Journal of Pathology and Translational Medicine

Case Study

Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review: Yuil Kim, Ha Young Park, Junhun Cho, Joungho Han, Eun Yoon Cho; Korean J Pathol. 2013;47(2):172-176. Published online April 24, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.2.172

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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.

Citations

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Congenital peribronchial myofibroblastic tumor (CPMT): a case report with long term follow-up and next-generation sequencing (NGS)
Ping Zhou, Shuang Li, Weiya Wang, Yuan Tang, Lili Jiang
BMC Pediatrics.2023;[Epub] CrossRef
Neonatal congenital lung tumors — the importance of mid-second-trimester ultrasound as a diagnostic clue
Stephan L. Waelti, Laurent Garel, Dorothée Dal Soglio, Françoise Rypens, Michael Messerli, Josée Dubois
Pediatric Radiology.2017; 47(13): 1766. CrossRef
Congenital peribronchial myofibroblastic tumor: Case report and review of literature
Jolanta Jedrzkiewicz, Eric Scaife, Bo Hong, Sarah South, Mouied Alashari
Journal of Pediatric Surgery Case Reports.2015; 3(4): 154. CrossRef
Perinatal Thoracic Mass Lesions: Pre- and Postnatal Imaging
Evan J. Zucker, Monica Epelman, Beverley Newman
Seminars in Ultrasound, CT and MRI.2015; 36(6): 501. CrossRef
Prenatal imaging and immunohistochemical analysis of congenital peribronchial myofibroblastic tumor
Y.‐A. Tu, W.‐C. Lin, H.‐J. Chen, J.‐C. Shih
Ultrasound in Obstetrics & Gynecology.2015; 46(2): 247. CrossRef
A Congenital Peribronchial Myofibroblastic Tumor Detected in a Premature Infant at 28 Weeks but That Resolved in the Late Stage of Pregnancy
Bo Xia, Gang Yu, Chun Hong, Lei Zhang, Jing Tang, Cuifen Liu
Medicine.2015; 94(42): e1842. CrossRef
Congenital peribronchial myofibroblastic tumor
Yuka Hotokebuchi, Kenichi Kohashi, Satoshi Toyoshima, Naoko Matsumoto, Toshinori Nakashima, Yoshinao Oda
Pathology International.2014; 64(4): 189. CrossRef

Review

Pathologic Review of Cystic and Cavitary Lung Diseases: Na Rae Kim, Joungho Han; Korean J Pathol. 2012;46(5):407-414. Published online October 25, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.5.407

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Abstract PDF

Pulmonary cystic and cavitary lesions caused by diverse etiologies are commonly encountered in chest imaging. The terms "cyst" and "cavity" are used to describe air-filled regions in the center of a nodule or consolidation of the lung. To date, only radiologic aspects of these lesions have been addressed. The morphologies of pulmonary cystic and cavitary lesions exhibit a broad spectrum, ranging from benign to malignant pulmonary diseases of acquired or congenital origin, including variable infectious diseases. In this review, we summarized the differential diagnosis of pathological entities to provide pathologists and radiologists with an overview of the diseases most commonly associated with pulmonary cystic and cavitary lesions in adults and children. The results showed slightly different patterns in the distribution of the diseases in the two groups. The most common causes of cavitary lesions include malignancy and infection in adults, and congenital malformation in children. Therefore, identification of pathologic entities correlating with the radiologic findings, clinical course, and location of the lesion is important in the evaluation of cystic and cavitary lung lesions in order to avoid unnecessary surgical procedures or delayed treatment.

