Fetal hydrops associated with congenital pulmonary myofibroblastic tumor

Horikoshi, Toru; Kikuchi, Akihiko; Matsumoto, Yasuhiro; Tatematsu, Mikiko; Takae, Kentaro; Ogiso, Yoshifumi; Nakayama, Masahiro; Unno, Nobuya

doi:10.1111/j.1447-0756.2005.00335.x

Cited by 23 publications

(21 citation statements)

References 10 publications

(19 reference statements)

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“…Histopathological findings are similar in the literature; i.e., cellular spindled myofibroblastic cells proliferating around the bronchial cartilage. Central necrosis was reported in three cases, all at the centre of the tumors (10,11,12). Thus, this is a coagulation necrosis, rather than a tumor necrosis.…”

Section: Discussionmentioning

confidence: 88%

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

Acikalin¹,

Gümürdülü²,

Bağır³

et al. 2013

Balkan Med J

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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 ...

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Section: Discussionmentioning

confidence: 88%

“…This term was more useful, because of the tumor's favourable outcome, in spite of its morphology. A summary of the English-language reported cases is given in Table 1 (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14).…”

Section: Discussionmentioning

confidence: 99%

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

Acikalin¹,

Gümürdülü²,

Bağır³

et al. 2013

Balkan Med J

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show abstract

“…The tumors typically measure 5-7 cm in diameter and occupy the majority of the involved hemithorax, exerting mass affect with mediastinal shift (Travis et al 2004). Hydrops fetalis is reported in 36 % and polyhydramnios in 27 % of cases (Horikoshi et al 2005). CPMT has a heterogeneously hyperechoic appearance on ultrasound and a heterogeneous appearance on CT without cysts or calcifications (Travis et al 2004).…”

Section: Congenital Peribronchial Myofibroblastic Tumormentioning

confidence: 97%

“…The tumor is typically detected in the neonatal period, and antenatal presentation with fetal hydrops has been reported (Horikoshi et al 2005). Although histologically benign, CPMT can exhibit certain aggressive features such as frequent mitoses, necrosis, and infiltrative growth, leading some cases to be reported as malignant entities such as bronchopulmonary fibrosarcoma (Kim et al 2013).…”

Section: Congenital Peribronchial Myofibroblastic Tumormentioning

confidence: 99%

Pulmonary and Extrathymic Mediastinal Tumors

Lyons

Guillerman

McHugh

2014

Pediatric Chest Imaging

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“…CPMT is very rare with approximately 25 cases reported in the literature [ 5 ] and the largest case series detailing 11 patients [ 80 ]. The mass is typically detected in the neonatal period, and prenatal detection has also been reported [ 80 ].…”

Section: Clinical Features and Epidemiologymentioning

confidence: 98%