Enigmatic Lesion Research Articles

Prevalence of Ameloblastoma in Sub-Saharan Africa: A systematic review and meta-analysis

IntroductionAmeloblastoma is the most common odontogenic tumor (OT) accounting for about 1- 3 % of all tumors and tumor-like lesions of the head and neck region. Contrasting reports from documented studies have observed that the relative frequency of ameloblastomas is higher in the black population as compared to Caucasians. This is a systematic review and meta-analysis of Sub-Sahara African prevalence of Ameloblastoma. MethodsThe MEDLINE, SCOPUS, and AJOL databases were searched for relevant studies published from 1980 till date. Papers selected for full-text review were included in the systematic review if they provided a hospital or population-based prevalence of Ameloblastoma. Manual searching of selected articles’ reference list was also performed to include additional studies. Two individuals independently performed abstract and full-text reviews, data extraction, and quality assessment of the papers. Random-effects models and/or meta-regression were used to generate pooled estimates by country, sex, and year of data collection. ResultsOf 264 abstracts screened, 166 articles were selected for full-text review. A total of 22 studies met the inclusion criteria and all articles were hospital-based. The pooled prevalence for Ameloblastoma was 12 % (CI 95 %: 9 % - 15 %). Increasing participant age was not associated with a higher Ameloblastoma prevalence. Prevalence was higher in Nigeria (13 %, CI 95 %: 10 % - 17 %) than in other sub-Saharan countries (9 %, CI 95 %: 6 % - 14 %). Sex, country, and year of data collection were not associated with statistically significant different estimates of prevalence. ConclusionSignificant gaps in data collection and overall knowledge about its epidemiology were identified, particularly about the population-based incidence of Ameloblastoma in sub-Saharan countries. Accurate estimates of the prevalence and incidence of Ameloblastoma are needed to plan for the health and social services that will be required to deal with this enigmatic lesion.

Rapid presentation of a de novo intracranial aneurysm: illustrative case.

Intracranial aneurysms are prevalent, particularly with advancing age. De novo aneurysms, occurring independently from the initial lesion, pose a unique challenge because of their unpredictable nature. Although risk factors such as female sex, smoking history, and hypertension have been proposed, the mechanisms underlying de novo aneurysm development remain unclear. A 79-year-old female developed a de novo saccular aneurysm within a year after management of a ruptured vertebral artery dissecting aneurysm. Her complex clinical course involved subarachnoid hemorrhage with diffuse vasospasm, stent occlusion of a dissecting aneurysm, discovery of a right 7- to 8-mm de novo middle cerebral artery aneurysm at the 1-year magnetic resonance angiography follow-up, and successful coil embolization. This rare occurrence challenges established timelines, as most de novo aneurysms manifest over a longer interval. Studies have attempted to identify risk factors, yet consensus remains elusive, particularly regarding the influence of treatment modality on de novo formation rates. This unique case urges reconsideration of posttreatment surveillance protocols, proposing shorter intervals for imaging and more vigilant follow-up strategies to detect asymptomatic de novo aneurysms. Timelier identification could significantly impact patient outcomes by averting potential ruptures. This emphasizes the need for further research to delineate effective monitoring and preventive measures for these enigmatic lesions.

A Study of Characteristic Features and Diagnostic Roles of X-ray and MRI in Calcifying Tendinitis of the Shoulder.

Background: Calcifying tendinitis (CT) is an enigmatic lesion with several obscure aspects and it is a common disorder of the upper extremity characterized by the presence of calcifications in rotator cuff tendons and synovial tissues. In this study, we aimed to review the demographic and clinical characteristics, as well as radiologic and treatment history in CT patients who were referred with shoulder pain. Methods: In this cross-sectional study, a total of 146 patients who were referred with a shoulder CT were included. The definitive diagnosis was based on a combination of plain radiograph and magnetic resonance imaging (MRI). A predesigned independent t test was used to capture demographic and clinical data, as well as radiologic and treatment histories, and a chi-square test was utilized to assess the statistical correlation between qualitative variables. Results: The median age of the patients was 42.5 years. The female to male ratio was 2.3 to 1. The complaint of restricted shoulder movement was recorded in 107 (73.3%) patients and more frequently in women (p = 0.042). Night pain was present in 109 (74.7%) patients. The current and previous diagnoses matched in 36.1% (13 out of 36) of patients who only had MRI and in 63.6% (35 out of 55) patients who had both MRI and plain radiograph with them. Supraspinatus tendon was the main site of calcified deposition 65% (95 out of 146). Conclusion: CT is frequent at the age of around 40 and in the female gender. The diagnosis should be based on a combination of radiography and MRI and not based on MRI alone. The efficacy of different conservative treatments remains to be unwrapped.

