Epidemiological and histopathological characteristics of thyroid carcinoma in a Tunisian health care center.

Background: Thyroid carcinoma (TC) accounts for almost 0.5%-1% of total malignancies. Its incidence is increasing rapidly worldwide. Several studies have drawn up the epidemiological profile of TC and its clinical and pathological features. However, to date, no similar studies have been conducted in Tunisia. Aims: To establish an epidemiological profile of TC in a Tunisian health care institute and to analyze its clinical and histopathological characteristics in our institute. Materials and Methods: We present a retrospective study reviewing the cases of TC diagnosed in our institution in a 4-year period. Results: We collected a sample of 192 cases of TC. It consisted of 31 males and 161 females (83.8%) with a sex-ratio M/F of 0.19. The mean age was 46.4 years. Papillary thyroid carcinoma was the most frequent histological subtype. The multifocality rate was 33.8%. The mean size of TC was 2.2 ± 1.9 cm. 60.9% of TC were staged pT1 and 20.3% had nodal involvement. Papillary thyroid microcarcinomas were noted in 37.5% of cases. Conclusion: Our results were consistent with those of the literature. A high proportion of pT1 and pN0 tumors were noted in our series, suggesting that TC's diagnosis and management was performed at an early stage of the disease in our institution. In addition, our study enabled us to notice the impact of the Coronavirus disease 19 crisis on the management of TC in our institution. Further studies are needed to establish the epidemiological profile of TC in Tunisia and to assess its clinical and pathological features.

Pleomorphic adenoma of the parotid gland concomitant with tuberculosis infection: A case report and review of the literature.

Tuberculosis of the salivary glands and particularly of the parotid gland is a localization that remains rare even in endemic countries. The association of intra parotid tuberculosis with a benign tumor has been found only in rare cases in literature. A 50-year-old woman with a history of normal pressure hydrocephalus treated surgically, non-smoker, presented with a right parotid swelling progressively increasing in size for 3 years. Clinical examination revealed a 4 cm long, firm, mobile, painless parotid swelling without inflammatory signs and without accessory lymphadenopathy. The oropharyngeal examination was without abnormalities. Ultrasound showed a mass of 31 × 27 mm suggesting a pleomorphic adenoma. MRI confirmed the suspicion of a pleomorphic adenoma of both lobes. The patient underwent a conservative total parotidectomy. The extemporaneous examination was in favor of a pleomorphic adenoma while the final pathology showed the coexistence of active tuberculosis lesions. The patient was put on long-term antituberculosis treatment with good clinical evolution. The clinical presentation of parotid tuberculosis is nonspecific mimicking any other tumor and the diagnosis can only be made by histological examination. Therapeutic management is based on long-term antituberculosis treatment.

A rare coexistence of sarcoidosis with overlap syndrome and sarcoidosis with primary biliary cholangitis and Sjogren's syndrome: Two distinct case reports.

