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INTRODUCTION: Hemangiomas of the small intestine are rare and usually present in young people. They are very difficult to diagnose preoperatively. We report a rare case of mixed intestinal hemangioma (IH) causing intussusception in a pediatric patient. CASE PRESENTATION: A 3-month-old girl, with no prior medical or surgical history, was admitted with rectal bleeding and paroxysmal crying due to intermittent abdominal pain. An urgent abdominal ultrasound revealed ileo-ileal intussusception. Operative findings confirmed the intussusception, and a segmental resection of the intussuscepted jejunum was performed. Histopathological examination found a mixed hemangioma. The postoperative course was uneventful. DISCUSSION: Clinical presentation may include intestinal bleeding leading to anemia, obstruction, intussusception and perforation. Intussusception caused by small bowel hemangioma is extremely rare. Notably, we didn't find any cases of small bowel hemangioma revealed by intussusception in children. The main treatment for hemangiomas is surgical resection of the affected segment. No evidence in the literature on postoperative recurrence of hemangiomas. CONCLUSION: Intussusception secondary to intestinal hemangiomas is extremely rare. Preoperative diagnosis is challenging as they are often undetectable with traditional techniques. Enhanced awareness and understanding of this condition can facilitate earlier diagnosis and improve management outcomes.
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Adenocarcinoma , Neoplasias da Vesícula Biliar , Humanos , Neoplasias da Vesícula Biliar/diagnóstico por imagem , Neoplasias da Vesícula Biliar/patologia , Neoplasias da Vesícula Biliar/cirurgia , Adenocarcinoma/patologia , Adenocarcinoma/diagnóstico por imagem , Feminino , Masculino , Pessoa de Meia-Idade , Idoso , Células Caliciformes/patologiaRESUMO
INTRODUCTION AND IMPORTANCE: Large cell neuroendocrine carcinomas of the colon (LCNECC) are exceptionally rare, comprising only 0.2 % of all colonic carcinomas. Their diagnosis poses a significant challenge due to their propensity to mimic colonic adenocarcinomas. Typically diagnosed at advanced stages, LCNECCs carry a grim prognosis. Herein, we present a rare case of LCNECC and aim to elucidate its clinico-pathological characteristics. CASE PRESENTATION: A 56-year-old female patient presented with complaints of constipation, abdominal pain, and weight loss. On physical examination, a sizable mass was palpable in the right flank. Colonoscopy revealed a polyp in the descending colon and a friable multinodular stenosing mass in the ascending colon. Microscopic examination of the biopsy from the ascending colon mass exhibited a poorly differentiated large cell carcinomatous proliferation with positivity for synaptophysin and CD56, along with a Ki-67 proliferation index of 50 %. The polyp in the descending colon was consistent with a low-grade dysplastic tubular adenoma. A diagnosis of LCNECC with synchronous low-grade dysplastic tubular adenoma was established. A right hemicoloctomy was performed. Final pathological examination confirmed LCNECC invading the muscularis propria, with lymph node metastases. The tumor was classified as pT2N1M0 (Stage III). CLINICAL DISCUSSION: LCNECCs often mimic adenocarcinomas clinically, endoscopically, and radiologically. Pathological examination is the key for diagnosis. An immunohistochemical study using neuroendocrine markers is imperative to prevent overlooking the diagnosis of LCNECC. CONCLUSION: LCNECCs represent rare aggressive carcinomas. Their diagnosis might be challenging. A better knowledge of this rare entities would enable early diagnosis.
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Sino-nasal respiratory epithelial adenomatoid hamartomas (REAHs) are rare entity. They are benign tumors with excellent results after complete excision. We report a case of a 57-year-old male with a history of endoscopic surgery for right nasal polyps 20 years ago. The patient presented nasal obstruction that persisted for 10 years without anosmia nor epistaxis. Nasal endoscopy found a tissular mass filling the right nasal cavity extending to the nasopharynx. CT scan and MRI demonstrated soft tissue opacification of the right maxillary sinus and the homolateral anterior ethmoid cells with extension to the nasal cavity. The suspected diagnosis on imaging was an Inverted papilloma with a wide implantation base on the posterior part of the nasal septum. No endocranial or orbital extension was noted. The patient underwent endoscopic sinus surgery with complete extirpation of the tumor and a right ethmoidectomy. Histopathological assessment showed features consistent with REAH. No recurrence was noted at 1 year follow-up.
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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.
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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.
