Aim/objective: Mucosal neuroma (MN) is a benign peripheral nerve tumour that usually occurs as a symptom of multiple endocrine neoplasia type 2B (MEN2B) (MEN2B-associated MN). MN that is not associated with MEN2B is termed solitary mucosal neuroma (SMN). We present a case of SMN in the upper gingiva and examine previously reported cases. Methods: The study included nine patients (eight from seven articles and one from this study) with SMN. Age of onset, sex, disease duration, tumour site, clinical findings including subjective symptoms, diagnostic method, treatment, prognosis and relationship with MEN2B were investigated in these nine patients. Results and conclusion: SMN is thought to be caused by the overproduction of nerve growth factors due to trauma or chronic continuous stimulation, whereas MEN2B-associated MN is caused by GNDF-induced hyperplasia. Considering the pathogenesis of their development, both diseases exhibit hyperplasia of the nerve fibre bundles and have the same histological type of hypertrophy. MNs (SMN and MEN2B-induced MN) must be differentiated from benign peripheral neurogenic tumours. Morphological recognition and immunohistopathological analyses make this differentiation possible.
Objectives: Mid-regional pro-adrenomedullin (MR-proADM) and monocyte CD169 (CD169) are valuable prognostic indicators of severe COVID-19. Methods: We assessed the predictive ability of a single measurement of MR-proADM and CD169 at emergency department (ED) admission to forecast in-hospital and 60-day mortality in adult COVID-19 patients. We analyzed clinical and laboratory data, with in-hospital mortality as the primary endpoint and 60-day mortality as the secondary endpoint. We examined associations with clinical and laboratory variables through univariate and multivariate analyses. Results: Data from 382 patients over 14 months were analyzed. Significant predictors of in-hospital mortality included age ⩾ 70 years (hazard ratio [HR] 8.1; 95% confidence interval [CI] 2.2-29.5), CD169 ratio ⩾ 20 (HR: 2.4; 95%CI: 1.6-5.6), MR-proADM ⩾ 1.1 mmol/L (HR: 5.1; 95%CI: 1.7-15.6), the need for invasive mechanical ventilation (HR: 6.8; 95%CI: 2.4-19.1), and active cancer (HR: 5.2; 95%CI: 1.8-15.2). For 60-day mortality, only elevated MR-proADM levels showed predictive value (HR: 6.7; 95%CI: 1.7-25.0), while high serologic titer was protective (HR: 0.4; 95%CI: 0.1-0.9). Conclusion: A single MR-proADM and CD169 measurement upon ED admission has prognostic value for in-hospital mortality, with MR-proADM also predicting 60-day mortality.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive neoplastic process of precursor plasmacytoid dendritic cells. The diagnostic evaluation of this heterogenous entity is challenging, requiring a comprehensive approach of incorporating clinical, morphologic, immunohistochemical, and molecular/cytogenetic evaluations. Optimal management of BPDCN remains controversial, and clinical outcomes continues to be poor. Pediatric cases of BPDCN are rare and to our knowledge, this is the second case of BPDCN described in a Hispanic child, first one was described outside the US in Peru. Here, we report a case of a juvenile patient of Hispanic origin presenting with cutaneous and bone marrow involvement and initially misdiagnosed as a cutaneous infection that resulted in subsequent delaying of necessary chemotherapy for 2 months. Biopsy of the lesion showed diffuse infiltration of immature cells involving the dermis with classical sparring of epidermis. A huge panel of immunohistochemical stains were performed to reach the diagnosis of BPDCN. Staging bone marrow biopsy also revealed involvement by BPDCN. Treatment was not only delayed in this patient but also due to the rarity of BPDCN in pediatric population, the subsequent therapeutic decisions were challenging for the primary oncology team as it was based solely on published literature on adult population. Our case report will not only add one more case in the pediatric age group, but also will also emphasize that although BPDCN has a grave prognosis in the elderly, timely diagnosis with prompt treatment is the key to complete remission in pediatric BPDCN population.