Sarcoidosis and the overlap syndrome of autoimmune hepatitis and primary biliary cholangitis (PBC) share common clinical, biological, and histological features. The simultaneous occurrence of these diseases have been reported in few cases and suggests that a common pathway which may contribute to granuloma formation in both conditions. We report the cases of two female patients having an association of sarcoidosis and inflammatory liver diseases. The first case is of a 61-year-old woman had been monitored for an overlap syndrome of PBC and autoimmune hepatitis (AIH). Therefore, treatment with azathiprine has been initiated associated with ursodeoxycholic acid (UDCA). Azathioprine had to be discontinued due to digestive intolerance, specifically chronic diarrhea and abdominal pain. The patient remained clinically stable on UDCA and her liver function tests were stable for years, until she developed symptoms of progressive dyspnea without any other associated signs. Chest computed tomography (CT) revealed mediastinal enlargement, bilateral pulmonary nodules, and symmetrical adenomegalies in the mediastinum. The bronchoalveolar lavage (BAL) revealed increased cellularity, with a notable elevation in lymphocyte count (48 %) and a CD4/CD8 ratio of 4. The patient underwent mediastinoscopy; a biopsy of the right laterotracheal (4R) adenomegaly was performed. Histological examination of the lymph node showed epithelioid and giant-cell tuberculoid lymphadenitis without necrosis, compatible with sarcoidosis. Ophthalmological and cardiac assessments were normal. Plethysmography test was normal and there was no need for corticosteroid treatment; a surveillance was planned. Treatment with UDCA was pursued. The second case is of a 50-year-old woman with no medical history presented symptoms including dry eyes and mouth, inflammatory-type polyarthralgia affecting knees and wrists, bilateral Raynaud's phenomenon, right hypochondrium pain, and worsening dyspnea over six months. Liver analysis showed elevation of alkaline phosphatase (ALP) to three times upper limit of normal (ULN) and gamma-glutamyltransferase (GGT) to 5 times ULN. This cholestasis was associated with an increase in transaminase activity to 5 times ULN for over six months. Immunological tests revealed positive anti-nuclear antibodies (ANA), anti-Ro52, anti-M23E, and anti-centromere antibodies. Chest-CT showed multiple bilateral bronchiolar parenchymal micronodules mostly in the upper and posterior regions without any mediastinal adenomegaly. Bronchial endoscopy was normal, and biopsies indicated chronic inflammation. The BAL revealed increased cellularity, characterized by a high lymphocyte count (51.7 %) and a CD4/CD8 ratio of 2.8. Biopsy of minor salivary gland revealed grade 4 lymphocytic sialadenitis. Skin biopsy revealed an epithelioid granuloma without caseous necrosis. Liver biopsy performed in the presence of cytolysis and moderate hepatic insufficiency, revealed granulomatous hepatitis and cholangitis lesions along with septal fibrosis suggestive of PBC. The diagnosis of cutaneous and pulmonary sarcoidosis with PBC and Sjögren's syndrome was retained. The spirometry and diffusing capacity for carbon monoxide value were normal. Treatment involved UDCA, corticosteroids, and azathioprine, leading to clinical and biological improvement. Sarcoidosis shares some clinical manifestations with autoimmune liver diseases, primarily PBC. A hepatic granuloma with a different appearance and location can guide the diagnosis. Early diagnosis and appropriate management can avoid serious complications and improve prognosis.

A dataset of tumour-infiltrating lymphocytes in colorectal cancer patients using limited resources.

In the realm of cancer research, specifically focusing on colorectal carcinomas (CRCs), a novel diagnostic test referred to as 'Immunoscore' (IS) has emerged. This test relies on assessing the density of tumour-infiltrating lymphocytes, specifically CD3 and CD8, in both the centre of the tumour (CT) and its invasive margin (IM). IS holds promise as a potential prognostic factor. A retrospective descriptive study was conducted within the Pathology Department of Habib Thameur Hospital in Tunis, Tunisia. The study's aim was to evaluate the prognostic efficacy of IS for patients with CRC by means of a comprehensive survival analysis. This publication introduces the immunoscore in colorectal cancer (ISCRC) dataset, which was meticulously compiled during the aforementioned study. The ISCRC dataset comprises digital slide images sourced from biopsies of 104 patients diagnosed with CRC. Using the tissue microarray technique, an immunohistochemical investigation involving anti-CD3 and anti-CD8 markers was performed in regions designated as 'Hot Spots' within the CT and IM. The images were captured using a smartphone camera. Each marker's percentage presence within its respective region was quantified. The IS was estimated utilizing a semi-quantitative method. The ISCRC dataset encompasses anonymized personal data, along with macroscopic and microscopic attributes. The captured images, acquired through manual efforts using smartphones, stand as a valuable asset for the advancement of predictive algorithms Importantly, the potential applications of these models extend beyond mere prediction capabilities. They lay the groundwork for innovative mobile applications that could potentially revolutionize the practices of pathologists, particularly in healthcare settings constrained by resources and the absence of specialized scanning equipment. Database URL: https://figshare.com/s/5b4fa3e58c247a4851d4.

