SUN-432 A Case of Sellar Plasmacytoma Masquerading as Pituitary Carcinoma with Hypopituitarism

Abstract Background: Sellar plasmacytomas are rare tumors arising from clonal plasma cells localized to the sellar/parasellar region. They may mimic other pituitary pathology such as pituitary adenomas, chordomas, meningiomas or metastatic carcinomas in both clinical manifestations and radiographic evidence. Certain features such as predominance of cranial nerve palsies and absence of hypopituitarism appear to be more common with sellar plasmacytomas. We present an interesting case of a sellar plasmacytoma, initially mistaken for pituitary carcinoma, with rapid development of hypopituitarism and systemic multiple myeloma. Case: A 75 year old healthy male presented with sudden onset left eye diplopia and retro-orbital pain. Left eye ptosis and diplopia with left lateral and upward gaze were noted on exam. Initial MRI brain revealed a 17mm soft tissue mass involving pituitary and clivus, extending into the left superior orbital fissure, concerning for pituitary carcinoma. With the exception of low LH (1.2) and testosterone (25), baseline pituitary hormone labs, complete blood count and metabolic panel were within normal limits. Within 1 month, he acutely presented with fever, confusion, weakness, and bony pains. Repeat pituitary labs showed low ACTH < 5, AM cortisol 4.3, TSH 0.07, FT4 0.5. New findings of acute renal injury, hypercalcemia, anemia with elevated Mspike, Kappa light chain 39.7 (0.3-2.0mg/dl), kappa/lamda ratio 66.2 (0.3-1.6) and beta2microglobulin 4484 were found. Repeat MRI brain noted multifocal osseous metastatic disease with left sellar and cavernous sinus lesion contiguous with clival osseous lesion displacing pituitary gland, concerning for plasmacytoma. PET/CT showed focal lytic lesions in clivus and posterior left iliac bone. Bone marrow biopsy confirmed 70% myeloma involvement with kappa monotypic plasma cell population. He was started on hydrocortisone, levothyroxine, chemotherapy and XRT. Conclusion: The rarity of plasmacytomas, combined with their clinical presentation and imaging characteristics similar to other sellar tumors, can often result in misdiagnosis. Accuracy of diagnosis is critical, as sellar plasmacytomas and underlying multiple myeloma (present in 50% of cases) require XRT and systemic chemotherapy respectively rather than surgery. Although pituitary function is usually preserved in plasmacytomas, our case shows that acute anterior pituitary dysfunction can be an early presenting sign.

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Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Blood ◽

10.1182/blood-2016-01-690636 ◽

2016 ◽

Vol 127 (22) ◽

pp. 2672-2681 ◽

Cited By ~ 430

Author(s):

Jean-François Emile ◽

Oussama Abla ◽

Sylvie Fraitag ◽

Annacarin Horne ◽

Julien Haroche ◽

...

Keyword(s):

Dendritic Cell ◽

Clinical Manifestations ◽

Rare Disorders ◽

Molecular Alterations ◽

Imaging Characteristics ◽

Rosai Dorfman Disease ◽

New Findings ◽

Wide Range ◽

Guidelines And Recommendations

Abstract The histiocytoses are rare disorders characterized by the accumulation of macrophage, dendritic cell, or monocyte-derived cells in various tissues and organs of children and adults. More than 100 different subtypes have been described, with a wide range of clinical manifestations, presentations, and histologies. Since the first classification in 1987, a number of new findings regarding the cellular origins, molecular pathology, and clinical features of histiocytic disorders have been identified. We propose herein a revision of the classification of histiocytoses based on histology, phenotype, molecular alterations, and clinical and imaging characteristics. This revised classification system consists of 5 groups of diseases: (1) Langerhans-related, (2) cutaneous and mucocutaneous, and (3) malignant histiocytoses as well as (4) Rosai-Dorfman disease and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. Herein, we provide guidelines and recommendations for diagnoses of these disorders.

