A 75-year-old Korean man was referred to our hospital with cramping abdominal pain. His chest X-ray showed an abnormal air shadow above the diaphragm, and computed tomography showed an abdominal viscera in the pericardium. We performed surgery and confirmed peritoneopericardial diaphragmatic hernia with small bowel strangulation. Postoperative course was uneventful. Peritoneopericardial diaphragmatic hernia is very rare in humans, so we report the case with a literature review. A peritoneopericardial diaphragmatic hernia is one that permits direct communication between the peritoneal and pericardial cavities through a defect in the diaphragm. It is very rare in humans. The cause of peritoneopericardial diaphragmatic hernia varies, as some authors have reported -5. We heA 75-year-old Korean male patient was referred to our emergency department with cramping abdominal pain for three days. He also had dyspnea and chest discomfort. He had diabetes mellitus and no history of trauma. Upon his arrival at the emergency department, his blood pressure was 150/100 mmHg, heart rate 102 per minute, respiratory rate 22 per minute and body temperature 37.5°C. On physical examination there was direct tenderness on the whole abdomen and we could hear rale in both lower lung fields. Laboratory studies showed leukocytosis and a leukocyte count of 1,500. An x-ray of the chest showed abnormal air shadows above the diaphragm (Figure A peritoneopericardial diaphragmatic hernia is one that permits direct communication between the peritoneal and pericardial cavities through a defect in the diaphragm. It is very rare in humans. Peritoneopericardial hernia may be congenital, arising from the failure of development of the transverse septum , or may A case of small bowel strangulation due to peritoneopericardial hernia and successfully corrected surgically is reported. Abnormal air shadow above the diaphragm on chest x-ray led us to suspect a type of diaphragmatic hernia. Computed tomography is one of the best diagnostic methods. The clinician should be aware of peritoneopericardial diaphragmatic hernia, however rare this condition. If peritoneopericardial diaphragmatic hernia is confirmed, surgical repair should be performed.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.JH and SW wrote the draft of the manuscript and obtained the written consent. JH performed the literature review and participated in the manuscript writing and helped to the final writing of the paper and gave final approval of the manuscript. Both authors have read and approved the final manuscript.


Fungi are unusual causes of pedal osteomyelitis in children and adolescents. Eumycetoma is a chronic cutaneous and subcutaneous infection caused by various genera of fungi. A provisional diagnosis of foot mycetoma is made after clinical assessment. Radiologic-pathologic correlation is an essential supplement for the accurate diagnosis of osteoarticular infections. This paper aims to sensitize orthopedic surgeons, radiologists, and pathologists to the importance of correlative imaging findings in relation to surgical and microscopic pathology in osteoarticular infections, specifically eumycetoma osteomyelitis of the foot. From our review of the published data, the present case is the first report of radiologic-pathologic correlation in eumycetoma osteomyelitis of the calcaneus. This paper describes a case of eumycetoma osteomyelitis of the calcaneus in a child in which diagnostic X-rays and magnetic resonance imaging (MRI) were correlated with the surgical and microscopic pathologic features, for establishing an appropriate diagnosis and treatment. We conclude that there is a significant agreement between radiologic and pathologic evaluation for assessment of eumycetoma osteomyelitis of the calcaneus. Radiologic-pathologic correlation amplified our interpretation of imaging information available on plain radiographs and MRI and augmented diagnostic confidence. Similarly, anatomic-histopathological correlations consolidated diagnostic accuracy. Eumycetoma is an unusual chronic cutaneous and subcutaneous infection caused by various genera of fungi. Approximately 40% of mycetomas worldwide are eumycotic rather than actinomycotic . The disease is characterized by marked progressive destruction of soft tissue and bones causing functional disability. The most common site of occurrence is the foot . A proviWe assume that radiologic-pathologic correlation is an essential supplement for the accurate diagnosis and management of osteoarticular infections. From our review of the published data, the present case is the first report of radiologic-pathologic correlation in eumycetoma osteomyelitis of the calcaneus. The purpose of this review is to demonstrate why performing this correlation is an essential part of the diagnostic process of osteoarticular infections. This paper aims to sensitize orthopedic surgeons, radiologists, and pathologists to the importance of correlative imaging findings in relation to surgical and microscopic pathology in eumycetoma osteomyelitis of the foot. Our patient was informed that data concerning the case would be submitted for publication. Staphylococcus aureus, the patient was diagnosed with nonspecific bacterial calcaneal osteomyelitis. The patient was treated with multiple courses of oral and intravenous antibiotics over the following six years, to which he did not respond. The patient walked with painful limp that deteriorated over the past years till he finally used crutches permanently and became a household ambulatory. The patient was nonimmunosuppressed and had no systemic manifestations or other skeletal complaints. Blood and serum chemistry were also unremarkable.A 14-and-a-half-year-old boy from Fayoum Governorate in rural Egypt complained of an initially painless right foot swelling for the past six years. Multiple sinus tracts developed with an initial purulent discharge and an eventual extensive black granular discharge. He was previously subjected to multiple bony debridement procedures in other institutions to which the he did not respond. Based on the clinical/radiologic suspicion and results of culture and sensitivity from discharging sinuses that revealed Gram positiveBy examination, there was diffuse right foot oedema and tenderness especially over the hind foot. Two discharging sinuses were observed in relation to the hind foot. There was painful passive motion of the subtalar and ankle joints.X-ray findings of the foot and ankle revealed massive bony destruction of the calcaneus consistent with chronic osteomyelitis. MRI of the right ankle and foot was performed to characterize and evaluate the extent of the disease. Technique of examination included sagittal T1-, STIR, and T2-weighted images, axial T1- and T2-weighted images, and coronal T2-weighted images. The salient abnormality was the markedly altered marrow signals seen almost totally replacing the marrow texture of the calcaneus and to a lesser extent involving the opposing posteromedial aspect of the talus eliciting abnormal low T1, high T2, and iso-to-high STIR signals with evident cortical breaching and associated large soft tissue component eliciting low T1 and high T2 and STIR signals Figures .In march 2015, we performed total calcanectomy through a heel splitting approach. Under general anesthesia, a high tourniquet was employed in the prone position. Anatomic pathology of the excised calcaneus is depicted in Figures The concept of radiologic-pathologic correlation was born in 1947 with the establishment of the Radiologic Pathology Department and Registry at the Armed Forces Institute of Pathology in Washington, DC. This method has become a key teaching tool to understand the radiological manifestations of disease, initially on plain films and later with cross-sectional techniques . ObviousMusculoskeletal infection is common in clinical practice. Osteomyelitis refers to infection of the bone and marrow usually by pus producing organisms. It is commonly caused by bacteria, but fungi are also considered. Usually, the diagnosis of fungal osteomyelitis is challenging. The clinical and radiological findings of osteoarticular infection may be conflicting and confusing. Even with the advancement of imaging technology, findings may remain inconclusive. There are a wide variety of presentations seen on imaging reflecting the balance between host and organism, disease duration, and institution of antimicrobial treatment. A good understanding of various stages of osteoarticular infection is essential to augment the interpretation of the vast amount of imaging information available to arrive at an accurate diagnosis and thereby institute timely and effective treatment to prevent destructive sequelae in the immature skeleton .In eumycetoma fungal osteomyelitis bones are almost always attacked from outside, in contrast to bacterial osteomyelitis, and periosteal reaction, cortical erosions, and bone texture abnormalities may then be seen. It is important to detect whether bone is infected as nonsurgical cure is uncertain in such cases. Conventional radiographs are used to determine whether bone is affected and to identify the limits of lesions in eumycetoma. Multiple radiographic changes can be detected . Abd El Several authors found that MRI is useful for visualizing soft tissue involvement and bone destruction; with MRI, multiple small spherical hyperintense lesions separated by tissue of low signal intensity appear. Some of these lesions show a central tiny hypointense focus. These hypointense foci were named “dot in circle” and were seen in 80% of patients, which made this appearance highly indicative of mycetoma , 4, 6, 7The MRI of our patient revealed thickening of the overlying skin with multiple defects, ulceration, and sinus tracts breaching the posterior and plantar posterior skin, subcutaneous fat, and plantar fascia. These findings clearly correlated with the intraoperative multiple small cavities and black grain discharge observed on the plantar surface of the calcaneus following calcanectomy. There was a significant correlation between the previous radiologic-pathologic findings and the black grain discharging sinuses observed on clinical examination.The MRI findings of our patient revealed markedly abnormal signal intensity involving the sinus tarsi and the subtalar articulation. The gross pathologic findings of the calcaneus revealed complete destruction of the articular cartilage of posterior subtalar joint, joint depression, and partial destruction of the middle facet. There was a significant correlation between the previous radiologic-pathologic findings and the preoperative painful passive range of motion of the subtalar joint.In 2012, El Shamy and colleagues reported a new grading system for MRI diagnosis of mycetoma. They found that actinomycetoma more often showed soft tissue microabscesses, bony periosteal reaction, and reactive sclerosis, while eumycetoma frequently exhibited soft tissue macroabscesses with bone cavitation; such differences were not statistically significant . The MRISince both actinomycetes and fungi are implicated as causative agents, it is important to distinguish them in order to ensure that correct treatments are given . MycetomCulture methods are still considered to be the gold standard in species identification of the causal agents of mycetoma. However, some agents are difficult to identify by morphology alone. Furthermore, most cultures methods are time consuming, contamination is common, and experience is needed to read results accurately . HistoloThe granules of actinomycetoma consist of fine, branching filaments, only about 1 micron thick, whereas the granules of eumycetoma are composed of septate hyphae 4-5 microns thick . This hiIn our patient, all of the above mentioned histopathological findings clearly correlated to the anatomic pathology aiding definitive species diagnosis.Agreement between radiology and pathology was recognizable. The radiologic-pathologic correlation may be used in explaining the role of plain radiographs and MRI in identifying eumycetoma osteomyelitis of the calcaneus in children. Establishment of a clear correlation between radiology and pathology may help differentiate eumycetoma from other infections and tumorous lesions. Radiologic-pathologic correlation augmented diagnostic confidence. Similarly, anatomic-histopathological correlations consolidated diagnostic accuracy.


