scholarly journals Pleuropulmonary Blastoma (PPB) in Child with DICER1 Mutation: The First Case Report in the State of Qatar

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Sara G. Hamad ◽  
Amal Al-Naimi ◽  
Mutasim Abu-Hasan

Pleuropulmonary blastoma (PPB) is a rare intrathoracic malignancy, which arises from the lung parenchyma and/or pleura. PPB has strong genetic association with mutations in DICER1 gene. Despite being rare, PPB is the most common lung tumor in children below 6 years of age. International registry of the disease has a total of 350 cases worldwide. We report the first case of PPB in the state of Qatar, which presented as a large cystic lung lesion. The patient was first thought to have benign congenital pulmonary airway malformation (CPAM) based on chest X-ray findings. The diagnosis of PPB was suspected based on chest CT scan findings and was confirmed after surgical resection of the cystic mass. The case highlights the need to consider PPB in the differential diagnosis of cystic lung lesions in children and the need for further radiological imaging (i.e., CT scan), genetic testing, and/or excisional biopsy to confirm the diagnosis.

2021 ◽  
Author(s):  
Deng ZhiJuan ◽  
Zhang Nan ◽  
He Le-jian ◽  
Yao Xing-feng ◽  
Jia Chao ◽  
...  

Abstract Background: The fetal lung interstitial tumor (FLIT) is a newly identified tumor and is extremely rare, with only five reports published in English. Here, we report 4 cases of FLITs in China and present a literature review, aiming to explore this rare tumor.Methods: The clinical and histological findings, immunophenotype, and ALK FISH of these 4 FLIT cases were evaluated, with a detailed review of the literature.Result: The 4 cases comprised 2 male and 2 female infants. Two cases had a lung or intrathoracic mass during pregnancy, as early as 28 gestational weeks, and tachypnea and groaning shortly after birth. The other two cases received clinical attention because of jaundice and weakness at 3 days and 10 minutes after birth. The two female patients had a high serum AFP level. CT showed a well-circumscribed solid-cystic mass in the lung. Surgical removal was performed at 6 days, 11 days, 18 days and 5 days respective. Grossly, the tumor was a well-0circumscribed, solid-to-spongy cut surface, and the largest diameter was 5.5-6.1 cm. Histological examination revealed an immature airspace and interstitium resembling fetal lung tissue at the canalicular stage (20-24 weeks of gestation). The epithelial cells coexpressed CK, EMA, TTF-1 and b-catenin, while the interstitial cells were strongly and diffusely positive for vimentin, with variable degrees of desmin, SMA, MSA and b-catenin. There was no ALK positivity among the four cases. One of the four cases had an ALK gene fusion by FISH. All four cases were in good condition after surgery.Conclusions: The FLIT is a recently reported type of congenital lung lesion with distinct pathological morphology. Some of the FLIT cases have high AFP levels, which represents an ALK gene fusion and may be related to ALK-positive tumors. The FLIT prognosis is good, and surgery is the preferred treatment. The differential diagnosis should include congenital pulmonary airway malformation (CPAM), pleuropulmonary blastoma (PPB), congenital inflammatory myofibroblastic tumor (CIMT) and pulmonary interstitial glycogenosis (PIG).


Author(s):  
Márcia Bohrer Mentz ◽  
Fernando Procianoy ◽  
Marcelo Krieger Maestri ◽  
Marilise Brittes Rott

We report the first case of human ocular sparganosis in the state of Santa Catarina, southern Brazil. A young female patient presented with three periocular moveable inflammatory masses in her right eye, during two years. By surgical excisional biopsy, a helminth larval stage was removed and identified as sparganum. Clinical, laboratory and epidemiological data on this parasite are presented.


2016 ◽  
Vol 02 (01) ◽  
pp. 23 ◽  
Author(s):  
Scott Bickel ◽  
◽  
Bethany Carnes ◽  
Matthew Thompson ◽  
Sheldon Bond ◽  
...  

Pleuropulmonary blastomas (PPBs) represent a rare, malignant neoplasm in infants and children. Despite their overall rarity, they are the most common primary lung malignancy in young children. Typically originating as cystic lesions that are amenable to easy resection, PPBs can progress to solid masses prone to metastases if not identified and treated early enough. In their early cystic form, PPBs are often difficult to distinguish from congenital pulmonary airway malformations based on clinical and radiographic findings. We report on an eight-month-old girl who presented to our institution with a large, right-sided, cystic lung lesion, which had not been apparent on a chest radiograph shortly after birth. After excision of the lesion, pathology demonstrated it to be a type I PPB, which was unique for its apparent extra lobar origin. We review the relevant literature concerning PPBs, as well as the debate surrounding resection of cystic lung lesions in infants and children.


2001 ◽  
Vol 125 (2) ◽  
pp. 282-285 ◽  
Author(s):  
Yuan Lin ◽  
Gilberto D. Rodrigues ◽  
John F. Turner ◽  
Mohammad A. Vasef

Abstract Non-Hodgkin lymphomas associated with acquired immunodeficiency syndrome are heterogeneous. Recently, a novel subtype of non-Hodgkin lymphoma occurring mostly in patients with acquired immunodeficiency syndrome has been described and designated as plasmablastic lymphoma. The histomorphologic and immunophenotypic findings of this distinct subtype of non-Hodgkin lymphoma have been characterized previously. Most patients present with oral cavity involvement. We report a case of plasmablastic lymphoma presenting as a lung tumor. To our knowledge, this is the first case report of this unusual subtype of diffuse large B-cell lymphoma in this location.


