Persistent Interstitial Pulmonary Emphysema-like Cyst Associated with Metastatic Synovial Sarcoma

2000 ◽  
Vol 3 (4) ◽  
pp. 391-393 ◽  
Author(s):  
Marta C. Cohen ◽  
Ricardo Drut

Interstitial pulmonary emphysema is characterized by the presence of gas dissecting the interstitial tissues of the lung. Clinically, it may be acute or persistent, and the latter can be further categorized as localized or diffuse. Usually, it appears in preterm neonates with a history of assisted ventilation or respiratory distress. Although far from frequent, the localized variety of persistent interstitial pulmonary emphysema (PIPE) can develop spontaneously in full-term babies or infants without any obvious underlying pulmonary disease. Histologically, PIPE is characterized by the presence of uni- and multinucleated histiocytes lining the inner surface of the cysts. In this report, we describe a 15-year-old male with synovial sarcoma (SS) of the right ankle diagnosed 4 years previously who developed pulmonary metastasis, one of which presented cystic changes with features of PIPE.

1999 ◽  
Vol 2 (6) ◽  
pp. 531-536 ◽  
Author(s):  
Marta C. Cohen ◽  
Rosa M. Drut ◽  
Ricardo Drut

Neonatal interstitial pulmonary emphysema (IPE) is a well-characterized lesion usually presenting in preterm newborns as a complication of respiratory distress syndrome and/or assisted ventilation. Occasionally, IPE may occur spontaneously in infants with no underlying pulmonary disease. Persistence of IPE (PIPE) may be diffuse or localized. Localized PIPE usually presents as multiple cysts 0.3 to 3 cm in one or more lobes of the lung. In this report, we describe four cases of unilocular large cysts (up to 5 cm in diameter) partially lined by uni- and multinucleated histiocytes in a foreign body type reaction and showing gas dissection of the surrounding parenchyma (present in case 1). These histological features favored the diagnosis of PIPE and the cases were interpreted as such, since no other clear-cut diagnosis could be defined. However, because the lesion was limited to one lobe, and the children were full term, asymptomatic at birth and without history of respiratory distress or assisted ventilation, differential diagnosis with other pulmonary cystic lesions of infancy is mandated.


2020 ◽  
Vol 18 ◽  
pp. 205873922092685
Author(s):  
Yunna Yang ◽  
Zheng Gu ◽  
Yinglun Song

Subdural osteomas are extremely rare benign neoplasms. Here, we report the case of a 35-year-old female patient with a right frontal and parietal subdural osteoma. The patient presented with a 2-year history of intermittent headache and fatigue. Computerized tomography (CT) scan showed a high-density lesion attached to the inner surface of the right frontal and parietal skull. Magnetic resonance imaging (MRI) demonstrated T1 hyperintensity and T2 hypointensity of the lesion. Intraoperatively, the hard mass was located in subdural space and attached to the dura mater. Histopathological examination revealed lamellated bony trabeculae lined by osteoblasts and the intertrabecular marrow spaces occupied by adipose tissue. The patient underwent neurosurgical resection and recovered without complication. Surgical excision is recommended to extract the symptomatic lesions with overlying dura mater.


2017 ◽  
Vol 50 (5) ◽  
pp. 1700610 ◽  
Author(s):  
Marc Miravitlles ◽  
Asger Dirksen ◽  
Ilaria Ferrarotti ◽  
Vladimir Koblizek ◽  
Peter Lange ◽  
...  

α1-antitrypsin deficiency (AATD) is the most common hereditary disorder in adults. It is associated with an increased risk of developing pulmonary emphysema and liver disease. The pulmonary emphysema in AATD is strongly linked to smoking, but even a proportion of never-smokers develop progressive lung disease. A large proportion of individuals affected remain undiagnosed and therefore without access to appropriate care and treatment.The most recent international statement on AATD was published by the American Thoracic Society and the European Respiratory Society in 2003. Since then there has been a continuous development of novel, more accurate and less expensive genetic diagnostic methods. Furthermore, new outcome parameters have been developed and validated for use in clinical trials and a new series of observational and randomised clinical trials have provided more evidence concerning the efficacy and safety of augmentation therapy, the only specific treatment available for the pulmonary disease associated with AATD.As AATD is a rare disease, it is crucial to organise national and international registries and collect information prospectively about the natural history of the disease. Management of AATD patients must be supervised by national or regional expert centres and inequalities in access to therapies across Europe should be addressed.


