Primitive Cystic Hepatic Neoplasm of Infancy with Mesothelial Differentiation: Report of a Case

Abstract

A malignant cystic neoplasm in the liver of a 6-month-old girl is reported. The neoplasm has unique clinical and morphological features, and despite ultrastructural and immunohistochemical studies, the tumor could not be clearly classified as any known primary hepatic neoplasm of infancy. A differentiated cystic component of the neoplasm had features of cystic peritoneal mesothelioma. Alpha-1-antitrypsin and alpha-fetoprotein were demonstrated in solid, anaplastic portions of the recurrent tumor by immunohistochemistry. The neoplasm recurred after surgical resection and proved fatal 11 months following diagnosis, despite multiple courses of chemotherapy.

Malignant transformation of “benign” cystic mesothelioma of the peritoneum

“…Furthermore, one patient reported in the literature that did not receive any treatment died from the disease 12 years after diagnosis [2]. Another patient reported with an initial diagnosis of cystic mesothelioma died of diffuse malignant mesothelioma 11 months later [3]. Additional evidence of a benign to malignant transformation of peritoneal cystic mesothelioma is provided by the following case report.…”

“…There has been only one other case reported that described a similar phenomenon [3]. The patient was a 6-month-old infant diagnosed with a cystic lesion growing into the liver parenchyma.…”

“…The suggestion that peritoneal cystic mesothelioma has an intermediate position within the clinico-pathological spectrum of mesothelial neoplasms, between the truly benign adenomatoid tumor and the diffuse forms of malignant mesothelioma, has greater relevance [2,16]. The 6-month-old infant reported by De Stephano et al [3] presented with simultaneous benign cystic and malignant mesothelioma components at the time of initial diagnosis. Consequently, one cannot know if the lesion had its origin in a benign component, and the possibility that malignant degeneration might have occurred cannot be determined.…”

Benign multicystic peritoneal mesothelioma in a cesarean-section scar presenting as a fungating mass

“…6,12,16,26 The clinical course of BMM is usually benign. No metastasis, but rare malignant transformation 16,18,19 and death 6 have been documented. BMM occurs more commonly in females (87%), particularly in the pelvic area of the cul-desac, uterus, and rectum.…”

Malignant peritoneal mesothelioma

“…However, it has been suggested that cautions should be made in the assessment of this condition as a ''benign'' process. This is mainly because of the high recurrence rate (27-75%) and because of the reported transition to conventional MPM [41][42][43][44].…”