scholarly journals Histiocytic Sarcoma: Challenging Course, Dismal Outcome

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 310
Author(s):  
Kim Francis Andersen ◽  
Lene Dissing Sjö ◽  
Peter Kampmann ◽  
Torben Bridstrup Pedersen

Histiocytic sarcoma (HS) is a rare hematopoietic neoplasm derived from non-Langerhans histiocytic cells of the monocyte/macrophage system. With an incidence of 0.17/million individuals and a slight male preference, HS presents with a wide age distribution. Most commonly, it occurs as a primary malignancy. In approximately 25% of the cases a presumed transdifferentiation of a preexisting hematolymphoid disorder can be demonstrated. The clinical presentation varies from a localized solitary mass to severe disseminated disease often with extranodal involvement including skin, soft tissue, the gastrointestinal tract and the hematopoietic system. Systemic symptoms in terms of weight loss, fever and night sweats often occur. The diagnostic work-up of HS is extremely challenging due to the rarity of the disease as well as a wide differential diagnosis in terms of a histologic overlap with diverse mimics. No standardized treatment for HS exists and especially in a disseminated disease the clinical course is overly aggressive with a dismal outcome. The median overall survival from the time of diagnosis is approximately six months. We report a 43-year-old previously healthy Caucasian male admitted to our hospitals with abdominal pain and a feeling of fatigue. We demonstrate both the challenges of a correct diagnosis and an effective treatment as well as the aggressive nature of histiocytic sarcoma.

2020 ◽  
Vol 29 (157) ◽  
pp. 190142
Author(s):  
Subha Ghosh ◽  
Atul C. Mehta ◽  
Sami Abuquyyas ◽  
Shine Raju ◽  
Carol Farver

Multiple synchronous lung nodules are frequently encountered on computed tomography (CT) scanning of the chest and are most commonly either non-neoplastic or metastases from a known primary malignancy. The finding may initiate a search for primary malignancy elsewhere in the body. An exception to this rule, however, is a class of rare primary lung neoplasms that originate from epithelial (pneumocytes and neuroendocrine), mesenchymal (vascular and meningothelial) and lymphoid tissues of the lung. While these rare neoplasms also present as multiple synchronous unilateral or bilateral lung nodules on chest CT, they are often overlooked in favour of more common causes of multiple lung nodules. The correct diagnosis may be suggested by a multidisciplinary team and established on biopsy, performed either as part of routine diagnostic work-up or staging for malignancy. In this review, we discuss clinical presentations, imaging features, pathology findings and subsequent management of these rare primary neoplasms of the lung.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Marialuigia Spinelli ◽  
Carmine Sica ◽  
Bruno Dallapiccola ◽  
Antonio Novelli ◽  
Letizia Di Meglio ◽  
...  

Background. Prenatal diagnosis of Optiz G/BBB syndrome (OS) is challenging because the characteristic clinical features, such as facial and genitourinary anomalies, may be subtle at sonography and rather unspecific. Furthermore, molecular testing of the disease gene is not routinely performed, unless a specific diagnosis is suggested.Method. Both familial and ultrasound data were used to achieve the diagnosis of X-linked OS (XLOS), which was confirmed by molecular testing ofMID1gene (Xp22.3) at birth.Results. Sequencing ofMID1gene disclosed the nucleotide change c.1285 +1 G>T, previously associated with XLOS.Conclusions. This case illustrates current challenges of the prenatal diagnostic work-up of XLOS and exemplifies how clinical investigation, including family history, and accurate US foetal investigations can lead to the correct diagnosis.


2020 ◽  
Author(s):  
Sandeep Segar ◽  
Daniel Bouland ◽  
Francesca Torriani ◽  
Kevin Kwak ◽  
Deepak Asudani ◽  
...  

Abstract Background:Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), was declared a global pandemic by World Health Organization in March 2020. Case Presentation:We report a case of a 51-year-old Chinese woman who was evacuated from Wuhan, China and diagnosed with COVID-19 infection at a Southern California quarantine facility. Clinical course was notable for high fevers, night sweats, productive cough, transient leukopenia, lymphopenia, thrombocytopenia, and transaminitis. Evolving hypoxia and infiltrates on chest imaging warranted the trial of an investigational antiviral drug - remdesivir. The patient recovered and was discharged after two weeks of hospitalization. Conclusion:This case highlights the patient’s clinical course including diagnostic work-up, medical management, and challenges in defining non-infectivity in a relatively unknown disease.


1997 ◽  
Vol 7 (3) ◽  
pp. 251-255 ◽  
Author(s):  
H. Mikkilä ◽  
I. Seppälä ◽  
M. Leirisalo-Repo ◽  
A. Karma

Purpose. To assess the utility of testing uveitis patients for anti-Borrelia antibodies in an area endemic for Lyme borreliosis. Methods. We examined 161 uveitis patients for serum antibodies to Borrelia burgdorferi by Lyme ELISA. Antibodies were determined in patients with uveitis of unknown etiology and non-selectively from patients with an established diagnosis. Results. Concentrations of antibodies to B. burgdorferi were elevated in 26 uveitis patients (16.1%), with elevated IgG in 11 of them (6.8%). In four of these patients Lyme borreliosis was a highly suggestive cause of uveitis because of a history of tick bites, systemic symptoms, response to antibiotic therapy, and/or a positive polymerase chain reaction result. Other causes of uveitis were ruled out. All these patients had vitritis. Conclusions. Non-selective testing of uveitis patients for Lyme antibodies is not reasonable even in endemic areas. We recommend using the Borrelia antibody test only in cases of uveitis of unknown cause, especially in patients with vitritis or other symptoms of Lyme borreliosis.