Exceptional outcome in cutaneous myeloid sarcoma.

Recognition of cutaneous myeloid sarcoma is important for all dermatologists to avoid further progression to acute myeloid leukemia. Nevertheless, we highlight the presence of a favorable clinical outcome in some patients with spontaneous regression.

EMERGENCY SURGERY FOR OBSTRUCTING COLON CANCER: MORBIDITY AND RISK FACTORS OF EARLY POSTOPERATIVE MORTALITY - A COHORT STUDY OF 118 CASES.

BACKGROUND: Occlusion is the most common complication of colon cancer. Surgical treatment is associated with the highest morbidity and mortality rate (10-27%) and has the worst prognosis. It is necessary for immediate management, avoiding colic perforation and peritonitis. The increase in mortality in emergency colon cancer surgery is multifactorial. AIMS: The aim of this study was to identify the risk factors for early postoperative mortality that highlights the therapeutic strategy in the management of obstructive colon cancer. METHODS: A retrospective study was performed on patients admitted from 2008 to 2020 at the Department of General Surgery due to obstructive colon cancer and operated on as an emergency (within 24 h of admission). RESULTS: In all, 118 patients with colon cancer were operated, and the early postoperative mortality was 10.2%. The univariate analysis highlighted that the American Society of Anesthesiology score III or IV, perforation tumor, one postoperative complication, and two simultaneous postoperative complications were considered significant risk factors for early postoperative mortality after emergent surgery. Multivariate analysis showed that only tumor perforation and the occurrence of two postoperative complications were significant risk factors. CONCLUSION: This study showed that postoperative complication is the leading cause of early postoperative mortality after emergency surgery for obstructive colon cancer. Optimizing the postoperative management of these higher risk patients is still necessary and may reduce the mortality rate.

Hemangioma of the umbilical cord: A case report on a rare entity.

Several hundred cases of placental hemangiomas have been reported in the literature. However, the umbilical cord is extremely uncommon as a site of occurrence. We present a case of postnatal discovery of giant hemangioma of the umbilical cord (HUM) in a Coronavirus Disease 2019 (COVID 19) positive mother. To our knowledge, this is the first reported case of HUM synchronous to a maternal infection with COVID 19. We aim, through this case and a review of the literature, to study the clinicopathological characteristics of this singular entity. Our patient, a 37-year-old woman, presented to the Department Of Obstetrics And Gynecology for respiratory distress and loss of fetal movements. Ultrasound examination concluded to intrauterine fetal desmise. After stabilization of the patient, a cesarean section was performed. A macerated fetus was extracted. Placenta showed a giant mass attached to the cord. It was submitted for pathological examination. Gross examination showed that the umbilical cord was inserted eccentrically with a fusiform dilation. Near its placental end, three cohesive solid angiomatous nodules were noted. Microscopic examination revealed lobules of dilated blood-filled capillaries set in a myxoid stroma. The diagnosis of HUM have been established. HUM arise from endothelial cells of the umbilical vessels. Their etiology, physio-pathology and pathways of tumorigenesis are not yet well defined. Further studies are needed to explore the pathways of tumorigenesis and to determin the implication of COVID-19 in HUM.

An unusual presentation of celiac disease in adult patient.

Pyoderma gangrenosum is among the exceptional extra-intestinal manifestations of celiac disease. We report a case of a 52-year-old patient who presented with pyoderma gangrenosum that turned out to be the initial presentation of celiac disease.

Incidental discovery of a Hodgkin lymphoma synchronous to a papillary thyroid carcinoma.