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Rathke cleft cyst apoplexy: a newly characterized distinct clinical entity

Journal of Neurosurgery ◽

10.3171/2010.5.jns091905 ◽

2011 ◽

Vol 114 (2) ◽

pp. 318-324 ◽

Cited By ~ 28

Author(s):

Joumana T. Chaiban ◽

Dima Abdelmannan ◽

Mark Cohen ◽

Warren R. Selman ◽

Baha M. Arafah

Keyword(s):

Pituitary Tumor ◽

Clinical Presentation ◽

Clinical Manifestations ◽

Sudden Onset ◽

Published Data ◽

Pituitary Hormone ◽

Sudden Increase ◽

Rathke Cleft Cyst ◽

Imaging Characteristics ◽

Pituitary Tumor Apoplexy

Object Although most Rathke cleft cysts (RCCs) remain asymptomatic, some present with compression of surrounding structures and pituitary hormone dysfunction. A rare, but distinct presentation of the RCC includes hemorrhage into the cyst—a presentation that mimics the clinical syndrome of pituitary tumor apoplexy. The objective of this article is to present the authors' experience on the prevalence and the clinical, biochemical, pathological, and imaging characteristics of hemorrhage into an RCC and review published reports on this entity. Methods An institutional database for patients with pituitary masses was reviewed and data on cases involving surgery for a subsequently documented RCC were identified. Patients with documented hemorrhage within the RCC were included in the analysis. Documentation included intraoperative visualization of hemorrhage by the surgeon and/or pathological confirmation of hemorrhage within the RCC. The clinical, biochemical, pathological, and imaging characteristics of these patients were extracted from the database. All published data on RCC were also reviewed and confirmed cases of hemorrhage were extracted and included in the review. Results A total of 11 cases with hemorrhage within an RCC were identified to have been managed at the authors' institution over a 10-year span. This figure represented 20% of all surgically treated cases of RCC from that period. Among published reports, a total of 10 confirmed cases were evaluated. The clinical presentation of patients with hemorrhage within an RCC are classical and include sudden onset of severe headache—or a sudden increase in headache severity—associated with visual disturbances, impairment in pituitary function, and occasionally alterations in mental status. Leakage of the cystic contents into the subarachnoid space can occur rarely and may lead to associated chemical meningitis. There are no known precipitating factors for this phenomenon. The clinical presentation of such patients is difficult to distinguish from that of patients with pituitary tumor apoplexy. However, patients with RCC apoplexy often present with less severe symptoms, have a lower prevalence of pituitary dysfunction, and generally have smaller mass lesions than those with pituitary tumor apoplexy. Management of patients with RCC apoplexy is similar to that of those with pituitary tumor apoplexy. Surgery can provide definitive diagnosis and relief of symptoms and often leads to recovery from hypopituitarism. All patients with this syndrome require long-term follow-up to treat any residual sequelae. Conclusions This article provides the first thorough review and characterization of patients with hemorrhage within an RCC. Based on the clinical manifestations of this presentation, we recommend using the term “Rathke cleft cyst apoplexy” to describe the syndrome.

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Clinical Manifestations and Radiological Characteristics on Rosai-Dorfman Disease: A Retrospective Observational Study

Medical & Clinical Research ◽

10.33140/mcr.05.05.005 ◽

2020 ◽

Vol 5 (5) ◽

Keyword(s):