Mpox (human mpox) is an opportunistic viral zoonosis that bears a strong resemblance to smallpox. The virus is divided into 2 distinct clades, clade I and clade II, which were originally confined to the moist forest regions of Africa. However, due to air travel and the exotic pet trade, these clades have spread globally. As immunity from smallpox vaccination declined, Mpox emerged as the most significant viral infection affecting humans within the Orthopoxvirus genus. While sporadic cases occurred worldwide, the largest Mpox outbreak outside Africa took place in 2022, prompting the WHO to declare a global public health emergency. Asia plays a pivotal role in both hosting and contributing to the spread of Mpox, driven by socio-economic factors such as high population density, widespread illegal wildlife trade, and a continuous rise in air travel. The increasing number of Mpox cases in Asia poses a significant challenge to healthcare systems, especially during times of global economic crisis. Strengthening diagnostic capabilities and infrastructure, while sustainably utilizing available resources to target high-risk populations, will be essential in addressing the Mpox threat in the region.
Background: The most prevalent subtype of non-Hodgkin lymphoma is diffuse large B-cell lymphoma (DLBCL). Germinal center B-cell (GCB) and non-germinal center B-cell (non GCB) are the two main biologically different molecular subtypes identified utilizing an immunohistochemistry-based approach. Aim: Our objective in this study is to analyze the impact of immunohistochemical subtypes of DLBCL (GCB or non GCB) on demographic and clinicopathological parameters, response to chemotherapy and survival outcomes. Subjects and methods: This is a retrospective study including 106 cases of DLBCL collected in the department of pathology, Hassan II university hospital, Fez (Morocco), over a period of 12 years (January 2010-September 2022). The subtypes of DLBCLs were defined according to Hans algorithm, using immunohistochemistry by three biomarkers (CD10, BCL6, MUM1). Statistical analysis used: Independent t tests and analyses of variance were used for the comparison of mean values. We employed the SPSS 26.0 program to achieve this. A statistically significant value was set at P < .05. Results: Seventy-five patients (71%) were non-GCB subtype, while thirty-one patients (29%) had the GCB immunosubtype. We have found a significant ( P < .05) correlations between DLBCL immunosubtypes and treatment responses on one hand and survival in the other hand. In the GCB subtype, the response rate and survival were significantly improved. A significant association was found between Ki 67 expression and survival on univariate analysis. On multivariate analysis, we note a correlation between Ki 67 expression, DLBCL immunohistochemical subtypes and survival outcome. Conclusion: Non GCB subtype is associated with poor response to treatment and inferior survival outcome compared to GCB subtype in Moroccan context, especially when combined with high expression of Ki 67 marker.
Introduction: Poroid neoplasms (PN) are a heterogeneous group of tumors deriving from sweat glands and folliculo-sebaceous units. Their histological classification and clinical features are challenging. Our aim was to report clinicopathological features of poroid neoplasms. Methods: It is a retrospective study including all cases of poroid neoplasms registered at our Pathology laboratory of Niamey National Hospital (February 2020-February 2024). Results: We registered 13 cases of benign poroid neoplasms: 10 classic poromas (CP) (76.9%), 2 poroid hidradenomas (PH) (15.4%) and 1 dermal duct tumor (DDT) (7.7%). Nine cases (69.2%) had preoperative clinical diagnosis of malignancy. The mean age was 41.1 years (range of 12-70 years) with a slight female predominance. Only 4/13 cases (30.8%) had classical palmoplantar locations. The tumors mean size was 3.7 cm (range of 0.4-8 cm). Clear cells were present in 7 cases (53.8%), apocrine ductal differentiation (mixed or pure) in 6 cases (46.2%), keratin horns in 2 cases (15.4%), squamous eddies in 6 cases (46.2%), melanin pigments in 1 case (7.7%) and sebaceous differentiation in 2 cases (15.4%). Conclusions: Unlike what is classically reported, our study shows that apocrine ductal differentiation, younger age and non-palmoplantar locations are common in poroid neoplasms.