In this report, we describe an extremely rare case of a patient with massive odontoameloblastoma arising in the maxilla and occupying maxillary sinus.Odontoameloblastoma is an extremely rare mixed odontogenic tumor with both epithelial and mesenchymal components. The term In 2013, an 11-year-old Japanese boy was referred to our department for a painless and large mass of the right maxillary region. A panoramic X-ray showed a unilocular cystic lesion in the right maxilla containing a calcified mass in the lesion associated with an impacted tooth. Computed tomography showed a cystic lesion that included calcified structures and measured 3.6×3.1×2.7 cm. In 2013, the patient underwent tumor extirpation combined with impacted tooth extraction. The histopathological diagnosis was an odontoameloblastoma. No recurrence was noted 27 months after the operation.The patient has undergone postoperative occlusal guidance and functional orthodontic treatment, and his postoperative condition is excellent. However, postoperative recurrence or malignant transformation can occur in cases of odontoameloblastoma, and close long-term follow-up will be continued for our patient. Odontoameloblastoma (OA) is an extremely rare mixed odontogenic tumor that is defined in the current World Health Organization tumor classification system as a tumor that includes odontogenic ectomesenchyme and odontogenic epithelium and resembles an ameloblastoma in both structure and behavior . GeneralOA is usually found in young patients and has no significant gender predilection , 7. ClinIn 2013, an 11-year-old Japanese boy was referred to our department for painless bone expansion in the right maxillary alveolus, delayed eruption of the permanent second molar teeth, and altered occlusion. He had no significant medical or family history. A panoramic X-ray showed a unilocular cystic lesion in the right maxilla containing a calcified large mass associated with an impacted tooth Fig. . A horizIn 2013, the patient underwent tumor extirpation combined with impacted tooth extraction. The incisional line was started from the labial mucosa in the right maxillary central and lateral incisor area. It extended to the gingiva in the right maxillary lateral incisor and canine area by arch-like incision, and to the gingiva of the right maxillary second molar by crestal incision with distal releasing incision. The goal of this procedure was to make a mucoperiosteal flap from the lower border of the pyriform aperture, vertically to the infraorbital foramen and surrounding areas, and horizontally to the lower border of the zygomatic bone. The infraorbital neurovascular bundles were preserved. Upon opening of the sinus from a thin plate of bone in the canine fossa and surrounding areas in the anterior wall of the right maxilla (the same level as the lower border of the pyriform aperture), bone-like hard tissues that were strongly adhered to the maxilla were found. When these tissues were separated from the surrounding areas and removed using a fissure bar, a solid, bone-like, hard odontogenic tumor (similar to a complex odontoma) was found to have occupied almost the whole sinus. The mass was too large to remove from the opening.For complete removal of the mass including the tumor capsule, which was adhered to the surrounding bone, the tumor was divided into several pieces using a fissure bar Fig. . There wNo recurrence was noted at 27 months after the operation. The patient has undergone postoperative occlusal guidance and functional orthodontic treatment, and his postoperative condition is excellent.OA is an aggressive odontogenic tumor characterized by simultaneous occurrence of an ameloblastoma and a compound or complex odontoma in the same tumor mass , 9. OA aet al. [The origin of an OA  has beenet al.  suggesteet al. [Ameloblastoma, complex odontoma, compound odontoma, and dentigerous cysts may be differentiated by imaging, but their diagnosis also requires histopathological examination. A definitive diagnosis is difficult to make on the basis of imaging and clinical findings alone; therefore, total excision is required. Masqueda-Taylor et al.  found reet al. . TherefoClinically, OA starts as a slow-growing, painless mass that expands the alveolus and vestibular cortex and prevents eruption of permanent teeth . Our patWe report an extremely rare case of a patient with massive OA arising in the maxilla and occupying the maxillary sinus.Written informed consent was obtained from the patient’s legal guardian(s) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25–54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle-shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low- to moderate-level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH. Pulmonary epithelioid hemangioendothelioma (PEH) is the current term for a rare neoplasm originally described by Dail and Liebow in 1975 as intravascular sclerosing bronchioalveolar tumor (IVBAT) of the lung . PEH is PEH is a rare low-grade malignant vascular tumor that occurs in the lungs and, due to its rarity, it is easy to clinically misdiagnose PEH as other lung diseases. In the present study, four cases of PEH, which were diagnosed and treated at Shanghai Chest Hospital, Shanghai Jiao Tong University , were observed and analyzed with respect to the clinical manifestations, imaging findings, histopathological characteristics, immunohistochemical phenotypes and prognosis. This study was performed according to the Declaration of Helsinki and was approved the the ethics committee of Shanghai Chest Hospital, Shanghai Jiao Tong University . Written informed consent was obtained from all patients.The present study describes four cases of PEH that were diagnosed at the Chest Hospital Affiliated to Shanghai Jiaotong University from 2006 to 2013. Two of the cases were surgical patients at the hospital, while the other two cases were consultation patients from other hospitals. Case 1 was a 54-year-old male with multiple lung nodules that were revealed during a preoperative examination for cholecystitis. Case 2 was a 54-year-old female who was admitted for chest tightness and fatigue, whereby X-ray examination revealed a right pleural effusion. Case 3 was a 46-year-old female with no obvious symptoms; yet irregular clumps were found below the pleura of the right upper lung on a chest computed tomography (CT) during a routine physical examination . Case 4 All of the specimens were fixed in 4% neutral formalin, embedded in paraffin and stained with hematoxylin and eosin. Briefly, following deparaffinization, rehydration, heat-induced epitope retrieval and endogenous peroxidase blocking, the slides were incubated with primary antibodies for 1 h. The primary monoclonal antibodies, including mouse anti-human cluster of differentiation 31 (CD31) (1:200 dilution), mouse anti-human CD34  (1:100 dilution), mouse anti-human CK7 (1:200 dilution), mouse anti-epithelial membrane antigen (EMA) (1:200 dilution), mouse anti-human calretinin (1: 200 dilution), mouse anti-human desmin (1:200 dilution), mouse anti-human thyroid transcription factor 1 (TTF1) (1:200 dilution) and mouse anti-human vimentin (1:200 dilution) and polyclonal rabbit anti-human factor VIII (F8) (1:200 dilution) were all purchased from Dako . The specimens were subsequently subjected to the polyclonal goat anti-rabbit anti-mouse secondary antibody (Dako) (dilution 1:500) for 30 min and visualized using 3,3′-diaminobenzidine tetrahydrochloride as chromogen using an Envision system (Dako)  expression.Angiosarcoma is common in skin and rare in the lungs, and tends to present as a large solitary mass. Tumor vascular lumina, luminal lining epithelioid malignant cells, highly atypical tumor cells and mitoses are commonly observed under the microscope, and large lesions with spindle cells are also observable. Some PEHs may show certain atypical morphologies that are similar to epithelioid angiosarcoma, forming a morphological continuity with epithelioid angiosarcoma .When the lesions of PEH inside one lung are relatively limited, surgery is the preferred treatment; however, when the lesions have invaded both lungs and cannot be completely resected, postoperative chemotherapy may be required, according to the condition of the patient. The average survival time of patients with asymptomatic pulmonary nodules is 15 years, and this can be >25 years in the best cases ,13. PatiIn conclusion, the clinicopathological features of four PEH cases were investigated and the associated literature was reviewed. This revealed PEH to be a rare yet diverse form of malignant vasular tumor with varying patient prognoses. The results of this study may improve our understanding of PEH. However, studies with a larger sample size are required in order to provide a more comprehensive understanding, which would improve the clinical treatment and prognosis for patients with PEH.


