Differential diagnosis of cystic lymphangioma of the pancreas based on imaging features.

Lymphangioma is a benign tumor, which is a consequence of lymphatic malformation with blockage of lymphatic flow. Most lymphangiomas occur in the neck and axillary region, and < 1% occur in the mesentery or retroperitoneum. Lymphangiomas arising from the pancreas are extremely rare. We report the case of a 34-year-old woman with cystic lymphangioma of the pancreas without major symptoms or signs. A 6 x 6 cm intra-abdominal cystic mass was incidentally revealed by sonography during a health examination. It is always a challenge to differentiate the lesion from other possible cystic-like pancreatic neoplasms. Differential diagnosis of cystic lymphangioma from other cystic-like tumors of the pancreas can be performed based on their imaging characteristics, including presence of septa, cystic or wall calcification, soft tissue, wall thickness, single or multiple loculation, and dilatation of the pancreatic duct. Post-gadolinium magnetic resonance imaging is excellent in defining the origin of intra-abdominal cystic mass and intracystic septa.     

Antepartum assessment of fetal cystic lymphangioma by magnetic resonance imaging

Few reports of fetal cystic lymphangioma have described assessment in utero by magnetic resonance imaging (MRI). We evaluated a fetus with cystic lymphangioma by this method. Complementing the characteristic features of cystic lymphangioma in ultrasonographic images, prenatal MRI provided a detailed view of anatomic relationships of cysts to surrounding tissues in this case. This anatomic evaluation facilitated planning of perinatal management and choice of manner of delivery. We found MRI very helpful in antepartum assessment of fetal cystic lymphangioma.     

Prenatal ultrasonographic diagnosis of abdominal cystic lymphangioma: A case report

Abdominal lymphangioma is a rare tumor of the lymphatic vessels. A case of an abdominal cystic lymphangioma identified at 22 weeks of gestation is reported. Ultrasonographic monitoring showed a progressive increase of mass size during the gestation. Pregnancy was terminated at 38 weeks and the newborn was submitted to a laparotomy with resection of all cystic structures. At the present time the infant is three years old and is doing well.     

Lymphangioma of the ovary after radiation due to Wilms' tumor in the childhood

OBJECTIVE: To report a case of lymphangioma of the ovary after radiation due to Wilms' tumor in the childhood. PATIENT: A 19-year-old nulliparous female. INTERVENTIONS: The vaginal ultrasound showed the left ovary enlarged to 4.4 cm x 2.9 cm x 4.5 cm in size including a 3.5 cm x 2.6 cm x 3.2 cm measuring cystic solid tumor without hypervascularity. For exclusion of a malignant tumor, a laparoscopy for excision of the tumor and deep incision of the left ovary with a bipolar needle was performed to exclude deeper tumor of stromal origin. The histological examination of the tissue showed a lymphangioma beside normal ovarian tissue. CONCLUSION: To our knowledge, this is the first report of lymphangioma of the ovary after radiation due to Wilms' tumor in the childhood. The impact of this finding on the patient's fertility remains unclear. As in other organs exposed to radiation, lymphangioma can also occur in the ovary. Careful follow up should be considered to this patients, because malignant transformation can not be excluded.     

Benign cystic lymphangioma presenting as a pelvic mass

We report a case of a 48-year-old woman with a complaint of chronic pelvic pain with a pelvic mass not related with uterus or adnexes. Preoperative ultrasonography or contrast enhanced computed tomography did not give accurate information on the origin of tumor. At laparotomy a cystic, retroperitoneal mass was totally resected without rupture. Final histopathological examination revealed that cystic spaces with abundant lymphocytes suggesting lymphangioma. No recurrence was detected in the follow up 6 months after total resection. As a result cystic lymphangioma should be considered as a rare cause of pelvic mass in women.     

