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Diffuse abdominal lymphangiomatosis is a rare and complex disease. It typically presents with non-specific gastrointestinal symptoms and characteristic cystic lesions or tumoral masses on imaging based on the literature to date. This report presents the rare case of a young man with an atypical form of diffuse abdominal lymphangiomatosis in the complete absence of cystic lesions or lymphangioma tumoral masses, thus presenting a unique diagnostic challenge. It was successively treated by surgery, gastric electrical stimulator, sirolimus, and imatinib., (© 2024. Japanese Society of Gastroenterology.)

Competing Interests: Declaration of competing interest All authors declare that they have no conflicts of interest.

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.)

Background: Scrotal and retroperitoneal lymphangioma (SRL) in children is relatively rare and its clinical symptoms are usually difficult to distinguish from other conditions such as hydrocele and incarcerated inguinal oblique hernia. This study aimed to explore the clinical diagnosis and treatment of abdominal scrotal lymphangioma in children, and thus, to increase our understandings of this disease in clinical practice., Method: This study enrolled nine boys, aged 1-10, who were admitted to Shanghai Children's Hospital from January 2019 to December 2020 and who were finally confirmed with lymphangioma in the inguinal area. The clinical manifestations, diagnosis, and treatment of these children were analyzed retrospectively. The length of diagnostic process ranged from 3 weeks to 20 months. We also reviewed other cases of initially misdiagnosed cases of SRL in English publications from 2000 to 2022., Results: The nine cases were misdiagnosed as hydrocele, hematoma, or inguinal hernia. Three patients received intracystic injection of bleomycin, three underwent laparoscopic mass resection, and three underwent resection of the inguinal lymphangioma under direct vision. Postoperative pathological analysis of the surgical specimens confirmed the diagnosis of benign cystic lesions and lymphangioma. Meanwhile, among the 14 cases of SRL in literature review, eight were misdiagnosed. Six were initially diagnosed as hydrocele, one as inguinal oblique hernia, and one as testicular tumor, all of which underwent ultrasonography scans. All cases were confirmed as lymphangioma after pathological examination., Conclusion: The non-specific clinical manifestations may contribute to the misdiagnosis of scrotal masses in children. A detailed and accurate medical history, careful physical examination, and imaging findings are important factors contributing to the preoperative differential diagnosis of scrotal lumps in children, but the final diagnosis is based on pathological examination., (© 2023. The Author(s).)

Lymphangiomas of pancreatic origin are rare and can often be found with abdominal symptoms or incidentally on imaging. A 46-years-old female presented with epigastric abdominal pain and vomiting, and twenty-pound weight loss over 3 months. Computed tomography of the abdomen showed a septated mass of the distal pancreas measuring 25.4 cm in largest diameter and extending superiorly into the mediastinum via a hiatal hernia. Endoscopic ultrasound demonstrated abutment but no invasion of the esophagus and stomach. She underwent an exploratory laparotomy with retroperitoneal mass excision, distal pancreatectomy, splenectomy, and hiatal hernia repair with Dor fundoplication. Pathology was consistent with a cystic lymphangioma. Patient was discharged on postoperative day 3 and remained recurrence free for over a year.

Background: Lymphangiomas are benign vascular tumors arising from the lymphatic system. They commonly affect the head and neck regions. Pancreatic involvement is extremely rare. Even though they are rare, it should be considered as a differential diagnosis for patients who present with pancreatic mass., Case Presentation: We report the case of a 6-year-old African male patient who presented with abdominal mass and dull aching pain of 6 months duration. He was examined and underwent excision, with biopsy showing pancreatic lymphangioma., Conclusion: These tumors are benign and slow growing and have potential to regress spontaneously. Thus, most literature agrees that surgical interventions should be reserved for symptomatic cases., (© 2023. The Author(s).)

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.)

A 26-year-old man with left inguinal pain and frequent urination was examined. An abdominal ultrasound revealed a cystic lesion. In further examinations, CT and MRI showed a large cystic lesion of about 20 cm in size, connected to mesenteric- derived blood vessels. We suspected a huge mesenteric lymphangioma and decided to perform a laparotomy. A tumor was seen in the mesentery of the jejunum and adhered to the duodenum widely. The tumor could be removed safely without resection of the duodenum by first sucking the contents and shrinking the tumor. The final pathological diagnosis was mesenteric lymphangioma. Adult mesenteric lymphangiomas measuring larger than 20 cm are relatively rare. We review the case in the context of the relevant literature.

