scholarly journals Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Andrew R. Orr ◽  
Daniel Lefler ◽  
C. Deshpande ◽  
Pallavi Kumar

Rosai–Dorfman disease (RDD) is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. However, a subset of patients with RDD will display extranodal manifestations that are highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress compared to nodal disease. While case reports of extranodal involvement in nearly every organ system exist, documented instances of mediastinal and pulmonary artery involvement are particularly rare. This study describes the case of a middle-aged woman presenting with new onset right heart failure who was found to have extranodal RDD in the form of a large mediastinal mass with invasion and occlusion of the main pulmonary arteries.

2020 ◽  
Vol 2 (3) ◽  
pp. 464-467
Author(s):  
Yogamaya Mantha ◽  
Mark Feldman ◽  
Andrew Assaf ◽  
Rakushumimarika Harada ◽  
David Musselman ◽  
...  

Author(s):  
Steven Lammers ◽  
Tosin Feyintola ◽  
Kendall Hunter ◽  
Emily Gibson ◽  
Tim Lei ◽  
...  

In pulmonary arteries (PA), mechanical function is largely driven by the underlying microstructure of the structural proteins collagen and elastin, which reside within the extracellular matrix (ECM) of the arterial tissue. It has long been established that much of the mechanical non-linearity associated with arterial tissue is the result of collagen mechanics. Arterial collagen is arranged within the vascular wall as tortuous fibrils with a bulk fiber orientation of roughly helical configuration. When arterial tissue is deformed, these collagen fibers become straightened in the direction of applied load. At some critical deformation, termed the transition stretch (λTrans), collagen fibers begin to carry load, thus significantly altering material stiffness. This in turn gives rise to the non-linear force-stretch (F-λ) response typical of these tissues, Figure 1. We have recently found that λTrans is significantly reduced in the hypoxia-induced pulmonary hypertensive (PH) rat model. We therefore propose that this model constitutes an ideal system to study the effect of collagen microstructure on the mechanics of arterial tissues in response to PH vascular remodeling. We hypothesize that quantitative characterization of collagen microstructure will predict pulmonary artery (PA) λTrans within this model system. By directly relating collagen microstructural changes to bulk tissue mechanics in response to PH-induced vascular remodeling we can better understand how changes in collagen structure impact pulmonary hemodynamic capacitance, a major component of cardiac load and contributing factor to right heart failure.


2021 ◽  
Vol 12 (1) ◽  
pp. 72-75
Author(s):  
Hubert Daisley Jr ◽  
Sailaja Golamari ◽  
Lilly Paul ◽  
Deandra Thomas-Romain ◽  
Dawn Meyers ◽  
...  

estombes–Rosai–Dorfman disease—often simply referred to, in the literature, as Rosai–Dorfman disease (RDD)—is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. A subset of patients with RDD, however, display extranodal manifestations highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress when compared to the nodal manifestations. This study describes the case of a young African male presenting himself with multiple nodules involving the anterior abdominal wall, who was found to have extranodal RDD. The current mode of diagnosis and the clinical management of RDD are reviewed.


F1000Research ◽  
2021 ◽  
Vol 7 ◽  
pp. 1483
Author(s):  
Joe M. Das ◽  
Apar Pokharel ◽  
Rashmi Sapkota ◽  
Manish Mishra ◽  
Ashish Babu Aryal

Background: There are a number of ways in which one can sustain a head injury. Even if you are doing simple household activities or going out for a morning walk, you cannot be sure of what type of injury awaits you. The source of injury may be a pressure cooker whistle acting as a projectile or a hailstone falling from the sky. Such injuries are common in Nepal, considering the socio-demographic and geographic conditions. In this article, we present two such very rare cases of head injury. Case Reports: The first case is a middle-aged woman who sustained an accidental injury to the face associated with fracture of frontal sinus and frontal contusion, following the impact from a high momentum projectile in the form of the pressure regulator of a pressure cooker. She underwent craniotomy and removal of the foreign body. In the second case, an elderly man sustained minor injury to the head following the fall of hail. The abrasions and contusions produced by the hail were managed conservatively. Since he did not have any clinical evidence of head injury, other than multiple abrasions with contusions in the scalp, he did not undergo any imaging studies. He did not have any neurological deficits. The postoperative period was uneventful for the first patient and she was followed up for one month. The second patient was lost to follow-up. Conclusion: Successful management of two very rare cases of head injuries from Nepal are reported. Proper care and maintenance of the house-hold utensils that are constantly used may protect people from head injuries.  Though natural calamities cannot always be avoided, simple measures like using an umbrella while going outdoors may protect individuals from head injuries due to hailstones.