Alpha-fetoprotein (AFP)-producing pancreatic neuroendocrine tumors (pNETs) are rare, and the few reported cases usually coexisted with other malignant components such as adenocarcinoma or hepatoid carcinoma. We present here the first case of pure AFP-producing pNET.A 56-year-old male patient underwent resection of the pancreatic tail and body because of elevated serum AFP levels and pancreatic mass. Microscopy showed that the tumor tissue consisted of large and small solid nests of polygonal cells. The tumor cells were positive for chromogranin A, synaptophysin, CD99, cytokeratin 19, pan-cytokeratin and β-catenin, and also showed diffuse immunoreactivity for AFP and human chorionic gonadotrophin. The mitotic rate was nearly 30 per 10 high-power fields and the Ki-67 index was nearly 25%. The histopathologic findings supported the diagnosis of an AFP-producing pNET. Other malignant components were not found. Serum AFP levels decreased to near-normal after operation and gradually increased to >1000 ng/ml at 5 months post-surgery. Recurrence and hepatic metastases were revealed by computed tomography. The patient died 21 months after surgery.This was the first case of pure AFP-producing pNET to be reported in the English literature. Serum AFP levels may provide useful information for monitoring the therapeutic effectiveness, early recurrence or metastases. Pancreatic neuroendocrine tumors (pNETs) are rare malignant tumors of the pancreas and account for approximately 1-2% of all pancreatic neoplasms . pNETs mAlpha-fetoprotein (AFP) has long been used as a tumor marker for hepatocellular carcinoma (HCC) and embryonic cell tumors. Elevated serum levels of AFP were also found in patients with carcinoma metastasis to the liver or non-neoplastic liver injury. Several cases of pNETs with elevated serum AFP levels have been reported, but most of these cases had liver metastasis at the time of diagnosis -8. Rare A 56-year-old man was admitted to the Peking University Third Hospital in December of 2011 because of abnormal imaging of the pancreas and high serum AFP levels found during routine health checkup. He had no symptoms of hypoglycemia, diarrhea or abdominal pain. Fifteen months earlier, he suffered from a liver abscess with normal serum AFP, which was treated successfully with antibiotics. Cholecystolithiasis was diagnosed at that time. One month earlier, he had undergone laparoscopic cholecystectomy for gallstones at another hospital. Enlargement of the tail and body of the pancreas was demonstrated by computed tomography (CT), and a diagnosis of pancreatitis was considered. The patient did not smoke but drank alcohol occasionally. Physical examination on admission revealed no specific findings.Laboratory tests showed that serum AFP levels were elevated to 321.4 ng/ml . The levels of other tumor markers  were all within normal limits. There was no serologic evidence of hepatitis B or C. Blood cell counts, erythrocyte sedimentation rate, and coagulation tests were normal. Levels of serum aminotransferases, alkaline phosphatase, gamma-glutamyl transferase, albumin, urea nitrogen, creatinine, amylase, lipase, glucose, human chorionic gonadotrophin (HCG), and immunoglobulins were all normal. Testicular ultrasonography revealed a cystic mass in the left epididymidis.Imaging examinations are shown in Figure Grossly, a grey ill-define nodular mass, measuring 5 × 4.5 × 3.5 cm, was shown at the body and tail of the pancreas Figure . NecrosiImmunohistochemistry showed patchy expression of chromogranin A (CgA) and synaptophysin (Syn). Tumor cells also showed diffuse immunoreactivity for AFP and focal strong immunoreactivity for hCG. Dot-like pan-cytokeratin (CKpan) reactivity near the nucleus, β-catenin reactivity at the cell membrane, diffuse CD99 expression in cytoplasm, and wide CK19 expression in tumor cells were observed. There was no expression of HapPar-1, CD10, CD56, or progesterone receptor (PR) in tumor cells. Ki-67 index was nearly 25%. The histopathology findings supported the diagnosis of a pure AFP-producing neuroendocrine carcinoma according to the forth edition of WHO Classification of neuroendocrine tumors .Serum AFP levels decreased to 23.15 ng/ml on day 29 post-surgery, but increased to 180.9 ng/ml on day 80. Lymph nodes metastasis and a recurrent mass in the residual pancreas were detected by CT. The patient was treated with a somatostatin (SS) analog (Sandostatin LAR) for 4 months. Serum AFP levels gradually increased to 1200 ng/ml 5 months after surgery, and liver metastases were found on CT. Hepatic metastases were treated with hepatic artery embolization and percutaneous radiofrequency ablation (RFA) in another hospital, and the serum AFP levels fluctuated around 700 ng/ml. His clinical condition gradually worsened, and he died 21 months after the original surgery. Other tumor markers (such as CEA and CA19-9) remained within the normal range during follow-up.AFP is a tumor marker for HCC and had been used in the clinical practice for a long time. Because there is a large population of patients with hepatitis B or C virus infection in China, serum levels of AFP are measured routinely during health checkups to screen for HCC. The high levels of AFP in this patient originally raised the question as to whether there was a tumor or another condition causing the expression of AFP.Pancreatic neuroendocrine neoplasms are rare, sporadic pancreatic tumors. Many pNETs are diagnosed at late stages because of the lack of symptoms in the case of non-functional tumors. The apparent incidence of pNETs had increased in recent years, likely due to improved detection methods, but it is still difficult to diagnosis pNETs at an early stage when the tumor do not invade adjacent tissues or produce distant metastases ,2. HistoOnly 23 cases of pNETs with elevated serum AFP levels have been described in the English literatures, and liver metastases were confirmed in most of these patients -14. AmonIn the present case, tumor cells exhibited dot-like CKpan reactivity near the nucleus, which is the classical CK expression pattern in neuroendocrine tumors. CD56 is usually expressed in pNETs , but wasSurgery is the only treatment that has the potential to cure patients with pNETs. Although the present patient was treated with radical surgery that achieved free resection margins and that did not exhibit regional lymph node metastases, the tumor relapsed and metastasized within months after surgery. More than 90% of pNETs express SS receptors, and biotherapy with long-acting SS analogs had been shown to slow tumor growth and improve survival in patients with pNETs ,20. HowePancreatic neuroendocrine neoplasms include well-differentiated (low- to intermediate-grade) neuroendocrine tumors and poorly differentiated (high-grade) neuroendocrine carcinomas (NECs). NECs are defined by the presence of >20 mitoses per 10 high-power fields  according to the WHO Classification of neuroendocrine neoplasms of the pancreas . On the In an analysis of the Surveillance Epidemiology and End Results (SEER) database, the 5-year survival of patients with poorly differentiated tumors was <38% for local disease, 21% for loco-regional disease, and <4% for metastatic disease, and the median survival for distant metastatic disease was only 5 months in patients with poorly differentiated carcinoma . SeveralWe described the first case of pure AFP-producing pNEC. Serum AFP levels may provide useful information for monitoring the therapeutic effectiveness, early recurrence or metastases.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


We analyzed the etiopathogenetic, clinical, radiographic, and histopathologic aspects of keratocystic odontogenic tumors, particularly in association with dental anomalies of number, with the aim of providing useful information for their correct diagnosis, treatment, and prognosis within a multidisciplinary approach.A 14-year-old Caucasian girl presented for observation of bilateral agenesis of the upper incisors, which was diagnosed by orthopantomography. Approximately one year after starting orthodontic treatment, the patient went to the emergency department because of a phlegmonous tumefaction of the lateroposterior upper left maxillary region. Diagnostic orthopantomography and axial computed tomography scan results of the facial skeleton revealed a large lesion occupying the left maxillary sinus, rhizolysis of dental elements 26 and 27, and dislocation of dental element 28. The lesion and infected sinus mucosa were removed through surgical antral-cystectomy with the Caldwell-Luc approach. Histological examination of the lesion confirmed the suspected diagnosis of keratocystic odontogenic tumor. The 12-month follow-up orthopantomography and computed tomography scan results showed good trabecular bone formation in the lesion area. The 24-month follow-up results showed optimal healing in the area of the lesion, positive pulp vitality tests for teeth 26 and 27, and good periodontal tissue healing, as verified through periodontal probing.Combined with our observations from a careful review of the literature, the results of the case study suggest that keratocystic odontogenic tumor and dental agenesis probably do not develop through a common genetic cause. More likely, they are caused by related environmental factors. Management of this case required the multidisciplinary collaboration of different specializations and careful planning to devise a correct therapeutic protocol and reach a favorable prognosis. The classification of maxillary cysts by the World Health Organization (WHO) in 1992 categorized keratocysts as maxillary cysts of dysembryonic odontogenic origin. In 2005, the WHO defined keratocysts as benign, uni- or multi-cystic intraosseous neoplasms of odontogenic origin . Keratocysts typically have a thick, parakeratinized, stratified, squamous epithelial lining, and a potentially aggressive and infiltrating behavior . The welKCOTs can develop in single or multiple form. Multiple keratocysts are characteristic of Gorlin-Goltz or nevoid basal cell carcinoma syndrome. Single keratocysts have a frequency of 5 to 15 percent, compared to 5 percent for multiple keratocysts. The frequency of recurrence ranges from 25 to 30 percent for single keratocysts, and is higher for multiple keratocysts. The likelihood of recurrence is currently predicted on the basis of histology; recurrence is rarely associated with orthokeratosis, but frequently found in parakeratosis. KCOTs have equal prevalence in males and females. They can affect individuals of all ages, with peaks in the second and fourth decades of life. They are more common in the posterior mandible (65 to 70 percent of cases), especially in the posterior body and ascending ramus [Clinically, a keratocyst manifests as a fast-growing aching tumefaction, with infiltration and expansion in the cortical bone. It may cause rhizolysis and dental dislocation. On radiography, a keratocyst appears as a uni- or multi-locular radiotransparency. A unilocular keratocyst can be connected to the tooth apex, simulating a periapical cyst. Alternatively, it can surround the crown of an impacted tooth, potentially being confused with a dentigerous cyst. If the keratocyst is localized between tooth roots, it may be misdiagnosed as a lateral periodontal or lateral