PURPOSE: When there is a mass in the superior temporal orbit area, a lacrimal gland tumor should be suspected. We report a rare case of orbital lymphatic malformation that was histologically diagnosed in a patient with typical clinical features of the lacrimal gland. CASE SUMMARY: A 55-year-old female with no underlying disease and no ophthalmic history visited our clinic with a right upper eyelid edema associated with an enlarged painless eyelid mass 1 month prior to her visit. The patient stated that she discovered the mass 1 year previously.The palpebral lobe of the lacrimal gland protruded slightly with congestion of the surrounding conjunctiva. Enhanced computed tomography showed a 3 cm well-defined heterogeneous mass in the right lacrimal gland area and several well-defined round calcifications within the mass. Orbital tissue or bone involvement was not observed. The pleomorphic adenoma of the lacrimal gland was the most clinically suspicious, so complete resection of the mass was performed using lateral orbitotomy. Histopathologically, lymphangioma (lymphatic malformation) originating from the lacrimal gland was diagnosed. CONCLUSIONS: Orbital lymphatic malformation can occur in the lacrimal gland. The present case showed that differential diagnosis can reveal the presence of an adult lacrimal gland tumor.
Adenoma, Pleomorphic ; Adult ; Conjunctiva ; Diagnosis, Differential ; Edema ; Estrogens, Conjugated (USP) ; Eyelids ; Female ; Humans ; Lacrimal Apparatus ; Lymphangioma ; Middle Aged ; Orbit

OBJECTIVE: To analyze the prognosis of fetuses with cystic hygroma (CH) or nuchal translucency (NT) or nuchal fold (NF) thickening detected by prenatal echography. METHODS: From January 2014 to December 2015, 124 fetuses with CH and NT/NF thickening on prenatal echography were enrolled from Women's Hospital of Zhejiang University School of Medicine. The basic clinical information, ultrasonic results, pregnancy outcomes and newborn follow-ups were analyzed. The cases were grouped by prognosis and the factors affecting prognosis were analyzed with logistic regression. RESULTS: There were 85 cases of labor induction including one stillbirth and 39 cases delivered. Except one infant who died after birth, all live births survived with good prognosis. Univariate analysis showed that the gestational age at diagnosis of poor prognosis group was earlier than that of good prognosis group (<0.01); and the former group also had higher hydrops fetalis rate and additional structural anomalies rate (all <0.01). Multivariate regression analysis showed that hydrops fetalis (=90.105, <0.05) and additional structural anomalies (=61.854, <0.05) were risk factors of poor prognosis in fetuses with CH and NT/NF thickening. CONCLUSIONS Fetuses with diagnosed CH or NT/NF thickening on prenatal ultrasonography are likely to be associated with chromosomal abnormality. Early gestational weeks, hydrops fetalis and additional structural anomalies may indicate poor prognosis.
Female ; Fetus ; Humans ; Hydrops Fetalis ; etiology ; Infant, Newborn ; Lymphangioma, Cystic ; complications ; diagnosis ; Nuchal Translucency Measurement ; Pregnancy ; Pregnancy Outcome ; Prognosis ; Ultrasonography, Prenatal

OBJECTIVE: To determine the frequency of chromosomal abnormalities and outcome of pregnancy for fetuses with increased nuchal translucency (NT). METHODS: Between July 2014 and February 2018, 247 fetuses with increased NT (>95th centile)were analyzed by chromosome microarray analysis (CMA). The fetuses were divided into ones with isolated increased NT (168 cases), increased NT with cystic hygroma (20 cases), increased NT with edema (12 cases) or increased NT with other abnormalities (47 cases). All couples were followed up by telephone calls. RESULTS: The rate of chromosomal abnormalities was 31.6% (78/247), which included 66 cases with chromosomal aneuploidies and 12 with copy number variants (CNVs). CNVs accounted for 31.4% (11/35) of total abnormalities among fetuses with isolated increased NT, whilst only 2.3% (1/43) of the total abnormalities among fetuses with non-isolated increased NT. Three fetuses with a normal CMA result had mental and physical retardation. Two of them were diagnosed with single gene disorders by whole exome sequencing. CONCLUSION CMA can detect more chromosomal microdeletion/microduplications among fetuses with isolated increased NT. Furthermore, fetuses with increased NT and anegative CMA result during pregnancy cannot exclude all adverse outcomes.
Aneuploidy ; Chromosome Aberrations ; Chromosomes ; DNA Copy Number Variations ; Edema ; Female ; Fetus ; Humans ; Lymphangioma, Cystic ; Microarray Analysis ; Nuchal Translucency Measurement ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis ; Ultrasonography, Prenatal