Neonatal ovarian lymphangioma

Ovarian cysts are primarily of follicular origin and ovarian lymphangioma is an extremely rare lesion. We report a case of prenatal diagnosis of unilateral ovarian cyst in which pathologic examination revealed an ovarian lymphangioma. Ovarian cystic lymphangioma should be included in the differential diagnosis of an ovarian cyst mass in neonate.     

Congenital abdominal cystic lymphangiomas: what is the correct management?

Introduction: Intrabdominal cystic lymphangioma is a rare benign lesion with a variable clinical presentation. The aim of this study was to evaluate the natural outcome of this lesion and to propose a decision-making protocol for antenatal suspected intrabdominal lymphangioma. We also would like to emphasize the role of laparoscopy in treatment. Materials and Methods: This retrospective study examined 14 cases of intraabdominal cystic lymphangioma in children underwent to our observation, at the Pediatric Surgery Department in Lapeyronie Hospital, Montpellier between 1996 and 2007. Results: Of the 14 patients, 12 were operated on (7 by laparoscopy and 5 in open surgery). In 2 patients we didn’t perform intervention because the lesion was localized in the mesenteric root: we observed a regression of the lesion on US-scan. Conclusion: The intrabdominal cystic lymphangioma is a rare lesion which is now sometimes suspected on antenatal US scan; consequently we observed at the present time an increasing number of lesion potentially asymptomatic: in these case the management has to be established and a risk-benefit balance has to be made before a surgical intervention. Our experience is limited to a small number of cases, and it is too premature to describe any final conclusion; however our suggestion is that clinical course of cystic abdominal lymphangioma is unknown, and we propose that if no complication occurs, a clinical and ultrasonographic monitoring should be done in case of mesenteric root localisation.     

Linfangioma quístico de ovario

Cystic lymphangioma of the ovary is an extremely rare neoplasm and is usually detected as an incidental finding during surgery. This mass mimics cystic tumors of the ovary such as teratoma, hemangioma or adenomatoid tumor. The case of a 31-year-old patient with intermittent abdominal pain of slight to moderate intensity is presented. Abdominopelvic ultrasound revealed a tumor with multiple cysts with a solid component inside. During laparotomy, a solid, encapsulated and gray tumor was observed. When the tumor was cut, multiple cystic spaces of different sizes and with yellow, serous fluid were observed. Histopathological examination revealed a tumor with characteristics of cystic lymphangioma of the ovary.     

Prenatal diagnosis of a retroperitoneal lymphangioma: a case and review

Prenatal sonographic findings of lymphangiomas characteristically appear as thin-walled, multiseptate hypoechogenic masses. In our case, a retroperitoneal hypoechogenic mass was detected at 26 weeks of gestation by sonography. Serial sonographic examinations demonstrated multiple septations at 29 weeks of gestation, which in retrospect was a classical finding of retroperitoneal lymphangioma. MRI at 38 weeks of gestation revealed a multilocular mass, which was homogeneous, low on T1-weighted and high on T2-weighted images, in the left retroperitoneal cavity. These findings were compatible with the diagnosis of a lymphangioma. This case shows the change in characteristic imaging features, from a unilocular to multilocular pattern, of a lymphangioma with regard to gestational age. It is important to observe the size and extension of such tumors in order to determine the prospect for neonatal prognosis, as well as to make decisions on the delivery timing and style.     

Abdominal cystic lymphangioma in a woman at 14 weeks' gestation: case report

Lymphangiomas are uncommon benign tumors that most commonly present early in life, and are even more exceptional in adults. We present a rare case of a 14-week pregnant woman who had a large septated cystic lesion adherent to the ileum. Despite analysis by ultrasound, the correct diagnosis was established only via laparotomy and she had surgical resection of an abdominal lymphangioma.     

Cystic hygroma and congenital diaphragmatic hernia: early prenatal sonographic evaluation of Fryns' syndrome.