Primary breast lymphoma (PBL) is a rare malignant lymphoid neoplasm limited to the breast, accounting for about 0.15% of all malignant breast tumors and 1.7% to 2.2% of extra-nodal lymphomas. PBL must be distinguished from conventional breast carcinomas due to different therapeutic approaches. A 25-year-old female presented with a left breast mass. Histopathology and immunohistochemical tests confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL). She had no similar lesions elsewhere in the body. She received 1 cycle of R-CHOP chemotherapy but absconded from the treatment and succumbed afterward while at home. Recent developments in DLBCL treatment have greatly improved patient outcomes by incorporating targeted medicines like rituximab, increased chemotherapy regimens, new drugs, and individualized treatment techniques. PBL appears to have a worse prognosis; thus, delay or abscondment from treatment is of serious concern when it comes to improving the prognosis of patients with PBL.
Cotyledonoid-dissecting leiomyoma, a very unusual form of uterine leiomyoma, often leads to misdiagnosis as a malignant tumor. Here, we describe a case of a 45-year-old nulliparous woman who underwent a laparoscopic biopsy of a large pelvic mass consisting of multiple flaps. Histologically, the mass was composed of smooth muscle fascicle nodules separated by hydropic connective tissue, and exhibited extensive stromal hyalinization. The tumor was diagnosed as a cotyledonoid-dissecting leiomyoma based on the laparoscopic, pathological, and image findings. Prior to performing radical laparotomy, two courses of leuprorelin were administered in anticipation of tumor reduction and hypoperfusion, and the tumor size reduced remarkably. We demonstrated the utility of laparoscopic biopsy, considering its minimal invasiveness and diagnostic accuracy. Furthermore, the preoperative use of Gonadotropin-releasing hormone (GnRH) analogs to reduce surgical stress may be useful for treating cotyledonoid-dissecting leiomyomas.
COVID-19 infection is still a mystery in terms of its long-term effect on health and its consequences on hematological disorders. Prior studies including ours have shown the abnormal changes in hematopoietic cells in COVID-19 patients. In this article, we are presenting 2 cases of pediatric B-lymphoblastic leukemia (B-ALL) with a previous history of COVID-19 infection. The first case describes a 22-month-old boy presenting with lymphadenopathy, neutropenia, and anemia with concurrent COVID-19 infection without any evidence of a hematolymphoid neoplasm as per bone marrow and lymph node biopsy. However, he presented after 2 months with bone marrow biopsy confirming B-ALL. The second case is that of a 4-year-old girl presenting with B-ALL who has had asymptomatic COVID-19 infection 5 months before this current presentation. Both the cases had complete resolution of COVID-19 infection during the time of presentation with acute leukemia. There were notably 2 rare findings along the course of the patients’ illnesses. First, the unusual plasmacytosis in the marrow during active COVID-19 infection in the first patient and the second, is predilection of development of B-ALL following COVID-19. In both the cases the fluorescence in situ hybridization (FISH) studies showed pathologic alteration of the RUNX1 gene. Overall, there are no literature to support a causal association between acute B-ALL and COVID-19. The diagnosis of B-ALL in these patients after COVID-19 infection may be totally unrelated. However, if we consider Greaves proposed 2-hit model for childhood acute leukemia, that an infectious agent can precipitate development of B-ALL in a genetically susceptible individual. Alteration of the RUNX1 gene in both the patients, opens a door for further exploration of the “second-hit” hypothesis regarding an infectious agent precipitating development of B-ALL in a genetically susceptible individual.