Blood Vessels ◽

Abdominal Cavity ◽

Clinical Manifestations ◽

Ct Images ◽

High Signal ◽

Radiological Features ◽

Imaging Characteristics ◽

Rosai Dorfman Disease ◽

Heterogeneous Enhancement ◽

The Masses

Background and Objective: Rosai-Dorfman disease (RDD) are usually misdiagnosed because of rarity and nonspecific clinical and radiological features. The aim of our study is to explore the clinical and imaging characteristics of RDD to improve diagnostic accuracy. Methods: Clinical and imaging data in 10 patients with RDD were retrospectively analyzed. 7 patients were underwent CT scanning and 3 patients were underwent MR examination. Results: 8 (8/10) patients presented with painless enlarged lymph nodes (LNs) or mass. 3 cases were involved with LNs, 5 cases were involved with extra-nodal tissues, and the remaining 2 cases were involved with LNs and extra-nodal tissue simultaneously. In enhanced CT images, enlarged LNs displayed mild or moderate enhancement, and 2 cases showed heterogeneous ring-enhancement. MR features of 3 patients with extra-nodal RDD, 2 cases showed a mass located in the subcutaneous and anterior abdominal wall respectively, and 1 case showed an intracranial mass. Besides, all lesions showed high signal foci on DWI images, and were characterized by marked heterogeneous enhancement with blurred edge. The dural/fascia tail sign and dilated blood vessels could be seen around all the lesions on enhanced MRI. Radiological features of 2 cases with LN and extranodal tissue involved, one case presented with the swelling and thickening of pharyngeal lymphoid ring and nasopharynx, meanwhile with enlarged LNs in bilateral submandibular area, neck and abdominal cavity, and also companied with osteolytic lesion in right proximal humerus. All these LNs displayed mild and moderate enhancement on CT images. Another case showed enlarged LNs in bilateral neck accompanied with soft tissue mass in the sinuses. Conclusions: RDD occurred commonly in young and middle-aged men and presented with painless enlarged LNs or mass.RDD had a huge diversity of imaging findings, which varied with different location. The radiological features, such as small patches of high signal foci in the masses on DWI images, heterogeneous enhancement and blood vessels around the masses, are helpful in diagnosis of extranodal RDD.

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Presepsin - New Marker of Sepsis Romanian Neonatal Intensive Care Unit Experience

Revista de Chimie ◽

10.37358/rc.19.8.7475 ◽

2019 ◽

Vol 70 (8) ◽

pp. 3008-3013

Author(s):

Silvia Maria Stoicescu ◽

Ramona Mohora ◽

Monica Luminos ◽

Madalina Maria Merisescu ◽

Gheorghita Jugulete ◽

...

Keyword(s):

Intensive Care Unit ◽

Intensive Care ◽

Neonatal Intensive Care Unit ◽

Neonatal Sepsis ◽

Predictive Value ◽

Neonatal Intensive Care ◽

Complete Blood Count ◽

Clinical Manifestations ◽

Receiver Operating Curve ◽

Predictive Values

Difficulties in establishing the onset of neonatal sepsis has directed the medical research in recent years to the possibility of identifying early biological markers of diagnosis. Overdiagnosing neonatal sepsis leads to a higher rate and duration in the usage of antibiotics in the Neonatal Intensive Care Unit (NICU), which in term leads to a rise in bacterial resistance, antibiotherapy complications, duration of hospitalization and costs.Concomitant analysis of CRP (C Reactive Protein), procalcitonin, complete blood count, presepsin in newborn babies with suspicion of early or late neonatal sepsis. Presepsin sensibility and specificity in diagnosing neonatal sepsis. The study group consists of newborns admitted to Polizu Neonatology Clinic between 15th February- 15th July 2017, with suspected neonatal sepsis. We analyzed: clinical manifestations and biochemical markers values used for diagnosis of sepsis, namely the value of CRP, presepsin and procalcitonin on the onset day of the disease and later, according to evolution. CRP values may be influenced by clinical pathology. Procalcitonin values were mainly influenced by the presence of jaundice. Presepsin is the biochemical marker with the fastest predictive values of positive infection. Presepsin can be a useful tool for early diagnosis of neonatal sepsis and can guide the antibiotic treatment. Presepsin value is significantly higher in neonatal sepsis compared to healthy newborns (939 vs 368 ng/mL, p [ 0.0001); area under receiver operating curve (AUC) for presepsine was 0.931 (95% confidence interval 0.86-1.0). PSP has a greater sensibility and specificity compared to classical sepsis markers, CRP and PCT respectively (AUC 0.931 vs 0.857 vs 0.819, p [ 0.001). The cut off value for presepsin was established at 538 ng/mLwith a sensibility of 79.5% and a specificity of 87.2 %. The positive predictive value (PPV) is 83.8 % and negative predictive value (NPV) is 83.3%.

