Congenital Peribronchial Myofibroblastic Tumor: A Case Report and Review of the Literature

Abstract

IntroductionCongenital peribronchial myofibroblastic tumor (CPMT) is a rare tumor of childhood that is thought to develop during the early weeks of intrauterine life. It is usually associated with hydrops fetalis and polyhydramnios, and can be detected during prenatal evaluation by ultrasonography. We have found 15 cases in the English-language clinical literature; six of these cases were reported as doing well at 3 months to 6.5 years after resection. In this report, we present the seventh surviving patient, with radiographic, gross, and microscopic features of the tumor, together with a review of the literature. Case ReportA 38 year-old female patient, gravida five, para two, abortus one (G5P2A1), at 35 weeks of gestation, was referred to the obstetrics department of our institute with polyhydramnios. She was married to a relative, a maternal cousin. Her first child had died 3 days after birth of unknown causes. Her second and third children are 17 and 15 years old, with no health problems. Her fourth pregnancy was a miscarriage at 21 weeks of gestation. An autopsy performed on this foetus had revealed omphalocele, olygodactyly of the left foot, and flexion deformity of the left hand.Our case was her fifth pregnancy. Prenatal ultrasonography at 35 weeks revealed polyhydramnios, hydrothorax, and a mass in the right lung. The male neonate was delivered by caesarean section at the 37 th gestational week. Birth weight was 3100 gr. The infant was cyanotic, and did not cry at birth; he required intubation and positive-pressure ventilation. Postnatal echocardiography revealed a mass in the right lung, hydrothorax, patent foramen ovale, and pulmonary hypertension. Postnatal computerised tomography showed a solid mass in the right lung, about 6 cm in diameter (Figure 1). Serum levels of alpha-fetoprotein (AFP) and neuron-specific enolase (NSE) were 15430 ng/mL (normal: 40-150 ng/mL) and 45.02 ng/mL (normal: 0-16.3 ng/mL), respectively.On day 12 after delivery, the neonate was extubated and was stable after surfactant therapy. A right middle lobectomy was performed by thoracotomy, during which a wellcircumscribed mass, originating from the right middle lobe, was found. Upper and lower lobes were quite normal, and a formal lobectomy was possible without any difficulty. No complications occurred in the postoperative period, and the infant breathed spontaneously. It has been 26 months since the surgery, and the patient is doing well, with no recurrence. Pathological FindingsThe mass removed at lobectomy was well circumscribed, with no true capsule, and was 6.5x5.5 cm in size (Figure 2). There was a small area of compressed lung tissue surrounding the mass. Its cut surface was solid and grey-tan, with necrotic areas at the centre.Microscopically, lung parenchyma was replaced by wellcircumscribed, non-encapsulated mesenchymal tumor (Figure 3). Tumor cells were uniformly spindle-shaped, with finely granular chromatin (Figure 4). There was no anaplasia or pleomorphism. Tumor cells were surrounding the bronchial walls, but not ...