2018 ◽  
Vol 35 (02) ◽  
pp. 136-142 ◽  
Author(s):  
G. Pretorius ◽  
Stuart Jamieson

AbstractChronic thromboembolic pulmonary hypertension occurs when acute thromboemboli fail to dissolve completely. The resulting fibrotic scar tissue within the pulmonary arteries is obstructive and eventually leads to right heart failure. Medical therapy for this condition is supportive, but surgery with pulmonary artery endarterectomy is curative, and carries a low mortality at experienced centers.


F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 1483
Author(s):  
Joe M. Das ◽  
Apar Pokharel ◽  
Rashmi Sapkota ◽  
Manish Mishra ◽  
Ashish Babu Aryal

Background: There are a number of ways in which one can sustain a head injury. Even if you are doing simple household activities or going out for a morning walk, you cannot be sure of what type of injury awaits you. The source of injury may be a pressure cooker whistle acting as a projectile or a hailstone falling from the sky. Such injuries are common in Nepal, considering the socio-demographic and geographic conditions. In this article, we present two such very rare cases of head injury. Case Reports: The first case is a middle-aged woman who sustained an accidental injury to the face associated with fracture of frontal sinus and frontal contusion, following the impact from a high momentum projectile in the form of the pressure regulator of a pressure cooker. She underwent craniotomy and removal of the foreign body. In the second case, an elderly man sustained minor injury to the head following the fall of hail. The abrasions and contusions produced by the hail were managed conservatively. Since he did not have any clinical evidence of head injury, other than multiple abrasions with contusions in the scalp, he did not undergo any imaging studies. He did not have any neurological deficits. The postoperative period was uneventful for the first patient and she was followed up for one month. The second patient was lost to follow-up. Conclusion: Successful management of two very rare cases of head injuries from Nepal are reported. Proper care and maintenance of the house-hold utensils that are constantly used may protect people from head injuries.  Though natural calamities cannot always be avoided, simple measures like using an umbrella while going outdoors may protect individuals from head injuries due to hailstones.


2003 ◽  
Vol 127 (7) ◽  
pp. e294-e297 ◽  
Author(s):  
Benjamin H. Lee ◽  
Suzanne George ◽  
Jeffery L. Kutok

Abstract Langerhans cell histiocytosis (LCH) is a neoplastic proliferation of Langerhans cells that occurs in a range of nodal and extranodal sites. Scattered reports of LCH within the thymus exist, typically among children within the setting of multifocal, multisystem disease. Rare cases of isolated LCH involving the thymus have occurred in adult patients with myasthenia gravis. We report a case of unifocal LCH involving the thymus in a middle-aged woman with a history of a resected leiomyosarcoma but no evidence of myasthenia gravis. Computed tomographic scans revealed an anterior mediastinal mass, which was excised and measured 9.0 cm. Histologic and immunophenotypic findings (CD1a, S100, and Fascin positive and CD68 negative) were consistent with LCH. To our knowledge, this is the first example of LCH occurring in a patient with a history of soft tissue sarcoma and one of the rare reported examples of LCH presenting as a large isolated lesion in the thymus of a nonmyasthenic adult.


2020 ◽  
Vol 16 (1) ◽  
pp. 27-32
Author(s):  
Oluwaseun A Akinseye ◽  
Mannu Nayyar ◽  
Pranab Das

We report a 54-year-old female with recently diagnosed multiple cardiac thrombi and pulmonary embolism that was treated with thrombolytics and anticoagulants. She presented again with worsening dyspnea and was found to have persistent large cardiac thrombi on echocardiogram. Surgical findings revealed a single right atrial mass originating from inferior vena cava and extending into the pulmonary artery. The mass was successfully removed. Final pathology revealed a benign smooth muscle and vascular mass with estrogen and progesterone receptor positivity favoring uterine intravenous leiomyoma. She was discharged on warfarin and leuprolide therapy. This diagnosis requires a high index of suspicion, especially in a middle-aged woman with right atrial mass and history of an existing leiomyoma, hysterectomy or myomectomy.


VASA ◽  
2018 ◽  
Vol 47 (5) ◽  
pp. 361-375 ◽  
Author(s):  
Harold Goerne ◽  
Abhishek Chaturvedi ◽  
Sasan Partovi ◽  
Prabhakar Rajiah

Abstract. Although pulmonary embolism is the most common abnormality of the pulmonary artery, there is a broad spectrum of other congenital and acquired pulmonary arterial abnormalities. Multiple imaging modalities are now available to evaluate these abnormalities of the pulmonary arteries. CT and MRI are the most commonly used cross-sectional imaging modalities that provide comprehensive information on several aspects of these abnormalities, including morphology, function, risk-stratification and therapy-monitoring. In this article, we review the role of state-of-the-art pulmonary arterial imaging in the evaluation of non-thromboembolic disorders of pulmonary artery.


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