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High-Resolution Computed Tomography of Cystic Lung Disease
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Seminars in Respiratory and Critical Care Medicine.2022; 43(06): 792. CrossRef
Miliary tuberculosis in a paediatric patient with psoriasis
Jacob Kilgore, Jonathon Pelletier, Bradford Becken, Stephen Kenny, Samrat Das, Lisa Parnell
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Atypical pulmonary metastases in children: the spectrum of radiologic findings
Michal Scolnik, Luda Glozman, Ronen Bar-Yoseph, Michal Gur, Yazeed Toukan, Lea Bentur, Anat Ilivitzki
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Radiographic and CT appearance of cavitary pulmonary lesions in a lamb
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Australian Veterinary Journal.2021; 99(12): 529. CrossRef
Community-acquired Achromobacter xylosoxidans infection presenting as a cavitary lung disease in an immunocompetent patient
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Yeungnam University Journal of Medicine.2020; 37(1): 54. CrossRef
Clinical Research of Pulmonary Langerhans Cell Histiocytosis in Children
Dong Wang, Lei Cui, Zhi-Gang Li, Li Zhang, Hong-Yun Lian, Hong-Hao Ma, Yun-Ze Zhao, Xiao-Xi Zhao, Tian-You Wang, Rui Zhang
Chinese Medical Journal.2018; 131(15): 1793. CrossRef
Benign features of infection‐related tumor‐like lesions of the lung: A retrospective imaging review study
Chun‐Chao Huang, Sho‐Ting Hung, Wei‐Chin Chang, Chin‐Yin Sheu
Journal of Medical Imaging and Radiation Oncology.2017; 61(4): 481. CrossRef
Cavitary lung disease in renal transplant recipients: A single center experience
Gizem Kumru, Serkan Akturk, Siyar Erdogmus, Aysegul Gursoy Coruh, Acar Tuzuner, Sule Sengul, Kenan Keven
Transplantation Reports.2017; 2(4): 19. CrossRef
Solitary lung cavities: CT findings in malignant and non-malignant disease
C.S. Nin, V.V.S. de Souza, G.R.T. Alves, R.H. do Amaral, K.L. Irion, E. Marchiori, B. Hochhegger
Clinical Radiology.2016; 71(11): 1132. CrossRef
Radial endobronchial ultrasound with a guide sheath for diagnosis of peripheral cavitary lung lesions: a retrospective study
Manabu Hayama, Norio Okamoto, Hidekazu Suzuki, Motohiro Tamiya, Takayuki Shiroyama, Ayako Tanaka, Takuji Nishida, Takashi Nishihara, Nobuko Uehara, Naoko Morishita, Kunimitsu Kawahara, Tomonori Hirashima
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An infant with a hyperlucent chest mass: An unexpected diagnosis
Zarmina Ehsan, Jaimie D. Nathan, Carolyn M. Kercsmar
Pediatric Pulmonology.2015; 50(12): E52. CrossRef
The Pseudocavitation Sign of Lung Adenocarcinoma
Tina D. Tailor, Rodney A. Schmidt, Keith D. Eaton, Douglas E. Wood, Sudhakar N. J. Pipavath
Journal of Thoracic Imaging.2015; 30(5): 308. CrossRef
A Case of Pulmonary Artery Sarcoma Presented as Cavitary Pulmonary Lesions
Daniel Min, Ji-Hyun Lee, Hye-Cheol Jeong, Jung-Hyun Kim, Suk-Pyo Shin, Hong-Min Kim, Kyu Hyun Han, Hye Yun Jeong, Eun-Kyung Kim
Tuberculosis and Respiratory Diseases.2014; 76(3): 136. CrossRef

Case Reports

Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return.: Seong Wook Hwang, Mee Seon Kim, Po Eun Park, Tae In Park; Korean J Pathol. 2011;45(6):650-653.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.6.650

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Abstract PDF: Congenital pulmonary lymphangiectasia (CPL) is very rare. It shows diffuse pulmonary lymphatic dilatation without lymphatic proliferation. CPL can occur as a primary disorder or arise secondarily from other diseases such as the obstruction of pulmonary veins or lymphatics. The prognosis of CPL is very poor. Approximately 50% of infants are stillborn and most others usually die within the first day of life. The present case showed diffuse lymphangiectasia in the subpleural, interlobular, and peribronchovascular areas. The flat lining cells were immunohistochemically positive for D2-40 and CD31. CPL is usually diagnosed by clinicoradiological or postmortem examinations. However, our case was diagnosed by an antemortem lung biopsy. We report a case of CPL with total anomalous pulmonary venous return.