The nested subtype of urothelial carcinoma (NS-UC), a rare and aggressive bladder cancer, mimics benign bladder lesions but behaves like high-grade urothelial carcinomas. The author reported a rare case of NS-UC, initially presenting with inguinal lymph node metastasis. The tumor cells of NS-UC exhibit minimal cellular atypia, forming small nests, while the tumor cells of lymph node metastatic carcinoma show greater cellular atypia with diverse structures. Immunohistochemistry is helpful in determining the origin of lymph node metastatic carcinoma. NS-UC often presents morphologically similar to benign lesions, which should be given sufficient attention.
Urinary symptoms are one of the most common reasons for emergency visits in females of pediatric age group and can be associated with various conditions like infections (most common), sexual trauma and rarely neoplastic processes. Here, we report a case of a 7-year-old female who presented in the emergency multiple times with the complaints of urinary symptoms and vaginal pain and was empirically treated with antibiotics and antifungals without symptomatic improvement. Her blood tests, physical examination during this time remained unrevealing. She was then transferred to our institution on her third emergency visit for further evaluation. On imaging studies, she was noted to have expansile lesions on her vertebral body at the L4 and T6 levels with compressive myelopathy with multiple bone and soft tissue lesions throughout her lower extremities. Patient developed saddle anesthesia requiring emergent decompression and biopsy of the epidural mass with the final pathology coming back as B-lymphoblastic leukemia/lymphoma. B-ALL/B-LBL is the most common pediatric hematologic malignancy and usually presents with fever, hepatosplenomegaly, lymphadenopathy, bone pain and bleeding. Occasionally, atypical presentations like bone and joint pain, osteoporosis, palpable paravertebral mass have been described. However, this is the first case report to describe a very unusual and unfamiliar presentation of this disease causing significant diagnostic difficulty resulting in delayed treatment. This case report can aid as a reminder that unusual pain or any nonspecific manifestations in pediatric patients, refractory to common treatment should be investigated with extreme diligence not to miss this neoplastic process.
Advanced Non-Small Cell Lung Carcinoma (NSCLC) patients with ROS1 gene rearrangement have shown significant therapeutic responses to tyrosine kinase inhibitors approved by the US Food and Drug Administration, with approximately 40 fusion partners documented in the existing literature. Our report highlights a novel fusion partner of ROS1 that has demonstrated a conclusive response to the current standard of treatment.
Background: Patients with acute-on-chronic liver failure (ACLF) who take entecavir (ETV) and tenofovir disoproxil fumarate (TDF) experience a reduction in hepatic events and mortality. The effectiveness of tenofovir alafenamide (TAF) was not well investigated. This study was aim to compare the antiviral efficacy and mortality between TAF and ETV in patients with ACLF caused by the hepatitis B virus (HBV). Methods: One hundred and six patients with HBV-ACLF who received TAF (25 mg/day) and ETV (0.5 mg/day) for 12 weeks were analyzed. The primary endpoints were overall mortality and liver transplantation (LT) at week 12. Biochemical responses, virologic responses, mortality, drug safety, and side effects were evaluated. Results: At 4 and 12 weeks of TAF treatment, patients showed significantly higher HBV-DNA reduction ( P < .001), higher HBV-DNA undetectability rates ( P < .001), and lower HBV DNA levels ( P < .001) in serum. Lower Child-Turcotte-Pugh (CTP) scores ( P = .003) were observed at 4 weeks in the TAF group, although the CTP scores showed no difference between TAF group and ETV group at 12 weeks ( P = 1.143). Lower alanine aminotransferase (ALT) levels of patients in the TAF group at week 4 and 12 were observed ( P = .023 and P < .0001, separately). The mortality of TAF group was lower after 4 weeks of treatment ( P = .038); however, the 2 groups had similar mortality rates at week 8 and 12. Among the causes of death in HBV-ACLF patients, we found the same incidence of liver-related problems in both groups ( P > .05). Conclusions: This study showed that ACLF patients with chronic HBV infection treated with TAF had a rapid decline in HBV DNA, a higher rate of ALT reduction and improved CTP scores compared to the ETV group, thereby improving patient survival.