Unusual angiomatous or lymphangiomatous vascular malformations are rarely seen. One of them is lymphangioma (LA), which is a rare benign lymphovascular abnormality. LA is usually seen in the types of circumscriptum (or capillary), cavernous and cystic. Here, we report a unique case of LA with a patchy appearance. The patient presented due to unusual symptoms and eccentric clinical manifestation of the lesion. Here, we present a new lymphatic entity which was diagnosed as LA with its clinical, radiological and pathological findings. Written informed consent of the patient was obtained for this report. To the best of our knowledge this macular form of cutaneous LA has not been previously reported in literature. Macular LA should be kept in mind when faced with a colored long-term macular lesion on the skin., (© 2022 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

Lymphangiomas are comprised of aggregates of lymphatic vessels, considered to represent either aberrant embryogenic remnants or developing secondary to obstruction. Lymphangiomas primary to the heart and pericardial are exceedingly rare, and to date sparingly reported in individual case reports. In this study, the histopathologic, clinical, and radiologic features of 35 cases of cardiac/pericardial lymphangiomas described in the literature to date together with four cases from our own institution (39 cases in total) are examined to provide clinicopathologic characterization. Cardiac/pericardial lymphangiomas were identified in both children and adults, with two cases initially discovered in utero. If presenting with symptoms, patients most commonly exhibited respiratory distress/dyspnea. By X-ray, a widened cardiac silhouette could be noted, and echocardiogram generally showed an echogenic mass with cystic and septal components. On computed tomography (CT) and magnetic resonance imaging (MRI), cystic and septal components were again observed, with CT showing an absence of calcifications or macroscopic fat. Most lymphangiomas were pericardial (specifically visceral) based, and frequently situated in the right atrioventricular groove. A majority of cases proceeded to surgical resection, with no evidence of recurrence post-operatively. Grossly, lesions had a median size of 6 cm and in almost all cases were multicystic/multilocular. Microscopically, the lymphangiomas were composed of lymphatic spaces lined by endothelial cells that specifically express podoplanin (D2-40) with immunoperoxidase staining. Further investigation with a larger and more uniformly organized cohort is required to better characterize the clinicopathologic features of lymphangiomas of this unusual anatomic location., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Salivary gland neoplasms are uncommon, and most exhibit epithelial differentiation. Mesenchymal neoplasms of the salivary gland are rare, and the incidence ranges from 1.9% to 5%. The aim of this study is to identify the types and clinical-pathological features of mesenchymal salivary neoplasm and review their differential diagnosis. A retrospective search for mesenchymal neoplasms of salivary glands from our institution's pathology archives from the 2004-2021 period and consultation files of one of the authors (AER) was performed. The clinical data were obtained from available medical records, and the histological slides and ancillary studies were retrieved and reviewed. We identified a total of 68 cases that form the study cohort. Thirty-five patients were male, and thirty-three patients were female, with a mean age of 48 years (range, 7 months-79 years), and the male to female ratio was 1:.94. Sixty-three (92.6%) of sixty-eight tumors were benign and included: 38 (56%) lipomas, 9 (13%) hemangiomas, 7 (10.3%) schwannomas, 3 (4.4%) neurofibromas, 3 (4.4%) lymphangioma, 2 (3%) solitary fibrous tumors, 1 (1.5%) myofibroma. Five of sixty-eight (7.4%) were malignant and included: 3 (4.4%) Adamantinoma-like Ewing sarcomas, 1 (1.5%) malignant peripheral nerve sheath tumor (MPNST), and 1 (1.5%) malignant solitary fibrous tumor. The involved sites included: parotid (55), submandibular gland (5), parapharyngeal space (5), buccal mucosa minor salivary gland (2), and sublingual gland (1). Sixty-seven patients underwent surgical resection. One patient with lymphangioma manifested a recurrence/persistence a week post-surgery. One patient with a parotid hemangioma developed post-operative numbness, and another patient developed chronic postauricular pain after surgery. Two patients with MPNST and one patient with adamantinoma-like Ewing sarcoma underwent neoadjuvant chemoradiation and were disease-free after treatment. The remaining 37 patients with available follow-up ranging from 7 days to 96 months (mean, 18 months) had a favorable outcome and were disease-free after treatment. Mesenchymal neoplasms of salivary gland are rare; most are benign and demonstrate adipocytic, endothelial, and schwannian differentiation; awareness of their development is important for adequate diagnosis. The mainstay of treatment is surgical excision, with the extent determined by tumor type. Adjuvant therapy is reserved for high-grade sarcomas and may be given in a neoadjuvant or adjuvant setting., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Acquired lymphangioma circumscriptum of the vulva is rare and can occur subsequent to malignancies of the anogenital and pelvic region. We sought to investigate the clinicopathologic characteristics of malignancy-associated acquired vulvar lymphangioma circumscriptum (AVLC)., Methods: We identified all cases of AVLC within our institution with history of prior malignancy between 2005 and 2021. A similar search was performed in the PubMed database to identify published cases to date. The clinical and histopathologic information was recorded., Results: A total of 71 cases were identified. The most common preceding malignancy was cervical carcinoma (71.8%, 51/71). Radiation therapy was given to 91.4% (64/70) of the patients and lymph node dissection was made on 70.2% (40/57). Median interval between the diagnosis of malignancy and the AVLC was 10 years (range 0-32 years). AVLC frequently presented as vesicular (31.6%, 18/57) or verrucous (28.1%, 16/57) lesions clinically. Common treatments for AVLC included excision (53.1%, 26/49) and laser therapy (16.3%, 8/49), with an overall recurrence rate of 42.9% (24/56) at a median follow-up interval of 1.8 years (range 0.04-32.3 years)., Conclusion: AVLC is a rare, late complication of anogenital and pelvic malignancies causing debilitating physical symptoms and psychological stress. Further studies are warranted to determine the most effective treatment modalities to mitigate recurrence., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The aim of this study was presenting significance of diagnostic process in doctor's daily routine. A 45-year-old patient for a planned laparotomy due to left ovarian cyst detected with a routine transvaginal ultrasound. She did not report any symptoms. Computer tomography of the abdomen and pelvis showed a cystic lesion with segmental wall thick¬ening, measuring 133 ☓ 83 ☓ 135 mm, adjacent to the left ovary and the uterus on the left side. At the laparotomy exploration, a giant cyst, ca. 20 cm in diameter was found above the uterus, on the left side, in the area between the lower and middle abdomen. The histological exam results showed lymphangioma of the small and large intestine mesentery. In this case, lymphangioma was found in a rare location, in the mesentery of the ileocecal valve. It did not present any signs and symptoms. CT used in the present case failed to identify the exact point of origin of the lesion.

