Congenital pulmonary myofibroblastic tumor—pathology and prenatal sonographic appearance

Reiss, Ari; Goldberg, Yael; Monichor, Mariana; Drugan, Arie

doi:10.1002/pd.1293

Cited by 11 publications

(9 citation statements)

References 4 publications

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“…The microscopic pattern of CPMT spans from that of uniform spindle cells4,12,13 to that harboring peribronchial cartilage proliferation6,9,14-16 and/or having a hemangiopericytoma-like area 2,10,17. The phenotype of the tumor cells has been related to that of myofibroblasts or fibroblasts, with variable reactivity to smooth muscle actin or muscle specific actin1,2,10,12-17 and the variable presence of cytoplasmic filaments in electron microscopy.…”

Section: Discussionmentioning

confidence: 99%

“…Since CPMT has been known to manifest certain features of malignant neoplasm, such as frequent mitosis (greater than 10/10 hpf2,5,7), necrosis,2,12,13,17 and infiltrative growth, some CPMT cases have been reported as malignant entities, such as bronchopulmonary fibrosarcoma2,3 and bronchopulmonary leiomyosarcoma 4,5. However, this neoplasm is benign, as it has not recurred or metastasized in patients surviving through the tumor-removal operation, even with positive surgical resection margins (current case and Huppmann et al16).…”

Section: Discussionmentioning

confidence: 99%

“…Bronchopulmonary fibrosarcoma is a low-grade malignancy, which presents as an endobronchial or intraparenchymal mass consisting of bundled spindle cells. Among 30 pediatric cases reported in the literature,2,19,20 four of five neonatal cases have been reclassified as CPMT 1,13. The recent, fifth neonatal case20 may also be considered a CPMT as it was composed of monotonous spindle cells with a high mitotic rate (20/10 hpf) and had not recurred during the 16-month follow-up.…”

Section: Discussionmentioning

confidence: 99%

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Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review

et al. 2013

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Congenital peribronchial myofibroblastic tumor (CPMT) is a benign pulmonary spindle cell neoplasm of intrauterine and perinatal period, which is thought to arise from primitive peribronchial mesenchyme. We present a case detected incidentally in a one-month-old infant. The solid and partially necrotic tumor involved the right middle and lower lobes of the lung with extension to the diaphragm. Histologically, the tumor was composed of fasciculated monotonous spindle cells, proliferating peribronchiolar cartilage and round cells with rich vasculature, and high mitotic activity was identified in the round cell area. Immunohistochemical and electron microscopic studies showed that the spindle cells were myofibroblastic in phenotype. Although the tumor showed several malignant pathological features, recurrence was not observed in the two-year follow-up period, consistent with the benign clinical behavior of CPMT.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review

et al. 2013

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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: 99%

“…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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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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Congenital peribronchial myofibroblastic tumor: prenatal imaging clues to differentiate from other fetal chest lesions

et al. 2013

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We present a prenatal case of congenital peribronchial myofibroblastic tumor referred as a congenital pulmonary airway malformation (CPAM) with hydrops and polyhydramnios at 30 weeks' gestational age. US and fetal MRI findings did not fit with the referral diagnosis, raising the possibility of intrinsic lung tumor. Fetal hydrops worsened and the baby was successfully delivered by ex utero intrapartum treatment (EXIT) to resection at 31 weeks' gestational age. To the best of our knowledge, this is a unique case of congenital peribronchial myofibroblastic tumor that underwent comprehensive prenatal evaluation and EXIT procedure with good outcome.

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Congenital pulmonary myofibroblastic tumor—pathology and prenatal sonographic appearance

Cited by 11 publications

References 4 publications

Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review

Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review

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

Congenital peribronchial myofibroblastic tumor: prenatal imaging clues to differentiate from other fetal chest lesions

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