Adrenal cystic lymphangiomas are extremely rare entities that are often identified incidentally, with less than 60 cases reported to date. We found a protruding ovoid mass consisting of a multiloculated cystic lesion within right adrenal gland in the cadaver of a 75-year-old Korean man. The epithelial cells lining the adrenal cyst were diffusely positive for cluster of differentiation 31 and podoplanin, and negative for pan-cytokeratin. The histopathological diagnosis confirmed a cystic lymphangioma arising from the adrenal gland. Post-mortem findings of the present case are discussed based on the clinicopathological features of adrenal cystic lymphangiomas.
Adrenal Glands ; Aged ; Cadaver ; Diagnosis ; Epithelial Cells ; Humans ; Lymphangioma ; Lymphangioma, Cystic ; Male

PURPOSE: To report a case of isolated conjunctival lymphangioma mimicking a recurrent conjunctival cyst. CASE SUMMARY: A 39-year-old male with a conjunctival cyst in the right eye lasted for 1 month visited our hospital. He had previously undergone aspiration of the cyst at another hospital 1 week before visiting our hospital. However, the cyst recurred, and he was referred to our hospital. On slit lamp biomicroscopy, yellow-colored turbid fluid and a hemorrhage were observed in the conjunctival cyst, but no specific finding was found in the fundus photography. The patient was initially treated with topical antibiotics and steroids. Three weeks later, absorption of the hemorrhage was noted, but there was no change in the size of the cyst. Therefore, surgical removal and histological examination of the cyst were performed. The histological examination revealed that the lesion was positive for CD 31 and D2-40, and the cyst was diagnosed as a cystic conjunctival lymphangioma. Thereafter, brain magnetic resonance imaging was performed to screen for orbital lymphangioma and systemic disease that could accompany a conjunctival lymphangioma. However, no specific findings were observed. There was no recurrence of the conjunctival cyst at 1 year and 6 months after surgical removal, and no other ophthalmic or systemic complication was observed. CONCLUSIONS: An isolated conjunctival lymphangioma is a rare lesion. In the diagnosis of lymphangioma, systemic examination is recommended for the identification of comorbid diseases, such as orbital lymphangioma.
Absorption ; Adult ; Anti-Bacterial Agents ; Brain ; Diagnosis ; Hemorrhage ; Humans ; Lymphangioma ; Magnetic Resonance Imaging ; Male ; Orbit ; Photography ; Recurrence ; Slit Lamp ; Steroids

PURPOSE: The management of lymphatic malformation (LM) in pediatric patients is challenging. Complete excision of LM is difficult to achieve in some cases. We reviewed our experience how to manage LM. METHODS: We retrospectively reviewed the patients who were treated for LM between 2010 and 2017. Medical records were reviewed about age of diagnosis, age of treatment age, gender, symptom, location of tumor, treatment modality, response and complication. RESULTS: Sixty-three patients (39 boys and 24 girls) were included. Mean age at diagnosis was 14.5±28.0 months (range, neonate-10 years). The involved lesion were head and neck in 27 patients (42.9%), abdominal cavity in 7 patients (11.1%), chest wall and abdominal wall in 11 patients (17.5%), buttock in 7 patients (11.1%), and extremities in 11 patients (17.5%). The treatment options were including surgical resection in 32 patients, sclerotherapy in 7 patients, surgical resection combined sclerotherapy in 19 patients, and close observation in 5 patients. We achieved complete remission in 39 patients. Fourteen patients showed partial remission and 6 showed recurrences. CONCLUSION: Despite surgical difficulty, meticulous excision with supportive treatment, and adjuvant sclerotherapy could get a favorable outcome without fatal complication. Decision should be based on surgeon's experience, location of LM, related symptoms, and consultation with patient's parents.
Abdominal Cavity ; Abdominal Wall ; Buttocks ; Diagnosis ; Extremities ; Head ; Humans ; Lymphangioma ; Medical Records ; Neck ; Parents ; Picibanil ; Recurrence ; Retrospective Studies ; Sclerotherapy ; Thoracic Wall