Papillary thyroid carcinoma is the most common type of thyroid cancer and accounts for almost 89.4% of all thyroid carcinomas. Hodgkin lymphoma is a heterogeneous group of neoplasms and represents 10% of lymphomas. These two cancers do not share the same risk factors. Some studies have reported the association of thyroid papillary carcinoma with lymphomas, mainly Hodgkin's lymphoma, treated with radiotherapy. However, to our knowledge less than 10 cases have illustrated synchronous papillary thyroid carcinoma and Hodgkin lymphoma with no history of radiotherapy. We present the case of a 49-year-old female patient, with no history of past exposure to radiation, who was incidentally diagnosed with Hodgkin lymphoma during the work up for papillary thyroid carcinoma. Our patient had total thyroïdectomy with cervical lymphadenectomy. The histopathologic examination concluded to a papillary thyroid carcinoma of classical variant. And the lymph node dissection enabled us to diagnose not only papillary thyroid carcinoma's lymph node metastasis, but also Hodgkin Lymphoma. This discovery of the Hodgkin lymphoma was totally incidental. The discovery of synchronous tumors in patients with papillary thyroid carcinoma has been reported in the literature. However, the diagnosis of Hodgkin through lymph node dissection for papillary thyroid carcinoma is extremely rare. This underlines the singularity and the importance of our case. The synchronous papillary thyroid carcinoma and Hodgkin lymphoma is a rare condition, which may pose significant diagnostic and treatment dilemmas. To date, there is no standardized approach due to lack of experience. The molecular mechanisms of this link are poorly understood and yet remain to be elucidated.

Splenogonadal fusion: A case series of two challenging diagnoses.

Splenogonadal fusion is an abnormal connection between the spleen and gonads. This rare entity can be easily confused with testicular tumors. It usually accompanies congenital malformations, such as cryptorchidism, making diagnosis more difficult. Surgeons must be aware of this entity to avoid unnecessary orchiectomy. In some cases, biopsy may help with diagnosis.

Comparison between signet-ring cell carcinoma and non-signet-ring cell carcinoma of the stomach: clinicopathological parameters, epidemiological data, outcome, and prognosis-a cohort study of 123 patients from a non-endemic country.

BACKGROUND: Signet-ring cell carcinoma of the stomach (SRCC) is a particular gastric cancer entity. Its incidence is increasing. Its diagnosis is pathological; it corresponds to adenocarcinoma with a majority of signet-ring cells component (> 50%). These histological features give it its aggressiveness characteristics. This has repercussions on the prognostic level and implications for the alternatives of therapy, especially since some authors suggest a potential chemoresistance. This survey aimed to identify the epidemiological, pathological, therapeutic, and prognostic characteristics of SRCC as a separate disease entity. METHODS: This was a retrospective study of 123 patients admitted for gastric adenocarcinoma to Habib Thameur Hospital in Tunis over 11 years from January 2006 to December 2016. A comparative study was performed between 2 groups: the SRCC group with 62 patients and the non-SRCC (non-signet-ring cell carcinoma of the stomach) with 61 patients. RESULTS: The prevalence of SRCC in our series was 50%. SRCC affected significantly younger patients (55 vs 62 years; p = 0.004). The infiltrative character was more common in SRCC tumors (30.6 vs 14.8%; p = 0.060), whereas the budding character was more often noted in non-SRCC tumors (78.7 vs 58.1%; p = 0.039). There was no significant difference in tumor localization between both groups. Linitis plastica was noted in 14 patients with SRCC against a single patient with non-SRCC (p = 0.001). The tumor size was more important in the non-SRCC group (6.84 vs 6.39 cm; p = 0.551). Peritoneal carcinomatosis was noted in 4.3% of cases in the SRCC group versus 2.2% of cases in the NSRCC group (p = 0.570). Total gastrectomy was more often performed in the SRCC group (87 vs 56%; p = 0.001). Resection was more often curative in the non-SRCC group (84.4 vs 78.3%; p = 0.063). Postoperative chemotherapy was more commonly indicated in the SRCC group (67.4 vs 53.3%; p = 0.339). Tumor recurrence was more common in the non-SRCC group (35.7 vs 32%; p = 0.776). The most common type of recurrence was peritoneal carcinomatosis in the SRCC group (62.5%) and hepatic metastasis in the non-SRCC group (60%; p = 0.096). The overall 5-year survival in the SRCC group was lower than in the non-SRCC group, with no statistically significant difference (47.1 vs 51.5%; p = 0.715). The overall survival was more important for SRCC in early cancer (100 vs 80%; p = 0.408), whereas it was higher for non-SRCC in advanced cancer (48.1 vs 41.9%; p = 0.635). CONCLUSION: Apart from its epidemiological and pathological features, SRCC seems to have a worse prognosis. Indeed, it is diagnosed at a more advanced stage and has a worse prognosis in advanced cancer than non-SRCC. It is therefore to be considered as a particular entity of gastric adenocarcinoma requiring a specific therapeutic protocol where the place of chemotherapy remains to be more investigated.