2021 ◽  
Author(s):  
Peter Dovjak ◽  
Michael Kroissenbrunner ◽  
Bernhard Iglseder

Abstract Background: A gruesome infection was found in a woman with advanced lung tumor and associated malnutrition. Worldwide, bacteremia with Wohlfartiimonas chitiniclastica was only found in 13 cases yet.Case presentation: This is the first case in Austria and the first case without infestation of maggots.Conclusions: This germ may be considered as an emerging human pathogen not only in patients with poor personal hygiene, difficult social circumstances, alcohol dependence or chronic wounds.AbstraktEine grauenhafte Infektion wurde bei einer Patientin mit fortgeschrittenem Lungentumor und Malnutrition gefunden. Bisher wurden weltweit erst 13 Fälle mit einer Wohlfahrtiimonas chitiniclastica - Bakteriämie beschrieben. Dieser erste Fall in Österreich manifestierte sich ohne eine Infestation mit Maden. Dieses Bakterium kann als neuer Krankheitserreger betrachtet werden, der nicht nur bei Patienten unter mangelnder Hygienebedingungen, schwierigen sozialen Verhältnissen, Alkoholabhängigkeit oder chronischen Wunden auftreten kann.


2003 ◽  
Vol 61 (1) ◽  
pp. 91-94 ◽  
Author(s):  
Oswaldo Ignácio de Tella Jr ◽  
Marco Antonio Herculano ◽  
Mirto Nelso Prandini ◽  
João Norberto Stavile ◽  
Antonio de Pádua Furquim Bonatelli

We present CT scan, MRI and histopathologic findings of two patients harboring a rare type of meningioma. In the first case, a 52 year-old male patient, a large parasellar mass invading the cavernous sinus, infiltrating the infratemporal fossa and extending as low as C2 was founded. The tumor was isointense on T1, enhanced strongly with gadolinium injection, and was hyperintense on T2. In the second case, a 19-year-old male patient, a large high density temporal right mass was disclosed by CT scan. Both patients were taken to surgery. In the first case, only a partial removal was possible to be accomplished due to a severe intra operative bleeding. In the second case, the tumor was totally removed. Both showed characteristic pathologic findings of a meningioma resembling a chordoma. Meningioma is a relatively common intracranial tumor, occurring most frequently in adults, showing a wide variety of growth patterns. We described a pattern that had a peculiar chordoma-like appearance. The pathological findings and the differential diagnosis from chordoma are discussed.


2020 ◽  
Vol 13 (3) ◽  
pp. 1097-1102
Author(s):  
Daisuke Nakamura ◽  
Ryoichi Kondo ◽  
Akiko Makiuchi ◽  
Hiroko Itagaki

We report on a giant pulmonary colloid adenocarcinoma successfully resected using a median sternotomy approach. A 69-year-old woman visited our hospital owing to a giant mass detected on chest radiography. A giant cystic mass measuring 115 × 90 mm was detected in the right upper lung using computed tomography. We suspected mucinous adenocarcinoma and performed right upper lobectomy and mediastinal lymph node dissection with median sternotomy. The surgical field of view for the tumor and superior vena cava was satisfactory, and compression but not invasion of the superior vena cava and chest wall by the tumor was observed. The tumor was pathologically diagnosed as a colloid adenocarcinoma of stage IIIA with pT4N0M0. The postoperative course was uneventful, with no signs of recurrence at one and a half years after operation. Thus, this case demonstrates that for giant lung tumor surgery, median sternotomy is useful and safe for improving the surgical field of view.


2020 ◽  
Vol 63 ◽  
pp. 29-42
Author(s):  
Jakub Łukaszewski
Keyword(s):  

The oldest Polish bookplate known today and imprinted directly from a woodcut block on book pages is one attributed to Wojciech Strzałkowski, a notary from Gniezno (dated to 1519–1520). It can be found in an incunabulum from the collection of the Cathedral Library in the Archdiocesan Archives in Gniezno (Formularium instrumentorum ad usum Curiae Romanae [Hagenau: Heinrich Gran, before 17 August 1492], in 4o, no. Inc. 37). In the article the author demonstrates that the sign in fact belonged to Jan Żdżarowski (–1551), a public notary as well as canon of Poznań, Włocławek and Kraków. In his notarial admission (of 26 January 1519) Żdżarowski used a woodcut block to make his notarial sign (at that time notaries usually drew their signs). He used the same block to make a bookplate. An analysis of the state of preservation of both prints demonstrates that the block used to make them originated around 1517–1518, certainly before 26 January 1519, perhaps in Kraków. This is the oldest known case of a block being used to make a notarial sign by a notary from Poland and, at the same time, the first case of the same tool being used also to make a bookplate. The annex features an edition of the text of Jan Żdżarowski’s admission.


2020 ◽  
Vol 13 (4) ◽  
pp. 205-219
Author(s):  
M.V. Tarasov

The topic of patriotic consciousness and patriotic education today claims to the status of a nationwide idea, so the research interest in this issue is unusually high. The study of patriotic consciousness should begin with an analysis of the subject which the patriotic feelings of citizens are directed on. This subject is the motherland and its image in the minds of citizens. The article gives an overview of the data, which is used for the semantic deferential method «Image of Motherland» and the procedure of studying of the image of the Motherland based on this method. The sample was 165 respondents. Based on the results obtained, it is concluded that the use of this methodology is a tool, which lets us to determine social ideas about the image of the Motherland. It has been proved that the image of the Motherland in consciousness reflects the ideas about the country and the state in which the respondents were born and raised, it is not associated with a so-called “Small homeland”, but with a certain commonality of territory, nature and culture. There is reason to believe that the image of the Motherland in the human mind can be viewed as an image “for oneself” and an image “for others”: in the first case, the Motherland is perceived as big and strong, interesting for life and comfortable, simple and cultural; Motherland “for others” is bold and friendly, strong and kind.


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