Ectomesenchymal Chondromyxoid Tumour of the Posterior Tongue

Ectomesenchymal chondromyxoid tumor (ECMT) is a rare benign neoplasm arising in the tongue. With only 45 cases reported in the literature, there are several unique features defining this lesion. Firstly, almost all patients present with an asymptomatic slow growing mass on the anterior dorsum of the tongue. At the microscopic level, it is recognizable as a well-circumscribed unencapsulated proliferation of uniform round to fusiform cells embedded in a chondromyxoid matrix. Lastly, the immunohistochemistry profile is characterised by positivity for glial fibrillary acidic protein and frequent positivity for S-100 and cytokeratins. We report a case of a mass located on the posterior dorsum of the tongue and meeting the aforementioned morphological and immunohistochemical criteria of ECMT.     

Ectomesenchymal Chondromyxoid Tumor: A Series of Seven Cases and Review of the Literature

Ectomesenchymal chondromyxoid tumors (ECT) are rare, benign, intraoral mesenchymal soft tissue tumors that can be diagnostically challenging. In this study the demographic, clinical, histopathologic, and immunohistochemical features of seven ECTs are examined. The differential diagnosis of this rare neoplasm is discussed and the literature is reviewed.     

Case Report of the positive exostosin-1 without B-cell lymphoma-2 gene expression of giant cell tumor lesion in hereditary multiple exostosis

IntroductionThe giant cell tumor, in which BCL-2 gene was expressed only in its malignant transformation, is a benign, primary skeletal neoplasm with variable biologic aggressiveness. The is of the giant cell tumor. A coexistence with hereditary multiple exostosis with expression of EXT-1 is very rare. The correlation between giant cell tumor in hereditary multiple exostosis is still not clearly determined.Presentation of caseA 31-years-old female presented with pain and lump on her left wrist and a coexistence of non tender multiple lump in the right and left knee. A wide excision of the tumor and reconstruction using non vascularized fibular graft was performed, followed by histopathology and immunohistochemistry of EXT-1 and BCL-2.DiscussionIn this case, the tumor showed negative BCL-2 and positive EXT-1 gene expression. Giant cell tumor and hereditary multiple exostosis also demonstrated associations of chromosomes 11 with a different pathological process.ConclusionGiant cell tumor in hereditary multiple exostosis revealed positive EXT-1 without BCL-2 expression. It still need more investigation to confirm the relationship between these tumors.     

A rare case report of an adenomatoid odontogenic tumor associated with odontoma in the maxilla

IntroductionThe adenomatoid odontogenic tumor (AOT) associated with odontoma has been described as a new entity with overlapping features of adenomatoid-like structures and odontoma. Presentation of the case: We report the second case of AOT associated with odontoma in the posterior maxillary region. A 22-year-old patient presented expansion of the vestibular and palatine cortical wall of maxilla. Radiographic examination and computed tomography confirmed erosion of maxillary walls, root resorption, displacement of a neighboring tooth and radiopacities within the lesion. Histopathological analysis showed a cystic cavity lined by odontogenic epithelium, organized as duct like structures and tubular dentin. The diagnosis established was AOT associated with odontoma.DiscussionAs fewer cases have been described, the aim of this study is to report clinical behavior and evolution of adenomatoid odontogenic tumor associated with odontoma, as it have not yet been described and its origin is not completely established. The hamartomatous or neoplastic origin of this tumor is under debate.ConclusionsTherefore, it is necessary similar cases to be published to increase the knowledge about the clinical behavior and evolution of this tumor, to enable such lesions to be more clearly defined in the next classification of odontogenic tumors.     

Primary leiomyosarcoma of the greater omentum: a case report

IntroductionGreater omentum leiomyosarcomas are rare tumors with only a few cases reported in literature.Presentation of caseWe report the case of a 68-year-old man who consulted complaining of diffuse abdominal pain without a palpable mass at physical examination. Imaging studies revealed a solid-cystic lesion in the right lower quadrant. Surgical resection was performed and the tumor was diagnosed as a leiomyoscarcoma by histological and immunohistochemical examinations.DiscussionSurgical resection of all lesions seems to be a reasonable therapeutic approach if resection is feasible. Chemotherapy may be used in selected cases.ConclusionMore cases are needed to define the best treatment approach of this disease.     