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The Role of Neuroimaging in the Diagnosis and Treatment of Depressive Disorder: A Recent Review

Current Pharmaceutical Design ◽

10.2174/1381612824666180727111142 ◽

2018 ◽

Vol 24 (22) ◽

pp. 2515-2523 ◽

Cited By ~ 4

Author(s):

Tianbin Song ◽

Xiaowei Han ◽

Lei Du ◽

Jing Che ◽

Jing Liu ◽

...

Keyword(s):

Magnetic Resonance ◽

Depressive Disorder ◽

Diffusion Tensor ◽

Single Photon ◽

Rapid Development ◽

Photon Emission ◽

Clinical Manifestations ◽

Brain Network ◽

Emission Computed Tomography ◽

Resonance Spectroscopy

Depression is a mental disorder with serious negative health outcomes. Its main clinical manifestations are depressed mood, slow thinking, loss of interest, and lack of energy. The rising incidence of depression has a major impact on patients and their families and imposes a substantial burden on society. With the rapid development of imaging technology in recent years, researchers have studied depression from different perspectives, including molecular, functional, and structural imaging. Many studies have revealed changes in structure, function, and metabolism in various brain regions in patients with depressive disorder. In this review, we summarize relevant studies of depression, including investigations using structural magnetic resonance imaging (MRI), functional MRI (task-state fMRI and resting-state fMRI), diffusion tensor imaging (DTI), magnetic resonance spectroscopy (MRS), brain network and molecular imaging (positron emission tomography [PET] and single photon emission computed tomography [SPECT]), which have contributed to our understanding of the etiology, neuropathology, and pathogenesis of depressive disorder.

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Multiple Myeloma or Brucellosis: A Case Report

Infectious Disorders - Drug Targets ◽

10.2174/1871526519666190307123047 ◽

2020 ◽

Vol 20 (1) ◽

pp. 102-105 ◽

Cited By ~ 1

Author(s):

Hossein A. Rahdar ◽

Mansoor Kodori ◽

Mohamad R. Salehi ◽

Mahsa Doomanlou ◽

Morteza Karami-Zarandi ◽

...

Keyword(s):

Multiple Myeloma ◽

Brucella Melitensis ◽

Severe Disease ◽

Correct Diagnosis ◽

Clinical Manifestations ◽

Bone Marrow Aspiration ◽

Major Health Problem ◽

Brucella Infection ◽

Wide Range ◽

Parental Treatment

Background: Brucellosis, a major health problem in developing countries, is a multisystem infection with a broad spectrum of clinical manifestations. Hematological complications, ranging from an intravascular coagulopathy to mild homeostasis disorders (such as gammopathy), have been reported in brucella infection. These signs and symptoms may lead to misdiagnosis of brucellosis with other hematological diseases. Case: A 65-year-old male whose occupation was shepherding was referred to our hospital as a known case of multiple myeloma with continuous fever, muscle weakness, and night sweating after taking 2 courses of chemotherapy. The laboratory diagnosis of multiple myeloma had been based on the observation of a high percent of plasma cells in the bone marrow aspiration. At follow- up, the result of patient's fever workup, with 2 sets of blood cultures, was positive for Brucella melitensis. Isolated brucella was confirmed as B. melitensis by 16S rRNA sequencing. Brucellosis serologic test was performed by agglutination test and positive results were obtained. The patient was discharged with the cessation of fever and general improvement after the end of the parental treatment phase of brucella bacteremia. Conclusions: Brucella infection may cause a severe disease, mimicking a primary hematological disease, which could complicate the correct diagnosis. In brucellosis cases, due to the wide range of symptoms, in addition to cultivation and serological methods, molecular methods should also be used to prevent inappropriate diagnosis and additional costs.