radicular cyst. A KCOT that develops along the midline simulates a nasopalatine duct cyst. Unilocular keratocysts and ameloblastomas are indistinguishable on X-ray .The aim of this report was to study the etiopathogenetic and clinical manifestations of KCOTs, particularly in association with dental anomalies of number. The overall goal was to provide useful information for the correct diagnosis, treatment, and prognosis of these lesions, within a multidisciplinary approach -9.Here, we report the case of a 14-year-old Caucasian girl, who presented with bilateral agenesis of the upper incisors, diagnosed via orthopantomography (OPG) Figure  revealedTo determine the origin of the lesion and the best surgical approach, an axial computed tomography (CT) scan of the facial skeleton was requested. The coronal section showed complete opacity of the left maxillary sinus, involvement of ethmoidal cells Figure , and disA large mucoperiosteal incision was made to create a mixed flap  from the central contralateral incisor to the maxillary tuberosity Figure . RemovinThe KCOT showed a typical undulated pattern in the epithelium, accompanied by parakeratinization and exfoliation of keratin in the lumen Figure . ClinicaThe orthodontic braces were removed, resulting in an increased gum volume and ‘gummy smile’. Therefore, we performed gingivectomy using an Nd: YAG laser source Figure .Sixty days after implant insertion, the peri-implant soft tissues were determined to be in an excellent condition, and the final prosthetic rehabilitation with a ceramic implant-crown system was performed Figure . At the In this manuscript, we present the case of a patient with a KCOT, an infrequent pathology of the maxillary sinus, associated with a dental anomaly (hypodontia). We primarily focused on the etiopathogenesis of the lesion, to detect a possible causal relationship between KCOT and agenesis.de novo. In rare cases, they develop on odontogenic cysts. Approximately 200 genes are involved in tooth development. Among them, fibroblast growth factor (FGF) and sonic hedgehog (SHH) are the most frequently altered genes in odontogenic neoplasms.No predisposing factors for keratocysts are known. Like other odontogenic tumors, keratocysts usually appear The question of whether keratocysts are malformations or neoplasms has been the subject of long debate. Whereas some authors claim that a keratocyst develops from a distal extension of the dental lamina, others think it is derived from the basal layer of the oral epithelium. Most authors agree that the neoformation shows an expanding growth type, similar to maxillary cysts ,11. KeraSMO), through the SHH ligand. The interaction between PTCH and SMO inhibits translation of the growth signal. If the normal functioning of PTCH fails, then the proliferation-promoting effects of SMO become dominant [SMO is an oncosuppressor that is important for the embryonic development of neural tubes, skeleton, limbs, lungs, hair follicles, and teeth. Mutations of SMO can be found in keratocysts in Gorlin-Goltz syndrome, as well as in sporadic keratocysts. The above findings constitute clinical and molecular evidence for the conclusion that keratocysts are neoplasms characterized by local aggressiveness (see [Patched (PTCH) is the receptor for secreted sonic hedgehog (SHH) protein. Normally, PTCH forms a receptor complex with the product of the oncogene Smoothened , and shess see -3,13,14.,14.SMO, et al.[MSX1) in chromosome 4 (4p.16), and Paired box gene 9 (PAX9) in chromosome 14 (14q.21q.13) [TGFA) [Concerning the etiopathogenesis of dental agenesis, the hereditary theory  highlig.21q.13) -18. Func.21q.13) ,20. OtheSome authors have suggested that a recessive autosomal transmission may be possible . SuarezStudies agree on the importance of pathology during the maturation and migration processes of cells involved in dental gem formation . Environmental factors can prevent follicle formation, due to internal factors  or external factors  ,26. ArreThe present study concentrated on clinical, imaging, and histological diagnosis, as well as on the therapy of the lesion and agenesis. Initially, healing the KCOT was the most important concern. We applied an aggressive approach , which prevented recurrence and encouraged bone repair. From a radiological perspective, this approach led to a progressive increase in trabecular bone formation during later follow-ups.The final phase of the intervention concentrated on the bilateral agenesis of the upper incisors through an implant-supported prosthetic treatment. From the biological, functional, and esthetic perspectives, implant therapy was considered to be the most desirable treatment. Prior to implant insertion, orthodontic treatment was used to achieve and maintain the space necessary for the implant-supported prosthetic reconstruction.The management of this case required multidisciplinary collaboration between different specializations. It necessitated careful planning to devise the ideal therapeutic protocol for a favorable prognosis, including absence of KCOT recurrence. Good functionality and esthetics were obtained through the implant-supported prosthetic rehabilitation of dental agenesis.Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: computed tomography; KCOT: keratocystic odontogenic tumor; OPG: orthopantomography.All authors declare that they have no competing interests.ML and VL contributed to the conception and design of the study, the analysis and interpretation of the data, and drafted the manuscript. ML, VL, and MC made the diagnosis of KCOT and established the treatment. AM, GM, and MC were involved in the data interpretation, and contributed to the revision of the drafted manuscript. All authors read and approved the final manuscript.


Local excision was curative in all cases except 5 stallions with SCCs. These stallions had extensive damage of the glans penis, free part of the penis and the inner lamina of the internal fold of the prepuce, and they underwent a partial phallectomy with successful outcome. Follow-up information was obtained by visit and telephone inquiries. In conclusion, penile and preputial neoplasms are commonly encountered in elderly male horses and SCCs are the most common type affecting male external genitalia. Partial phallectomy is effective for management of equine neoplasia if they are confined to the glans and body of the penis and there is no proximal spread or involvement to regional lymph nodes.Penile and preputial neoplasia in horses occurs infrequently and represents diagnostic and therapeutic challenges. The present study was carried out on a total number of 21 equids  suffered from different penile and preputial neoplasia. Diagnosis of neoplasms was based up on history of the case, clinical examination as well as histopathological evaluation. Animals with penile and preputial neoplasms were underwent local excision and partial phallectomy with a slightly modified version of the techniques described by William's. The diagnosed neoplasms were penile and preputial squamous cell carcinomas (SCCs;  Penile and preputial neoplasia is more prevalent in equine than in other domestic animals; they occupied about 6 and 10% of all neoplasms in equine . SquamouSCCs are the commonest neoplasm involving the penis, prepuce, or both . It reprFibrosarcoma is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. It is unusual mesenchymal tumor in male genitalia of man , horse , and catEquine sarcoid is a locally aggressive, fibroblastic skin tumour, which can appear as a flat or cauliflower-like growth either singularly or multiple growths, usually in young horses . SarcoidMelanocytic tumours have been reported to affect all parts of the penis and prepuce other than the glans , 9, 16. Recently, a standard protocol has been proposed to aid treatment selection of horses with penile and preputial tumours. Treatments were ranged from minimally invasive interventions  to radical surgery . SurgicaLocal excision is indicated to remove small, solitary tumours, such as focal SCCs, squamous papilloma, and sarcoid, where there is no evidence of metastasis to regional lymph nodes or invasion to deeper structures. Local excision is usually curative for horses with melanocytic nevi and solitary dermal melanomas , 23.Partial phallectomy, such as Scott's, Williams', or Vinsot's techniques, is indicated only if the distal portion of the penis (the glans and shaft of the penis) is involved and there is no proximal spread and without involvement of the prepuce and regional lymph nodes , 5. InvoThe present study aimed to examine various types of penile and preputial neoplasia in horses and donkeys in our region and investigate the results of the surgical intervention using a modified version of Williams's technique for partial phallectomy. The present study was carried out on a total number of 21 equids (14 horses and 7 donkeys) and diagnosed different penile and preputial neoplasia Tables . DiagnosClinical examination was performed of the penis and prepuce. Examination of the penis itself and the internal fold of the prepuce required sedation with intravenous detomidine 1%  in a dose of 0.01 mg/kg. body weight. Superficial inguinal lymph nodes were examined.Each animal was held off feed for 12 hrs preoperatively and received procaine penicillin  and phenylbutazone  prior to general anesthesia. μg/mL), 40 mg midazolam (0.04 mg/mL) and 2 gm ketamine (2 mg/mL) dissolved in 1 liter of dextrose 5% in animals either underwent local excision or partial phallectomy.Anesthesia was induced and maintained by infusion of a freshly prepared mixture of 3 mg medetomidine  no. 0 and appositional sutures (simple interrupted).This modified technique was performed in five stallions diagnosed SCCs at the glans penis (3 horses), free part of the penis, and the inner lamina of the internal fold of the prepuce (2 horses).Horses were positioned in dorsal recumbency. A catheter was placed in the urethra and advanced into the bladder as a guide to aid in dissection of the urethra and to evacuate urine at the end of the procedure. Soft-roll gauze was used to tie the free part of the penis to an overhead support to facilitate aseptic preparation. The glans and proximal free part of the penis were covered in a sterile bandage. A latex tourniquet was placed proximal to preputial ring. The penis, prepuce, and the surrounding ventral abdomen were aseptically prepared and the area was draped. A triangular incision (base 3 cms and sides 5 cms) was made through the penile skin on the ventral midline of the penis, with the apex pointing in a proximal direction. The base of the triangle was proximal to corona glandis and it was the site of penile transection. This triangle was bluntly dissected. A longitudinal incision was made into the urethra from the apex to the base of the triangle. The incised edges of the urethral mucosa were opposed to the epithelial edges of the sides of the triangle with simple continuous sutures  using poThe preoperative antibiotic and anti-inflammatory were continued for 5 days. A prophylactic dose of antitetanic serum was injected. Wound dressing using povidone iodine was performed twice daily. Animals were confined to a stall without exercise for 5 days after surgery.Samples of the excised masses were fixed in neutral buffered