AQP1在骨巨细胞瘤中的表达和意义

目的研究水通道蛋白1（aquaporin 1,AQP1）在骨巨细胞瘤中的分布及在骨巨细胞瘤组和对照组中的表达差别,并确定AQP1在骨巨细胞瘤各级中的表达差别,从而确定AQP1在骨巨细胞瘤中的作用。方法对42例骨巨细胞瘤标本和24例对照组标本,采用免疫组织化学方法,镜下观察AQP1在组织中的分布,应用自动图像分析检测AQP1在两组中表达情况。结果a）本免疫组化染色显示：AQP1表达于所有标本的肿瘤微血管和小血管内皮细胞上;AQP1表达于大多数骨巨细胞瘤的肿瘤细胞上,AQP1在对照组中无表达或表达很微弱;b）自动图像分析系统所得平均光密度在两组中差别显著（P〈0.01）,AQP1在骨巨细胞瘤中高表达;c）AQP1的表达在骨巨细胞瘤级和骨巨细胞瘤级差别显著（P〈0.01）,而AQP1的表达在骨巨细胞瘤其他各级之间无差别（P〉0.05）。结论大多数骨巨细胞瘤的肿瘤细胞高表达AQP1的原因不明;AQP1在骨巨细胞瘤中高表达,预示其在骨巨细胞瘤的生物学行为中的作用;AQP1的表达在骨巨细胞瘤级和骨巨细胞瘤级差别显著,提示AQP1可能有区分骨巨细胞瘤级和骨巨细胞瘤级的作用。     

Neuroendocrine tumor of the small intestine diagnosed with trans-abdominal ultrasonography: A case report

IntroductionTumors of the small intestine are rare. In addition, clinical symptoms are nonspecific and neoplasm-related symptoms occur late. We report a case of neuroendocrine tumor (NET) of the small intestine that was diagnosed early with trans-abdominal ultrasonography (US).Presentation of caseThe patient was a 61-year-old man. Abdominal contrast-enhanced computed tomography (CT) was performed because the patient complained of abdominal pain. The CT showed a tumor lesion in the mesentery. Trans-abdominal US was undertaken to evaluate this tumor lesion, and a tumor lesion of the small intestine was found nearby. A diagnosis of lymph-node metastasis of a small-intestine tumor was made as a preoperative diagnosis. A laparotomy was performed with partial resection of the ileum, together with the small-intestine mesentery including an enlarged lymph node. Histological examination revealed NET of the ileum and lymph-node metastasis.DiscussionWith the application of trans-abdominal US, we could diagnose lymph-node metastasis of a small-intestine tumor relatively early and before surgery.ConclusionTrans-abdominal US is useful in the diagnosis of small-intestine NET.     

胃粘膜良恶性病变中P53和bc1—2表达的研究

目的探讨P53与bc1-2基因蛋白在胃癌及癌前病变组织中的表达特点及相关性.方法收集活检胃粘膜标本及部分手术切除胃癌病理标本126例,其中浅表性胃炎(CSG)29例,萎缩性胃炎(CAG),肠上皮化生(IM),不典型增生(DyS)各20例,胃癌(GC)37例.应用SP免疫组织化学方法检测P53、bcl-2基因蛋白的表达.结果P53在CSG、CAG、IM、DyS、GC中的阳性表达率分别为6.9%(2/29)、10%(2/20)、15%(3/20)、30%(6/20)、45.95%(17/37).bc1-2在上述各组中的阳性表达率分别为10.34%(3/29)、10%(2/20)、20%(4/20)、25%(5/20)、40.54%(15/37).P53、bc1-2表达阳性率GC组显著高于CSG组(P＜0.01),高于IM组(P＜0.05),IM、DyS、GC组P53、bc1-2有共同表达,差异无显著性(P＞0.05)结论P53和bc1-2在胃癌与癌前病变的表达显著高于良性病变.     

Solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas involving the distal body and proximal tail: A case report

Introduction and importanceSolid Pseudopapillary Epithelial Neoplasm (SPEN) of the pancreas is a rare cystic exocrine tumor of the pancreas most commonly occurring in women between 30 and 40 years of age. This case report aims to demonstrate the clinicopathological findings encountered and the management of a patient diagnosed with SPEN.Case presentationAn 18-year-old woman with gradually progressive and intermittent abdominal pain in the epigastric region presented to our outpatient department. Physical examination elicited tenderness to palpation in the epigastric area, and imaging findings suggested SPEN of the pancreas involving distal body and proximal tail region of the pancreas. The tumor was resected, and the diagnosis was confirmed on histopathology examination.Clinical discussionSPEN is a slow-growing tumor with a low-grade malignant potential, found incidentally in asymptomatic patients and symptomatic patients present with abdominal pain. The average tumor size is about 4 to 6 cm in diameter. Imaging is essential for diagnosis, and distal pancreatectomy with splenectomy was the most commonly reported procedure.ConclusionIt is crucial to consider a diagnosis of SPEN in women with abdominal pain in the epigastric region as early surgical resection of the tumor results in resolution and excellent prognosis.     

