenhanced soft tissue was revealed in the anterior mediastinum. The venous feeding enhancement of the mass is very characteristic. The diagnosis of lymphangiohemangioma was made by pathologic study of the surgical specimen.

Study Design: Case report, minimally invasive technique., Objectives: Disseminated lymphangiomatosis is a rare disorder that can produce clinical manifestation secondary to soft tissue, visceral and bone involvement. The overall prognosis of this disorder is usually poor, and the current treatment options for its sequelae are limited and only palliative. In this report, we present the use of cementoplasty in the percutaneous treatment of a sacral lymphangiomatous bone lesion producing severe pain., Summary of Background Data: Disseminated lymphangiomatosis is a rare disorder that can produce clinical manifestation secondary to soft tissue, visceral, and bone involvement. Major morbidity related to skeletal involvement requiring surgical intervention is less common than that related to visceral involvement, but it has been reported in a few case reports to palliate neurologic sequelae secondary to vertebral involvement. We present case of osteoplasty used to treat a painful osteolytic sacral lesion in a patient with diffuse lymphangiomatosis. Computed tomography guided osteoplasty injecting acrylic bone cement into the lesion resulted in almost immediate reduction in pain., Methods: The technique and results of minimally invasive percutaneous computed tomography-guided cementoplasty of a painful osteolytic sacral lesion resulting from chylous reflux in a patient with lymphangiomatosis is presented., Results: Computed tomography was used to accurately position a 13-gauge needle into a dominant sacral osteolytic lesion. The injection of bone cement into the sacral lesion was then monitored by intermittent CT imaging. The patient reported substantial pain relief within several hours of the procedure., Conclusion: The case presented demonstrates the feasibility and efficacy of computed tomography-guided cementoplasty used to palliate unusual causes of benign osteolytic bone lesions. These procedures can be performed as outpatients with minimal recovery.

An 8-year-old boy who presented with a mediastinal mass, pulmonary infiltrates, and disseminated intravascular coagulation was diagnosed with lymphangiomatosis. Despite medical management, he developed multiple organ failure and died. The authors discuss the diagnostic findings, medical management, and pathology and review 52 additional cases of thoracic lymphangiomatosis from the literature. Patients presented with chylothorax (49%), a mass (47%), pulmonary infiltrates (45%), bone lesions (39%), splenic lesions (19%), cervical involvement (15%), disseminated intravascular coagulation (9%), and skin involvement (7%). Children (<16 years) had a worse prognosis than older patients (39% vs. 0% mortality). All patients who died had either parenchymal lung involvement or pleural effusion. Thoracic lymphangiomatosis should be included in the differential diagnosis of a mediastinal mass with pulmonary findings.

Implications: We describe a case of a parturient with disseminated lymphangiomatosis involving the thorax, retroperitoneum, and lumbar vertebrae who received epidural labor analgesia. Clinical presentations vary depending on the organ systems involved, the extent of the disease, and the stage of pregnancy. Anesthetic implications are discussed.

Lymphatic malformation is a developmental error usually noticed at birth or in early childhood. Lesions of the upper leg and lower trunk are the most difficult to remove, because they are often the largest ones encountered and also because they tend to extend proximally into the retroperitoneal tissues. Chyle reflux, usually associated with lymphedema of the extremity, has not been reported to be caused by lymphatic malformation. We report a case of intrapelvic retroperitoneal lymphatic malformation with an extension of gluteal-thigh soft-tissue involvement causing intractable chylorrhagia. The tumor was subtotally excised, and the defect was closed by a distally based, peninsular latissimus dorsi myocutaneous flap. The flap served both as a filling material and as a "bridge" between the residual tumor, including abnormal lymphatics, and normal lymph flow.

Lymphangiomas of the ovary are rare tumors, with only 13 cases reported. The diagnoses of these tumors have been based on histologic findings without immunohistochemical confirmation of endothelial cell origin. It is uncertain if these tumors are true neoplasms or if some represent reactive lesions. In this report, the literature is reviewed, and a 53-year-old woman with bilateral ovarian lymphangiomas is described. The ovarian masses were composed of numerous, thin-walled, cystic spaces containing a proteinaceous fluid, mature lymphocytes, and occasional erythrocytes. The cyst walls were lined by flat, benign-appearing cells that were immunoreactive for factor VIII-related antigen, CD34, and CD31. Further examination of the specimen showed absent fallopian tube fimbriae, tuboovarian adhesions, and chronic follicular salpingitis, suggesting that the lymphatic proliferation in the ovaries was a reactive change secondary to impaired regional lymphatic drainage.

Lymphangioma circumscriptum of the penis and scrotum is an unusual entity that may be indistinguishable from genital warts. After confirmation of the diagnosis, a treatment plan consisting of wide excision should be outlined. To lower the chance of recurrence, not only the affected skin but all the subjacent subcutaneous tissue, including the deeper components of the lymphatic malformation just above the deep fascia, should be removed.