Children ◽  
2021 ◽  
Vol 8 (10) ◽  
pp. 860
Author(s):  
Francesco Pecora ◽  
Luciana Abate ◽  
Sara Scavone ◽  
Irene Petrucci ◽  
Federico Costa ◽  
...  

Lymphadenopathy is an irregularity in the size and texture of the lymph nodes, which is quite common in childhood. When the enlargement of lymph nodes is caused by inflammatory and infectious processes, it is called lymphadenitis. The main objective of this manuscript is to summarize the common infectious etiologies and presentations of lymphadenitis in children providing a management guide for clinical practice. PubMed was used to search for all of the studies published up to April 2021 using keywords such as “lymphadenitis” and “children”. Literature analysis showed that the differential diagnosis for lymphadenitis in pediatrics is broad. Although lymph node enlargement in children is usually benign and self-limited, it is important to exclude malignant etiology. In most cases, history and physical examination allow to identify the correct diagnosis and start a proper treatment with a prompt resolution of the lymphadenopathy. However, particularly in the case of persistent lymphadenitis, determining the cause of lymph node enlargement may be difficult, and the exact etiology may not be identified despite extensive investigations. Further studies should develop and validate an algorithm to assist pediatricians in the diagnosis and timely treatment of lymphadenitis, suggesting situations in which a watchful waiting may be considered a safe approach, those in which empiric antibiotic therapy should be administered, and those requiring a timely diagnostic work-up.


VASA ◽  
2000 ◽  
Vol 29 (1) ◽  
pp. 75-76 ◽  
Author(s):  
Paolo Claudio Cassina ◽  
Hauser ◽  
Kossmann ◽  
Brunner

Ganglion cysts of the hip joint are uncommon synovial-lined fluid-filled juxtaarticular groin lesions. Whereas in the past the correct diagnosis was often made only at surgery there are now valuable imaging methods used for the diagnostic work-up. In experienced hands ultrasonography (US) combined with colour duplex Doppler ultrasonography (CDDS) as a real-time imaging technique easily performed at the patient’s bedside is a valid alternative to more expensive or invasive investigations. We report on a patient who presented with a ganglion cyst and in whom first supported by conventional US an aneurysm of the femoral artery was suspected. The diagnosis of a juxtaarticular ganglion was subsequently correctly made at our institution by CDDS and magnetic resonance imaging, respectively, and the cyst was exstirpated successfully. The differential diagnosis of a pulsating groin mass as well as the most useful and specific imaging methods in the diagnostic work-up in this clinical setting are discussed.


2021 ◽  
pp. 194187442110180
Author(s):  
Harry J. Han ◽  
Anna L. Parks ◽  
Maulik P. Shah ◽  
Gerald Hsu ◽  
Lekshmi Santhosh

We present a case of a healthy 62-year-old woman who developed recurrent seizures preceded by subacute cognitive slowing, ataxia, night sweats, and weight loss. She was found to have cytopenias, multifocal T2/FLAIR hyperintensities on magnetic resonance imaging (MRI), and magnetic susceptibility artifact lesions on susceptibility weighted imaging (SWI). Her symptoms, imaging and laboratory abnormalities all improved with high-doses of steroids and intravenous immunoglobulin (IVIG). But recurred several weeks after completing treatment. Despite extensive work-up, she required multiple hospitalizations and repeat diagnostic studies to arrive at a diagnosis. With an expert discussant in hematology and oncology, we review the differential diagnosis and stepwise approach of unexplained neuro-inflammatory syndromes with cytopenias and systemic symptoms. Our case highlights how time, empiric treatment response, and repeated diagnostic studies refine differential diagnoses and subsequent evaluation. After revealing the diagnosis, we discuss the heterogenous clinical manifestations of this disease process.


2020 ◽  
Vol 10 (2) ◽  
pp. 137-138
Author(s):  
Samiha Haque ◽  
Ishrat Jahan ◽  
Tufayel Ahmed Chowdhury ◽  
Muhammad Abdur Rahim ◽  
Mehruba Alam Ananna ◽  
...  

Rapidly progressive glomerulonephritis is one of the most dramatic and tragic presentations of lupus nephritis (LN) or renal manifestation of systemic lupus erythematosus (SLE). A 35-year-old Bangladeshi gentleman presented with worsening oedema, scanty, high colored, frothy urine and deteriorating renal function. He had puffy face, anaemia, oedema, normal jugular venous pressure (JVP), high blood pressure (150/90 mm Hg), ascites and bilateral pleural effusions. Diagnostic work-up confirmed SLE with class IV LN. His initial response to specific therapy showed improvement Birdem Med J 2020; 10(2): 137-138


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