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Deep Cerebral Venous Thrombosis—A Clinicoradiological Study

Journal of Neurosciences in Rural Practice ◽

10.1055/s-0041-1730109 ◽

2021 ◽

Author(s):

Sujana Gogineni ◽

Dhananjay Gupta ◽

R. Pradeep ◽

Anish Mehta ◽

Mahendra Javali ◽

...

Keyword(s):

Venous Thrombosis ◽

Care Center ◽

Tertiary Care ◽

Clinical Manifestations ◽

Arterial Occlusion ◽

Venous System ◽

Common Risk Factor ◽

Imaging Characteristics ◽

Deep Cerebral Venous Thrombosis ◽

High Index Of Suspicion

AbstractStroke is a common neurological emergency. Almost 80% of strokes are due to arterial occlusion. Venous thrombosis comprises less than 1–2% of all strokes. Involvement of the deep cerebral venous system is still rare and accounts for about 10.9% of all cerebral venous thromboses (CVT). CVT diagnosis is often delayed or missed, because of its variable clinical manifestations. We retrospectively (2015–18) and prospectively (2018–20) reviewed all the cases of CVT in a tertiary care center in south India. Out of a total of 52 CVT cases, 12 were due to the involvement of deep cerebral venous system. Their clinical presentation, imaging characteristics, and outcomes were assessed. The most frequent presentation was headache followed by seizures. Hyperhomocysteinemia was the most common risk factor noted. Imaging characteristics were variable, and a high index of suspicion was required for early diagnosis. All patients had favorable outcome in our study, and except one, all were treated conservatively.

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Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report

BMC Gastroenterology ◽

10.1186/s12876-021-01882-7 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Rachael Chan ◽

Stephanie Carpentier

Keyword(s):

Multiple Myeloma ◽

Clinical Symptoms ◽

Clinical Manifestations ◽

Left Gastric Artery ◽

Bleeding Complications ◽

Al Amyloidosis ◽

Gi Bleeding ◽

Reduced Ejection Fraction ◽

Repeat Endoscopy ◽

Tertiary Center

Abstract Background Amyloidosis is characterized by extracellular tissue deposition of fibrils, composed of insoluble low-molecular-weight protein subunits. The type, location, and extent of fibril deposition generates variable clinical manifestations. Gastrointestinal (GI) bleeding due to amyloid deposition is infrequent. Previous literature describes upper GI bleeding (UGIB) in patients with known amyloid disease. Here, we describe a case of recurrent UGIB that ultimately led to a diagnosis of GI amyloidosis and multiple myeloma in a patient with no history of either. Case presentation A 76-year-old male presented to the emergency department with frank hematemesis, melena, and a decreased level of consciousness. Management required intensive care unit (ICU) admission with transfusion, intubation, and hemodynamic support. Upper endoscopy revealed gastritis with erosions and nodularity in the gastric cardia and antrum. Hemostasis of a suspected bleeding fundic varix could not be achieved. Subsequently, the patient underwent computerized tomography (CT) angiography and an interventional radiologist completed embolization of the left gastric artery to address potentially life-threatening bleeding. Complications included development of bilateral pleural effusions and subsegmental pulmonary emboli. Pleural fluid was negative for malignancy. He was transferred to a peripheral hospital for continued care and rehabilitation. Unfortunately, he began re-bleeding and was transferred back to our tertiary center, requiring re-admission to the ICU and repeat endoscopy. Repeat biopsy of the gastric cardial nodularity was reported as active chronic gastritis and ulceration. However, based on the unusual endoscopic appearance, clinical suspicion for malignancy remained high. He exhibited symptoms of congestive heart failure following standard resuscitation. Transthoracic echocardiogram (TTE) demonstrated a reduced ejection fraction of 35–40% and a strain pattern with apical sparing. Following discussions between the treating gastroenterologist, consulting cardiologist, and pathologist, Congo Red staining was performed, revealing submucosal amyloid deposits. Hematology was consulted and investigations led to diagnosis of multiple myeloma (MM) and immunoglobulin light-chain (AL) amyloidosis. The patient was treated for MM for four months prior to cessation of therapy due to functional and cognitive decline. Conclusions GI amyloidosis can present with various non-specific clinical symptoms and endoscopic findings, rendering diagnosis a challenge. This case illustrates GI amyloidosis as a potential—albeit rare—etiology of UGIB.