formalin 10%. The specimens were sectioned at 5 microns, stained with hematoxylene and eosine according to Bancroft et al. .Follow-up information was obtained by visit and telephone inquiries. Owners were questioned as to the horse's general health status, recurrence of masses, complications from the surgical procedure, and the ability of the operated animals to urinate freely.n = 9) and preputial (n = 6) SCCs, penile fibrosarcoma (n = 1), penile (n = 1) and preputial (n = 3) sarcoid, and preputial melanoma (n = 1) which was detected in a stallion with SCCs  who underwent partial phallectomy  showed various types of penile and preputial neoplasms . The diallectomy . SCCs were detected in 15 equids representing 71.4% of the total recorded cases Tables . The aniLocal excision was curative in 10 cases with SCCs. The other five stallions showed 5-7 mm pedunculated masses present on glans penis, 4 mm friable mass within the urethral fossa, and 5 mm mass located on the free part of the penis. Preputial bleeding and an intense, pungent odour were found. These 5 stallions underwent partial phallectomy with A modified Williams technique. Good recovery without recurrence was obtained in three horses one year after surgery, while the other 2 horses showed regrowth of the neoplasia at the stump of the penis 5 and 7 months after surgery. Surgical excision was performed in the two cases for a second time with successful recovery without recurrence one year after surgery.Two horses and one donkey had been treated by local excision of the SCCs and showed regrowth of the neoplasia 3 months after surgery. Surgical excision was performed in the two cases for a second time with successful recovery without recurrence 9 months and 16 months after surgery.Penile fibrosarcoma was diagnosed in a stallion representing 4.7% of the total neoplastic cases. Examination of the penis revealed a large ulcerated soft tissue mass on the shaft of the penis. Microscopically, tumor cells showed mesenchymal origin characterized by spindle cells in nodular whorls or streams and bundles, high nuclear to cytoplasm ratio, and oddly shaped large nuclei, without inflammatory component and necrosis. Successful outcome was obtained after local excision without recurrence 13 months after surgery. Penile and preputial sarcoids were detected in 2 donkeys and 2 horses with an average age (2–5 years) and representing 19.2% of the total neoplastic cases. They appeared singular or multiple cauliflower like growths Figures . MicroscA case of melanoma was detected in one stallion with SCC  who underwent partial amputation of the penis. It appeared as a solitary, firm, nodular, and hairless mass with intact skin at the prepuce . The masPostoperative oedema was observed in 3 animals operated via partial phallectomy. It receded after 4 days and urination was not impeded. Intermittent haemorrhage associated with urination was observed frequently 2 weeks after partial phallectomy in one stallion, but it stopped spontaneously without the need for further surgery or any other specific therapy.Partial phallectomy is generally regarded as a salvage procedure in stallions for purposes other than breeding . VariousSurgical intervention aims to remove lesions and adequate margins to reduce the risk of recurrence . PartialUnsuccessful outcome  of SCCs treated by partial phallectomy is not uncommon 43.5%). This was possibly as a result of the limitation to the amount of tissue that can be removed combined with the frequent presence of the more proximal part of the penis or the prepuce of premalignant tumors, such as papillomas that may subsequently transform to SCCs [%. This wTwo horses and one donkey treated via local excision of the SCCs underwent a second surgery three months after the first surgery due to the regrowth of the mass. This was consistent with the finding of , 5. TumoCastration of stallions several weeks before phallectomy is advisable to help in modification of behavior , 32, 33.SCCs appear as single or multiple, raised and ulcerated. Advanced lesions often appear as florid, cauliflower-like masses with areas of necrosis, ulceration, and hemorrhage. They are the most common form of penile and preputial neoplasms in horses . RecurreIn this study, three horses that operated via a slightly modified phallectomy were cured without incidence of recurrence one year after surgery. While two cases showed a local recurrence of the masses at the stump of the penis and tunica albugenia, this may be due to that those horses were affected by a more advanced stage of disease at the time of surgery than that was appreciated. This might have involved spread to inguinal lymph nodes or the presence of tumour in the penile or preputial tissues remaining after surgery. In none of these horses, the superficial lymph nodes were palpably enlarged at the time of surgery, suggesting that palpation of the lymph nodes may not be effective in identifying early metastasis , 5, 12. Haemorrhage is frequently observed after phallectomy especially during and immediately after urination , 37. TheIn this study, local excision of the smaller penile and preputial neoplasms which are not invading the tunica albugenia was considered the radical method of treatment. It is economic, fast, and mostly curative. However, this sort of treatment has a high risk of incomplete removal followed by recurrence . If the In this study, a case of fibrosarcoma was recorded on the body of the penis which is an uncommon tumor of the external genitalia of male horses . Van DenFour animals in this study showed penile and preputial sarcoids. In the surgically treated cases, 75% of the treated sarcoids (3 cases) did not recur throughout the time of followup. This was considered a satisfactory success rate. However, this success rate could probably be due to the seat of the sarcoid on prepuce and removal of a wide margin of normal tissues around the sarcoid to avoid the recurrence of the lesion. This was in agreement with previous results obtained by , 39–41.Melanoma is a common, generally slow growing, locally invasive tumour, estimated to occur in approximately 80% of ageing grey horses , 23. GarIn conclusion, the results of this study confirmed that partial phallectomy with a slightly modified version of Williams's technique is an effective therapy for horses if neoplasia is confined to the glans and body of the penis and there is no proximal spread without involvement of the prepuce and regional lymph nodes. Penile and preputial tumours are commonly encountered in elderly male horses except sarcoids in 2–5 years old, and SCCs are by far the most common type of tumour affecting male genitalia. Fibrosarcoma is uncommon tumours of the equine penis.


An acetabular location of osteoid osteoma is rare and represents less than 1 % of cases. The purpose of this clinical case report is to highlight the unusual location of osteoid osteoma and the technical difficulty of its removal.We report a case of a 17-year-old Moroccan Arab boy who presented with pain in his right hip with lameness. The diagnosis of osteoid osteoma was made by imagery and confirmed by histological examination. The treatment consisted of a complete percutaneous resection scanno-guided of the nidus. The evolution was marked by complete healing with total and definitive disappearance of symptoms after 1 year.Osteoid osteoma of the acetabular roof is rare. The diagnosis is now easy because of the evolution of imaging. Treatment is exclusively surgical. Complete resection guarantees the absence of recurrence. The difficulty of the surgical procedure is due to the deep localization of the osteoid osteoma and because it is endosseous, it is also dangerous due to anatomical relationships and the small size of the osteoid osteoma. Osteoid osteoma is a common benign bone tumor. It represents 10 to 12 % of all benign bone tumors [A 17-year-old Moroccan Arab boy without significant medical history presented last year with pain in his right hip that was predominantly nocturnal and sensitive to aspirin. A clinical examination showed lameness while walking without limitation of hip movements. The results of laboratory tests were normal. Plain radiographs of his right hip did not show any visible lesion. A computed tomography (CT) scan showed a 10 mm osteoid osteoma located in the roof of the right acetabulum  is justified especially in cases of doubt in the diagnosis as in unusual localizations, such as juxta-articular or intramedullar. It shows not only the nidus but also the inflammatory reaction modifications in bone marrow and surrounding soft tissue. The use of dynamic sequences after gadolinium injection improves the sensitivity of MRI 92 %) so much that it becomes equal to that of CT [2 % so muTreatment of osteoid osteoma is surgical. Bloc resection has been the standard therapy. However, difficulty in locating the nidus, despite using preoperative or perioperative imaging, results in a wide bone resection compared to the small size of the lesion. Open surgical treatment is effective when the nidus is resected completely, but remains a relatively invasive technique which weakens the bones, especially long bones, which can justify resorting to a bone graft or internal fixation osteosynthesis and postoperative immobilization . The perPercutaneous resection under CT guidance is an effective technique and noninvasive. It is practiced in the scanner room under strict aseptic conditions and under local or general anesthesia. It is useful especially in deep locations such as the acetabulum because it avoids dislocating the hip for a direct approach. This procedure uses a coaxial system comprising a guidewire, a trocar mandrel and a toothed outer sheath to protect the soft tissue, a cannulated drill bit to penetrate the bone to the edge of the nidus in the case of a deep lesion, and a trephine of 3 to 8 mm in diameter for the excision of the nidus. Some use a motor to move the wick and the trephine.A CT control checks the total resection of the nidus. In a case of incomplete excision, a new borehole can be done. Histological evidence of the presence of a nidus within the resected bone core is reported in 50 to 100 % of cases depending on the series .Other less invasive alternatives have been developed, mostly under CT guidance, comprising alcoholization, thermocoagulation by radiofrequency, laser photocoagulation, and cryotherapy. Currently, surgery is reserved for percutaneously inaccessible locations  or locations adjacent to neurovascular structures (<1 cm) .From the first postoperative hours, the virtual disappearance of pain or complete attenuation supports the use of a total resection. Pain disappears after 24 hours in most cases, but can sometimes persist for a month . The diaOsteoid osteoma is a small benign bone tumor. It is rarely located on the acetabulum. CT is the gold standard for diagnosis. The use of MRI is justified if there is doubt in the diagnosis especially in unusual localizations, such as juxta articular or intramedullar forms. Treatment of osteoid osteoma is exclusively surgical. Percutaneous resection under CT guidance is an effective technique, especially in the forms with deep anatomical location, where it is extremely efficient. It allows perioperative control with high precision, lower complication rates, faster recovery and probably a reduction in recurrences. Several procedures and techniques for treating osteoid osteoma percutaneously are currently available to the clinician. Surgical treatment is reserved for percutaneously inaccessible locations or locations near neurovascular structures.CT, computed tomography; MRI, magnetic resonance imaging