The ex utero intrapartum treatment (EXIT) procedure was introduced to reduce fetal hypoxic damage while establishing an airway in fetuses with upper and lower airway obstruction. Delivery of the fetal head and shoulders while maintaining the uteroplacental circulation offers time to secure the fetal airway. Here, we report two cases of EXIT procedure for fetal airway obstruction, which were successfully managed with extensive preoperative planning by a professional multidisciplinary team.
Airway Obstruction* ; Fetal Therapies ; Fetus* ; Head ; Laryngeal Diseases ; Lymphangioma ; Placental Circulation ; Prenatal Diagnosis ; Shoulder

Fetal cystic lymphangioma, commonly called hygroma, is formed by multiple cysts ranging from few millimeters to several centimeters in size. Fetal cystic lymphangioma is a rare developmental congenital anomaly of the lymphatic system. Most are found in the neck and axilla, rarely in the retroperitoneum, abdominal viscera, limbs, bones, and cervico-mediastinal area. The prenatal diagnosis of cystic hygroma by ultrasound guidance is commonly localized in the nuchal region. We report a case of rare fetal axillary hygroma that was diagnosed at 27 weeks of gestation and delivered at 37 weeks of gestation.
Axilla ; Extremities ; Lymphangioma, Cystic* ; Lymphatic System ; Neck ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography ; Ultrasonography, Prenatal* ; Viscera

Cystic lymphangioma is a true, benign, congenital multicystic tumor arising from sequestrations of embryonic lymphatic tissue. Most often it occurs at birth or during the early years of life, is found most commonly in the neck, and its distribution coincides with that of the primitive lymph sacs. A 23-year-woman presented with a soft palpable mass on her chest wall. Physical and ultrasound examination revealed a well-circumscribed, mobile, nontender, soft subcutaneous mass on the right posterior chest wall beneath the latissimus dorsi muscle. A skin biopsy of the mass showed endothelium-lined lymphatic vessels and a loose, lace-like fibrous tissue stroma. The specimen was stained with D2-40 showing positive staining of lymphatics. These histopathological findings confirmed the diagnosis of a cystic lymphangioma. We report a rare case of a cystic lymphangioma on the chest wall in a 23-year-old woman.
Adult* ; Biopsy ; Diagnosis ; Female ; Humans ; Lymphangioma, Cystic* ; Lymphatic Vessels ; Lymphoid Tissue ; Neck ; Parturition ; Skin ; Superficial Back Muscles ; Thoracic Wall* ; Thorax* ; Ultrasonography ; Young Adult

Fibroepithelial polyp (FEP) and lymphangioma circumscriptum (LC) of the vulva are rare diseases that occur generally in young to middle-aged women. The pathogenesis of FEP is not clearly understood. Several previous cases have suggested that a hormonal influence may be a predisposing condition for developing FEP with or without chronic inflammation. A 68-year-old postmenopausal woman presented with a history of multiple raised lesions on the vulva with a 1-year duration. Additionally, a pedunculated mass had appeared 6 months earlier in the left lower labia minora. The patient underwent simple excision by electrocautery. The final pathological diagnosis was FEP with LC. In this case, the development of FEP was associated only with chronic inflammation induced by LC. Therefore, the case supports the hypothesis that a chronic inflammatory process may be related to the development of FEP even without hormonal influence.
Aged ; Diagnosis ; Electrocoagulation ; Female ; Humans ; Inflammation ; Lymphangioma* ; Neoplasms, Fibroepithelial ; Polyps* ; Polytetrafluoroethylene ; Postmenopause ; Rare Diseases ; Vulva*