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This research aims to compare and assess the clinical and radiological presentations of tuberous sclerosis complex (TSC)-associated lymphangioleiomyomatosis (LAM) and sporadic LAM. A retrospective medical record review was conducted for 90 patients with confirmed LAM diagnoses. Radiologists who were blinded to the LAM type evaluated CT images of the chest and abdomen for the presence of four CT phenotypes: multiple sclerotic bone lesions (SBLs), multifocal micronodular pneumocyte hyperplasia (MMPH), hepatic fat-containing lesions, and cardiac fat-containing lesions. Statistical analyses were then completed to analyze the differences between TSC-LAM and sporadic LAM. Sporadic LAM patients reported a greater number of clinical symptoms at the time of diagnosis than TSC-LAM patients. All four CT phenotypes were present among the TSC-LAM patient population, whereas hepatic fat containing lesions were the only phenotype present in sporadic LAM patients evaluated in this study. The clinical and radiological presentations of sporadic LAM and TSC-LAM differ significantly, suggesting that the diagnostic criteria for sporadic LAM and/or TSC itself could be adapted accordingly. However, the similarities in the presentation of the LAM types are also important to note as these trends inform theories surrounding the potential underlying pathogenic mechanisms of sporadic LAM.

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BACKGROUND: Chromoblastomycosis (CBM) is a chronic infection of skin and subcutaneous tissue. CBM cases have been reported in local literature from Pakistan with heterogenous demographic, diagnostic and therapeutic information. The objective of this study is to share the experience of CBM from a large tertiary care hospital laboratory in Pakistan. METHOD: This was a retrospective observational study. Histopathology and microbiology data of suspected CBM between 2016 and 2022 was retrieved. Patients' demographics, site of involvement, histopathological findings and positive microbiology cultures were assessed. Literature search on Google Scholar, PubMed and PakMediNet was done between 1990 and 2023 with multiple terms. RESULT: A total of 16 CBM cases were identified; 14 were histopathology positive and two were both histopathology and culture positive. The median age was 21 years, and 11 patients were male. The predominant site was lower extremities followed by the face. Severe acanthosis, hyperkeratosis and granuloma with sclerotic bodies were observed in all histopathology slides. Alternaria spp. and Phialophora spp. were isolated from two culture-positive cases. A total of nine cases of CBM were reported from Pakistan in PubMed non-indexed journal. CONCLUSION: CBM is not a commonly thought of disease when evaluating skin lesions in Pakistan. A high index of suspicion when assessing patients who may have a history of trauma, exposure to soil and suggestive lesions is reasonable. An integrated approach between clinicians, histopathologist and microbiologist is required to do early identification and therapeutic interventions.

Asunto(s)

Cromoblastomicosis , Humanos , Cromoblastomicosis/microbiología , Cromoblastomicosis/diagnóstico , Cromoblastomicosis/patología , Cromoblastomicosis/epidemiología , Pakistán/epidemiología , Masculino , Estudios Retrospectivos , Femenino , Adulto , Adulto Joven , Adolescente , Centros de Atención Terciaria/estadística & datos numéricos , Piel/microbiología , Piel/patología , Persona de Mediana Edad , Niño , Antifúngicos/uso terapéutico