Lymphangioendothelioma, also known as acquired progressive lymphangioma, is a rare vascular neoplasm of lymphatic origin. Although in light-skinned individuals lesions typically present as erythematous to violaceous papules or plaques, in dark-skinned patients lesions often appear hyperpigmented. Histopathologic distinction of lymphangioendothelioma from early Kaposi sarcoma and angiosarcoma is imperative considering the therapeutic and prognostic implications. Herein, we present a 71-year-old woman with slowly progressive hyperpigmented papules and histopathology demonstrating thin-walled vascular spaces interspersed between collagen bundles, consistent with lymphangioendothelioma. We describe the clinical and histologic findings of this rare entity and highlight histologic mimics that might result in diagnostic delay.

Background: Urinary bladder lymphangioma is a rare and benign lesion that is often causes symptoms related to irritation and urinary tract obstruction. Because a lymphangioma may resemble a true neoplasm of the urinary bladder clinically, the lesion must be removed for accurate histologic diagnosis and to rule out malignancy., Case Presentation: We present a case of a 40-year-old female who was evaluated for painless gross hematuria. Clinical and diagnostic work up revealed a sharply defined mass involving the wall and bulging into the cavity on the dome of the bladder. Partial cystectomy was performed and histologic findings were compatible with cavernous lymphangioma. The symptom of hematuria relieved after the procedure and the patient was in good status without evidence of recurrence by cystoscopy at follow-up 6 months later., Conclusions: Lymphangioma of the urinary bladder is treated with surgical excision and seems to have no recurrence once completely resected, but long-time follow-up may be needed., (© 2021. The Author(s).)

In vivo reflectance confocal microscopy (RCM) findings of lymphangiomas have been scarcely reported. We report a lymphangioma circumscriptum (LC) with some new observations., (© 2021 The Australasian College of Dermatologists.)