Coexistence of Intrapulmonary Bronchogenic Cyst and Congenital Cystic Adenomatoid Malformation: A Case Report.: Mee Hye Oh, Eun Ah Jung, Ji Hye Lee, Hyun Deuk Cho, Ki Hyun Seo, Seock Yeol Lee, Young Tong Kim; Korean J Pathol. 2011;45(1):92-95.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.1.92

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Abstract PDF

Congenital cystic lesions of the lung are uncommon and a conjunction of two or more lesions is very rare. We report here on a case of coexisting intrapulmonary bronchogenic cyst and congenital cystic adenomatoid malformation in a 13-year-old female with a cystic mass in the right upper lobe of the lung. Computed tomography showed a cystic lesion measuring 2.5 cm with an air fluid level and surrounding multicystic lesions in the right upper lobe. On gross examination, the cut surface showed a cystic mass containing inspissated mucinous material, and the cystic mass was surrounded by multiple small cysts. Microscopically, the larger cystic cavity was lined with pseudostratified ciliated columnar epithelium. The submucosal tissue contained mucinous glands and plates of cartilage. The surrounding smaller cysts or irregular spaces were lined with bronchiolar-type respiratory epithelium. We propose that this hybrid lung lesion may represent the missing link in a common embryologic pathway determined by the timing of mesenchymal and epithelial interactions.

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Case 2: Coexisting Cystic Lesions of Lung in a Term Neonate: A Management Dilemma
Bichitrananda Raut, Aakriti Soni, Susanta Kumar Badatya, Satish Saluja, Manoj Modi, Arun Soni
NeoReviews.2018; 19(9): e542. CrossRef

VATER Association: Three autopsy case reports with imusual defects.: Mi Ja Lee, Myeong Cherl Kook, Je G Chi; Korean J Pathol. 1995;29(5):678-683.

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Abstract: VATER association represents vertebral defects, anal atresia, tracheo-esophageal fistula with without esophageal atresia, renal defects and radial limb dysplasia. The probability of the simultaneous occurrence of any three of these defects is so unlikely that it suggests a sporadic non-random association. This non-random association appears to be related to some chromosomal anomalies, the caudal regression syndrome, mesodermal defects in early developmental period or the matemal use of sex hormones during embryogenesis. We report three autopsy cases of the VATER association that showed most of the known major and minor defects as well as an unusual concurrence of other defects, i.e., scoliosis, talipes varus, absent penis, urethral agenesis and stenosis, rectourethral fistula, rib anomaly, single umbilical artery, Meckel's diverticulum, diaphragmatic hemia, absent rectum, short neck, simian crease, low set ear, and hypoplastic lung.

Original Article

Infantile Myofibromatosis(Congenital Generalized Fibromatosis): Associated with multiple congenital malformations and basaloid follicular hamartomas in the skin.: Eun Sook Nam, Yoo Hun Kim, Han Kyeom Kim, Insun Kim, Je Geun Chi; Korean J Pathol. 1995;29(6):776-782.

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Abstract PDF: Infantile myofibromatosis with systemic involvement is a very rare disease and is characterized by numerous nodules composed of spindle cells of a myofibroblastic nature. There are often disseminated throughout the subcutis, muscle, skeleton and viscera. We report an autopsy case of infantile myofibromatosis in a stillborn female fetus of 32 weeks of gestation. The nodules, Imm to 2 cm, were found over the whole body and viscera. The involved viscera were the heart, tongue, esophagus, gastrointestinal tract, portal areas of the liver, spleen anc pancreas. There were also associated malformations, viz., frontal meningoencephalocele, flexion defer-mities, syndactyly, cleft palate, agenesis of corpus callosum, pachygyria, diaphragmatic hemia, renal hypoplasia, etc. Multiple basaloid follicular hamartomas of the skin were noted on the face and extremeties. There are no previous reports in the literature of infantile myofibromatosis in conjunction with the above skin lesion and congenital malformations.

Case Report

Congenital Uterine Cyst: A case report.: Chang Ho Cho; Korean J Pathol. 1996;30(10):954-956.

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Abstract PDF: I experienced a case of a congenital intramural cyst of the uterine fundus. On examination by light and electron microscope it was composed of a single layer of thin atrophied lining epithelium without secretory activity and was found to be derived from the paramesonephric duct. This case is presented with a brief review of the literature.