The differential diagnosis of cystic echinococcosis should be considered in the suspected case of a pelvic mass presenting with vague symptoms. Primary pelvic hydatid cyst is rare, and the achievement of early diagnosis is important, as the involvement of bone in the pelvis is difficult to treat, and the probability of recurrences is greater. This case report presents a rare case of a primary pelvic hydatid cyst in a male in his 30s. The patient presented vague symptoms of difficulty in micturition and chronic pelvic pain. First-line radiological imaging techniques diagnosed it as a neoplastic mass and tuberculosis, while MRI revealed it as a cystic disease, probably a hydatid cyst. Surgical excision and histopathological examination confirmed the diagnosis of a primary pelvic hydatid cyst. Acquaintance with the varied presentation of cystic echinococcosis helps to obtain an accurate diagnosis and thus decreases the severe complication associated with the disease. This case report highlights the importance of considering parasitic infections in the differential diagnosis of cystic pelvic masses, even in regions with low endemicity.
Introduction: Blood culture is the gold standard for diagnosing bacteremia and direct the physicians to select appropriate antimicrobials. In hospitals, blood culture contamination (BCC) is a common problem that has a detrimental effect on patient outcomes. Hence, we implemented strategies in our tertiary care setup, for training phlebotomists and nurses in proper blood sampling techniques, and assessed their effectiveness in reducing BCC rates. Methods: This interventional study was conducted at the Indus Hospital, Karachi, Pakistan from 1 st January 2021 to 30 th June 2023. All blood cultures received from different departments of the hospital were included. The 2.5-year study period was divided into pre-intervention and intervention periods, with monthly monitoring of BCC. The BCC data between 1 st January 2021 and 31 st December 2021 was taken as the baseline pre-intervention period and the next 1.5 years comprised the intervention period (1 st January 2022-30 th June 2023). To improve compliance, various strategies were implemented, such as regular training sessions, didactic sessions, and re-competencies. Results: A total of 86 774 Blood cultures were received from all departments of the hospital, out of which n = 30 672 were received in the pre-intervention period whereas, n = 56 102 were received in the intervention period. Mean BCC rate in the pre-intervention period was found to be 4.6%. However, after the implementation of different measures to reduce BCC, the contamination rate decreased to a mean of 3.1% by the end of the intervention period. Emergency department accounted for the highest proportion of BCC in the pre-intervention and intervention periods. Conclusion: We decreased BCC in our tertiary care setup by implementing a simple and inexpensive collaborative intervention, and came to the conclusion that the higher incidence of BCC was probably caused by factors unique to the emergency department and provided measures to successfully address them.
The COVID-19 pandemic-led worldwide healthcare crisis necessitates prompt societal, ecological, and medical efforts to stop or reduce the rising number of fatalities. Numerous mRNA based vaccines and vaccines for viral vectors have been licensed for use in emergencies which showed 90% to 95% efficacy in preventing SARS-CoV-2 infection. However, safety issues, vaccine reluctance, and skepticism remain major concerns for making mass vaccination a successful approach to treat COVID-19. Hence, alternative therapeutics is needed for eradicating the global burden of COVID-19 from developed and low-resource countries. Repurposing current medications and drug candidates could be a more viable option for treating SARS-CoV-2 as these therapies have previously passed a number of significant checkpoints for drug development and patient care. Besides vaccines, this review focused on the potential usage of alternative therapeutic agents including antiviral, antiparasitic, and antibacterial drugs, protease inhibitors, neuraminidase inhibitors, and monoclonal antibodies that are currently undergoing preclinical and clinical investigations to assess their effectiveness and safety in the treatment of COVID-19. Among the repurposed drugs, remdesivir is considered as the most promising agent, while favipiravir, molnupiravir, paxlovid, and lopinavir/ritonavir exhibited improved therapeutic effects in terms of elimination of viruses. However, the outcomes of treatment with oseltamivir, umifenovir, disulfiram, teicoplanin, and ivermectin were not significant. It is noteworthy that combining multiple drugs as therapy showcases impressive effectiveness in managing individuals with COVID-19. Tocilizumab is presently employed for the treatment of patients who exhibit COVID-19-related pneumonia. Numerous antiviral drugs such as galidesivir, griffithsin, and thapsigargin are under clinical trials which could be promising for treating COVID-19 individuals with severe symptoms. Supportive treatment for patients of COVID-19 may involve the use of corticosteroids, convalescent plasma, stem cells, pooled antibodies, vitamins, and natural substances. This study provides an updated progress in SARS-CoV-2 medications and a crucial guide for inventing novel interventions against COVID-19.
