scholarly journals Coexistence of Craniopharyngioma and Cranial Fibrous Dysplasia: A Clinicopathological Study of 5 Cases and Literature Review

Author(s):  
Yang-Hua Fan ◽  
ZHI LI

Abstract Background: Craniopharyngioma (CP) and cranial fibrous dysplasia (CFD) are both rare embryonic benign cranial diseases that most commonly present during childhood or adolescence. Co-existence of CP and CFD is extremely rare, which has never been reported before.Methods: We retrospectively reviewed the data of 5 patients coexisted with CP and CFD in Beijing Tiantan Hospital from January 2003 to January 2021. Their clinicopathological features, treatment modalities, and outcomes were summarized. Moreover, a comprehensive literature review was conducted, and in order to explore the potential connection leading to this coexistence, the CFD characteristic GNAS gene and corresponding Gsα protein were tested in the CPResults: There were 4 males and 1 female (median age, 39 years) in the present series. The symptoms mainly included headache, dizziness, fatigue, polyuria/polydipsia, hypogonadism and blurred vision. Sphenoidal bone is the most common involved bone by CFD (n =4). Four patients had undergone surgery to remove the CP (1 transsphnoid and 3 transcranial). Complete and subtotal resection were achieved in 2 cases respectively. The tumor subtypes were 3 adamantinomatous, 1 unknown subtype. The common postoperative complications are pan-hypopituitarism, diabetes insipidus, and hypothyroidism. The mean follow-up time was 57.2 months. Postoperative hormone replacement was required in 2 patients. 3 patients underwent a genetic study of tumor specimens. GNAS mutations were not detected, but they were positive for Gsα protein. Conclusions: Though the definite causative relationship has not been proved, the coexistence of CP and CFD should not completely be excluded potential interplay or atypical FD course for the uncommon manifestations of CPs. Prompt diagnosis and appropriate treatment are more challenging than for solitary CPs for the deformations of skull base, as of now, management strategies are aimed at surgical treating the CP and regularly monitoring the CFD.

Author(s):  
David Cutter ◽  
Martin Scott-Brown

The variety of conditions that are considered to be ‘cancer’ is extremely wide, with marked variation in the management approach from disease to disease. A common feature in the management of malignant conditions, however, is the involvement of a wide range of medical professionals at different stages of the patient pathway. This commonly includes physicians, surgeons, radiologists, pathologists, medical oncologists, radiation oncologists, and specialist nurses, as well as a plethora of other allied disciplines. As such, a practice that has been widely adopted is to work as a multidisciplinary team (MDT), with regular meetings to decide the appropriate treatment for each patient with a cancer diagnosis, on an individual and case-by-case basis. The main treatment modalities for the treatment of cancer are surgery, radiotherapy, and chemotherapy. While these are often combined to form a multimodality therapy, they are all, in isolation, potentially radical (curative) therapies for certain conditions. For example, surgery (in the case of a Stage I colon adenocarcinoma), radiotherapy (in the case of early laryngeal squamous cell carcinoma), and chemotherapy (in the case of acute lymphoblastic leukaemia) are all curative as single-modality treatments. It is commonly the case, however, for a patient to require more than one mode of therapy to achieve the best outcome, for example a combination of surgery, chemotherapy, and radiotherapy for early breast cancer. It can also be the case that two or more different management strategies are thought to give equivalent oncological results, for example surgery or radiotherapy for early prostate cancer. In this situation, the MDT and the patient need to decide on the ‘best’ management plan for the individual, based on their personal and professional opinions and on the differing toxicity profiles of the alternate treatments.