Original Articles

Congenital Anomalies Observed by Autopsies at the Seoul National University Children's Hospital.: Jin Haeng Chung, Jeong Wook Seo, Chong Jai Kim, Chul Woo Kim, Je G Chi; Korean J Pathol. 1997;31(2):93-99.

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Abstract PDF: A retrospective analysis was performed on the 968 cases of fetal or pediatric autopsies over five year period (1990-1994), at the Seoul National University Children's Hospital. Age/mode distribution of cases were artificial abortus(30.6%), spontaneous abortus(12.0%), stillbirth(21.9%), neonates(29%), infants(2.8%) and children(0.9%). Male/female ratio was 1.21. Overall incidence of congenital anomalies was 60.8% and 34.0% of all cases had anomalies involving multiple organ systems. Percentage of cases with any anomaly was 71.6% in artificial abortus, 35.3% in spontaneous abortus, 59% in still births, 65.5% in neonates and 38.9% in infant and children. Common organ systems involved were the cardiovascular system (39.0%), musculoskeletal system (23.6%), nervous system (22.6%), gastrointestinal system (19.9%), and urinary system (14.6%). From these results, we found that the congenital anomalies were most significant diseases of the perinatal period and the cardiovascular anomalies were the most common anomalies of them.

Congenital Cystic Disease of the Kidney overview and a classification.: Mee Joo, Yeon Mee Kim, Chong Jai Kim, Yeon Lim Suh, Jeong Wook Seo, Je Geun Chi; Korean J Pathol. 1997;31(3):233-243.

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Abstract PDF: The congenital renal cystic disease encompasses a complex group of pathologic and clinical entities. We retrospectively reviewed 42 cases of congenital renal cystic lesions classified into four Potter types in a series of 2,063 consecutive autopsies from 1981 to 1996. According to our study based on morphologic, clinical, genetic features and associated anomalies, type I and III are relatively compatible with Potter's original definition. However, it was reasonable that type II and IV are classified to the same group because of: 1) very similar histologic findings representing dysplastic kidney, 2) many associated anomalies, 3) no evidence of inheritance, and 4) presence of a combined type. Syndrome associated cysts, such as Meckel-Gruber syndrome, were also separately classified. If the dysplastic evidence was insufficient for diagnosis to the dysplastic kidney in type II and IV, then these cases would be better classified into a cystic disease associated with congenital hydronephrosis. We propose a classification of the congenital cystic disease of the kidney to be: 1) dysplastic kidney, 2) cystic disease associated with congenital hydronephrosis, 3) polycystic kidney, and 4) syndromic cystic disease.

Case Reports

Congenital Hepatic Fibrosis with Caroli's Disease.: Yoon Jung Kim, Soon Ae Oak, In Chul Lee; Korean J Pathol. 1997;31(3):275-279.

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Abstract PDF: Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.

Congenital Mesoblastic Nephroma.: Seok Hoon Jeon, Seung Sam Paik, Nam Hoon Kim, Moon Hyang Park, Jung Dal Lee; Korean J Pathol. 1997;31(4):375-378.

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Abstract PDF: Mesoblastic nephroma is an important differential diagnosis of a renal mass occurring in the neonatal period or in early childhood. It is a rare monomorphous congenital renal neoplasm most commonly recognized during the first 3 months of life. With the widespread application of ultrasound imaging, many cases are recognized prior to birth. We report a case of mesoblastic nephroma detected by ultrasonograph at 36 weeks of intrauterine fetal life and removed after birth. It showed a well circumscribed, grayish white, solid mass measuring 4x3x2 cm. The tumor was predominantly a classic type with a focal cellular pattern. Immunohistochemical and electron microscopic studies were done.

Achondrogenesis Type 2: An autopsy case.: Joon Mee Kim, Young Chae Chu, Soo Kee Min, Hee Jeung Cha, Je Geun Chi; Korean J Pathol. 1997;31(5):482-488.

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Abstract PDF: Achondrogenesis type 2 is a lethal form of congenital skeletal dysplasia characterized by severe short-limbed dwarfism, decreased vertebral ossification and normal ossification of the skull. We report an autopsy case of achondrogenesis type 2 in a female fetus terminated at 29 weeks of gestation. External morphology revealed a relatively large head, short upper and lower extremities, short neck, and distended abdomen. The x-ray finding showed normal calvarial ossification, hypoplastic ilium and unossified ischium, and metaphyseal flares of the femur and tibia. Histologically, chondrocytes were large and irregular with increased vascularity.

