Unusual Presentation of Lymphangioma Circumscriptum of the Vulva: In Association With Crohn Disease.

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.

Benign Orofacial Vascular Anomalies: Review of 47 Cases in Enugu, Nigeria.

BACKGROUND: A study of oral vascular anomalies has not been conducted in Nigeria to provide baseline data for comparison with reports in the literature. AIMS: To study the prevalence and distribution of benign orofacial vascular anomalies at a tertiary hospital in Enugu. MATERIALS AND METHODS: This is a 10-year retrospective observational study of consecutive patients with orofacial vascular anomalies, diagnosed by histology. The clinic-pathologic information was obtained from records archived in the department, and descriptive analysis was used to determine the frequency, tables for categorical variables, and a Chi-square test to determine the statistical significance. RESULT: There were 47 cases of benign vascular anomalies out of 897 orofacial lesions giving a prevalence of 5.2%. There were 35.4% (17) male and 64.6% (31) female patients. The mean age in this series was 37.4 ± 19.8 (range: 1 to 76 years). Pyogenic granuloma was the most common vascular lesion 78.7% (37), followed by hemangioma 14.9% (7) and lymphangioma 6.4% (3). The gingiva was the most frequent site of oral occurrence 65.9% (31), especially maxillary gingivae 48.9% (23). The type of orofacial vascular anomalies was significantly associated with the anatomical site of occurrence, P = 0.00. The mean ages for the occurrence of pyogenic granuloma, hemangioma, and lymphangioma were 37.7 ± 18.3, 50.7 ± 16.9 years, and 3.3 ± 3.2 years, respectively. Pain was a frequent occurrence in 36.2% (17) of anomalies. CONCLUSION: Oral vascular anomalies predominantly presented as pyogenic granuloma on the gingivae, while oral hemangioma was observed in adults, and lymphangioma was infrequent.

Diagnóstico mediante resección endoscópica de una entidad extraña / Gastric lymphangioma. Diagnosis by endoscopic resection of a foreign entity

The lymphangioma is a rare and very uncommon benign tumor at the gastric level. Its diagnosis typically involves imaging tests and endoscopy, and its treatment usually involves surgery. We present a case of an 82-year-old patient who presented with chronic anemia, with a large gastric polyp detected during the initial gastroscopy. Subsequently, an endoscopic resection was performed, confirming histologically that it was a lymphangioma.(AU)

Focal hemolymphangioma of the rectum: A case report and literature review.

RATIONALE: Gastrointestinal hemolymphangiomas are very rare, especially in the rectum. Only 3 cases of rectal hemolymphangioma, all of which are diffuse lesions, have been reported in the English literature on PubMed. Our case is the first focal lesion of the rectum. It is important for radiologists to correctly identify the imaging features of rectal hemolymphangiomas. PATIENT CONCERNS: A 51-year-old woman visited our hospital because of intermittent constipation for 3 years. DIAGNOSES: Colonoscopy revealed a prominent lesion on the left anterior wall of the lower rectum. Transvaginal color Doppler ultrasonography showed that the posterior vaginal wall area had a mixed-echo mass, and blood flow signals of the dots and stripes could be seen. Pelvic magnetic resonance imaging showed that the cystic space-occupying lesion in the region between the left anterior wall of the lower rectum and the posterior wall of the middle and lower vagina had a clear boundary. INTERVENTIONS: The patient underwent surgery to remove the rectal lesions. The surgical specimen was finally diagnosed as local hemolymphangioma by pathological analysis. LESSONS: Localized hemolymphangioma of the rectum is very rare, and imaging examination is essential for the diagnosis and evaluation of the extent of lesion invasion.

Gastric lymphangioma. Diagnosis by endoscopic resection of a foreign entity.

The lymphangioma is a rare and very uncommon benign tumor at the gastric level. Its diagnosis typically involves imaging tests and endoscopy, and its treatment usually involves surgery. We present a case of an 82-year-old patient who presented with chronic anemia, with a large gastric polyp detected during the initial gastroscopy. Subsequently, an endoscopic resection was performed, confirming histologically that it was a lymphangioma.