Oral Warty Dyskeratoma-A Systematic Review of the Literature.

Objective: To systematically review the clinicopathological features of oral warty keratoma based on published literature. Materials and Methods: PubMed and Scopus databases were searched for reports of oral warty dyskeratoma. Of the 52 identified articles, only 25 articles (43 cases) satisfied the selection criteria (case report/series in the English language reporting clinicopathologically diagnosed oral warty dyskeratoma/oral focal acantholytic keratosis/oral isolated dyskeratosis follicularis in humans). Risk of bias was assessed using the Joanna Briggs institute critical appraisal checklist for case reports and case series. Results: Most cases had well-circumscribed, white, nodular verruco-papillary lesions with a central depressed/crater-like area. Alveolar ridges were the most frequent sites of occurrence and tobacco was the most commonly associated risk factor. Histopathologically, the most pathognomonic feature was the supra-basal clefting. The cleft had dyskeratotic acantholytic cells (corps ronds, and grains). Below the cleft were projections of the connective tissue villi lined by basal cells. The basal cells in a few cases exhibited hyperplasia in the form of budding into the stroma, but epithelial dysplasia was not reported. The surface epithelium had crypts filled with keratin debris. Conclusion: Oral warty dyskeratoma is a rare solitary self-limiting benign entity, which due to its clinical and histopathological resemblance and associated habit history could be misdiagnosed as leukoplakia or carcinoma. None of the assessed articles provided molecular data, which in turn could be the reason for the lack of insight into the etiopathogenesis of this enigmatic lesion.

Sclerosing Polycystic Adenoma of Salivary Glands: A Novel Neoplasm Characterized by PI3K-AKT Pathway Alterations-New Insights Into a Challenging Entity.

Sclerosing polycystic adenoma (SPA) is a rare salivary gland neoplasm originally thought to represent a non-neoplastic lesion. Recently we have encountered an index case of apocrine intraductal carcinoma of parotid gland of 62-year-old man with invasive salivary duct carcinoma component arising from SPA, a combination of tumor entities that has never been published so far. Here, we further explore the nature of SPA by evaluating 36 cases that were identified from the authors' consultation files. The patients were 25 females and 11 males aged 11 to 79 years (mean, 47.8 y). All tumors originated from the parotid gland. Their size ranged from 11 to 70 mm (mean, 28 mm). Histologically, all cases revealed characteristic features of SPA, such as lobulated well-circumscribed growth, focal hyalinized sclerosis, presence of large acinar cells with abundant brightly eosinophilic intracytoplasmic granules, and ductal components with variable cytomorphologic characteristics, including foamy, vacuolated, apocrine, mucous, clear/ballooned, squamous, columnar and oncocyte-like cells. In all cases, there were foci of intraluminal solid and cribriform intercalated duct-like epithelial proliferations with variable dysplasia which were positive for S100 protein and SOX10, and fully enveloped by an intact layer of myoepithelial cells. In addition, 14/36 cases (39%) had focal intraductal cribriform and micropapillary apocrine-type dysplastic epithelial structures composed of cells positive for androgen receptors and negative for S100/SOX10. The intraductal proliferations of both types showed focal mild to severe dysplasia in 17 cases (17/36; 47%). Two cases showed overt malignant morphology ranging from high-grade intraductal carcinoma to invasive carcinoma with an apocrine ductal phenotype. Next generation sequencing using ArcherDX panel targeting RNA of 36 pan-cancer-related genes and/or a TruSight Oncology 170/500 Kit targeting a selection of DNA from 523 genes and RNA from 55 genes was performed. Tumor tissue was available for molecular analysis in 11 cases, and 9 (9/11; 82%) of them harbored genetic alterations in the PI3K pathway. Targeted sequencing revealed HRAS mutations c.37G>C, p.(Gly13Arg) (2 cases) and c.182A>G, p.(Gln61Arg) (2 cases), and PIK3CA mutations c.3140A>G, p.(His1047Arg) (3 cases), c.1633G>A, p.(Glu545Lys) (1 case), and c.1624G>A, p.(Glu542Lys) (1 case). Moreover, mutations in AKT1 c.49G>A, p.(Glu17Lys) and c.51dup, p.(Tyr18ValfsTer15); c.49_50delinsAG, p.(Glu17Arg) (as a double hit) were found (2 cases). In addition, germinal and somatic mutation of PTEN c.1003C>T, p.(Arg335Ter); c.445C>T, p.(Gln149Ter), respectively, were detected. Gene fusions were absent in all cases. These prevalent molecular alterations converging on one major cancer-related pathway support the notion that SPA is a true neoplasm with a significant potential to develop intraluminal epithelial proliferation with apocrine and/or intercalated duct-like phenotype. The name SPA more correctly reflects the true neoplastic nature of this enigmatic lesion.

