scholarly journals Composite phaeochromocytomas—a systematic review of published literature

Author(s):  
K. Dhanasekar ◽  
V. Visakan ◽  
F. Tahir ◽  
S. P. Balasubramanian

Abstract Introduction Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre’s records and presents a systematic literature review of composite phaeochromocytomas. Methods In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist. Results There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4–86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5–168) months. Conclusion Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.

Author(s):  
Doniparthi Pradeep

The authors present an excellent retrograde analysis of a rare condition of a phenomenal number of cases and their surgical outcomes. A majority of the studies in published literature are anecdotal case reports which are a rare and dreadful entity. A comprehensive countrywide view of the UK National Adult Cardiac Surgery Audit database is presented in this study. This study represents the changing trends in the risk factors, management strategies, and outcomes of ventricular septal rupture for over 23 years in a nutshell.


2019 ◽  
Vol 8 (2) ◽  
Author(s):  
Anna Garofalo ◽  
Paolo Petruzzelli ◽  
Michela Chiadò Fiorio Tin ◽  
Silvia Parisi ◽  
Giulia Garofalo ◽  
...  

Abstract Background Symptomatic myomas during pregnancy are a rare condition that could however turn into an emergency because of torsion, necrosis, growth and compression leading to acute abdomen, potentially threating for pregnancy. Surgeons are usually reluctant to perform myomectomy during pregnancy because of an increased uterine blood flow and volume can give rise to a potential risk for haemorrhagic complications, while uterine manipulation can determine adverse pregnancy outcome. However, in some rare cases surgery is compulsory. Case presentation Here, we described a case of a successful laparotomic myomectomy performed during pregnancy at 11 weeks of gestation when an acute abdomen occurred. Surgery was followed by regular obstetrics follow-up ended with a spontaneous vaginal delivery with no pregnancy complications. Conclusion Although few case reports are described in literature, other authors have performed a myomectomy during pregnancy, and fewer cases have had a subsequent vaginal delivery, so that nowadays there is no clinical evidence on which to base best practice. This case shows that vaginal delivery after a laparotomic myomectomy performed during pregnancy, in selected cases, can be considered as a feasible option.


Author(s):  
Rita Sarabando ◽  
Natacha Sousa ◽  
Ana C. Borges ◽  
Cristina Nogueira-Silva

Persistent genital arousal disorder is a rare condition characterized by unwanted intrusive symptoms of sexual arousal without specific context. Their possible aetiologies and treatments are multiple and mostly based on case reports. We aim to do a comprehensive review of persistent genital arousal disorder and describe a case of a postmenopausal woman who developed this disease and, during the follow-up, was diagnosed with advanced endometrial cancer, reminding physicians to keep in mind the possibility of multiple diagnosis in the same patient, including malignancy. Although there is no description of this association in the literature, the possible aetiologies of persistent genital arousal disorder are diverse, and we sought this rare case should be disclosed.


2012 ◽  
Vol 126 (6) ◽  
pp. 641-644 ◽  
Author(s):  
H J Kim ◽  
B J Lee ◽  
J C Lee ◽  
C K Yeo

AbstractBackground:Cervical sparganosis is a rare condition that presents as a lateral neck mass. Its radiological findings have not previously been investigated. Thus, the important radiological findings of cervical sparganosis are presented herein.Methods:We report two patients with cervical sparganosis who presented with cervical masses, and we review the relevant head and neck literature. Computed tomography was performed three times over 13 months of follow up for one patient.Results:On follow-up radiological examination, a migratory lesion with a tubular appearance, seen on serial images, should be considered significant for cervical sparganosis.Conclusion:Radiologically, a migratory cervical mass in the head and neck area with a tubular appearance is suggestive of cervical sparganosis.


2020 ◽  
Vol 36 (11) ◽  
pp. 2635-2640
Author(s):  
Francesca Olivero ◽  
Thomas Foiadelli ◽  
Sabino Luzzi ◽  
Gian Luigi Marseglia ◽  
Salvatore Savasta

Abstract Background Pai syndrome is a rare idiopathic developmental condition characterized by midline craniofacial abnormalities. It was originally described as the presence of a median cleft lip, cutaneous polyps of the nasal mucosa and face, and midline lipomas of the central nervous system, mostly at the corpus callosum. However, there is great phenotypical variability and these characteristics are rarely all present at once. Objective The aim of this review was to analyze the available evidence regarding Pai syndrome in order to better delineate this rare condition and its features. Methods We analyzed the PubMed database using the words “Pai syndrome”, “frontonasal dysplasia”, “cleft lip”, “nasal polyp”, “facial polyp”, and “corpus callosum lipoma”, including reviews, case reports and case series. Conclusion There is no consensus regarding the diagnostic criteria of Pai syndrome up to date. It is usually diagnosed at birth, and its incidence is often underestimated. At present, the etiology of Pai syndrome is unknown. Several hypotheses regarding its genetic background have been made; however, there are not enough data yet to elucidate this point. An improved awareness could help in diagnosing the condition and performing the necessary investigations. These patients should have a multidisciplinary follow-up.