Mesenteric desmoid tumour presenting with recurrent abdominal abscess and duodenal fistula: A case report and review of literature

IntroductionDesmoid tumors are locally destructive but histologically benign. Their management involves close observation and surgical, medical, or hormonal treatment.Presentation of the caseA 36-year-old male was admitted for abdominal pain and fever. A CT scan showed fluid collections and air within a mesenteric mass. Diagnostic laparotomy was performed with drainage of the abscess and biopsy of the mass. The pathology suggested a desmoid tumor. His fever and abdominal pain persisted. An endoscopy was performed, which demonstrated a fistula track in the third part of the duodenum. After a multidisciplinary discussion, consensus was to pursue surgical intervention. The patient underwent an en bloc resection of the tumor including a portion of the wall of the third part of the duodenum. The final pathology confirmed a desmoid tumor with a fistula track to the duodenum. The patient had a re-laparotomy on POD2 for intra-abdominal bleeding but was discharged without further events on POD7. He had no evidence of recurrence on follow-up at 11 months.DiscussionDesmoid tumors are rarely complicated by abscess formation or fistulization. The management of intra-abdominal desmoids in this setting is challenging, as patients are often symptomatic and unresponsive to medical management. Percutaneous drainage and antibiotics are often initiated as first-line treatment, followed by surgery or medical therapy after evaluation of resectability and tumor stage.ConclusionRare complications can arise with intra-abdominal desmoid tumors. Principles of infection control should be applied in combination with optimization of oncologic outcome. A multidisciplinary approach helps to achieve both these objectives.     

Giant cell tumor of soft tissues: A case report of extra-articular diffuse-type giant cell tumor of the quadriceps

IntroductionGiant cell tumors of soft tissue (GCTs) are a relatively rare entity. It is a distinct but uncommon group of neoplasms morphologically identical to osseous giant cell tumor. The diffuse type of extra-articular GCT arising within muscle is a rare benign soft tissue tumor with a wide spectrum of clinical presentation.Presentation of caseThis article reports a rare case of a 44-year-old woman with a mass arising from her right thigh. MRI showed only a few areas of low T2 signal in a mass that was hyper intense to muscle. Histopathology of this lesion located within the right quadriceps muscle revealed admixture of multinucleated giant cell with mononuclear cells. This patient was treated by surgical resection and followed up for recurrence.DiscussionDiffuse-type GCTs are commonly located in the periarticular soft tissues, but on rare occasions these lesions can be purely intramuscular or subcutaneous and can be challenging to diagnose. Characteristic findings include gradient echo secondary to hemosiderin deposition, and the low signal on T2.ConclusionBecause extra-articular diffuse-type GCTs are rare, the differential diagnosis is challenging. The clinical outcomes of diffuse-type GCTs are unclear because of their rarity. Benign clinical course is expected if the lesion is excised adequately.     

Bronchial carcinoid tumor in the era of covid-19 pandemic: A case report

IntroductionBronchial carcinoid tumors are rare, slow growing, malignant neuroendocrine tumors which arise from Enterochromaffin (Kulchitsky) cells. Early diagnosis is extremely important as the main stay of treatment is surgical excision.Presentation of caseWe present a rare case of bronchial typical carcinoid tumor in a 27-year-old male who presented with a complaint of intermittent dry cough of 2 weeks’ duration associated with shortness of breath and low grade fever. He was initially misdiagnosed as covid-19 pneumonia and was admitted to covid-19 treatment center. Right lung bi-lobectomy with regional lymph node resection was done and he was discharged home in good condition.DiscussionMajority of typical carcinoids are located in the central airways leading to bronchial obstruction with recurrent pneumonia, chest pain and wheezing. In the era of covid-19 pandemic, lung cancer patients are at higher risk of being affected by covid-19 and, early identification and differential diagnosis is extremely difficult in the absence of comprehensive evaluation and work up as the clinical and imaging findings of covid-19 may resemble lung cancer. Although hilar and mediastinal lymph nodes are the most common metastatic sites for typical carcinoids most lymphadenopathies are caused by a reactive inflammatory reaction.ConclusionBronchial carcinoids are rare, malignant neuroendocrine tumors with complete surgical resection being the only curative management. During the Covid-19 pandemic crisis, diagnosing rare lung diseases like carcinoid tumor is real challenge especially in resource limited set up and needs high index of suspicion with meticulous diagnostic work up. The outcome of typical carcinoids with lymph node metastasis is excellent with complete resection.     