We report a case of cystic hygroma and diffuse lymphangiectasia detected by sonogram at 12 weeks' gestation. Fetal karyotype was normal. At 20 weeks' gestation, herniation of the bowel into the chest was noted. At delivery, the infant was diagnosed as having Fryns' syndrome. This is the first reported case of Fryns' syndrome presenting with cystic hygroma.     

Magnetic resonance image findings of placental lake: report of two cases

Two cases with a large cystic mass within the placenta are reported. By ultrasonography, it was found that both women had a subchorionic hypoechoic lesion (11.0 x 4.8 x 4.0 cm and 6.6 x 3.7 x 2.2 cm, respectively) at 24 and 35 weeks of gestation, respectively. In both cases, turbulent blood flow generated by a pulsatile jet flow (pulse rate; 40 to 60 beats per minute) into the cystic lesion seen on real-time imaging and lesions being low intensity on T1-weighted and isointensity on T2-weighted magnetic resonance image suggested that they contained fresh maternal blood. In both cases, the sonolucency of the lesions did not change until cesarean deliveries of females, both of whom were small-for-gestational-age infants (1940 g at 37 weeks and 2195 g at 37 weeks, respectively). Biochemical analysis of the fluid in the cystic lesion sampled during the cesarean section in the latter case confirmed that the fluid had originated from the maternal blood. These lesions histologically corresponded to large avillous areas surrounded by normal villi. Thus, a huge placental lake was diagnosed in both cases.     

Axillary cavernous lymphangioma in pregnancy and puerperium

BACKGROUND: Cavernous lymphangioma is a form of benign congenital abnormality grouped under lymphatic malformations, which can clinically manifest as a cystic tumor. Common areas are the neck and axillary region. Most cases are diagnosed in children under the age of 2, and occasionally the diagnosis is made in utero. CASE: We present the case of a 29-year-old primigravid woman with an axillary cavernous lymphangioma which rapidly increased in size during pregnancy, and describe the treatment plan in this unusual situation. CONCLUSION: The treatment of a large lymphangioma during pregnancy has to be well planned, considering the welfare of the mother and baby. Most of the current therapies have never been tested in pregnant or lactating women. We discuss the possible pathomechanisms for the growth of lymphangioma during pregnancy with the overproduction of cytokines such as vascular endothelial growth factor.     

Primary retroperitoneal mucinous cystadenoma of borderline malignancy: a case report and review of the literature.

A case of primary retroperitoneal mucinous cystic tumor of borderline malignancy is reported. A 36-year-old, nulliparous woman complained of abdominal fullness. Physical examination revealed a cystic mass 12-cm x 8-cm in size. At laparotomy, a cystic tumor was observed in the right retroperitoneal space. Both ovaries appeared normal and the uterus was almost normal except for small myomatous nodules. Removal of the retroperitoneal tumor, an appendectomy, and a myomectomy were performed. The histologic diagnosis was a primary retroperitoneal mucinous cystic tumor of borderline malignancy, similar to findings for ovarian tumor. There was no evidence of disease 6 months after the surgery.     

Prenatal and perinatal aspects of a giant fetal cervicothoracal lymphangioma.

A massive cervicothoracal lymphangioma was diagnosed in a fetus at 25 weeks of gestation. On ultrasound study, the mass showed septated, cystic components and extended from the right submandibular region to the right anterolateral thoracic wall including the right axilla and right scapula. Close sonographic follow-up revealed an increase in the size of the lymphangioma without fetal hydrops. An interdisciplinary approach including a pediatric surgeon, neonatologist, perinatologist and anesthesiologist was chosen. Elective cesarean section under general anesthesia was planned at 37 + 0 weeks of gestation. Surgical correction of the lymphangioma was successfully performed on the 4th day of life. Possible differential diagnoses and the obstetrical management are presented.     