2020 ◽  
Vol 0 ◽  
pp. 1-4
Author(s):  
Rijami Godwin Ekpo ◽  
Otei O. Otei ◽  
Mba O. Ozinko

Erysipelas, a variant of cellulitis, is a superficial dermal bacterial infection. This superficial cutaneous infection may cause lymphangitis with resultant lymphedema in poorly treated cases. Reports of erysipelas preceding lymphedema are rare, rather many have reported the occurrence of erysipelas in patients with subclinical or overt lymphedema. Two case reports of erysipelas preceding lymphedema after appropriate parenteral antibiotics therapy and limb elevation are here presented. Following diagnosis, admission, and commencement of therapy, the second patient (Case 2) discontinued hospital treatment halfway for native treatment and returned after about 3 weeks. All patients recovered successfully but later presented with lymphedema after 8 weeks and 6 weeks, respectively. Both patients made a sustained functional recovery of their limbs with conservative management including elastic compression stocking and limb elevation. Erysipelas infection preceding lymphedema may develop when it occurs, prompt and appropriate treatment modalities for erysipelas infection may forestall the development of lymphedema.


Author(s):  
Xuefeng Wei ◽  
Xu Zhang ◽  
Zimu Song ◽  
Feng Wang

Abstract Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.


Dermatology ◽  
2020 ◽  
Vol 237 (4) ◽  
pp. 618-628
Author(s):  
Philip Surmanowicz ◽  
Sean Doherty ◽  
Arunima Sivanand ◽  
Nikoo Parvinnejad ◽  
Jean Deschenes ◽  
...  

<b><i>Background:</i></b> Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (SMPLPD) is a provisional entity within the 2016 World Health Organization classification of primary cutaneous lymphomas. The condition is currently classified as a lymphoproliferative disorder to emphasize its benign course and discourage aggressive, systemic treatment modalities. <b><i>Objective:</i></b> To provide a relevant synthesis for the dermatological practitioner on the prevalence, presentation, and treatment of SMPLPD. <b><i>Methods:</i></b> We conducted an updated systematic literature review and a retrospective chart review of diagnosed cases of SMPLPD from 2 Canadian academic cutaneous lymphoma centers. <b><i>Results:</i></b> A total of 23 studies with 136 cases were extracted from the systematic review and 24 patients from our retrospective chart review. SMPLPD proved relatively common accounting for 12.5% of all cutaneous T-cell lymphomas encountered in our cutaneous lymphoma clinics, second in frequency only to mycosis fungoides. The typical clinical presentation was that of an older individual (median age 59 years) with an asymptomatic solitary lesion on their upper extremity. The most common clinical differentials were cutaneous lymphoid hyperplasia, basal cell carcinoma, and lymphoma unspecified. T follicular helper markers were reliably detected. The main treatment modalities were surgical excision, local radiation therapy, and topical or intralesional steroids. Cure was achieved in the vast majority of cases. <b><i>Conclusions:</i></b> SMPLPD is an underdiagnosed T-cell lymphoma with an overtly benign clinical course. The condition has an excellent prognosis and responds well to skin-directed therapies. Practitioners should be aware of this condition to avoid aggressive systemic treatments.


2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
M Aker ◽  
A Askari ◽  
M Rabie ◽  
M Aly ◽  
S Adegbola ◽  
...  

Abstract Introduction Colorectal anastomotic leaks (AL) are an unfortunate occurrence and are associated with a high mortality. The aim of this multi-centre study is to explore the different management strategies used and compare outcomes in the management of AL. Method All patients who had an AL were included at seven hospitals across the East of England. Morbidity, mortality, and survival were compared across the different management strategies. Results A total of 247 consecutive patients were included of which 60.3% were male and the median age was 68 (IQR 57-77). Half of patients were initially managed conservatively, a further 10.5% had a radiological procedure. 39.7% required surgery as an initial treatment. Of those who initially did not have a surgical intervention (n = 149), 10.7% (n = 16/149) eventually required laparotomy. Ultimately, 42.7% (n = 106/248) required a laparotomy. The 30- and 90-day across the entire population mortality were 3.6% and 4.9% respectively. There were no significant differences in mortality or long-terms survival between the different initial treatment modalities. Conclusions Despite initial conservative, antibiotic and radiological intervention being successful in the majority of patients, two out of five patients will still require a laparotomy. A consensus approach is required to standardise management in these difficult scenarios.