Cardiac tumors, either benign or malignant, are difficult to diagnose due to their rarity, variety, and nonspecific presentation. Since primary cardiac sarcoma remains an unusual diagnosis, the literature on its presentation, diagnosis, and optimal management remains scarce. To our knowledge the following case of cardiac perivascular epithelioid cell tumor is the fourth reported case found in the literature. Although complete surgical resection remains the gold standard for cardiac sarcomas, our case demonstrates that not all of them can be completely resected. The prevalence of primary cardiac tumors is 0.001–0.03% in autopsy series, with malignant tumors accounting for 25% of cases [Resection remains the primary mode of treatment for primary cardiac sarcomas (PCSs). However, PCS usually has a high recurrence rate of up to 50% even after resection, and the prognosis remains dismal . Early dA 64-year-old Caucasian female with a past medical history significant for hypertension and hyperlipidemia presented to our institution with progressive shortness of breath on exertion, bilateral lower extremity edema, and a chronic dry cough for the past 3 months. She also complained of a decreased appetite and a 12-pound weight loss over the past 2 months. She denied orthopnea or paroxysmal nocturnal dyspnea. She had no previous smoking history and no significant surgical or family history. Physical examination revealed sinus tachycardia, bilateral pitting pedal edema, and basilar crackles in both lungs. Laboratory diagnosis showed leukocytosis (13.2), microcytic anemia , and thrombocytosis (524).Chest X-ray displayed cardiomegaly with increased interstitial pulmonary markings and small bilateral pleural effusions see . On lateCardiac magnetic resonance imaging (CMRI) showed that the mass was cystic and solid in nature and again arising near anterior pericardium see . A CT guAfter discussion with the patient and her family, the decision was made to perform surgery for a complete resection of the tumor. However, at surgery it was discovered that the mass was actually arising from the pericardium and wedged underneath it. Attempts to debulk the tumor demonstrated that it had already infiltrated the myocardium. Thus, the cardiac tumor was deemed unresectable.Pathology results of the partial tumor excision showed high-grade sarcoma with features favoring malignant perivascular epithelioid cell tumor see . The debPostoperatively, the patient required vasopressor support and intubation for respiratory failure. Vasopressors were weaned over the following 48 hours and she was eventually extubated. She refused any further chemotherapy or radiotherapy and opted for home with hospice care. She died 6 months following surgery.Primary cardiac sarcomas may occur in any chamber of the heart. Clinical presentation is often nonspecific and depends upon the location of the tumor. Patients can present with generalized symptoms including subjective fevers, weight loss, generalized weakness, and fatigue. Additionally, tumors can infiltrate the myocardial wall and lead to cardiomyopathy and heart failure symptoms as in our case. Furthermore, tumor infiltration into the neural pathways can cause arrhythmias and atrioventricular block. Also, intracavitary tumors, that is, when they protrude inside the atrium or the ventricle, can result in cerebrovascular accidents and pulmonary emboli when they become detached. In some rare cases, the first manifestation of a cardiac tumor is sudden cardiac death .Generally, clinical history and echocardiography can help the diagnosis in most cases. Lab findings can be nonspecific and include leukocytosis, anemia, thrombocytosis, and elevated ESR. Echocardiography represents a substantial imaging technique for the detection of cardiac tumors with high sensitivity and specificity  . Newer tSurgery remains the backbone of management for cardiac sarcomas . The mosThe response of soft tissue sarcomas to radiation has been well documented and currently adjuvant radiation is recommended along with surgical resection to improve overall survival . ChemothUnfortunately, survival from the time of diagnosis varies from 7 months to a maximum of 2 years and there is insufficient evidence to define optimal treatment , 7. On lHeart transplantation can occasionally be considered as a final treatment option in individual cases, provided distant metastases can be ruled out , 8. One Perivascular epithelioid cell tumor (PEComa) is recognized as a very rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells , 10. PerA number of the morphologic features of PEComas, including the admixture of spindled and epithelioid forms, the occasionally prominent nucleoli, and the presence of multinucleated cells, are also seen in melanoma and clear cell sarcoma. PEComas can histopathologically be confused with carcinomas, smooth muscle tumours, adipocytic tumours, and gastrointestinal stromal tumours (GIST) .Based on our literature research, this perivascular epithelioid cell tumor is the fourth reported case found in the heart . The preGeramizadeh and colleagues  describeTazelaar et al. described a case of a 29-year-old man with a seven-year history of atrial fibrillation presented with symptoms of mitral stenosis . ThoracoTai et al. reported a case of cardiac PEComa in a 10-year-old girl who had presented with 8 years of cardiac murmur and 2 weeks of increasing dyspnea. Chest computed tomography showed a solid mass located in the left atrioventricular groove. At surgery a 5.5 × 3 × 4.8-cm bulging mass was detected in the left atrioventricular groove. The base of the neoplasm was fixed in the posterolateral wall of the left cardiac ventricle. The slightly firm tumor was well defined with a smooth external surface. The tumor protruded from the cardiac wall into the pericardial cavity with massive effusion. A total tumorectomy was performed, and the postoperative course was uneventful. The patient is alive and well without evidence of disease 18 months after surgery .When a cardiac tumor is confirmed, it is crucial for oncologists and surgeons to collaborate. These tumors are of a particular concern due to the fact that overt signs and symptoms occur rather late in the course, precluding effective tumor eradication as in our case. Perhaps if neoadjuvant therapy was attempted, complete surgical resection may have been possible.


Free perforation is one of the most feared complications of the intestinal tuberculosis. The terminal ileum is the most common site of perforation, while the majority of (90%) perforations are solitary. Herein, we describe a case of a 25-year-old male who presented with generalized peritonitis requiring an emergency exploratory laparotomy, which revealed pan-enteric perforation characterized by multiple perforations of the small bowel extending 10–15 cm from the DJ flexure up to the terminal ileum. The perforations were primarily closed, while 6–8 cm of the diseased terminal ileum was resected and the two ends were brought out as double-barreled ostomy. To the best of our knowledge, such an extensive tuberculous perforation of the small bowel has not been previously reported in the literature before. Intestinal tuberculosis is a disease of underdeveloped countries. However, the incidence is increasing in the Western countries due to increased influx of immigrants as well as increased incidence of HIV infection and increased use of immunosuppressive drugs. Perforation is an uncommon but life-threatening complication of intestinal TB, associated with high morbidity and mortality. We describe a patient who developed acute tuberculous pan-enteric perforation. Such a manifestation of intestinal TB has not been previously reported in the literature.A 25-year-old male presented to the emergency department with complaints of progressively worsening abdominal pain for the past 3 days, occurring on the background of fatigue and weight loss for the past 8 months.6. His electrolytes were within normal limits, but his serum albumin was 1.6 g/L. An erect chest X-ray showed free air under diaphragm , hypotensive (80/50 mmHg), and tachycardic (110 bpm). His abdomen was diffusely tender with board-like rigidity, while digital rectal examination (DRE) was unremarkable. His hematologic investigations revealed low hemoglobin (9 g/dL) and TLC of 15.4 × 10iaphragm .After initial assessment a diagnosis of generalized peritonitis secondary to hollow viscus perforation was made. He underwent emergency laparotomy, with intraoperative findings of multiple small bowel perforations, starting 10–15 cm from duodenojejunal (DJ) junction, extending up to the terminal ileum . On closSince the patient was hemodynamically unstable and severely wasted, such an extensive small bowel resection was not an option due to the high intraoperative and postoperative mortality risk. Therefore, a more conservative approach was selected. The perforations were primarily closed by Lambert sutures, using Vicryl 2-0 , while tHe was subsequently started on antituberculous therapy based on clinical suspicion, and parenteral nutrition was also initiated. The histopathologic examination of the resected bowel specimen and the mesenteric lymph nodes was consistent with intestinal tuberculosis. The patient was closely watched for signs and symptoms of postoperative leak; however, he made a slow but progressive recovery and is currently being followed up in the clinics with ultimate plan of ileostomy closure.Intestinal TB is generally a disease of underdeveloped countries; however, due to an increase in the number of immigrants as well as HIV-infected patients, the incidence of intestinal TB is rising in the Western countries . Only a Free perforation is a complication of severe untreated disease, occurring in 1–15% of patients with intestinal TB , 4. The Dasgupta et al. studied tissue samples from patients of abdominal TB that had surgical intervention and perforation was seen in 80% of the cases, mostly of the terminal ileum and being solitary in nature, with epithelioid cell granuloma visible in lymph nodes and the intestinal tract [Antituberculous chemotherapy is the mainstay of intestinal TB management; however, the duration of treatment is not uniform, ranging from 6 to 12 months . Our patSurgery is reserved for complication cases, such as obstruction, hemorrhage, perforation, abscess, and fistula formation. The most effective surgical therapy in perforated cases is resection of the affected segment with end to end anastomosis . HoweverIn summary, the case presented here is the first one to be reported in the literature that describes such an extensive perforation of almost the entire small bowel. Considering the fact that the incidence of intestinal TB is rising in both underdeveloped and Western countries, the aim of this study is to make the surgical community aware of such an atypical manifestation of the intestinal TB so that they are more prepared if such a case is encountered in the future.