Lipoadenoma of the Parathyroid Gland: Characterization of an Institutional Series Spanning 28\xa0Years

Parathyroid lipoadenomas (PLAs) are rare tumors, and case descriptions are limited, < 80 to date. As a consequence, scarce information regarding the etiology of these enigmatic lesions is available. We searched our departmental pathology database for PLAs diagnosed between 1992 and 2020, reexamined the histology of each case, and recorded clinical parameters from the patients’ medical charts. As the diagnostic criteria of this lesion vary over this time period, we defined PLA as an enlarged parathyroid gland with > 50% fat on histologic examination with preoperative evidence of primary hyperparathyroidism (PHPT). A total of 8 bona fide PLA cases and 2 equivocal PLAs (close to 50% fat) were included. As approximately 4000 conventional parathyroid adenomas were diagnosed at our department during the same time interval, the prevalence of PLA was 0.20%. PLA patients were predominately female (63%) and presented with classical PHPT-related symptoms. Majority of cases were successfully located preoperatively and had an average tumor weight of 445 mg. Histologically, all PLAs consisted of > 50% mature adipose tissue, except a single case with brown fat. Of note, PLA patients exhibited a body mass index in line with PHPT patients in general, but a relatively high, near-significant prevalence of arterial hypertension was observed when compared to tumors with less fat (P = 0.0584). Future studies on this finding might be warranted. To summarize, we present one of the largest institutional PLA case series to date, and conclude that PLAs are rare, sporadic tumors mirroring many clinical aspects of conventional adenomas—with a potential coupling to hypertension worthy of follow-up studies.

Oral proliferative verrucous leukoplakia: An enigmatic lesion with emphasis on its differential diagnosis

Oral proliferative verrucous leukoplakia (OPVL) is a distinct clinical form of oral leukoplakia (OL). It is one of the rare clinicopathological entities that is considered as a potentially malignant oral mucosal disorder with an increased rate of progression to oral squamous cell carcinoma (OSCC) and a very high recurrence rate. Initially, the condition develops as focal clinical hyperkeratosis that progresses gradually and becomes a wide multifocal disease with gross exophytic features. It is a slow-growing, long-term progressive, and enigmatic lesion that is difficult to define and disclose. We report here, a case of OPVL in the right mandibular alveolar ridge, clinical and diagnostic aspects, differential diagnosis, and histologic features of this relatively rare entity.