Intramuscular lipomas, typically found in subcutaneous tissue, rarely affect deeper muscular planes, especially those of the head and neck region. The following are 3 cases of intramuscular lipomas involving the sternocleidomastoid muscle. The first 2 patients presented with painless, palpable masses confirmed by diagnostic imaging as well-circumscribed intramuscular lipomas. One was treated surgically, while the other was managed conservatively with monitoring and close follow-up. The third patient reported dysphagia associated with occasional dyspnea and mild pain. The mass was identified as infiltrative lipoma and was resected surgically. Complete tumor removal with no recurrence at 6 months was observed for the first and last cases. The second case was serially followed at 3 and 6 months with no interval changes. We report the largest case series on intramuscular lipomas of the sternocleidomastoid muscle to enhance our understanding of this rare entity.
The most critical step in the clinical diagnosis workflow is the pathological evaluation of each tumor sample. Deep learning is a powerful approach that is widely used to enhance diagnostic accuracy and streamline the diagnosis process. In our previous study using omics data, we identified 2 distinct subtypes of pure seminoma. Seminoma is the most common histological type of testicular germ cell tumors (TGCTs). Here we developed a deep learning decision making tool for the identification of seminoma subtypes using histopathological slides. We used all available slides for pure seminoma samples from The Cancer Genome Atlas (TCGA). The developed model showed an area under the ROC curve of 0.896. Our model not only confirms the presence of 2 distinct subtypes within pure seminoma but also unveils the presence of morphological differences between them that are imperceptible to the human eye.
Appendiceal diverticulitis is an uncommon condition that clinically resembles acute appendicitis. However, it is an incidental finding in histopathological studies and is rarely diagnosed preoperatively by imaging studies. In this article, we present the clinical and imaging findings of a male patient presenting with right upper quadrant pain with a preoperative imaging diagnosis of appendiceal diverticulitis. He underwent laparoscopic appendectomy and confirmed the diagnosis of appendiceal diverticulitis. This is a rare preoperative diagnosis. The management is often like typical appendicitis which is appendectomy. It is important to differentiate it from diverticulitis of the small intestine or colon because these diseases usually require only conservative treatment.
Adamantinoma (AD) is a rare, slow-growing primary malignant bone tumor characterized by a biphasic morphology of clusters of epithelial cells and spindle cell osteofibrous components. A strong relationship between AD and osteofibrous dysplasia (OFD) has been proposed, while fibrous dysplasia (FD) has been rarely associated with AD. We present an AD case that was followed and histologically evaluated 3 times over 6 years with different morphological patterns. The tumor in the primary biopsy and after complete resection showed classical features of AD and osteofibrous-like pattern, while the recurrent lesion presented with exclusively spindle cell morphology and was thus diagnosed as FD. However, the extensive immunohistochemical analysis in all 3 lesions revealed strong reactivity for pancytokeratin, vimentin, p63, and podoplanin, which are characteristic for AD. Although, in the FD-like section of the tumor from the first recurrence the positivity of podoplanin was stronger than pancitokeratin, which was variably positive on spindle cells. The present case highlights the problem of diagnosing AD based on a single biopsy with one tumor’s component predominating over the other, and at the same time emphasizes the importance of using immunohistochemical staining for keratin and podoplanin when the histopathological features of (osteo)fibrous lesion can be linked to AD.