2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Martin G. D. Kelleher ◽  
Deborah I. Bomfim ◽  
Rupert S. Austin

The prevalence and severity of tooth wear is increasing in industrialised nations. Yet, there is no high-level evidence to support or refute any therapeutic intervention. In the absence of such evidence, many currently prevailing management strategies for tooth wear may be failing in their duty of care to first and foremost improve the oral health of patients with this disease. This paper promotes biologically sound approaches to the management of tooth wear on the basis of current best evidence of the aetiology and clinical features of this disease. The relative risks and benefits of the varying approaches to managing tooth wear are discussed with reference to long-term follow-up studies. Using reference to ethical standards such as “The Daughter Test”, this paper presents case reports of patients with moderate-to-severe levels of tooth wear managed in line with these biologically sound principles.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Michel Ibrahim ◽  
Michael Fattouh ◽  
Omar Siddiqi ◽  
Alice K Jacobs

Background: The evidence on recurrent pregnancy-related pericarditis is limited, and management strategies are based mainly on case reports and expert opinion. Case: A 25-year-old G2P1, 28-week pregnant female, with a history of presumed viral pericarditis complicated by pericardial tamponade and recurrent colchicine resistant pericarditis which was successfully treated with a prolonged steroid taper in the postpartum period, now 1 year in remission presents with shortness of breath and pleuritic chest pain with elevated inflammatory consistent with prior presentation of myo-pericarditis. A trans-thoracic echocardiogram (TTE) revealed a mild pericardial effusion without evidence of systolic dysfunction, and pericardial tamponade. Decision-Making: Given prior history of pregnancy related colchicine resistant pericarditis which was complicated by pericardial tamponade around her prior delivery time, it was decided by an interdisciplinary team involving rheumatology, cardiology and obstetrics, to initiate prednisone 10 mg daily. Symptoms subsequently subsided with a down trend of cardiac and inflammatory biomarkers. Daily 10 mg prednisone was to be continued up through delivery but within 2 months she presented yet again with a similar clinical picture and was diagnosed with recurrence of disease. Her prednisone was increased to 20 mg daily with symptom resolution. Two weeks later, she went into labor and received stress dose steroids. She had a normal spontaneous vaginal delivery without any complications. She continued the same dose of 20 mg of prednisone until her follow-up with rheumatology when the decision was made to initiate azathioprine and slowly titrate off the steroids. Conclusion: The case highlights not only the rare association between pregnancy and recurrent pericarditis but also the complexity of its management. The case of our patient underscores the importance of family planning, shared-decision making, and management by an interdisciplinary team comprised of rheumatology, obstetrics/gynecology, and cardiology. There are currently no known well controlled trials of therapy for pregnancy related idiopathic recurrent acute pericarditis.


2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Mohammad Jawad H. Rahal ◽  
Karam M. Karam ◽  
Selim M. Nasser ◽  
Jihad A. Daher ◽  
Hicham G. Abdel Nour ◽  
...  

We report the case of a sixty-eight-year-old female patient who presented for left ankle pain; X-rays and MRI showed a benign calcaneal cyst, found to be delayed distant metastasis from primary papillary thyroid carcinoma. Patient required surgical excision of the cyst. Results of histological examination showed metastatic papillary thyroid carcinoma. The patient underwent total thyroidectomy 12 years prior to presentation, with the same pathology. Postoperatively, she was treated with radioactive iodine. At 10-year follow-up post calcaneal mass excision, she was found to have a right proximal tibial mass and found to be recurrent with the same pathology. This case reports a rare condition that will be taken into consideration in bone metastasis with thyroid cancer.


2020 ◽  
pp. 014556132092317
Author(s):  
Shaowen Lv ◽  
Dehuan Xie ◽  
Zheng Wu ◽  
Lei Wang ◽  
Yong Su

Lymphoepithelial carcinoma (LEC) of the salivary gland is a rare malignancy which is identical to undifferentiated nasopharyngeal carcinoma. However, most patients are treated with surgery as primary treatment, which is impossible for some very locoregionally advanced patients. And there are few reports of patients treated by an induction chemotherapy (IC) and concurrent chemoradiotherapy (CCRT) approach. This report describes 3 cases of advanced stage LEC of the salivary gland. All patients presented with a palpable mass of variable duration and underwent induction CCRT. All cases were positive for Epstein-Barr virus–encoded small RNAs. After IC, all cases had reached partial response and all achieved complete response after CCRT. All patients remained local–regional recurrence-free after 6-month follow-up for case 1, 50-month for case 2, and 14-month for case 3 up to our last follow-up. No serious adverse events were found.


2012 ◽  
Vol 18 (2) ◽  
pp. 193-199
Author(s):  
DGM Akaiduzzaman ◽  
Md Zahedul Alam ◽  
Manilal Aich

Bachground: Ectopic thyroid is a rare entity resulting from maldescent of the thyroid gland & can be found anywhere between the foramen cecum & the normal pretracheal position of the thyroid gland, as well as in distant places such as the mediastinum and the subdiaphragmatic organs. Although most cases are asymptomatic, obstructive symptoms may appear. Any disease affecting the thyroid gland may also involve the ectopic thyroid, including malignancy. Hypothyroidism is frequent but hyperthyroidism is an exceptionally rare finding. The clinician must distinguish between ectopic thyroid and metastatic deposits emerging from an orthotopic gland, as well as other benign or malignant masses. Thyroid scintigraphy plays the most important role in diagnosing ectopy, but ultrasonography contributes as well. Treatment is indicated in the presence of symptoms & consists initially of full replacement thyroid hormone therapy. Severe or unresponsive cases require excision.Case reports: We present here two case reports & review of the literature.Conclusion: This review provides current understanding about the wide clinical spectrum of this rare condition, optimal diagnostic approach, differential diagnosis, and management strategies. DOI: http://dx.doi.org/10.3329/bjo.v18i2.12016 Bangladesh J Otorhinolaryngol 2012; 18(2): 193-199


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