Pediatric and Developmental Pathology 17, 406-408, 2014 DOI: 10.2350/14-06-1501-CR.1 © 2014 Society fo r Pediatric Pathology
Pediatric Gastrointestinal PEComas: A Diagnostic Challenge M
arco
P izzi, 1 * I d a
di
L orenzo , 2 Em anuele S.
d ' A more , 3
Pao lo D 'A ngelo , 4
and
R ita A
la g g io 1
d e p a rtm e n t of Medicine-DIMED, General Pathology & Cytopathology Unit, University of Padova, Padova, Italy 2Pathology Unit, ARNAS Civico, Di Cristina and Benfratelli Hospital, Palermo, Italy 3Pathology Department, "San Bortolo” Hospital, Vicenza, Italy 4Pediatric Hematology and Oncology Unit, ARNAS Civico, Di Cristina and Benfratelli Hospital, Palermo, Italy
Received June 1, 2014; accepted July 14, 2014; published online July 14, 2014. ABSTRACT
CASE REPORT
Perivascular epithelioid cell tumors (PEComas) in the pediatric population are very rare and frequently arise in the gastrointestinal tract. These tumors are characterized by variable morphological and immunohistochemical features, which may pose significant problems in differential diagnosis with other gastrointestinal mesen chymal tumors in pediatric patients. In presenting an unusual pediatric case of gastrointestinal PEComa, we also discuss the possible differential diagnosis of this rare entity.
An 8-year-old Caucasian girl presented at Di Cristina and Benfratelli Hospital (Palermo, Italy) with mild abdominal tenderness and epigastric pain. Computed tomographic scan revealed an intraperitoneal mass (greater diameter 8 cm) that was partially adherent to the transverse colon. After surgical excision (complete resection with negative margins [RO]), gross examination revealed a roundish, pedunculated lesion with focal hemorrhagic areas. Histologic examination revealed that the tumor was composed of a fascicular proliferation of spindle to epithelioid cells, focally arranged in a storiform growth pattern. Intratumor blood vessels were tiny and incon spicuous, with fascicles of neoplastic cells often arranged around them. Despite the presence of occasional hemor rhagic areas, tumor coagulative necrosis was consistently absent. The neoplastic population was composed of medium-sized, spindle to polygonal elements, with amphophilic cytoplasm, elongated nuclei, finely dispersed chromatin, and an absence of evident nucleoli. The mitotic rate was extremely low (
Pediatric Gastrointestinal PEComas: A Diagnostic Challenge M
arco
P izzi, 1 * I d a
di
L orenzo , 2 Em anuele S.
d ' A more , 3
Pao lo D 'A ngelo , 4
and
R ita A
la g g io 1
d e p a rtm e n t of Medicine-DIMED, General Pathology & Cytopathology Unit, University of Padova, Padova, Italy 2Pathology Unit, ARNAS Civico, Di Cristina and Benfratelli Hospital, Palermo, Italy 3Pathology Department, "San Bortolo” Hospital, Vicenza, Italy 4Pediatric Hematology and Oncology Unit, ARNAS Civico, Di Cristina and Benfratelli Hospital, Palermo, Italy
Received June 1, 2014; accepted July 14, 2014; published online July 14, 2014. ABSTRACT
CASE REPORT
Perivascular epithelioid cell tumors (PEComas) in the pediatric population are very rare and frequently arise in the gastrointestinal tract. These tumors are characterized by variable morphological and immunohistochemical features, which may pose significant problems in differential diagnosis with other gastrointestinal mesen chymal tumors in pediatric patients. In presenting an unusual pediatric case of gastrointestinal PEComa, we also discuss the possible differential diagnosis of this rare entity.
An 8-year-old Caucasian girl presented at Di Cristina and Benfratelli Hospital (Palermo, Italy) with mild abdominal tenderness and epigastric pain. Computed tomographic scan revealed an intraperitoneal mass (greater diameter 8 cm) that was partially adherent to the transverse colon. After surgical excision (complete resection with negative margins [RO]), gross examination revealed a roundish, pedunculated lesion with focal hemorrhagic areas. Histologic examination revealed that the tumor was composed of a fascicular proliferation of spindle to epithelioid cells, focally arranged in a storiform growth pattern. Intratumor blood vessels were tiny and incon spicuous, with fascicles of neoplastic cells often arranged around them. Despite the presence of occasional hemor rhagic areas, tumor coagulative necrosis was consistently absent. The neoplastic population was composed of medium-sized, spindle to polygonal elements, with amphophilic cytoplasm, elongated nuclei, finely dispersed chromatin, and an absence of evident nucleoli. The mitotic rate was extremely low (