Trisomy 16 in a mid-trimester IVF foetus with multiple abnormalities

An 18 week foetus with multiple system abnormalities was found to have full trisomy 16. This appears to be only the third reported case surviving into mid-gestation; typically, this common aneuploidy dies post-implantation. Similarities exist in the abnormalities found in the three cases suggesting that there is a 'surviving' trisomy 16 phenotype. It is characterised by: absent hemidiaphragm, pulmonary hypoplasia/aplasia, major cardiac defect, small chest, vertebral and rib defects, cystic kidneys, absent gall bladder, multiple spleens and imperforate anus, together with cleft palate, nuchal webbing/cystic hygroma, microcephaly, marked dysmorphic facial features and dorsiflexed great toe.     

Persistent pulmonary hypertension in a neonate with cystic adenomatoid malformation of the lung following lobectomy: survival with prolonged extracorporeal membrane oxygenation therapy.

A full-term neonate is reported with congenital cystic adenomatoid malformation of the lung treated by lobectomy with development of pulmonary hypertension. The infant was successfully treated with extracorporeal membrane oxygenation (ECMO) for persistent pulmonary hypertension, which developed postoperatively. An 18-day course of venovenous ECMO was necessary to effectively reverse the severe pulmonary hypertension. This was probably a result of significant pulmonary hypoplasia of the compressed lung. Although not all congenital cystic adenomatoid malformations of the lung are associated with pulmonary hypoplasia and persistent pulmonary hypertension, this is one case where severe pulmonary hypertension developed secondary to a mass effect by a large lesion in the chest.     

Postmenopausal hyperandrogenism caused by a benign cystic teratoma: a case report

BACKGROUND: Mature, benign cystic teratomas of the ovary are common in reproductive-age women, but they are very rarely associated with androgen production and subsequent development of hirsutism or virilization. We describe a case of postmenopausal hirsutism and hyperandrogenism caused by a mature cystic teratoma as well as the 7 previously reported cases. CASE: A 55-year-old, postmenopausal woman presented with hirsutism and unilateral lower extremity edema. Pelvic ultrasound showed a complex cystic mass in the left ovary measuring 6.0 x 7.0 x 10 cm, and laboratory evaluations revealed progressively increasing testosterone levels. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy, with resection of a large, complex mass originating in the left ovary. The pathology department found 2 left, mature ovarian cystic teratomas containing a layer of Leydig cells. Postoperatively the patient experienced rapid normalization of the elevated testosterone level. CONCLUSION: Although rare, ovarian production of androgens resulting in hirsutism or virilization can occur with a hormonally active mature cystic teratoma.     

Rare Case of Ovarian Cystic Lymphangioma

Lymphangiomas are rare, generally benign tumors of the lymphatic system comprised of multiple cystic spaces lined with endothelium. Lymphangiomas may arise in any part of the body. Lymphangioma of the ovary is rare; we have identified only 13 reports in a 50-year literature survey (PubMed 1959–2009). Typically, lymphangiomas are slow-growing tumors that remain asymptomatic for a long time. They are most often found incidentally in abdominal or pelvic imaging studies or at surgery or autopsy. Wide excision of the lesion with microscopically clear margins is the best approach when feasible. A postmenopausal woman had a symptomatic pelvic mass. Imaging studies demonstrated a complex left ovarian cyst. Complete removal of a cystic lymphangioma was successfully performed at laparoscopy. Cystic lymphangiomas should be included in the differential diagnosis of an ovarian cystic mass, and laparoscopic excision may be the method of treatment.     

Giant benign mucinous cystadenoma growing during pregnancy: a case report

A 32-year-old woman, gravida 4, para 2, was diagnosed with a benign right ovarian mucinous cystadenoma. It was diagnosed in the tenth week of pregnancy, measured 47 x 69 mm, and reached 190 x 152 mm at 23 weeks of gestation. Laparotomy was performed and the ovarian cystic mass was removed in the second trimester. The patient had postoperative tocolytic therapy and progesterone medication. She is at the 35th gestation week and no other antenatal complication has been reported. A case of giant mucinous cystadenoma is presented with clinical follow-up details.