A multidisciplinary approach to giant soft tissue sarcoma of the chest wall: A case report

IntroductionSoft tissue sarcomas of the chest wall are exceptionally rare entities that present as painless slow growing masses. Resection is often precarious due to involvement of vital structures, and patients are left with large chest wall defects postoperatively requiring extensive reconstruction.Presentation of caseWe present a case report of a 29 year-old man who presented with a giant soft tissue sarcoma of the chest that had been growing slowly for one year prior to presentation. The patient had a biopsy that was positive for sarcoma, and PET CT demonstrated a large lobulated mass in the left chest wall with an SUV of 6.7. He received 50 Gy of radiation therapy; however, the mass continued to grow in size. He subsequently underwent an en-bloc resection of the mass with latissimus and serratus muscle primary reconstruction. Final pathology showed a 27 cm high-grade fibrosarcoma with prominent myxoid component. To our knowledge, this is the largest soft tissue sarcoma of the chest wall reported in the literature. Postoperatively, the patient received 6 cycles of adjuvant chemotherapy.DiscussionSurgery is the mainstay of treatment, and chemotherapy and radiation are used in specific circumstances. Risk of recurrence is dependent on many factors, including histologic subtype, grade, and size of tumor. Long term surveillance with physical exam and imaging is recommended.ConclusionWe feel that the multidisciplinary approach is crucial for optimal management of large soft tissue sarcomas. We recommend this approach to all patients with chest wall sarcomas.     

Denosumab salvage therapy in an 11-year-old boy with locally recurrent unresectable giant cell tumor of the lumbar spine after surgery

Giant cell tumors (GCTs) of the bone are locally aggressive primary bone tumors with a benign character. Spinal involvement is rare which accounts for approximately 5% of all primary bone tumors and it is quite rare in the lumbar spine. An 11-year-old boy patient presented with pain of low back and bilateral low extremities. Lumbar CT and MRI revealed a lytic lesion of the L4 vertebra corpus. The patient earned remarkable and timely recovery with 2 surgical interventions and the use of denosumab. Surgical resection for GCTs is still preferable as the initial treatment, denosumab should be utilized after tumor resection whether based on the purpose of prevention or treatment of tumor recurrence.     

Inhabitual presentation of Sertoli-Leydig cell tumor of the ovary with xeroderma pigmentosum: Case report with review of literature

IntroductionSertoli-Leydig cell tumors (SLCTs) are rare sex-cord stromal tumors of the ovary. Heterologous components may be present, most commonly in the intermediate differentiated and poorly differentiated groups. Because of their scarcity, SLCTs with heterologous differentiation represent a challenge in both diagnosis and management, with limited available experience.Presentation of caseWe report a case of a 27-year-old, Tunisian woman, followed in the Dermatology Department since the age of six months for xeroderma pigmentosum, with a history of basal cell carcinoma of the face operated on several times. The patient presented with abdominal pain and bloating associated with a medium abundance ascites on physical exam. Ultrasound showed a large left adnexal mass associated with an elevated cancer antigen 125 on serological exam. The patient underwent unilateral salpingo-oophorectomy with resection of two omental nodules. Microscopic examination concluded to poorly differentiated Sertoli-Leydig tumor with rhabdomyomatous differentiation. Adjuvant chemotherapy was performed and there was no clinical evidence of tumor recurrence during the three years of follow-up.DiscussionSLCTs with rhabdomyomatous differentiation on the setting of xeroderma pigmentosum are exceptional, microscopic diagnosis and management is challenging, considering the tumor scarcity.ConclusionFurther case reports and retrospective studies are required to more understand the pathogenesis of SLCTs and to determine their optimal treatment regimen.     