Breast rescue after necrotizing fasciitis.

Necrotizing fasciitis is a dangerous and rapidly spreading infection of soft tissue involving skin, subcutaneous tissue and fascia; muscles can be concerned but often omitted. It's considered as emergency due to its fulminant nature. The necrotizing fasciitis of the breast is exceptional. Management is based on surgical debridement and, in the case of breast, mastectomy in most cases is inevitable. We describe a case-report of breast necrotizing fasciitis with prompt management and with satisfactory cosmetic result owing to dermal autograft.

Emergency surgery for obstructing colon cancer: morbidity and risk factors of early postoperative mortality - a cohort study of 118 cases / Cirurgia de emergência para obstrução do câncer do cólon: morbidade e fatores de risco de mortalidade pós-operatória precoce - estudo de coorte de 118 casos

ABSTRACT BACKGROUND: Occlusion is the most common complication of colon cancer. Surgical treatment is associated with the highest morbidity and mortality rate (10-27%) and has the worst prognosis. It is necessary for immediate management, avoiding colic perforation and peritonitis. The increase in mortality in emergency colon cancer surgery is multifactorial. AIMS: The aim of this study was to identify the risk factors for early postoperative mortality that highlights the therapeutic strategy in the management of obstructive colon cancer. METHODS: A retrospective study was performed on patients admitted from 2008 to 2020 at the Department of General Surgery due to obstructive colon cancer and operated on as an emergency (within 24 h of admission). RESULTS: In all, 118 patients with colon cancer were operated, and the early postoperative mortality was 10.2%. The univariate analysis highlighted that the American Society of Anesthesiology score III or IV, perforation tumor, one postoperative complication, and two simultaneous postoperative complications were considered significant risk factors for early postoperative mortality after emergent surgery. Multivariate analysis showed that only tumor perforation and the occurrence of two postoperative complications were significant risk factors. CONCLUSION: This study showed that postoperative complication is the leading cause of early postoperative mortality after emergency surgery for obstructive colon cancer. Optimizing the postoperative management of these higher risk patients is still necessary and may reduce the mortality rate.

RESUMO RACIONAL: A oclusão é a complicação mais comum do câncer de cólon. A cirurgia está associada à elevada morbimortalidade (10-27%) e pior prognóstico. É necessário indicação imediata, evitando perfuração cólica e peritonite. O aumento da mortalidade na cirurgia de emergência do câncer de cólon é multifatorial. OBJETIVOS: Identificar os fatores de risco de mortalidade pós-operatória precoce que levaram a destacar a estratégia terapêutica no manejo do câncer de cólon obstrutivo. MÉTODOS: Estudo retrospectivo em pacientes admitidos no Departamento de Cirurgia Geral, entre 2008 e 2020, por câncer de cólon obstrutivo e operados de emergência (dentro de 24 horas da admissão). RESULTADOS: Foram operados 118 pacientes e a mortalidade pós-operatória precoce foi de 10,2%. A análise univariada destacou que escore American Society of Anesthesiology III ou IV, tumor perfurado, uma complicação pós-operatória e duas complicações pós-operatórias simultâneas foram considerados fatores de risco significativos de mortalidade pós-operatória precoce após cirurgia de emergência no câncer de cólon obstrutivo. Na análise multivariada, apenas a perfuração tumoral e a ocorrência de duas complicações médicas pós-operatórias foram fatores de risco significativos. CONCLUSÃO: Este estudo mostrou que a complicação pós-operatória é a principal causa de mortalidade pós-operatória precoce após cirurgia de emergência de câncer de cólon obstrutivo. Otimizar o manejo pós-operatório desses pacientes de alto risco ainda é necessário e pode reduzir a taxa de mortalidade.