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BACKGROUND: Evaluation of glenoid bone loss following recurrent anterior shoulder dislocations is normally performed using cross sectional imaging. OBJECTIVES: To assess how anteroposterior (AP) and Bernageau view radiographs compare to computed tomography (CT), magnetic resonance imaging (MRI) and arthroscopy for evaluating glenoid bone loss in patients with recurrent anterior shoulder dislocation. MATERIALS AND METHODS: A prospective observational study was performed on 32 patients over two years at a tertiary orthopedic center. The loss of sclerotic glenoid rim (LSGL) on AP radiograph and the percentage relative glenoid bone loss on the Bernageau radiograph were assessed. The percentage glenoid bone loss and anterior straight line (ASL) were calculated using a best fit en face circle method using CT and MRI. Percentage glenoid bone loss was also calculated during arthroscopy in multiples of 5%. RESULTS: In our study, 90.6% (29) patients were males, while only 9.4% (3) were females. This can be attributed to the involvement of the males in outdoor activities and sports. Also, the maximum number of patients were found to belong to 21-30 years of age, with the mean age being 28.66 years. Of the 32 patients, loss of sclerotic glenoid line (LSGL) on AP radiographs correlated with glenoid bone loss on cross-sectional imaging in 27 patients. Three patients had equivocal LSGL and 2 patients with glenoid bone loss on CT did not demonstrate LSGL. The difference between the two modalities was not statistically significant (p value = 0.002). The glenoid bone loss on Bernageau view correlated with glenoid bone loss on cross sectional imaging in all but one patient. The bone loss as evaluated by radiograph Bernageau view was found to have strong correlation (correlation coefficient r = 0.948, p value < 0.0001). CONCLUSION: AP and Bernageau radiographic views for anterior shoulder dislocations demonstrate good correlation with glenoid bone loss on cross-sectional imaging. They may also be used as an adjunct to predict overall bone loss on CT and at arthroscopy.

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Castleman's disease is a rare lymphoproliferative disease that usually presents as a solitary mass in the mediastinal or cervical region. Castleman's disease can be usually of two types: unicentric type (which involves only one site of lymph nodes) and multicentric type (which involves multiple sites of lymph nodes). We report the case of a 26-year-old female with multiple sclerotic bone lesions in unicentric Castleman's disease. The definitive diagnosis was made by excisional biopsy with immunohistochemistry, 18F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) study, and MRI scan. This case report emphasizes the need for proper workup for systemic manifestations in unicentric Castleman's disease.

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Esclerosis Tuberosa , Humanos , Esclerosis Tuberosa/complicaciones , Esclerosis Tuberosa/diagnóstico , Femenino , Masculino , Osteosclerosis/diagnóstico por imagen , Osteosclerosis/etiología

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Vertebral body enhancement is occasionally seen on postcontrast CT imaging in the absence of osseous pathology. This enhancement can mimic sclerotic osseous metastatic disease, leading to a diagnostic dilemma for radiologists and increasing the chance of misinterpretation. Existing literature has focused on the association between this enhancement and concomitant central venous system obstruction. We report a 61-year-old woman with a history of nasopharyngeal carcinoma presenting with an epidural abscess who exhibited vertebral body enhancement resembling sclerotic metastatic disease without imaging evidence of central venous obstruction or vertebral osseous metastatic disease. Awareness of this unique presentation may prevent the incorrect diagnostic errors and their associated negative effects on patients.

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Superficial fibromas are a group of mesenchymal spindle cell lesions with pathomorphological heterogeneity and diverse molecular backgrounds. In part, they may be indicators of an underlying syndrome. Among the best-known entities of superficial fibromas is Gardner fibroma, a plaque-like benign tumor, which is associated with APC germline mutations and occurs in patients with familial adenomatosis polyposis (Gardner syndrome). Affected patients also have an increased risk to develop desmoid fibromatosis (DTF), a locally aggressive neoplasm of the deep soft tissue highly prone to local recurrences. Although a minority of DTFs occur in the syndromic context and harbor APC germline mutations, most frequently their underlying molecular aberration is a sporadic mutation in Exon 3 of the CTNNB1 gene. Up to date, a non-syndromic equivalent to Gardner fibroma carrying a CTNNB1 mutation has not been defined. Here, we present two cases of (sub-)cutaneous tumors with a hypocellular and collagen-rich Gardner fibroma-like appearance and pathogenic, somatic CTNNB1 mutations. We aim to differentiate these tumors from other fibromas according to their histological appearance, immunohistochemical staining profile and underlying somatic CTNNB1 mutations. Furthermore, we distinguish them from locally aggressive desmoid fibromatosis regarding their biological behavior, prognosis and indicated therapeutic strategies. Consequently, we call them CTNNB1-mutated superficial fibromas as a sporadic counterpart lesion to syndromic Gardner fibromas.