A Sialoblastoma Associated with a Hepatoblastoma: An autopsy case report.: Sun Lee, Youn Wha Kim, Jae Hoon Park, Yong Koo Park, Ju hie Lee, Moon Ho Yang; Korean J Pathol. 1997;31(11):1222-1226.

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Abstract: Sialoblastoma is defined as a rare, congenital or perinatal, aggressive and potentially low-grade malignant, basaloid gland neoplasm that occurs in the major salivary glands. We report a case of a congenital sialoblastoma in the left parotid gland, associated with a hepatoblastoma in a female infant. At birth, a huge mass in the left neck and hepatomegaly were noted. Grossly, the neck mass was well-circumscribed, lobulated and gray tan. Microscopically, the tumor was composed of basaloid aggregates of primitive uniform cells with focal ductal differentiation. The liver showed a well-circumscribed gray tan tumor with extensive hemorrhage and cystic change. Microscopically, the liver revealed characteristic findings of hepatoblastoma. To the best of our knowledge, this is the first case of coexistence of a congenital sialoblastoma and a hepatoblastoma, reported in the literature.

Congenital Sialoblastoma: A case report and review.: Jong In Yook, Hee Jeong Ahn, Jin Kim; Korean J Pathol. 1997;31(11):1227-1232.

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Abstract: A congenital salivary gland tumor, sialoblastoma, is extremely rare. A sialoblastoma of the parotid gland, occurring in a 28-week old fetus, is described. The histologic, immunohistochemical, and ultrastructural features of this tumor were studied. The tumor was characterized by solid nests or sheets of tumor cells intermingled with ductal structures lined by a columnar cells. Some of the tumor cells showed squamous differentiation. Immunohistochemically, these epidermoid cells reacted positively with anti-cytokeratin. But anti-S-100, anti- vimentin, anti-smooth muscle actin, anti-GFAP positive cells were not found. The ultrastructure was characterized by primitive epithelial cells. Although various names have been proposed, we favored the term "sialoblastoma". The histogenesis of this tumor is also discussed.

Original Article

Cystic Hygroma of the Neck Pathologic study of 26 autopsy cases.: Yeon Lim Suh, Je Geun Chi; Korean J Pathol. 1997;31(12):1256-1263.

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Abstract PDF: Cystic hygroma is a congenital malformation of the lymphatic system appearing single or multiloculated fluid-filled cavities, most often around the neck. They often progress to hydrops and cause fetal death, and frequently associated with chromosomal abnormalities and other congenital malformations. The purpose of our study is to delineate the nature of cystic hygroma and determine the relationship between cystic hygroma and associated anomalies including fetal hydrops. We used data from 26 cases of cervical cystic hygroma in autopsy files of SNU Children's Hospital. Most of cystic hygroma were found in stillborn or premature infants. The fetal cases had been dead for a quite a long period since there was discrepancy between the true gestational age and the developmental age estimated from the body length. Of 26 fetuses only 2 were studied chromosomally and both of them showed 45X. Of 26 cystic hygromas 23 occurred in the posterior neck and 3 in the anterior neck. They ranged from 2.5 to 14 cm (mean: 7.9 cm). The cystic hygroma of the posterior neck consisted of two symmetric sacs on both sides and in the nape and extended to the occipital region. The cystic hygromas of the anterior neck were unilateral or bilateral, and multiloculated and extended into the adjacent cheek. Cystic hygromas of posterior neck were always associated with hydrops, while no recognizable hydrops was found in cystic hygromas of anterior neck. The cystic hygromas were larger in patients with severe hydrops than in patients with less severe hydrops. Associated abnormalities, found in 88%, included hydrops fetalis(88%), growth retardation(80%), cardiovascular anomalies(27%), horseshoe kidney(23%), skeletal anomalies(12%) and hypoxic changes(31%) in visceral organs. In summary, when a hygroma is detected during fetal life, careful sonographic examination for associated congenital anomalies, fetal karyotyping and consideration of artificial abortion are indicated.

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