Despite cardiac metastases are found in about 20% of cancer deaths, the presence of primary cardiac tumors is rare. Most primary tumors are benign, and malignant tumors comprise about 15%. We report a 21-year-old man with fever, dyspnea, and hemoptysis that was diagnosed with angiosarcoma of the right atrium and pulmonary metastasis. Patient was submitted to surgical tumor resection without adjuvant therapy and died four months after diagnosis. Sarcomas are the main malign primary heart tumor , with hiAfter approval by the Ethics and Research Committee, we describe the case of a 21-year-old man with hemoptysis, fever (38°C), dyspnea, and weight loss not quantified, with any changes in the physical examination. The symptoms were treated as community acquired pneumonia, with partial improvement after antibiotic therapy. After recurrence of symptoms, chest X-ray showed new hypotransparency juxtaposed to the heart area. Magnetic resonance imaging (MRI) confirmed a large heterogeneous solid mass measuring 12 × 10 × 9 cm, located above the right atrium (RA) and superior vena cava (SVC) with extrinsic compression of the joint without cleavage plane. Evidenced still small lesion measuring 3 cm, capturing contrast, in the posterior segment of upper lobe of the right lung, a calcified nodule measuring 1 cm, not capturing contrast, in the middle lobe and residual pleural effusion. The electrocardiogram (ECG) showed sinus tachycardia and nonspecific repolarization changes. In the National Cancer Institute, patient underwent transthoracic biopsy, with diagnosis of solitary fibrous tumor. During hospitalization, the patient developed massive right hemothorax, diagnosed by Computed Tomography (CT) scan without contrast, as shown in The peroperative transesophageal echocardiogram showed hemodynamic compromise caused by compression of RA with resolution after resection Figures . CytopatThe patient developed pulmonary sepsis and vasodilatatory shock complicated with ischemic hepatitis and renal failure requiring hemodialysis and was discharged after clinical improvement and awaiting adjuvant therapy, which was not performed. By telephone contact 4 months after surgery, we were informed by relatives that the patient was admitted at emergency department because of respiratory failure two weeks before the telephone contact. He died.Angiosarcoma is a disease of difficult diagnosis and reserved prognosis , correspThe most common symptoms include dyspnea, chest pain, heart failure (mainly right), palpitations, fever, and myalgia. Other manifestations are less frequent: pericardial effusion with or without tamponade, obstruction of the vena cava, conduction disorders , hemoptyComplementary exams are useful in the diagnosis of primary tumor, in assessing the extent of the disease and the presence of metastasis . The ECGThe approach of pericardial fluid allows the analysis of cytopathology, positive in 75–87% of cases. Pericardioscopy guided biopsy has a yield of around 93–97%. A less invasive procedure is the transvenous endocardial biopsy, but it is often false negative . This prDue to the rarity, studies to establish the treatment of cardiac angiosarcomas are scarce . SurgeryOrthotopic transplantation could allow complete resection of the tumor, being performed in selected cases due to risk of tumor or metastatic recurrence related to immunosuppressive treatment .The case described is consistent with those found in the literature. Cardiac tumors have nonspecific signs and symptoms. Most clinical presentation reflects hemodynamic impaired of local attempt. Diagnosis is established by image and histology analysis of the tumor. Prognosis is reserved with scarce evidence. The surgery has brought relief of symptoms of the disease and local control. The patient survival of 11 months was already expected.


FLCN) gene. Clinical manifestations of BHD include skin fibrofolliculomas, renal cell cancer, lung cysts and (recurrent) spontaneous pneumothorax (SP). All clinical manifestations usually present in adults > 20 years of age.Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominantly inherited disorder caused by germline mutations in the folliculin  pneumothorax starting at age 14 accompanied by multiple basal lung cysts on thoracic CT underwent Although childhood occurrence of SP in BHD is rare, these two cases illustrate that BHD should be considered as cause of SP in children. FLCN) gene located on chromosome 17p11.2. Clinical manifestation usually appears after the age of 20 years. We here present two cases of BHD with episodes of recurrent pneumothorax of which the first episode occurred at the age of 14 years.Birt-Hogg-Dubé syndrome (BHD) is a rare tumour predisposition syndrome first described in 1977  is a rare autosomal dominant inherited disorder caused by germline mutations in the  have published different recommendations for treatment of primary SP in adults as well paediatric patients. Multiple studies in adult literature advocate aspiration as a first treatment option in PSP. For secondary SP or recurrent SP the ACCP and BTS recommend tube thoracostomy and surgical recurrence prevention ,25. WhetIn conclusion we report here two cases of Birt-Hogg-Dubé syndrome in children with recurrent pneumothorax. As SP in the paediatric population is relatively rare, BHD should be considered as underlying cause, especially when there is a positive family history for pneumothorax, skin fibrofolliculomas or renal cell cancer. BHD patients are at increased risk for renal cell cancer and therefore diagnosis is important for the affected children and their family members. Therefore we suggest that a thorough family history, low dose chest CT (with the restraints of using CT scans in the paediatric population) and easy accessible genetic testing for BHD are important options for paediatric patients with (recurrent) SP should be performed, even when skin manifestation are absent. We also suggest more research is needed on the prevalence of BHD in the pediatric population with a history of (recurrent) SP.Written informed consent was obtained from both patients for publication of this Case report and any accompanying images. Both patients were over the age of 18 when the manuscript was submitted to this journal.The retrospective data collected for this Case report was part of patient care and was performed in accordance with the Declaration of Helsinki. Therefore exemption was granted by our ethics committee. We obtained written informed consent from both patients for publication of this Case report and any accompanying images. Copy of written consent forms are added as additional supplement.As SP in the paediatric population is relatively rare, BHD should be considered as underlying cause, especially when there is a positive family history for pneumothorax, skin fibrofolliculomas or renal cell cancer. BHD patients are at increased risk for renal cell cancer and therefore diagnosis is important for the affected children and their family members. Therefore we suggest that a thorough family history, low dose chest CT (with the restraints of using CT scans in the paediatric population) and easy accessible genetic testing for BHD are important options for paediatric patients with (recurrent) SP should be performed, even when skin manifestation are absent. We also suggest more research is needed on the prevalence of BHD in the pediatric population with a history of (recurrent) SP.BHD: Birt-Hogg-Dubé syndrome; FLCN: Folliculin; SP: Spontaneous pneumothorax; CT: Computed tomography; MRI: Magnetic resonance imaging; VATS: Video assisted thoracoscopic surgery; BTS: British thoracic society; LCH: Langerhans cell histiocytosis; LAM: Lymphangioleiomyomatosis; LIP: Lymphocytic interstitial pneumonia; LCDD: Light chain deposition disease; ACCP: American College of Chest Physicians; PSP: Primary spontaneous pneumothorax.All authors have no conflicts of interest to disclose.PCJ collected the data, provided a substantial contribution to conceptation, design and drafting/writing of the manuscript, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. BEEMvandenB provided a substantial contribution to the drafting/writing of the manuscript, reviewed and revised it critically, provided the clinical data of patient case 1 as attending pulmonologist, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. JJPG provided the genetic testing and analysis on both patients. Provided a substantial contribution to review and revise the manuscript critically, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. AFN provided a substantial contribution to review and revise the manuscript critically, supervised the analysis and interpretation of the clinical patients data, supervised as attending pediatrician, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. JHTMvanW, provided a substantial contribution to review and revise the manuscript critically, provided and interpreted the thoracic imaging of both patients as attending radiologist, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. MAP, provided a substantial contribution to review and revise the manuscript critically, provided the clinical data of patient case 1 and 2 as attending thoracic surgeon, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. RJAvanM, provided a substantial contribution to review and revise the manuscript critically, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. FHM, provided a substantial contribution to review and revise the manuscript critically provided the genetic testing on Folliculin of patient case 1 and 2, contributed as the attending clinical geneticist of both families, gave final approval of the version to be published and agreed to be accountable for all aspects of the work. PEP, provided a substantial contribution to conceptation, design and drafting/writing, contributed to review and revise the manuscript critically, contributed as attending pulmonologist of patient case 2, gave final approval of the version to be published and agreed to be accountable for all aspects of the work.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2431/14/171/prepub


In this case report we present a 60-year-old male patient with overt midgastrointestinal bleeding of a primary ileal pleomorphic liposarcoma diagnosed by video capsule endoscopy (VCE). Clinical work-up for final diagnosis and the pathological background of this uncommon tumorous entity of the small bowel will be discussed in this paper. Severe gastrointestinal bleeding is a medical emergency situation, asking for rapid diagnosis and treatment. In case of suspected mid-gastrointestinal bleeding, following negative gastroscopy and colonoscopy, video capsule endoscopy (VCE) is known to be an effective examination for the diagnosis of the hemorrhagic site .In this case report we present the clinical and the pathological findings of a rare tumor of the small bowel that lead to severe gastrointestinal bleeding—a primary ileal pleomorphic liposarcoma.We report a 60-year-old male patient who was hospitalized due to gastrointestinal bleeding. The patient initially presented to the referring hospital with melaena and symptomatic anemia (hemoglobin 6.3 g/dL). Similar episodes of melaena, however non-Hb-relevant, were reported by the patient since 2009. The patient's medical history showed chronic renal failure, hypothyreosis, and hypertension. The patient was on regular therapy with acetylsalicylic acid (100 mg/day), thyroxin, blood pressure medications, and a statin.Gastroscopy and colonoscopy showed no source of hemorrhage; however, fresh blood was detected in the terminal ileum.At time of initial admission to our hospital, the patient's hemoglobin value was still low (6.7 g/dL), although he had already received two packed red blood cells (RBCs). The patient received four more RBCs during the first three days of hospital stay; afterwards Hb value proved to be stable and no more RBCs were needed.Following admission to our hospital, we conducted emergency single-balloon enteroscopy (Olympus SIF Q180) of the proximal small bowels, reaching into the distal jejunum, without identification of the source of hemorrhage. In addition, colonoscopy with deep penetration into the ileum (approximately 30 cm) was repeated without detection of the hemorrhagic site. There was no evidence of fresh or old blood in the ileum or colon.Subsequent video capsule endoscopy  was not able to detect the bleeding site due to insufficient bowel cleansing, because of feces and hematin in the middle/distal small bowels, even though laxative preparation was adequate for prior colonoscopy. After additional laxative bowel cleansing  of the intestines and after ceasing of hemorrhagic signs, second capsule endoscopy  revealed a centrally exulcerated, subepithelial tumorous lesion with luminal growth in the ileum . Again, Supplementary contrast-enhanced computed tomography (CT) for staging purpose was not able to detect the tumorous lesion of the small bowels—there were no signs of local or distal metastases.The patient was referred for surgery. Following median laparotomy, the tumor could be palpated approximately 40 cm orally to the ileocecal valve. Second a Meckel's diverticulum was found aborally to the tumor without signs of inflammation. Ileum-segment resection (30 cm) and side-to-side ileoileostomy were performed. Seven days postoperatively the patient could be discharged without any residual impairment or further blood loss.Histologically the small tumor (max. diameter 1.8 cm) showed well-defined borders and consisted of a mostly solid and haphazard proliferation of undifferentiated cells in a mostly solid, partly pericytoma-like growth pattern. The tumor emerged from the submucosa with polypoid protrusion of the mucosa , withoutIn general, liposarcomas are one of the most common soft-tissue sarcomas in adults. However, manifestation in the small bowel is rare , 3. The In the detection of small bowel hemorrhage, video capsule endoscopy (VCE) and balloon-assisted enteroscopy (BAE) are techniques with a similar diagnostic yield . HoweverIn clinical practice, VCE is usually used as the first diagnostic approach to the small bowels, enabling localization and assessment of the bleeding site, and can then be followed by BAE for endoscopical haemostasis . Hereby,Therefore, in this case of severe hemorrhage, we decided to perform antegrade BAE as the primary approach to the small bowel in order to achieve rapid endoscopical haemostasis. In the retrospective, and this underlines the importance of VCE in severe bleeding, VCE performed prior to balloon enteroscopy could have predicted the bleeding site and antegrade balloon-assisted enteroscopy would not have been necessary. Moreover, retrograde balloon-assisted enteroscopy or surgery could have been performed at an early stage.Retrograde balloon-assisted enteroscopy was not performed in this case, because the tumor was already diagnosed by VCE and the indication for primary surgery was decided, based on estimated intraoperative identifiability of the tumor. However, some  tumors might not be easily palpated during surgery. Thus, retrograde BAE after VCE might have been useful in order to mark the tumor for scheduled surgery. Since the liposarcoma was located only approximately 40 cm orally to the ileocecal valve, the effort for BAE could be regarded justifiable to eventually ensure a safer subsequent surgical resection.Complete surgical resection is the best treatment of liposarcomas of the small intestine, given that it is diagnosed at an early stage with no evidence of distant metastasis . In our In conclusion, the proper application of video capsule endoscopy is an important method for the diagnosis of small bowel hemorrhage. In the current case VCE revealed the uncommon finding of a primary liposarcoma of the small bowel. Surgical resection was conducted and the patient is expected to have a good prognosis.