Asunto(s)

Fibroma , beta Catenina , Humanos , beta Catenina/genética , Fibroma/genética , Fibroma/patología , Masculino , Femenino , Mutación , Persona de Mediana Edad , Fibromatosis Agresiva/genética , Fibromatosis Agresiva/patología , Adulto , Síndrome de Gardner/genética , Síndrome de Gardner/patología , Mutación de Línea Germinal

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Storiform collagenoma, also known as sclerotic fibroma, is a relatively rare benign cutaneous tumor consisting of a proliferation of fibroblasts that shows increased production of type I collagen. It may appear as a solitary, sporadic lesion, or, especially when multiple, associated with Cowden syndrome. Giant cell collagenoma has a histopathologic appearance similar to that of storiform collagenoma with the addition of floret-type giant cells. Herein, we report the finding of multiple giant cell collagenomas arising in an individual with Cowden syndrome. In a review of the published literature, this histopathologic variant appears to be rarely observed in association with Cowden syndrome.

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Fibroma , Síndrome de Hamartoma Múltiple , Enfermedades de la Piel , Neoplasias Cutáneas , Humanos , Síndrome de Hamartoma Múltiple/complicaciones , Síndrome de Hamartoma Múltiple/patología , Enfermedades de la Piel/patología , Fibroma/patología , Neoplasias Cutáneas/patología , Células Gigantes/patología

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OBJECTIVE: To investigate the CT features of incidental rib enhancement (RE) and to summarize the CT characteristics for distinguishing the RE from sclerotic metastasis (SM) in patients with malignancies. MATERIAL AND METHODS: This retrospective observational study enrolled 79 patients with RE (involved 133 ribs) during October 2014 and December 2021. Another 53 patients with SM (160 SM) in the same period were selected randomly for comparison. The location, enhancement patterns of RE were reviewed. The CT values of RE regions and SM were measured and statistically analyzed. RESULTS: Most REs (70 patients, 88.6%) were in the 1st to 6th ribs. 50 patients had solitary RE and 29 with multiple REs in a regional distribution. All the REs were closely connected to the intercostal venous plexus (ICVP) ipsilateral to the injection site. No visible abnormalities on unenhanced scans were detected in all REs. One hundred and twenty REs (90.2%) had nodular/patchy enhancement. The CT value of RE regions in the venous phase was lower than that in the arterial phase (589.8 ± 344.2 HU versus 1188.5 ± 325.3 HU, p < 0.001). During the venous phase, most REs (125, 94.0%) shrank or disappeared. SM appeared similar on both contrast-enhanced and unenhanced scans in terms of shape and CT values. CONCLUSION: The RE demonstrated characteristic CT features. The manifestations of nodular/patchy enhancement in the arterial phase, decreased density and shrinkage or disappearance during the venous phase, and no abnormality on unenhanced scans, as well as a close connection with the ICVP, may help differentiate RE from SM.

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Neoplasias Óseas , Hallazgos Incidentales , Costillas , Tomografía Computarizada por Rayos X , Humanos , Femenino , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Costillas/diagnóstico por imagen , Diagnóstico Diferencial , Tomografía Computarizada por Rayos X/métodos , Anciano , Adulto , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/secundario , Estudios de Factibilidad , Medios de Contraste , Esclerosis/diagnóstico por imagen