Bone island and hand involvement - A short review

Bone island or enostosis is an enigmatic lesion affecting the skeleton system. As these anomalies have limited clinical implications owing to an asymptomatic course with most cases diagnosed incidentally, the exact etiopathogenesis is not clearly understood. A lot has been studied regarding radiological features and morphology despite scarce clinical cases and relevant studies. More research is warranted to provide in-depth details of these lesions that has potential to enlighten us with newer aspects of bone metabolism and properties. These lesions might just not be an aberration and may offer important clues about bone behavior and morphogenesis. Bone islands, when in large number, are associated with equally fascinating disorder called osteopoikilosis. This review, however, does not include osteopoikilosis on purpose. Hand involvement, although rarer, has been described and a short review is attempted to acknowledge that apart from relevant facts on diagnosis and management.

All things rhabdoid and SMARC: An enigmatic exploration with Dr. Louis P. Dehner

Over the past several decades, our understanding of malignant rhabdoid tumors (MRT) and the central nervous system equivalent atypical teratoid/rhabdoid tumor (ATRT) has undergone considerable refinement, particularly in terms of genetic characterization. MRT (both renal and extra-renal) and ATRT share phenotypic similarities and a common genetic signature, that being inactivating alterations of the SWI/SNF complex component SMARCB1 (or rarely SMARCA4). Unfortunately, a wide array of tumors bears significantly overlapping phenotypic characteristics to MRT/ATRT, posing a formidable diagnostic challenge. Likewise, the list of tumors bearing SMARC-related alterations has grown at a dizzying pace, and the original assumption that SMARCB1 alterations were unique to MRT/ATRT has been essentially negated. It should come as no surprise that Dr. Louis P. Dehner, no stranger to enigmatic lesions, participated significantly in this pathologic controversy, and the circuitous journey of entity discovery and clarification. This review aims to (1) summarize our current knowledge of MRT and ATRT with an emphasis on genetic characterization, (2) present insight into so-called "composite rhabdoid tumors" (CRTs), and (3) and provide an updated account of others tumors bearing SMARC alterations.

Expression of C-KIT, CD24, CD44s, and COX2 in benign and non-invasive apocrine lesions of the breast.

Benign apocrine metaplasia (AM) of the adult breast is a very common, but enigmatic lesion. It has been speculated that AM might be a precursor of malignancy or an indicator of a susceptibility of the breast tissue to develop neoplasia, mainly based on comparing the frequency of AM in breast cancer and non-breast cancer patients [1]. Studies using comparative genomic hybridization have supported this by showing similar molecular alterations in benign and malignant apocrine lesions [2]. Few studies, however, have compared expression of biomarkers involved in tumor progression in AM and progressively more advanced atypical apocrine lesions. The expression of C-KIT, COX2, CD24, and CD44s was evaluated by immunohistochemistry in formalin-fixed, paraffin-embedded material of 9 AM, 20 apocrine ductal intraepithelial neoplasia (DIN1c-3) and 40 atypical apocrine lesions (not qualifying for DIN1c-3) and compared to expression of the same biomarkers in adjacent normal ductal epithelium. Of the 66 apocrine lesions, 62 (94%) did not express C-KIT compared to 4/63 (6%) of the normal glands (Fisher's exact, p < 0.001). COX2 was expressed in a significantly higher proportion of apocrine lesions than of normal glands (49 vs. 14%, p < 0.001), and the number of apocrine lesions positive for CD24 was found to be higher with increasing aggressiveness of the lesions (Spearman, p < 0.001). In conclusion, benign and non-invasive proliferative apocrine lesions of the breast display immuno-phenotypical characteristics previously ascribed mainly to malignant transformation. This could lend support to the theory that AM is an early step towards malignant transformation, albeit associated with slow progression to carcinoma.

Surgical Neuropathology of Focal Epilepsies: Textbook and Atlas.

Surgical Neuropathology of Focal Epilepsies: Textbook and Atlas Ingmar Blumcke, Harvey B. Sarnat, and Roland Coras John Libbey Eurotext, Montrouge, France, 2015 157 pages; $99.50 (90.00 €) This is a concise and practical gui-de to the neuropathological diagnosis of epileptogenic lesions. This book would be a particularly valuable addition to the shelf of any neuropathologist faced with evaluation of these often enigmatic lesions. The 157 pages of text contain 70 high-quality figures. The first 81 pages comprise a textbook containing a brief introduction followed by 5 chapters add-ressing the major categories of epileptogenic …

Cholesterol Granuloma in Odontogenic Cyst: An Enigmatic Lesion.