2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Xiaohua Jiang ◽  
Qi Huang ◽  
Jianguo Tang ◽  
Matthew R. Hoffman

A 58-year-old man presented with a six-month history of intermittent blood-stained posterior nasal discharge. Five years ago, he had a three-week episode of fitful light headaches. Nasal ventilation, olfactory sensation, and facial sensation were normal; there were no ophthalmological complaints. Coronal computed tomography (CT) scans revealed soft masses in the bilateral sphenoid sinuses with bone absorption. The patient underwent bilateral functional endoscopic sinus surgery and resection of right nasal papillary masses. Papillary masses and mucosa in both sphenoid sinuses were also removed. The mass in the left sphenoid sinus was diagnosed as two separate entities, one being a primary monophasic epithelial synovial sarcoma and the other an inverted papilloma, while the mass in the right sphenoid sinus was an inverted papilloma. After surgery, the patient underwent radiotherapy and chemotherapy. At the 50-month follow-up visit, there were no signs of recurrence.


Hand ◽  
2021 ◽  
pp. 155894472097513
Author(s):  
Arthur Samia ◽  
Joshua Scarcella ◽  
Richard Zeri ◽  
Yifan Guo

There have been 8 synovial sarcomas of the median nerve reported. We report a case of a 15-year-old male with synovial sarcoma of the right-hand median nerve. Patient presented with a 2-month history of enlarging mass at the base of the right thenar eminence associated with numbness in the median nerve distribution. Physical examination revealed a soft mass over the thenar eminence and paresthesia in the median nerve distribution. He underwent excision of the tumor, which revealed a well-encapsulated lesion encompassing the median nerve, involving the first, second, and radial aspect of the third web space as well as recurrent branches of the median nerve. Following excision of the tumor, a thorough metastatic workup was negative for metastatic disease. He was staged as III, T2b, N0, M0—poorly differentiated monophasic synovial sarcoma of the right median nerve. Postoperatively the patient was started on chemotherapy and radiation. Intraneural synovial sarcoma is extremely rare. Our case is the youngest with the longest follow-up. He is currently at a status of 3 years posttreatment with no signs of recurrence and excellent use of his right hand. This case is of particular interest due to the rarity of the disease along with this being the best outcome reported in the literature to-date.


2020 ◽  
pp. 247412642097255
Author(s):  
Katherine E. Holekamp ◽  
Nancy M. Holekamp

Purpose: We discuss a peripheral combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with a macula-involving TRD that was repaired by vitrectomy surgery. Methods: A case report is presented. Results: A 15-year-old white girl with no significant medical or ocular history presented to the retina clinic with a 1-month history of progressive loss of inferior visual field in the right eye. A large, elevated CHRRPE was found in the superior midperipheral retina. On involvement of the macula, urgent vitrectomy surgery with peeling of the cortical vitreous membrane to the margins of the hamartoma was performed. Eighteen months later, vision had returned to 20/16 and the retina, relieved of traction, continued to reattach with trace remaining cystic changes at the fovea on optical coherence tomography. Conclusions: Physicians should consider vitrectomy surgery with membrane peeling of the cortical vitreous for TRD due to CHRRPE.