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OBJECTIVES: Cowden syndrome (CS) is a multisystem disease with an elevated lifetime risk of internal malignancy. We aim to assess the role of PTEN immunostain as a screening test for CS in a variety of common CS-associated neoplasms, with a particular focus on cutaneous tumors. METHODS: We retrospectively searched for patients meeting criteria for CS and/or demonstrating germline PTEN mutation from 2008 to 2022. We then performed PTEN immunostains on tumors of these patients as well as control cases. RESULTS: Our study included 30 patients with CS who had a total of 25 CS-associated malignancies (13 thyroid, 8 breast, and 4 endometrial carcinomas). Specifically, there were 11 patients with biopsy-confirmed CS-associated cutaneous neoplasms, including 1 patient with multiple trichilemmomas and 3 with multiple sclerotic fibromas. In total, 45 CS-associated tumors (6 trichilemmomas, 7 sclerotic fibromas, 5 thyroid carcinomas, 18 adenomatous thyroid nodules, 6 breast carcinomas, and 3 endometrial carcinomas) and 31 non-CS cases (9 trichilemmomas, 5 sclerotic fibromas, 8 adenomatous thyroid nodules, and 3 thyroid, 3 breast, and 3 endometrial carcinomas) were available for PTEN immunohistochemical staining. PTEN expression was lost in 43 (96%) of 45 CS-associated lesions and retained in 30 (97%) of 31 sporadic tumors. The overall sensitivity and specificity of PTEN loss of expression as a screening test for CS were 96% and 97%, respectively. CONCLUSIONS: PTEN immunohistochemistry on CS-associated tumors, especially trichilemmomas, can serve as a readily accessible and cost-effective screening test for CS.

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Síndrome de Hamartoma Múltiple , Inmunohistoquímica , Fosfohidrolasa PTEN , Humanos , Síndrome de Hamartoma Múltiple/diagnóstico , Síndrome de Hamartoma Múltiple/patología , Síndrome de Hamartoma Múltiple/genética , Fosfohidrolasa PTEN/metabolismo , Fosfohidrolasa PTEN/genética , Femenino , Persona de Mediana Edad , Adulto , Estudios Retrospectivos , Masculino , Anciano , Biomarcadores de Tumor/metabolismo , Biomarcadores de Tumor/análisis , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/metabolismo , Neoplasias de la Tiroides/diagnóstico , Neoplasias de la Tiroides/patología , Neoplasias de la Tiroides/metabolismo , Neoplasias de la Tiroides/genética , Neoplasias de la Mama/diagnóstico , Neoplasias de la Mama/patología , Neoplasias de la Mama/metabolismo , Neoplasias de la Mama/genética

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INTRODUCTION: The aim of this case-control study was to examine the relationship between type 2 diabetes mellitus (DM) and the occurrence of VRFs. The crack extension, dentin sclerosis, and chemical characteristics of root dentin in teeth with VRF from patients with/without DM were also compared. METHODS: One hundred and thirty-two patients diagnosed with VRF in crowned root filled posterior teeth were selected. The study was conducted in 2 parts. In Part-1: The cases were matched with control teeth (1:1) for age (±5 years), sex, tooth type, apical extent of root filling, time period after root filling to a diagnosis of VRF, presence or absence of intracanal post and abutment status. The presence or absence of type 2 DM (HbA1c > 6.5) was recorded. In Part-2: The extracted teeth with VRF from the case control study were used to evaluate the extension of VRF, presence of sclerotic dentin and isthmus using a microscopic analysis; while the levels of pentosidine, collagen cross-linking ratio and mineral-collagen ratio were determined by Fourier-transform infrared spectroscopy. The distribution of DM between cases and controls was analyzed using Pearson Chi-Square test and Odds Ratio estimated. Chemical composition data was analyzed using Mann-Whitney test. The extent of sclerotic dentin was analyzed using Pearson Chi-Square test. RESULTS: When compared to patients without DM, patients with DM had 2.67 (95% CI: 1.6-4.45) folds higher odds for occurrence of VRF. Pentosidine (P = .014), collagen cross-linking ratio(P = .047), mineral-collagen ratio (P = .009) and sclerotic dentin extent (P = .0009) were significantly higher in patients with DM and VRF. CONCLUSIONS: Type 2 DM was more often associated with VRFs in root canal treated teeth with crowns. Root dentin from patients with type 2 DM and VRF had higher levels of pentosidine, collagen cross-linking ratio, mineral to collagen ratio and sclerotic dentin.