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.)

Acquired lymphangiectasia of the vulva is very uncommon. Owing to the non-specific papillomatous manifestation and the vast array of possible differential diagnoses, lymphangioma circumscriptum (LC) still presents a diagnostic challenge. In this report, we present a very rare form of acquired vulvar LC in a 71-year-old patient with a longstanding history of asymptomatic lesions over the labia majora that had been previously treated as genital warts. On examination, the patient had multiple clustered translucent papules up to 15mm in diameter, morphologically reminiscent of vesicles, that oozed clear fluid throughout her groin and swollen labia majora. The patient also suffered concomitant bilateral lower-extremity lymphedema. A skin biopsy showed multiple, irregular-shaped lumina containing eosinophilic material in the upper dermis. Dilated lymphatic channels were lined by a single layer of flattened endothelial cells and the overlying epidermis showed acanthosis, hyperkeratosis, focal mild pseudoepitheliomatous hyperplasia. There is still no consensus on the optimal management of LC. Our patient was referred to a plastic surgeon for further evaluation and treatment. Although there are a variety of therapeutic modalities for LC, positive results are few and relapses are observed.

Competing Interests: Competing interests: None declared.

Competing Interests: Competing interests: None declared.

Introduction:  Vascular endothelial growth factor (VEGF) and its receptor act as a major contributor to lymphangioma, but their role on nonrecurrent and recurrent lymphangiomas remain unclear. We aim to investigate those factors in the generation of recurrent lymphangioma., Materials and Methods:  Patients diagnosed with lymphangioma from January 2005 to December 2012 in our hospital were collected and divided into nonrecurrent and recurrent lymphangiomas. The clinical characteristics including age, sex, symptoms, location, and size of lymphangioma were collected. Surgical resection samples were collected for histology, protein and mRNA detection of VEGF-C, VEGF receptor-3 (VEGFR-3), and neuropilin 2 (Nrp2). Follow-ups including lymphangioma recurrent and the local symptoms such as ulcer were reviewed., Results:  A total of 80 patients aged from 5 months to 12 years were enrolled in this study, 51 patients had no recurrence and other 29 patients suffered from recurrent lymphangioma. There was no significant difference in demographic data and clinical characters between the two groups ( p  > 0.05). Immunohistochemistry staining showed that VEGFR-3 remained unchanged between nonrecurrent and recurrent lymphangiomas ( p  > 0.05), and VEGF-C and Nrp2 were significantly increased in recurrent lymphangioma compared with nonrecurrent lymphangioma ( p  < 0.05). The same expression trend was proved as detected by protein and mRNA levels., Conclusion:  The VEGF-C/Nrp2 axis was significantly increased in the recurrent lymphangioma, indicating that VEGF-C/Nrp2 targeted therapy may serve as a potential therapeutic strategy for recurrent lymphangioma., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Lymphangiomas are rare, congenital malformations of the lymphatic system which have a marked predilection for the head and neck. In this region, they most commonly occur on the dorsum of the tongue, followed by the lips, buccal mucosa, soft palate, and floor of the mouth. Lymphangiomas of the tongue are commonly present at birth; however, they may go unnoticed until after eruption of the dentition or even puberty. They may present as a defined mass or as macroglossia with impaired speech, difficulty in mastication, and, in extreme cases, airway obstruction. Clinically, lymphagiomas of the tongue are characterized by clusters of pebbly, vesicle-like nodules. A benign proliferation of lymphatic vessels is identified histologically. A classic case of a lymphangioma of the dorsal tongue is presented.

Competing Interests: Competing interests: None declared.

Lymphangioma insults in adults are uncommon and the occurrence in the oropharynx is extremely rare. Although disease history and clinical symptoms may help in distinguishing it from malignant tumors, the pathophysiology of lymphangioma via histological examinations should be established for convenient and precise diagnosis. We present a 20-year-old male with lymphangioma in the right oropharynx, and the multiple-angle examination and treatment plan used. The case further emphasizes the need to consider lymphangioma in the differential diagnosis of an oropharyngeal mass. Awareness that lymphangioma can occur in adults is important for its proper management, which includes complete surgical removal to prevent recurrence., (Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

We describe a case of gastric lymphangioma coexisting with mucosal gastric cancer. A 76-year-old man was diagnosed with an advanced gastric cancer (cT2N0M0, Stage I) presenting a nodular protruded lesion with irregular central depression. The surgically resected specimen showed a 4 × 3 cm sessile protruded lesion in the anterior wall of the lower stomach. Histopathological examination revealed a mucosal adenocarcinoma was located immediately above the lymphangioma and was elevated by the cystic component of lymphangioma without admixture, which suggested that the two components arose separately. The mucosal adenocarcinoma was masquerading as an advanced gastric cancer due to the protrusion by the lymphangioma.