Misdiagnosis of scrotal and retroperitoneal lymphangioma in children.

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.

Neonatal alveolar lymphangioma: A rare benign oral disease case report and literature review.

BACKGROUND: Neonatal alveolar lymphangioma (NAL) is a rare benign condition most often reported in the oral cavity of neonates of African descent. CASE DESCRIPTION: The authors present a case report of bilateral NAL, including follow-up images at 6 months and 17 months. The clinical differential diagnosis includes dental lamina cyst, hemangioma, congenital epulis of the neonate, and melanotic neuroectodermal tumor of infancy. Key differences to assist in making the distinction are described. PRACTICAL IMPLICATIONS: The distinguishing characteristics of NAL make a clinical diagnosis possible. Accurate clinical recognition of NAL is essential because biopsy is not typically indicated. Because this is a clinical diagnosis, follow-up for 1 year or until complete resolution; biopsy is suggested if clinically indicated for residual or recurrent lesion.

Anomalía linfática generalizada en un paciente adulto, un diagnóstico eminentemente radiológico / Generalized lymphatic anomaly in adult patients: an eminently radiological diagnosis

La anomalía linfática generalizada (ALG) es una enfermedad congénita poco frecuente, secundaria a la proliferación de vasos linfáticos en cualquier órgano a excepción del sistema nervioso central, mostrando un amplio abanico de formas de presentación clínicas y radiológicas. Las lesiones osteolíticas son una constante, y las costillas, el hueso más frecuentemente afectado. Se diagnostica principalmente en niños y adultos jóvenes; no obstante, en raras ocasiones la enfermedad puede ser asintomática y detectarse de forma incidental en pacientes de mayor edad. Presentamos un caso inusual de ALG en un paciente de 54 años, asintomático y con presencia de lesiones óseas de distribución atípica en la tomografía computarizada (TC). La naturaleza puramente quística de las lesiones, evidenciada mediante la medición de las unidades Hounsfield, permitió establecer el diagnóstico de sospecha de ALG, que posteriormente se confirmó con resonancia magnética, tomografía por emisión de positrones/TC, punción aspiración con aguja fina guiada por TC y biopsia vertebral percutánea con guía fluoroscópica. Finalmente, se obtuvo el diagnóstico anatomopatológico definitivo tras la resección quirúrgica de una de las lesiones.(AU)

Generalized lymphatic anomaly (GLA) is an uncommon congenital disease secondary to the proliferation of lymphatic vessels in any organ except the central nervous system. GLA has a wide spectrum of clinical and radiological presentations, among which osteolytic lesions are the most widespread, being the ribs the most commonly affected bone. GLA is diagnosed mainly in children and young adults; nevertheless, on rare occasions it can remain asymptomatic and be detected incidentally in older patients. We present an unusual case of GLA in an asymptomatic 54-year-old man who had atypically distributed, purely cystic bone lesions on CT; measuring the Hounsfield (HU) of these lesions enabled us to suspect GLA. This suspicion was confirmed with MRI, PET/CT, CT-guided fine-needle aspiration biopsy, and fluoroscopy-guided percutaneous vertebral biopsy. After surgical resection of one of the lesions, histologic study provided the definitive diagnosis.(AU)

Unexpected Axillary Lymph Node Uptake During Lower-Limb Lymphoscintigraphy in a Case With Vulvar Lymphangioma Circumscriptum.

ABSTRACT: Lymphoscintigraphy is a safe, minimally invasive, and well-established imaging modality for evaluating lymphatic drainage in patients with suspected lymphedema. We report a case of an 8-year-old girl with lymphangioma circumscriptum of the labium majora. She was referred to lymphoscintigraphy for swelling of the lower extremities. Scintigraphy showed widespread dermal backflow at bilateral lower limbs, aberrant uptake in the abdominal lymph nodes, and unexpected uptake in the right axillary lymph nodes.