Asunto(s)

Dentina Secundaria , Diabetes Mellitus Tipo 2 , Fracturas de los Dientes , Humanos , Estudios de Casos y Controles , Raíz del Diente , Diabetes Mellitus Tipo 2/complicaciones , Fracturas de los Dientes/complicaciones , Fracturas de los Dientes/diagnóstico , Colágeno , Minerales

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The SP7 gene encodes a zinc finger transcription factor (Osterix), which is a member of the Sp subfamily of sequence-specific DNA-binding proteins, playing an important role in osteoblast differentiation and maturation. SP7 pathogenic variants have been described in association with different allelic disorders. Monoallelic or biallelic SP7 variants cause Osteogenesis imperfecta type XII (OI12), a very rare condition characterized by recurrent fractures, skeletal deformities, undertubulation of long bones, hearing loss, no dentinogenesis imperfecta, and white sclerae. Monoallelic or biallelic SP7 variants may also cause sclerotic skeletal dysplasias (SSD), partially overlapping with Juvenile Paget's disease and craniodiaphyseal dysplasia, characterized by skull hyperostosis, long bones sclerosis, large ribs and clavicles, and possible recurrent fractures. Here, we report the long-term follow-up of an 85-year-old woman presenting with a complex bone disorder including features of either OI12 (bone fragility with multiple fractures, severe deformities and short stature) or SSD (striking skull hyperostosis with optic atrophy, very large ribs and clavicles and long bones sclerosis). Exome sequencing showed previously undescribed biallelic loss of function variants in the SP7 gene: NM_001173467.2(SP7): c.359_362del, p.(Asp120Valfs*11); NM_001173467.2(SP7): c.1163_1174delinsT, p.(Pro388Leufs*33). RT-qPCR confirmed a severely reduced SP7 transcription compared to controls. Our report provides new insights into the clinical and molecular features and long-term outcome of SP7-related bone disorders (SP7-BD), suggesting a continuum phenotypic spectrum characterized by bone fragility, undertubulation of long bones, scoliosis, and very heterogeneous bone mineral density ranging from osteoporosis to osteosclerosis.

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Hiperostosis , Osteogénesis Imperfecta , Femenino , Humanos , Anciano de 80 o más Años , Estudios de Seguimiento , Esclerosis/patología , Osteogénesis Imperfecta/genética , Huesos/patología , Hiperostosis/patología

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Sclerotic fibroma (SF) is a rare subset of dermal fibromas that occurs sporadically or in association with Cowden syndrome (CS). We report a case of a patient with known CS and a solitary lesion on the scalp. Histologic examination demonstrated a well-circumscribed lesion with sclerotic dermis and a whorled collagen pattern, multinucleated giant cells, and dendritic spindle cells. Nuclear atypia or mitotic figures were not noted. The giant cells were negative for Melan-A, SOX-10, EMA, SOX-10, and factor XIIIa. These findings are consistent with a giant cell collagenoma (GCC). Despite possible overlap with SF, GCC has not been associated with CS. This makes our case unique and suggests that GCC should be included in the spectrum of CS-associated cutaneous lesions. The diagnosis of SF may lead to the identification of previously undiagnosed CS; accordingly, GCC, even when present as a solitary lesion, may indicate the need for further work-up and screening for CS.

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Fibroma , Síndrome de Hamartoma Múltiple , Nevo , Enfermedades de la Piel , Neoplasias Cutáneas , Humanos , Síndrome de Hamartoma Múltiple/diagnóstico , Neoplasias Cutáneas/patología , Fibroma/complicaciones , Fibroma/patología , Enfermedades de la Piel/patología , Células Gigantes/patología