Expanding the clinical phenotype associated with NIPAL4 mutation: Study of a Tunisian consanguineous family with erythrokeratodermia variabilis-Like Autosomal Recessive Congenital Ichthyosis.

Erythrokeratodermia variabilis (EKV) is a rare disorder of cornification usually associated with dominant mutations in the GJB3 and GJB4 genes encoding connexins (Cx)31 and 30.3. Genetic heterogeneity of EKV has already been suggested. We investigated at the clinical and genetic level a consanguineous Tunisian family with 2 sisters presenting an autosomal recessive form of EKV to better characterize this disease. Mutational analysis initially screened the connexin genes and Whole-exome sequencing (WES) was performed to identify the molecular aetiology of the particular EKV phenotype in the proband. Migratory shaped erythematous areas are the initial presenting sign followed by relatively stable hyperkeratotic plaques are the two predominates characteristics in both patients. However, remarkable variability of morphological and dominating features of the disease were observed between patients. In particular, the younger sister (proband) exhibited ichthyosiform-like appearance suggesting Autosomal Recessive Congenital Ichthyosis (ARCI) condition. No causative mutations were detected in the GJB3 and GJB4 genes. WES results revealed a novel missense homozygous mutation in NIPAL4 gene (c.835C>G, p.Pro279Ala) in both patients. This variant is predicted to be likely pathogenic. In addition, in silico analysis of the mutated 3D domain structure predicted that this variant would result in NIPA4 protein destabilization and Mg2+ transport perturbation, pointing out the potential role of NIPAL4 gene in the development and maintenance of the barrier function of the epidermis. Taken togheter, these results expand the clinical phenotype associated with NIPAL4 mutation and reinforce our hypothesis of NIPAL4 as the main candidate gene for the EKV-like ARCI phenotype.

Pedunculated hepatic focal nodular hyperplasia: A case report and review of the literature.

Focal nodular hyperplasia (FNH) is a common asymptomatic benign hepatic tumor encountered in middle-aged women. However, pedunculated FNH is exceedingly rare and more frequently associated with complications. That is why surgical management is mandatory in this form.

SMARCA4-deficient thoracic sarcoma revealed by metastasis to the small intestine: a diagnostic dilemma.

SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a recently identified aggressive subtype of sarcoma. We present the case of a 44-year-old man who underwent a surgery for a perforated small intestine. Compued tomography scan revealed a tissular mediastino-pulmonary mass.Histopathological examination of the intestinal mass shown a malignant tumour with a typical epithelioid and rhabdoid cells, numerous mitoses and large necrosis. A large panel of immunohistochemistry revealed loss of SMARCA4 and SMARCA2 and allowed the diagnosis of SMARCA4-DTS. It is important to consider SMARCA4-deficient thoracic sarcoma in the differential diagnosis of tumours showing suggestive morphologic features in patients of all ages, especially in the case of metastasis associated with thoracic mass.

A pseudotumoral form of Crohn's disease: A case report and review of the literature.

In front of a colonic tumor, the diagnosis of a pseudotumoral form of Crohn's disease must be considered. However, it is a rare form, especially when inaugural and a neoplasia must be eliminated before retaining the diagnosis.

Primary and isolated cutaneous precursor B-lymphoblastic lymphoma in an infant.

Cutaneous lesions may represent the initial sign of various neoplasms in children, including histiocytosis, neuroblastomas, and lymphomas. Primary cutaneous lymphoblastic lymphomas are rare and involve mostly the B-cell phenotype. Herein, we report a case of isolated, primary B-lymphoblastic lymphoma in a 7-month-old infant and emphasize the importance of early diagnosis.