2021 ◽  
Vol 11 (7) ◽  
pp. 99
Author(s):  
Gian Piero Turchi ◽  
Marta Silvia Dalla Riva ◽  
Luisa Orrù ◽  
Eleonora Pinto

Starting from statistical data derived from the oncological field, some articles have highlighted the importance of communication in the patient–caregiver dyad and have considered the various roles involved in a cancer diagnosis situation. Thus, the question of how to intervene in terms of “quality of life” from the time of diagnosis to the recovery or death of a cancer patient, beyond the sanitary and physical dimensions, has become relevant. Therefore, the present narrative review aims to offer an overview of the state of the art in terms of the psychological treatment modalities of cancer patients, from the diagnosis to the post-surgery period. A total of 67 articles were collected and analyzed, in relation to (1) psychological constructs employed in the oncological field, (2) intervention models and (3) quality of life and well-being measurement and evaluation tools. We described these articles, differentiating between those focusing on the role of (1) the patient, (2) the caregiver, (3) the patient–caregiver dyad and (4) healthcare professional roles. The oncological diagnosis and its repercussions in the lives of the patient and caregiver were explored and critical aspects that emerged from the literature were highlighted. In conclusion, the analysis allowed some considerations about the need to define research protocols and useful management strategies for increasing the overall health of patients with cancer diagnoses and the people who surround them.


2021 ◽  
Vol 28 (1) ◽  
pp. 640-660
Author(s):  
Grace Lassiter ◽  
Cole Bergeron ◽  
Ryan Guedry ◽  
Julia Cucarola ◽  
Adam M. Kaye ◽  
...  

Multiple myeloma (MM) is a hematologic malignancy characterized by excessive clonal proliferation of plasma cells. The treatment of multiple myeloma presents a variety of unique challenges due to the complex molecular pathophysiology and incurable status of the disease at this time. Given that MM is the second most common blood cancer with a characteristic and unavoidable relapse/refractory state during the course of the disease, the development of new therapeutic modalities is crucial. Belantamab mafodotin (belamaf, GSK2857916) is a first-in-class therapeutic, indicated for patients who have previously attempted four other treatments, including an anti-CD38 monoclonal antibody, a proteosome inhibitor, and an immunomodulatory agent. In November 2017, the FDA designated belamaf as a breakthrough therapy for heavily pretreated patients with relapsed/refractory multiple myeloma. In August 2020, the FDA granted accelerated approval as a monotherapy for relapsed or treatment-refractory multiple myeloma. The drug was also approved in the EU for this indication in late August 2020. Of note, belamaf is associated with the following adverse events: decreased platelets, corneal disease, decreased or blurred vision, anemia, infusion-related reactions, pyrexia, and fetal risk, among others. Further studies are necessary to evaluate efficacy in comparison to other standard treatment modalities and as future drugs in this class are developed.


2016 ◽  
Vol 24 (4) ◽  
pp. 431-439 ◽  
Author(s):  
Khawla Abu Samra ◽  
Armin Maghsoudlou ◽  
Ramak Roohipoor ◽  
Manuel Valdes-Navarro ◽  
Stacey Lee ◽  
...  

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Mohammed Hammoudeh ◽  
Hanan Al Rayes ◽  
Adel Alawadhi ◽  
Kamel Gado ◽  
Khalid Shirazy ◽  
...  

Data on spondyloarthritis (SpA) from the Middle East are sparse and the management of these diseases in this area of the world faces a number of challenges, including the relevant resources to enable early diagnosis and referral and sufficient funds to aid the most appropriate treatment strategy. The objective was to report on the characteristics, disease burden, and treatment of SpA in the Middle East region and to highlight where management strategies could be improved, with the overall aim of achieving better patient outcomes. This multicenter, observational, cross-sectional study collected demographic, clinical, laboratory, and treatment data on 169 consecutive SpA patients at four centers (Egypt, Kuwait, Qatar, and Saudi Arabia). The data collected presents the average time from symptom onset to diagnosis along with the presence of comorbidities in the region and comparisons between treatment with NSAIDs and biologics. In the absence of regional registries of SpA patients, the data presented here provide a rare snapshot of the characteristics, disease burden, and treatment of these patients, highlighting the management challenges in the region.


Sign in / Sign up

Export Citation Format

Share Document