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Myocardial amyloidosis following multiple myeloma in a 38-year-old female patient: A case report

Open Medicine ◽

10.1515/med-2020-0125 ◽

2020 ◽

Vol 15 (1) ◽

pp. 396-402

Author(s):

Qisi Zhang ◽

Yingli Qiao ◽

Dongmei Yan ◽

Yuhui Deng ◽

Mengyang Zhang ◽

...

Keyword(s):

Multiple Myeloma ◽

Plasma Cells ◽

Laboratory Data ◽

Young Patients ◽

Clinical Manifestations ◽

The Elderly ◽

First Episode ◽

Rapid Progression ◽

Congo Red Staining ◽

Impaired Cardiac Function

AbstractMultiple myeloma (MM) is an immunoglobulin-producing tumor of plasma cells, which occurs commonly in the elderly. The incidence of myocardial amyloidosis with MM is extremely low and early clinical manifestations are nonspecific. The diversity of clinical manifestations and first episode symptoms often cause misdiagnosis in young patients with myocardial amyloidosis following MM. In this study, we analyzed the clinical data of a young woman with MM and impaired cardiac function combined with echocardiography, electrocardiography (ECG), laboratory data, cell Congo Red staining, and other manifestations to diagnose amyloidosis. Considering the rapid progression, short survival, and poor prognosis in most patients, a clear, definitive, and timely diagnosis is essential for the treatment of patients with MM complicated with myocardial amyloidosis.

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Tropheryma whipplei, Immunosuppression and Whipple's Disease: From a Low-Pathogenic, Environmental Infectious Organism to a Rare, Multifaceted Inflammatory Complex

Digestive Diseases ◽

10.1159/000369538 ◽

2015 ◽

Vol 33 (2) ◽

pp. 190-199 ◽

Cited By ~ 10

Author(s):

Thomas Marth

Keyword(s):

Heart Valves ◽

Clinical Manifestations ◽

Systemic Infection ◽

Tropheryma Whipplei ◽

Molecular Techniques ◽

Whipple’S Disease ◽

Whipple's Disease ◽

New Findings ◽

The Central Nervous System ◽

Body Compartments

Background: The actinobacterium Tropheryma whipplei was detected 20 years ago by molecular techniques, and following its culture has been characterized as the cause of a systemic infection known as Whipple's disease (WD). T. whipplei occurs in the environment, is prevalent only in humans, is believed to be transmitted via oral routes and to be host dependent. Key Messages: The classical form of T. whipplei infection, i.e. classical WD (CWD), is rare. It is well defined as slowly progressing chronic infection with arthralgia, diarrhea and weight loss, mostly in middle-aged men. However, current research revealed a much broader spectrum of clinical features associated with T. whipplei infection. Thus, T. whipplei may cause acute and transient infections (observed primarily in children) and the bacterium, which is found in soil and water, occurs in asymptomatic carriers as well as in CWD patients in clinical remission. In addition, T. whipplei affects isolated and localized body compartments such as heart valves or the central nervous system. Subtle immune defects and HLA associations have been described. New findings indicate that the progression of asymptomatic T. whipplei infection to clinical WD may be associated with medical immunosuppression and with immunomodulatory conditions. This explains that there is a discrepancy between the widespread occurrence of T. whipplei and the rareness of WD, and that T. whipplei infection triggered by immunosuppression presents with protean clinical manifestations. Conclusions: This review highlights recent findings and the clinical spectrum of infection with T. whipplei and WD, focusing specifically on the role of host immunity and immunosuppression. Current concepts of the pathogenesis, diagnosis and therapy are discussed.

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