Cholesterol granuloma (CG) is the outcome of the foreign body type of response to the accumulation of cholesterol crystals and is frequently present in conjunction with chronic middle ear diseases. Recently, cases of CG in jaws have been reported, but still, very few cases have been found of CG in dental literature. This article presents three rare cases of CG in the wall of odontogenic cysts emphasizing on its possible role in expansion of the associated lesion and bone erosion. It also lays stress on the fact that more cases of CG should be reported so that its nature and pathogenesis in the oral cavity become more perceivable.

An Ambiguous Asymptomatic Swelling in The Maxillary Region-A Case Report

Central giant cell granuloma (CGCG), formerly called giant cell reparative granuloma, is a nonneoplastic proliferative lesion of unknown etiology. It occurs most commonly in the mandible but can also occur in maxilla. The case reported here resembled a wide variety of conditions that led to a misdiagnosis both on clinical and radiographic examinations but was histopathologically diagnosed as CGCG. We describe a case of CGCG arising from the posterior maxilla to highlight the importance of histopathology in the diagnosis of this enigmatic lesion.

Exophytic oral verrucous hyperplasia: a new entity.

Exophytic oral verrucous hyperplasia (OVH) is a new entity described by an expert working group from South Asia. First reported in Taiwan, there are no reports so far from an Indian population. The aim was to use the microscopic features described by the expert group to differentiate OVH from other oral verruco-papillary lesions in an Indian archive. In a retrospective multicentre study, using pathology archives, 188 verruco-papillary lesions were retrieved from pathology archives. A proforma listing histopathological criteria for OVH based on published guidelines (Annals of Dentistry, University of Malaya, 2013) was used. Patients' demographic and clinical data were transcribed from patient charts. The Pearson chi-square test was used to determine associations between clinical and histopathological features. Of 188 oral verruco-papillary lesions that were evaluated, based on microscopic features the cases were reclassified as OVH (57), verrucous carcinoma (VC) (84), oral squamous cell carcinoma (16), and other verruco-papillary lesions (31). Both OVH (70%) and VC (60%) showed male predominance and commonly affected buccal mucosa (OVH 74% and VC 57%). Absence of downward growth of the hyperplastic epithelium into lamina propria when compared with the level of the basement membrane of the adjacent normal epithelium was a distinct feature in OVH. Keratin plugging, epithelial dysplasia and subepithelial lymphocytic infiltration were found to be significantly different (P<0.05) in OVH versus VC. The sample size of other verruco-papillary lesions was insufficient for statistical comparison. Apart from the absence of an endophytic growth pattern in OVH, we noted the presence of dysplasia in OVH. This significant observation does institute a debate as to whether this enigmatic lesion could possibly be a precedent of oral squamous or verrucous carcinoma. We propose OVH is a distinct entity in our Indian population and should be considered in the classification of oral potentially malignant disorders.

Minor and repetitive head injury.

Traumatic brain injury (TBI) is the leading cause of death and disability in the young, active population and expected to be the third leading cause of death in the whole world until 2020. The disease is frequently referred to as the silent epidemic, and many authors highlight the "unmet medical need" associated with TBI.The term traumatically evoked brain injury covers a heterogeneous group ranging from mild/minor/minimal to severe/non-salvageable damages. Severe TBI has long been recognized to be a major socioeconomical health-care issue as saving young lives and sometimes entirely restituting health with a timely intervention can indeed be extremely cost efficient.Recently it has been recognized that mild or minor TBI should be considered similarly important because of the magnitude of the patient population affected. Other reasons behind this recognition are the association of mild head injury with transient cognitive disturbances as well as long-term sequelae primarily linked to repeat (sport-related) injuries.The incidence of TBI in developed countries can be as high as 2-300/100,000 inhabitants; however, if we consider the injury pyramid, it turns out that severe and moderate TBI represents only 25-30 % of all cases, while the overwhelming majority of TBI cases consists of mild head injury. On top of that, or at the base of the pyramid, are the cases that never show up at the ER - the unreported injuries.Special attention is turned to mild TBI as in recent military conflicts it is recognized as "signature injury."This chapter aims to summarize the most important features of mild and repetitive traumatic brain injury providing definitions, stratifications, and triage options while also focusing on contemporary knowledge gathered by imaging and biomarker research.Mild traumatic brain injury is an enigmatic lesion; the classification, significance, and its consequences are all far less defined and explored than in more severe forms of brain injury.Understanding the pathobiology and pathomechanisms may aid a more targeted approach in triage as well as selection of cases with possible late complications while also identifying the target patient population where preventive measures and therapeutic tools should be applied in an attempt to avoid secondary brain injury and late complications.