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INTRODUCTION AND IMPORTANCE: Locally advanced jejunal stromal tumors stand as a captivating and relatively rare entity, garnering attention for several reasons. Their inaccessible location by conventional endoscopy poses a diagnostic challenge. Further, treatment decisions necessitate a multidisciplinary approach, compounded by the absence of high-level evidence studies. CASE PRESENTATION: A 54-year-old patient was admitted to our surgical department with abdominal pain and chronic anemia. Abdominal CT imaging confirmed the presence of a non-metastatic sizable jejunal tumor. The patient underwent laparotomy, revealing a locally advanced jejunal tumor contracting the ileum and the ascending colon. A monobloc oncological resection was performed, followed by the restoration of digestive continuity. Anatomopathological analysis delineated a locally advanced Stromal Tumor with a high risk of recurrence. The patient underwent a course of tyrosine kinase inhibitors for 3 years, with no reported recurrence during the subsequent 3-year follow-up. DISCUSSION: Locally advanced jejunal stromal tumors are rare. Most patients present with unspecific symptoms. Diagnosis remains challenging due to their intricate anatomical location. Decisions regarding management must be deliberated within a multidisciplinary framework, tailored to each patient's unique characteristics. While combined therapeutic modalities have demonstrated efficacy in recent studies, prudence is advised given the heightened incidence of both short and long-term complications. CONCLUSION: In the absence of randomized controlled trials, the management of locally advanced jejunal stromal tumors underscores the imperative of multidisciplinary collaboration in treatment deliberations. A wide, sometimes mutilating excision is only permissible if it is complete.
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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.
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Neoplasias Colorretais , Linfócitos do Interstício Tumoral , Humanos , Linfócitos do Interstício Tumoral/patologia , Neoplasias Colorretais/genética , Neoplasias Colorretais/patologia , Estudos Retrospectivos , Análise de SobrevidaRESUMO
Pelvic leiomyoma in male patients is a very rare occurrence and its association with prostate cancer is a unique clinical situation. This study reports a new case of a 9 cm pelvic Leiomyoma associated with intermediate-risk localized prostate cancer in a 68-year-old patient. The leiomyoma was discovered fortuitously after an MRI was performed in the context of prostate cancer work-up. Surgical excision of the pelvic mass along with a radical prostatectomy were performed and the diagnosis of leiomyoma was confirmed by histopathology.
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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.
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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.
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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.
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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.
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Primary squamous cell carcinoma of the stomach is a rare type of gastric malignancies. Diagnosis criteria are well defined but diagnosis is generally late being made at an advanced stage with metastases explaining its poor diagnosis.
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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.
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Sarcoma , Neoplasias Torácicas , Adulto , Biomarcadores Tumorais/genética , DNA Helicases , Humanos , Imuno-Histoquímica , Intestino Delgado/cirurgia , Masculino , Proteínas Nucleares/genética , Sarcoma/diagnóstico , Sarcoma/genética , Sarcoma/cirurgia , Neoplasias Torácicas/diagnóstico , Neoplasias Torácicas/genética , Neoplasias Torácicas/cirurgia , Fatores de Transcrição/genéticaRESUMO
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.
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Linfoma de Células B , Leucemia-Linfoma Linfoblástico de Células Precursoras , Neoplasias Cutâneas , Criança , Humanos , Lactente , Pele , Neoplasias Cutâneas/diagnósticoRESUMO
Gastric lipoma should be considered as a rare differential diagnosis in patients presenting with gastrointestinal bleeding. Diagnosis is mainly done by radiologic and endoscopic findings. It presents characteristic pathognomonic endoscopic signs.
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BACKGROUND: While the incidence of gastric cancer has decreased worldwide in recent decades, the incidence of poorly cohesive carcinoma (PCC) is rising. The prognostic significance of gastric PCC remains a subject of debate. OBJECTIVE: To analyze the prognosis of gastric PCC in a Tunisian cohort. METHODS: A total of 122 gastric adenocarcinoma patients who underwent curative gastrectomy from 2001 to 2014 at Habib Thameur hospital in Tunis, Tunisia were included. The clinicopathological parameters and prognosis of PCC were analyzed in comparison with non PCC (NPCC). RESULTS: Sixty one patients (50%) presented PCC. Patients were younger in PCC group (p = 0,001). There was no difference in sex distribution between the two groups. PCC was more likely to be stage T4 (55.7% vs 34.4%; p = 0.033), N3 (67.8% vs 30%; p < 0.001) and have a higher metastatic lymph node ratio (p < 0.001). Hepatic metastases were more frequent in NPCC group (p = 0.031) whereas peritoneal carcinomatosis was more common in PCC group (p = 0.004). Perineural invasion was more frequent in PCC group (p = 0.001). Resection margins were more often positive in PCC group (31.1% vs 9.8%; p = 0.004). There was no difference in recurrence rate between the 2 groups (p = 0.348). The 5-year survival was similar in the NPCC and PCC (respectively 43% vs 23 %; p = 0.247). Survival rates were also comparable in early stage (100% vs 80% respectively for PCC and NPCC; p = 0.527) as well as for advanced stage (16% vs 35% respectively for PCC and NPCC; p = 0.538). PCC was not a prognostic factor for survival. Interestingly, advanced age, adjacent structures invasion, positive resection margins were specific prognostic factors for PCC. CONCLUSION: In our study PCC was not a prognostic factor for survival. Advanced age, adjacent structures invasion and positive resection margins were specific prognostic features for this histological subtype.