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The WHO substantially redefined cemento-osseous dysplasia (COD) in 2017. The descriptions of COD in the 2005 and 2017 WHO classifications are quite different. In this study, we compared the difference in COD description between the 2005 and 2017 editions of the WHO classifications in detail. There are remarkable differences in the terminology, definition, synonyms, epidemiology, classifications, clinical features, radiation/pathology, prognosis, and predictive factors of COD between the two versions. At present, the surgical treatment of COD is less defined, and there is no clear guidance for the treatment of sclerotic bone. In this study, we extracted the affected teeth without removing the sclerotic bone when the bone absorption line can be only found between the root and the lesion, and we extracted the teeth as well as the lesion and curetted the granulation tissue when a bone absorption line could be seen between the lesion and the alveolar bone. According to our observation, the position of the bone absorption line can be used as a reference for the selection of sclerotic bone treatment. Sclerotic bone preservation did not increase its volume and density after tooth extraction. Sclerotic bone was composed of highly mineralized tissue with less blood vessels and cells. The position of the bone resorption line can be used as a basis for treatment selection. The high mineralization of the lesion may weaken its anti-infection ability.

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Background: Osteosclerotic metaphyseal dysplasia (OSMD, OMIM 615198) is an extremely rare autosomal recessive osteopetrosis disorder resulting in a distinctive pattern of osteosclerosis of the metaphyseal margins of long tubular bones. To date, only thirteen cases have been reported (eight molecularly confirmed). Five homozygous sequence variants in the leucine-rich repeat kinase 1 (LRRK1) gene have been identified to cause OSMD. We present two male siblings with OSMD with a novel LRRK1 variant. Cases: The index case, now aged 6 years, was referred aged 9 months when diffuse sclerosis of the ribs and vertebral bodies, suggestive of osteopetrosis, was incidentally identified on a chest radiograph for suspected lower respiratory tract infection. Parents were consanguineous and of Pakistani origin. Further evaluation revealed developmental delay, nystagmus with bilateral optic nerve hypoplasia and severe visual impairment. Skeletal survey confirmed typical changes of OSMD, with widespread diffuse sclerosis and Erlenmeyer flask deformity of long bones. His older sibling, now aged 12 years, was 7 years at the time of referral and had similar clinical course and skeletal findings. Additionally, he had a chronic progressive osteonecrosis of the left mandible that required debridement, debulking and long-term antibiotics. Skeletal survey revealed findings similar to his sibling. Neither sibling had significant skeletal fractures or seizures. Unlike most previous reports suggesting sparing of the skull and lack of visual impairment, our patients had evidence of osteosclerosis of the cranium. Genetic screening for the common autosomal recessive and dominant pathogenic variants of osteopetrosis was negative. Whole Exome Sequencing (WES) followed by Sanger sequencing, identified a novel homozygous LRRK1 c.2506C>T p. (Gln836Ter) nonsense variant predicted to result in premature truncation of LRRK1 transcript. Conclusion: Our cases confirm the autosomal recessive inheritance and expand the spectrum of genotype and phenotype of OSMD reported in the literature. Increasing reports of LRRK1 variants in this phenotype raise the question of whether LRRK1 should be included in targeted osteopetrosis panels. Bone histology in previous cases has shown this to be an osteoclast rich form of osteopetrosis raising the possibility that haematopoietic stem cell transplantation may be an appropriate treatment modality.

Asunto(s)

Osteopetrosis , Osteosclerosis , Humanos , Masculino , Mutación , Nervio Óptico , Osteopetrosis/complicaciones , Osteopetrosis/genética , Osteosclerosis/complicaciones , Osteosclerosis/genética , Osteosclerosis/diagnóstico , Proteínas Serina-Treonina Quinasas/genética , Costillas , Esclerosis , Trastornos de la Visión , Niño