Juvenile Ossifying Fibroma of Maxilla; An Uncommon Entity - A Case Report

In the group of confounding fibro-osseous lesions juvenile ossifying fibroma JOF is the most enigmatic lesion. It is the most uncommon clinical entity distinctly in craniofacial skeleton with unique histological presentation. Because of its characteristic early onset in children and young adolescent aggressive local behaviour and high recurrence rate it is important to make an early diagnosis apply the appropriate treatment and follow-up the patient over the long-term. Here we are reporting a case of Juvenile Ossifying Fibroma of left maxilla in 8-year old girl. Surgical excision of lesion was done with safe margins under general anaesthesia amp further regular follow up was done

Sacrococcygeal neoplastic lesions

Sacrococcygeal neoplastic lesions are very rare entities and therefore, a diagnostic and therapeutic challenge. They include developmental cysts, inflammatory, neurogenic, osseous and miscellaneous lesions. In this review, we discuss the incidence, symptoms, characteristics, diagnostic methods and treatment strategies of these enigmatic lesions. We have to highlight the high index of suspicion that is necessary when dealing with chronic sacrococcygeal pain or multiple perianal abscesses or persistent perianal drainage, because any misinterpretation may result in unfavorable outcomes.

Histiocyte-rich Xanthomatous Pseudotumor Mimicking Relapse on Positron Emission Tomography Imaging in an Adolescent With Primary Mediastinal Diffuse Large B-cell Lymphoma

Inflammatory pseudotumors (IPTs) are rare, enigmatic lesions that may develop as a late manifestation of a reparative process. We describe the case of a teenager with primary mediastinal subtype of diffuse large B-cell lymphoma who developed an IPT at the site of the original lymphoma, mimicking relapse of disease on positron emission tomography/computed tomography imaging. This is the first report of IPT in a teenager with mediastinal lymphoma. This case is an important reminder of the limitations of positron emission tomography/computed tomography imaging in patients with lymphoma and stresses the importance of histologic confirmation of suspected treatment failure or relapse.

Letter regarding Flecher et al. “Cancer on a pacemaker lead” — Is it MICE (Mesothelial Incidental Cardiac Excrescence)?

This article interested me. The authors describe a 3.5×3×4cm mass on a pacemaker lead [1]. The photomicroscopy images are very good and from them I wondered if this could be an example of a MICE (Mesothelial Incidental Cardiac Excrescence) [2]. These enigmatic lesions have been described and may display pleomorphism with mitotic activity. The cells in the photographs resemble mesothelial cells to me and these can be cytokeratin positive. This could explain the occurrence of this lesion in the absence of an underlying cancer.

Anterior mandibular swelling – A Case Report

Predilection of lesions to occur at certain specific sites is of great aid in arriving at a logical diagnosis. However tendency of lesions to appear at particular site does not follow a rule book. Enigmatic lesions like ameloblastomas have varied presentation. Here is an unusual case report of a patient who presented to us with an anterior mandibular swelling. Although clinical and radiographic features were suggestive of central giant cell granuloma, histopathological diagnosis was of ameloblastic carcinoma. Ameloblastomas are considered to be benign lesions; however, some can be reclassified as malignant when metastases occur or present with a very aggressive behavior. A detailed deliberation of differential diagnosis of anterior mandibular swellings is also done.Key words: ameloblastoma, anterior, carcinoma, giant cell granuloma, mandible