Bullous lesions have been only rarely described in Kaposi's sarcoma (KS), and their histopathologic features have never been described in detail. We report a case of bullous lesions of KS in an 82-year-old Italian woman. The patient had typical smooth pale reddish-grey slightly-raised KS plaques on the legs, present for at least 10 years. Several dull grayish-pink blisters (0.5 to 2 cm in diameter) affected both dorsa of her feet and ankles symmetrically. Two punch biopsies were taken, one from an infiltrated KS plaque on the right buttock and the other from a bullous lesion on the right foot. Histopathologically, the late KS plaque on the buttock showed typical features of KS, with an increased number of spindle cells arranged in short bundles and extravasation of erythrocytes. The bullous lesion on the foot showed a full-thickness vascular neoplasm involving the upper and lower dermis and the subcutaneous fat. The upper portion of the lesion contained many newly formed, highly-dilated blood vessels, touching the overlying epidermis and separated from it by a narrow band of collagen and endothelial cells; wide, empty spaces characterized the superficial dermis, in which preexisting venules and bands of collagen associated with non-atypical endothelial cells floated. All these findings would suggest a lymphangiomatous lesion, if the presence of specific diagnostic criteria of KS were not recognizable at a deeper level of the lesion. Various criteria actually suggest that the bullous lesion may be regarded as an epiphenomenon of a KS plaque lesion: (a) full-thickness involvement of the reticular dermis and, in this case, also of the subcutaneous fat; (b) dense and patchy lymphoplasmocytic infiltrate typical of plaque lesions and, much less frequently, of patch lesions; (c) presence of ectatic blood vessels, filled with plasma and erythrocytes (pseudoangiomatous findings), a nonpathognomonic but highly characteristic finding of the plaque lesion; and (d) as in the KS plaque lesions, in the bullous lesion as well the reticular dermis was characterized by an increased number of anastomosing bizarrely shaped vascular spaces lined by non-atypical endothelial cells. We hypothesize that the prevalence of lymphangiomatous differentiation in the upper dermis represents one of the many features of KS lesions. When present, it may correlate with the clinical feature of a blister.

The clinical and pathological features of seven cases of lymphangioma-like Kaposi's sarcoma (KS) are reported. As with the other subtypes of KS, the lymphangioma-like variant occurs more often in men aged 59-80 years. Clinically, the lesion appears intermingled with the classical forms of KS, but a "bulla-like" appearance recognized in seven of 13 cases has been considered as a clinical hallmark of this variant. Although occasional cases have shown aggressive behavior, the most frequent clinical course is slowly progressive with localized or diffuse involvement of lower limbs. The histological pattern, characterized by permeation of dermal collagen by labyrinthine vascular channels lined by a flattened endothelium, must be differentiated from spindle cell hemangioendothelioma, low-grade angiosarcoma, targetoid hemosiderotic hemangioma, and benign lymphangioendothelioma.

Lymphangiomas are uncommon congenital tumors of the lymphatic system which usually occur in the head and neck; involvement of the tongue is rare. We report the case of a six-year-old Asian girl with a lymphangioma of the tongue. The differential diagnosis and treatment of tongue lymphangioma are also reviewed. It is important for emergency physicians to recognize unusual lesions such as lymphangioma, because early recognition and surgical excision of these tumors are essential for an optimal outcome.

Study Design: This is a report of a solitary lymphangioma in a vertebral body treated by excision and autogenous bone grafting. The literature for solitary lymphangioma is reviewed., Summary of Background Data: An adolescent patient presented with low back pain and an abdominal mass associated with a radiographic lytic process in the L3 vertebral body. Direct continuity of the mass with the L3 body and without involvement of neural elements was demonstrated by computed tomography. Excision of the cystic mass and L3 body with subsequent reconstruction using cancellous iliac autograft was completed. At 20 months' follow-up, the patient was without pain and without evidence of recurrence., Conclusions: This is the first reported case of the occurrence of this entity in the vertebral body. Excision and bone grafting is accepted treatment. Careful observation for local recurrence is needed.

Cavernous lymphangiomas of the thorax are extremely rare. We encountered an 11-year-old boy with such a disorder. Magnetic resonance imaging (MRI) showed numerous small rounded and tubular structures within the mediastinal mass; we believe these represent dilated lymphatic channels.

A 14 yr old girl presented with a large cystic mass in the transverse mesocolon which was completely excised. Cystic lymphangioma was diagnosed, based on the specific histologic, immunoperoxidase and ultrastructural findings.

Lymphangioma of the upper extremity is rare; its treatment is unstandardized. We reviewed five female and one male patient with cavernous lymphangioma of the hand and forearm. Each of them underwent at least one surgical procedure. Five patients had satisfactory results with cosmesis and hand function. Satisfactory results are expected in those treated initially in early childhood, as most lymphangiomas tend to increase gradually in size and infiltrate previously uninvolved normal tissues.