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BACKGROUND: Patients with dysplastic bone diseases, including fibrous dysplasia (FD), represent a particular challenge for placement of dental implants. This is due to structural bony changes that may compromise the bone blood supply and plasticity, thus potentially affecting the process of osseointegration. This case report describes a novel approach for dental-implant-based rehabilitation of the posterior maxilla affected by craniofacial fibrous dysplasia (CFD), with 7 years of treatment follow-up. CASE PRESENTATION: A 35-year-old female patient was referred due to a suspected unidentified bone lesion affecting the left side of the maxilla. A clinical and radiographic diagnosis of fibrous dysplasia was confirmed through a wedge bone biopsy. Particulate bone substitute was packed into a box-shaped ostectomy area of the lesion in the affected maxillary alveolar ridge. This was followed by the placement of four implants 6 months post operation. The implants were successfully integrated, as confirmed by clinical examination over 7 years of follow up. CONCLUSION: this treatment approach may be considered as a predictable and efficient treatment modality for dental implant rehabilitation in patients with a variety of fibro-osseous lesions, including fibrous dysplasia, which affect the alveolar bone.

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Chromoblastomycosis (CBM) presenting with linear distribution and with underlying bony destruction is rare. Herein, we report such a presentation in a farmer who presented with ulcerated nodules over the right leg and swelling of the right foot. Potassium hydroxide (KOH) preparation and histopathological examination of biopsy from nodule revealed characteristic sclerotic bodies on Gomori methenamine silver and periodic acid Schiff stain (PAS), which confirmed the diagnosis of chromoblastomycosis. X-ray of right foot revealed osteolytic destruction of right third metatarsophalangeal joint. Work-up for systemic involvement did not reveal any involvement. He was placed on combination therapy of itraconazole and terbinafine and is under follow-up.

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In the fields of forensics, the identification of human remains is a recurrent problem. The estimated age at death is one of the copious criteria to be evaluated. In adult teeth, the height of the root dentin transparency is used to estimate age. However, in archaeological material, this phenomenon appears inconstant. The aim of this work was to observe the structural modifications of the sclerotic dentin in the teeth for different postmortem intervals. The study included two parts (retrospective and prospective study) with 21 human monoradicular teeth, from bodies donated to medical science with postmortem intervals (PMIs) of 0, 1, 2 and 5 years and archeological excavation. After inclusion based on resin, section and polishing, the samples were analyzed with a scanning electron microscope (SEM) JSM-7800F®, and the procedure was completed via a semiquantitative analysis of calcium and phosphorus using EDX microanalysis. The analysis showed the existence of tubular and chemical modifications of sclerotic dentin at different PMIs. Our SEM study allowed us to observe a difference in tubule aspects linked to an increased PMI: the loss of peritubular collar and the lumen obstruction of tubules with a hyperdense material. Microanalysis highlighted variations in phosphocalcic ratios among the different groups, especially in the pulp area and the canine. Our hypotheses that explain these differences are based on the postmortem modifications of the crystals of the mineral phase of sclerotic dentin under the influence of chemical and/or bacterial action.

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BACKGROUND: Marginal zone lymphoma (MZL) is an indolent non-Hodgkin B cell lymphoma with various architectural pattern including perifollicular, follicular colonization, nodular, micronodular, and diffuse patterns. A sclerotic variant has not been previously reported and represents a diagnostic pitfall. CASE SUMMARY: A 66-year-old male developed left upper extremity swelling. Chest computed tomography (CT) in September 2020 showed 14 cm mass in left axilla. Needle core biopsy of axillary lymph node showed sclerotic tissue with atypical B lymphoid infiltrate but was non-diagnostic. Excisional biopsy was performed for diagnosis and showed extensive fibrosis and minor component of infiltrating B cells. Flow cytometry showed a small population of CD5-, CD10-, kappa restricted B cells. Monoclonal immunoglobulin heavy chain and light chain gene rearrangement were identified. Upon being diagnosed with MZL, patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone and achieved complete remission by positron emission tomography/CT. CONCLUSION: This is an important case report because by morphology this case could have easily been overlooked as non-specific fibrosis with chronic inflammation representing a significant diagnostic pitfall. Moreover, this constitutes a new architectural pattern. While sclerotic lymphomas have rarely been described (often misdiagnosed as retroperitoneal fibrosis), we do not know of any cases describing this architectural presentation of MZL.