2021 ◽  
Vol 14 (7) ◽  
pp. e243760
Author(s):  
Mohsin F Butt ◽  
Maggie Symonds ◽  
Ruhaid Khurram

Unilateral pleural effusions are uncommonly reported in patients with SARS-CoV-2 pneumonitis. Herein, we report a case of a 42-year-old woman who presented to hospital with worsening dyspnoea on a background of a 2-week history of typical SARS-CoV-2 symptoms. On admission to the emergency department, the patient was severely hypoxic and hypotensive. A chest radiograph demonstrated a large left-sided pleural effusion with associated contralateral mediastinal shift (tension hydrothorax) and typical SARS-CoV-2 changes within the right lung. She was treated with thoracocentesis in which 2 L of serosanguinous, lymphocyte-rich fluid was drained from the left lung pleura. Following incubation, the pleural aspirate sample tested positive for Mycobacterium tuberculosis. This case demonstrates the need to exclude non-SARS-CoV-2-related causes of pleural effusions, particularly when patients present in an atypical manner, that is, with tension hydrothorax. Given the non-specific symptomatology of SARS-CoV-2 pneumonitis, this case illustrates the importance of excluding other causes of respiratory distress.


2019 ◽  
Vol 2019 (7) ◽  
Author(s):  
Chairat Burusapat ◽  
Nutthadon wongprakob ◽  
Rapeepat Sapruangthong ◽  
Chatchai Pruksapong ◽  
Viriya Kaewkangsadan ◽  
...  

AbstractPrimary breast angiosarcoma is an extremely rare tumor type (0.05% of primary breast cancers) for which diagnosis can be difficult. They arise within the breast parenchyma and typically present as a breast mass. Here, we present the case of a 30-year-old female with spontaneous hypervascular skin on her right breast with progressive enlargement presenting as an expanding hematoma. A chest computed tomography revealed a hypervascular mass in the right breast abutting the pectoralis muscle and cystic changes within the mass. A core needle biopsy revealed an angiosarcoma. In this case study, we report a patient who, with no history of any known risk factors, presented with a benign vascular skin lesion as the first sign of angiosarcoma followed by an expanding hematoma, which is an extremely rare manifestation of the disease. Microscopic examination demonstrated a low-grade angiosarcoma on the skin, while a high-grade angiosarcoma was found in the breast parenchyma.


2002 ◽  
Vol 14 (4) ◽  
pp. 335-337 ◽  
Author(s):  
Janet C. Patterson-Kane ◽  
Lenn R. Harrison

A 5-month-old male Thoroughbred foal with a history of chronic septic arthritis of the tibiotarsal joint and recent respiratory distress was euthanized and a postmortem examination performed. A giant diverticulum communicating with the lateral aspect of the right atrial cavity of the heart was observed. Histologically, the wall was comprised of myocardial tissue containing cavernous vascular spaces. There was gross and histologic evidence of right-sided heart failure. Congenital right atrial diverticula are rare anomalies in humans and have not previously been reported in foals.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Yoichiro Shinohara ◽  
Ryo Mukai ◽  
Shinji Ueno ◽  
Hideo Akiyama

Introduction. We report the clinical features and clinical course of melanoma-associated retinopathy (MAR), in which autoantibodies against the transient receptor potential cation channel subfamily M member 1 (TRPM1) were detected. Case Presentation. A 74-year-old man was referred to our hospital for treatment of bilateral vision loss. The best-corrected visual acuity was 20/100 in the right eye and 20/200 in the left eye. His electroretinogram (ERG) showed a reduced b-wave and a normal dark-adapted a-wave in both eyes. Optical coherence tomography (OCT) revealed loss of the interdigitation zone in both eyes. We strongly suspected MAR based on the markedly reduced b-wave in the ERG and a history of intranasal melanoma. The diagnosis was confirmed after autoantibodies against TRPM1 were detected in his blood serum. Fifteen months later, his ERG remained unchanged, and OCT showed bilateral cystic changes in the internal nuclear layer. The visual acuity in both eyes also remained unchanged. Conclusions. Anti-TRPM1 autoantibodies were detected in a patient diagnosed with MAR who had negative flash ERG and retinal microstructural abnormalities, and the impairment did not recover during the follow-up period. Identification of anti-TRPM1 antibodies was helpful in confirming the diagnosis of MAR.


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