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.

A 5-year old boy presented with a picture of communicating hydrocele and was discovered to have an abdominoscrotal lymphangioma after undergoing hydrocele surgery. Upon initial presentation the lymphangioma was missed and the child underwent inguinal approach surgery for hydrocele. The lymphangioma was then noticed as an abdominal lump due to a rapid increase in size within 1 week following the hydrocele surgery. The sudden enlargement of the lymphangioma was due to intra-cystic hemorrhage post-operatively. The lymphangioma was then completely excised with no recurrence noted after 1 year of follow up. This is a rare case of a retroperitoneal, abdominoscrotal lymphangioma masquerading as a communicating hydrocele. Keywords: Communicating Hydrocele, Abdominoscrotal lymphangioma, Scrotal Swelling.

Intussusception in lymphatic vessels has received less attention than in blood vessels. In tumors and pseudotumors of blood vessels with intravascular papillary structures, including sinusoidal hemangioma and intravascular papillary endothelial hyperplasia, we observed exuberant intussusceptive angiogenesis, as well as the similarity between papillae (term used by pathologists) and pillars/folds (hallmarks of intussusceptive angiogenesis). A similar response could be expected in lymphangiomas (lymphatic malformations and reactive processes rather than tumors) with papillae. The aim of this work is to assess whether papillae/pillars/folds and associated structures (vessel loops and septa) are present in lymphangiomas, and to establish the characteristics and formation of these structures. For this purpose, we selected lymphangiomas with intraluminal papillae (n = 18), including cystic, cavernous, circumscriptum, and progressive types, of which two cases of each type with a greater number of papillae were used for serial histologic sections and immunohistochemistry. The studies showed a) dilated lymphatic spaces giving rise to lymphatic-lymphatic vascular loops, which dissected and encircled perilymphatic structures (interstitial tissue structures/ITSs and pillars/posts), b) ITSs and pillars, surrounded by anti-podoplanin-positive endothelial cells, protruding into the lymphatic spaces (papillary aspect), and c) splitting, remodeling, linear arrangement, and fusion of papillae/pillars/folds, forming papillary networks and septa. In conclusion, as occurs in blood vessel diseases, the development of lymphatic vessel loops, papillae/pillars/folds, and septa (segmentation) supports intussusceptive lymphangiogenesis and suggests a piecemeal form of intussusception. This intussusceptive lymphangiogenesis in lymphatic diseases can provide a basis for further studies of lymphatic intussusception in other conditions, with clinical and therapeutic implications. Anat Rec, 302:2003-2013, 2019. © 2019 American Association for Anatomy., (© 2019 American Association for Anatomy.)

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is an extremely rare recently described disorder characterized by diffuse congenital skin and gastrointestinal vascular lesions that may be associated with gastrointestinal bleeding and thrombocytopenia. We herein present a case report of multifocal lymphangioendotheliomatosis without thrombocytopenia or extensive extracutaneous involvement (gastrointestinal bleeding). Given the high morbidity and mortality associated with this disease, it is important for clinicians to recognize this disorder in order to select the most appropriate therapeutic approach., (© 2019 Wiley Periodicals, Inc.)

Lymphangioma circumscriptum is a benign hamartomatous malformation involving the lymphatic system of skin and subcutaneous tissue. It can involve any part of the body with maximum predilection for proximal parts of limbs. Vulvar involvement is quite infrequent. Based on the aetiopathogenesis, it can be either primary or secondary, with the secondary form being relatively more common in vulva. We report an exceedingly rare case of primary lymphangioma circumscriptum in a post-menopausal female presenting with multiple warty, papulo-nodular lesions on both labia majora. The patient was diagnosed histopathologically and managed surgically by wide local excision with primary closure., (© 2019 The Australasian College of Dermatologists.)

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.