A recurrent solitary fibrous tumor of the thigh with malignant transformation: A case report

IntroductionWe describe an unusual case of a uniformly high-grade malignant solitary fibrous tumor (SFT) of the thigh with recurrence after wide resection in a 31-year-old man.Presentation of caseOur current case showed a long-term benign course before the operation, although the subcutaneous tumor was larger than 10 cm at presentation. The SFT was diagnosed by needle biopsy, and wide resection was performed. Histological findings showed proliferation of capillaries surrounded by masses of spindle-shaped cells without any cytologic atypia, and the percentage of MIB-1-positive nuclei was 2.1%. However, a rapidly enlarging recurrent tumor was observed 11 months after the operation. A second wide resection for the recurrent tumor was performed. Histologically, the tumor cells uniformly displayed significant cytologic atypia and pleomorphism, and had 40–50 mitoses per 10 high-power fields. The proportion of MIB-1-positive nuclei was 48%. Consequently, the tumor was diagnosed as a SFT with malignant transformation.DiscussionThe malignant transformation described in past studies showed high-grade areas within benign, low-grade, or intermediate-grade SFTs. Therefore, in contrast to our case, uniformly high-grade malignant histological findings at recurrence were not described.ConclusionEven if a tumor is non-malignant during the clinical course, as confirmed by tissue biopsy, the possibility of tumor progression to high-grade sarcoma at recurrence should be considered, and the treatment strategy should be determined carefully.     

A massive hepatic tumor demonstrating hepatocellular,cholangiocarcinoma and neuroendocrine lineages: A case report and review of the literature

IntroductionMixed hepatocellular and cholangiocarcinoma tumors (MHCC) are described in the literature, as are the more rare mixed adenoneuroendocrine carcinomas (MANC) of hepatobiliary origin. Only two cases of tumors with characteristics of all three histologies/phenotypes have been previously described in one Chinese study.Presentation of caseHerein we report clinical, microscopic and molecular features of a 25 cm mixed hepatic tumor with hepatocellular, cholangiocarcinoma and neuroendocrine differentiation arising in an otherwise healthy 19-year-old North American Caucasian male without any identifiable risk factors.DiscussionThe patient underwent multimodality imaging and the tumor was biopsied preoperatively, and it was initially interpreted to be hepatocellular carcinoma fibrolamellar type. A left trisegmentectomy with lymphadenectomy was performed and the tumor was definitively diagnosed based on the surgically resected specimen. Integrated microscopic and molecular features defined the differing biological aggressiveness of growth pattern components. Cases in the literature of MHCC and rare cases of MANC have largely undergone aggressive surgical resection as well, however the majority of studies on mixed hepatic tumors to date reflect Eastern patient cohorts and populations with underlying liver disease, thereby limiting extrapolation on management or outcomes in this case.ConclusionThis is one of the only reports of a hepatic tumor arising from hepatocellular carcinoma, cholangiocarcinoma and neuroendocrine lineages. Increased awareness of this tumor type may optimize improve future management.     

Carney triad: A case report,characteristics and literature review of this rare entity

Introduction and importanceCarney triad is a rare entity of unknown etiology, characterized by the association of tumors with low incidence such as: gastric leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma. We show a case of Carney triad diagnosed in our center that has some different characteristics to the typical presentation of this patology, and a review of the literature.Case presentationWe present the case of a 47-year-old men who was admitted to our hospital for upper gastrointestinal bleeding and anemia. The patient was diagnosed of gastric GIST (gastrointestinal stromal tumor), pulmonary nodule and retroperitoneal nodule close to iliac bifurcation. A review of his medical history revealed that he had undergone surgery at 18-year-old for gastric leiomiosarcoma, subtotal gastrectomy performed with gastroenteric anastomosis type Billroth II.The patient underwent total gastrectomy with a end-lateral esophago-jejune mechanical anastomosis and retroperitoneal tumor was also excised. Pathological result confirmed gastric GIST, and extra-adrenal paraganglioma.Clinical discussionWe review the available literature to determinate the characteristics and the most appropriate management for this syndrome. The trend is surgical treatment of gastric GIST and paraganglioma, preferred conservative treatment in pulmonary chondroma.It is under discussion whether gastric tumors Carney syndrome is considered GIST, because the Carney triad lesions in stomach are different clinically, pathologically, and etiologically from GIST of stomach.ConclusionCarney's triad is a low prevalence entity, with a limited number of publications, so the presentation of the syndrome may be different from the presentation described in the literature.