A Rare Presentation of Isolated Congenital Splenic Hemangioma With Kasabach-Merritt Syndrome.

INTRODUCTION: Splenic hemangiomas (SHs) are the most common benign neoplasms of the spleen. However, they are rare in the newborn period. We present an extremely rare case of congenital SH complicated by Kasabach-Merritt syndrome. CASE PRESENTATION: A 2.93 kg male infant was delivered at term with a prenatal diagnosis of a left infrarenal mass diagnosed by ultrasound at 35 weeks of gestation. Magnetic resonance imaging demonstrated a well-defined splenic mass with multiple flow voids and scattered areas of high intensity suggestive of hemorrhage. He developed anemia, thrombocytopenia, and coagulopathy which required transfusion with packed red cells, platelets, cryoprecipitate, and fresh frozen plasma. Excision biopsy of the spleen led to resolution of anemia, thrombocytopenia, and coagulopathy. The diagnosis of SH was confirmed by histopathology. At 2 months outpatient follow-up, the patient was growing well without any evidence of tumor recurrence. CONCLUSIONS: Congenital SH is a rare entity that can be fatal if the potential complication of Kasabach-Merritt syndrome is not anticipated, evaluated, and promptly treated. Our patient had a favorable outcome with early surgical excision of the SH.

Ruptured splenic cavernous hemangioma in a neonate.

We present a case of a term neonate with hypovolemic shock after spontaneous vaginal delivery. Hemodynamic instability persisted despite resuscitation with packed red cells, fresh frozen plasma, and platelets. An ultrasound scan at 48 hours after birth followed by a computed tomographic scan demonstrated a splenic lesion and hemoperitoneum. She underwent an emergency laparotomy and splenectomy for splenic rupture. Histologic findings demonstrated a ruptured cavernous hemangioma of the spleen. Exsanguinating intraabdominal hemorrhage in the newborn infant is rare. The diagnosis and management, with particular reference to splenic cavernous hemangioma and splenic rupture, is discussed.

Congenital occurrence of solitary infantile myofibromatosis of the spleen.

Infantile myofibromatosis (IM) is a rare soft tissue tumor of infancy and childhood. We report the case of a newborn girl with an abdominal tumor discovered at 32 weeks of gestation by fetal ultrasound. She underwent a laparotomy for an unexplained abdominal mass 20 days after birth. The tumor originated from the spleen and was removed by splenectomy. There were no other abnormal findings on diagnostic modalities. Based on the histological examinations, the tumor was diagnosed as an IM. Although extremely rare during the neonatal period, solitary type IM should be considered as a differential diagnosis of the splenic tumor.

Bilateral adrenal cystic neuroblastoma with hepatic and splenic involvement in a newborn.

Bilateral cystic adrenal neuroblastoma is an unusual variant of neuroblastoma, and only two cases have been reported in the neonatal period until now. The authors describe a newborn with splenic and hepatic involvement of bilateral adrenal cystic neuroblastoma.

[Acute peritonitis: a rare presentation of chylous ascites]. / Péritonite aiguë: un mode de révélation rare d'ascite chyleuse.

Chylous ascites is a rare case of peritonitis. We report here a case arising in a 21-year-old lady.

Hereditary melanoblastoma in miniature pigs and its successful therapy by devitalization technique.

Applying selective breeding for 10 years we established the MeLiM (Melanoblastoma-bearing Libechov Minipigs) strain. Melanoblastoma (MB) in this strain shows a hereditary occurrence. Cutaneous tumours are usually nodular, multiple and distributed on various parts of body. They appear in darkly pigmented animals already at the birth or during two months thereafter (57% of all animals). Numerous organ metastases mainly into the spleen, lymph nodes and lungs are regularly ascertained in animals with cutaneous MB. Tumour cells were surprisingly found also in the inner organs of phenotypically healthy minipigs in which no cutaneous MBs were observed visually (27% of all animals). About 34% of all affected piglets die during the first 2 months of age. These features document a malignancy of this tumour in the MeLiM strain. Original surgical technique was applied in more than 40 affected minipigs at 1-2 months of age. It consists in a devitalization (ischemization) of one of cutaneous tumours by the mattress sutures conducted around the tumour base without any excision of tumour tissue. This simple procedure causes a total destruction of MB cells in all cutaneous tumours as well as in all organ metastases during 4-6 months. Animals treated by this technique were fully healed of tumour cells and no relapses were observed. This technique could bring similar positive results also in therapy of human MB.

Diffuse splenic and visceral hemangiomas complicated by chronic consumption coagulopathy.

The case of a 7-year-old girl with a 2 year history of easy bruising associated with thrombocytopenia is reported. On admission she presented with ecchymoses, abdominal distention and splenomegaly. Hemostasis investigation revealed a consumption coagulopathy. Several radiological studies failed to confirm the diagnosis of diffuse splenic and visceral hemangiomatosis, which was eventually established by an explorative laparotomy. Platelet count and the other coagulation abnormalities progressively returned to normal after splenectomy, although the remaining hemangiomas were extensive.

Splenic hemangioma with thrombocytopenia in a newborn.

The case report of a newborn infant with a splenic hemangioendothelioma with the life-threatening complications of thrombocytopenia, anemia, and disseminated intravascular coagulation is presented together with a review of the literature. Removal of the tumor via splenectomy, despite the known risk of subsequent overwhelming sepsis due to encapsulated organisms in the young child, is the treatment of choice for splenic hemangiomas complicated by thrombocytopenia. The use of enhanced CT with delayed filling is a diagnostic tool in the workup of suspected hemangiomatous lesions.