Congenital Pulmonary Myofibroblastic Tumor: A Case Report with Cytogenetic Analysis and Review of the Literature

Alobeid, Bachir; Beneck, Debra; Sreekantaiah, Chandrika; Abbi, Rakesh; Slim, Michel S.

doi:10.1097/00000478-199705000-00016

Cited by 43 publications

(41 citation statements)

References 10 publications

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“…Immunohistochemically, tumor cells were positive for vimentin in all of the studied cases. Focally, SMA positivity was also reported in three cases (10,13,14), but was negative in others, as in our case. Electron microscopic evaluation suggested a myofibroblastic differentiation.…”

Section: Discussionsupporting

confidence: 72%

“…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: 87%

“…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%

“…Electron microscopic evaluation suggested a myofibroblastic differentiation. Ultrastructurally, spindle-shaped cells' cytoplasm contained rough endoplasmic reticulum, mitochondria, ribosomes, lysosomes, and a few lipid droplets; some showed bundles of actin filaments (10).…”

Section: Discussionmentioning

confidence: 99%

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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: Discussionsupporting

confidence: 72%

Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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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“…8 The neoplastic cells in this case stained for vimentin, desmin, and smooth muscle actin, as has previously been reported for neoplastic myofibroblasts. 1,5,9 Electron microscopic features such as intracytoplasmic microfilaments and electron-dense bands running parallel to the plasma membrane are consistent with myofibroblasts or smooth muscle cells. 8 Criteria used to diagnose this neoplasm are similar to those used in human cases and include vimentin and actin expression, the finding of bundles of electron-dense myofibers running parallel to the plasma membrane, and intracytoplasmic microfilaments.…”

mentioning

confidence: 89%

Abdominal Spindle Cell Sarcoma of Probable Myofibroblastic Origin in a Horse

et al. 1999

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An 18-year-old gelding Thoroughbred was referred to Virginia-Maryland Regional College of Veterinary Medicine for evaluation of anorexia, pyrexia, chronic weight loss, and a distended abdomen. Three years previously, this horse was diagnosed with acute septic hepatocholangitis based on physical, bacteriologic, biochemical, and histopathologic findings. Streptococcus viridans was isolated from the liver biopsy samples. Treatment included intravenous fluids, B vitamins, and antibiotics. The horse recovered and had normal blood chemistry and complete blood counts within 5 months of this episode. After recovery, the horse developed intermittent dermal photosensitization involving the lightly pigmented skin of the distal hind limbs.Current physical examination findings included mild depression, lethargy, patchy alopecia and scaling, abdominal distention, generalized muscle atrophy, and poor body condition. There were bilaterally symmetrical 10 ϫ 5 ϫ 1-cm raised swellings in the subcutaneous tissue of the prepuce. Serum biochemical analysis revealed hyperproteinemia (8.8 g/dl). The horse had a leukocytosis (20.1 ϫ 10 3 cells/l) characterized by mature neutrophilia (17.69 ϫ 10 3 cells/l). Results of serum electrophoresis were consistent with chronic inflammation. All other blood parameters were considered normal. Rectal palpation identified a large nodular mass in the caudal abdomen that was confirmed by transrectal and transabdominal ultrasound examination. No distinct organ involvement was identified, and the liver appeared normal. Abdominocentesis yielded a voluminous yellow/orange turbid fluid with an elevated protein level (4.7 g/dl) but a normal total nucleated cell count (300 cells/l). Neutrophils and small lymphocytes were identified on cytologic preparations. Ultrasound examination of the parapreputial swellings demonstrated subcutaneous multiloculated masses. Cytologic examination of fluid obtained by fine-needle aspiration from these masses revealed a monomorphic population of small lymphocytes and occasional erythrophagocytic macrophages.Because of the rapid and severe deterioration of the patient and a high suspicion of abdominal neoplasia, the horse was euthanized and a postmortem examination was performed. Necropsy examination revealed significant muscle atrophy and loss of body fat stores, with grossly observable prominent bony processes and ribs. The abdominal cavity contained approximately 250 ml of clear yellow fluid in as- Received for publication June 11, 1998. sociation with a multiloculated, encapsulated, cystic 81.82-kg mass within the intestinal mesentery (Fig. 1). The mass was cavitated and contained 5-7 liters of red fluid admixed with gelatinous tan material and blood clots. The capsule was thick, firm, and white and composed of variable amounts of fibrous connective tissue, which surrounded and encapsulated many of the enlarged, swollen mesenteric lymph nodes. Numerous fibrous adhesions extended from the capsular surface of the mass to the serosal surface of loops of both the small and l...

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Identification of novel ALK rearrangement A2M–ALK in a neonate with fetal lung interstitial tumor

Onoda

Miyako

Sato

et al. 2014

Genes Chromosomes & Cancer

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Fetal lung interstitial tumor (FLIT) is a recently reported type of congenital lung lesion comprising solid and cystic components. The pathological features include unique interstitial mesenchyme-based cell proliferation, and differ from other neoplasms represented by pleuropulmonary blastoma or congenital peribronchial myofibroblastic tumor. FLIT is extremely rare and its gene expression profile has not yet been reported. We provide the first report of a novel chromosomal rearrangement resulting in α-2-macroglobulin (A2M) and anaplastic lymphoma kinase (ALK) gene fusion in a patient with FLIT. The tumor cells contained a t(2;12)(p23;p13) and were mesenchymal in origin (e.g., inflammatory myofibroblastic tumors), suggesting the involvement of ALK in this case of FLIT. Break apart fluorescence in situ hybridization demonstrated chromosomal rearrangement at ALK 2p23. Using 5'-rapid amplification of cDNA ends, we further identified a novel transcript fusing exon 22 of A2M to exon 19 of ALK, which was confirmed by reverse-transcription polymerase chain reaction. The corresponding chimeric gene was subsequently confirmed by sequencing, including the genomic break point between intron 22 and 18 of A2M and ALK, respectively. Discovery of A2M as a novel ALK fusion partner, together with the involvement of ALK, provides new insights into the pathogenesis of FLIT, and suggests the potential for new therapeutic strategies based on ALK inhibitors.

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Congenital Pulmonary Myofibroblastic Tumor: A Case Report with Cytogenetic Analysis and Review of the Literature

Cited by 43 publications

References 10 publications

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

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

Abdominal Spindle Cell Sarcoma of Probable Myofibroblastic Origin in a Horse

Identification of novel ALK rearrangement A2M–ALK in a neonate with fetal lung interstitial tumor

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