Pilomatrixoma is a common benign soft tissue neoplasm arising from hair follicle cells, typically not exceeding 3 cm and located mainly within the head and neck regions. Lesions greater than 3 cm or those located elsewhere are rare and are often not thought of or high on a differential diagnosis. Moreover, the radiographic features of pilomatrixoma are very nonspecific making the diagnosis even more difficult and rarely described in the radiology literature. We present the largest reported case of pilomatrixoma measuring 24 cm arising from the posterior thorax. Our hope is to increase awareness of this diagnosis for slow-growing soft tissue masses not located in the classically described locations of head and neck, explore the radiographic features on various imaging modalities, and review the current radiology literature. The white count was slightly elevated 14.5 (4.5–11.0 K/μL) but normalized quickly after a short course of antibiotics. Surgery was consulted and a contrast enhanced CT of the chest was ordered to delineate the full extent of the mass and affected surrounding tissues. The CT revealed a massively large, heterogeneously enhancing, partially calcified, centrally necrotic mass immediately deep in the skin in the subcutaneous tissue of the thoracolumbar back region (We present a 52-year-old gentleman with a large mass in the upper back, which originally started as an “itchy bump” 9 years ago and has progressively doubled in size each year. He presented to the urgent care clinic because of bleeding and purulent discharge from the mass and subsequently he was referred to the emergency department for further workup. Lab results showed normocytic anemia (RBC 3.29 (4.7–6.1 M/k region . This mak region . This reWide-excision surgery was performed and pathology revealed a massive soft tissue tumor of the back consistent with pilomatrixoma and with clean margins. The tumor measured 24 cm × 21 cm × 9 cm and was yellow-tan, well marginated, encapsulated, and firm. There was a cavity in the center of the tumor containing debris and pus Figures  consistePilomatrixoma was first termed as “calcified epithelioma of Malherbe” thought to originate from sebaceous glands by Malherbe and Chenatais in 1880. This was subsequently renamed by Forbis and Helwig in 1961 after demonstrating that its origin was actually from the hair follicle matrix cells . PilomatHistologically, pilomatrixomas are nodular aggregates of epithelial cells scattered within a connective matrix with each nodule composed of two types of differently organized epithelial cells. The peripheral part is densely packed basophilic cells producing keratin; the central part contains eosinophilic cells known as “ghost” or “mummified” cells , 5. CytoThe radiographic features of pilomatrixomas are very nonspecific findings regarding a soft tissue mass within the subcutaneous tissue demonstrating heterogeneity with variable enhancement and calcification on CT examination. Differential diagnosis would include malignancies such as malignant fibrous histiocytoma or synovial cell sarcoma versus benign tumors such as lipoma, fibromatosis, or hemangioma versus other causes like myositis ossificans, hematoma, or abscess , 8.Pathology is the most definitive means of diagnosis with tissue sampling through either fine needle aspiration or open biopsy. CT would be helpful in surgical staging of malignant tumors or even large benign tumors as in our case.


Dermoid cysts in oral cavity are unusual lesions. Their etiology is not yet clear and can be associated with trapped cells as a result of the inclusion error resulting in the development into the ectoderm, mesoderm, and endoderm tissues. The aim of this case report is to evidence the presence of a dermoid cyst in the floor of mouth surgically removed. In the present case, the lesion showed soft consistency, floating, regular borders, smooth surface, and the same color as the adjacent mucosa, asymptomatic and measuring 4.5 × 5.5 cm in its greatest diameter. The initial diagnostic was ranula in consequence of the similarity with clinical characteristics and localization. After surgical removal lesion, a fibrotic capsule was identified with a friable material with intensive yellow color. The microscopic exam showed cystic lesion with cavity lined by squamous stratified epithelium hyperorthokeratinized. Cutaneous attachments, such as sebaceous glands and hair follicles, were present in connective adjacent tissue. Surgical intervention is elective in these situations. All dentists must have a thorough knowledge of this unusual lesion. Dermoid cysts are developmental lesions that arise either by entrapped pluripotent cells or by implantation of epithelium, with the former being termed congenital and the latter as acquired –3. NowadConsidering the histological aspects, the lesions are classified as epidermoid cyst (lined by only by stratified squamous epithelium and composed by ectodermic layer), dermoid cyst (lined by stratified epithelium with skin adnexa), and teratoid  , 5. ThesUsually these lesions are asymptomatic; however, their slow enlargement can cause obstruction with consequent dysphagia, dysphonia, and at last dyspnea. The size of dermoid cysts is very variable (up to ten cm in diameter) and it depends on their first clinic manifestation , 8. The A 29-year-old black man reported to the Semiology Clinic at Dental School of the University of Cuiabá, Cuiabá-MT. During the intrabuccal exam, the dentist noted a volumetric increase with slow growth in the floor of the mouth, laterally to tongue. The lesion showed soft consistency, floating, regular borders, smooth surface, and the same color as the adjacent mucosa, asymptomatic and measuring 4.5 × 5.5 cm in its greatest diameter . The patBefore the biopsy, the suspected diagnosis was ranula due the similarity to clinical characteristics and localization. An aspiration puncture with a thick needle was done and no material was collected. Based on this, the possibility of a solid or a cystic lesion with semisolid container was considered .The treatment administered in the present case was surgical and the lesion was completely removed . InitialWhen found in oral cavity, dermoid cysts are classified as nonodontogenic lesions and about 7% occur in head/neck region, among them 23% are located at floor of mouth and can be found either lateral to tongue or in the midline , 12. TheThe differential diagnosis of lesions that present as a cyst or pseudocyst of the floor of the mouth includes mucocele, ranula, cystic hygroma, thyroglossal duct cyst, brachial cleft cyst, infectious process, lymphatic malformation, tumors, hemangioma, salivary lesions, and Ludwig's angina only in cases of inflammatory complications . The cliThe treatment of choice is surgical enucleation via an intraoral or extraoral approach, which is facilitated by the presence of a capsule . An intrBased on the case report, it was possible to observe the importance of differential diagnosis in relation to other nodule mass lesions and that the histological aspects are conclusive to define the treatment. Surgical intervention is elective in these situations.


Dear SirA 2-day-old male neonate, born full term through Caesarian section, presented to our department with bilious vomiting since birth and unable to pass meconeum. On examination, the patient had distended abdomen. A mass was palpable in the hypogastrium and left iliac region. His bowel sounds were not audible; rectal examination showed no meconium. His baseline investigations were within normal ranges. Abdominal radiograph showed few gas shadows. Ultrasound of the abdomen was unremarkable except for a large cystic mass in pelvis which was ‘megacystis’ according to radiologist. Upon exploration, a large fluid containing mass was encountered occupying whole left side of the abdomen and was pushing intestine towards right . The mass was the distended urinary bladder with dilated and tortuous both ureters. Intestine of patient was malrotated with multiple adhesion bands. Caliber of distal small intestine, caecum and large intestine were micro sized. However whole length of intestine was patent and no atresia was identified. On the basis of these findings the diagnosis of Megacystis Microcolon Intestinal Hypoperistalsis (MMIHS) was made and an ileostomy was made. Postoperatively total parenteral nutrition was provided to the patient. The biopsies taken from ileostomy site, ascending colon and sigmoid colon, all showed normal ganglion cells. The patient is now 1 month old and is still on total parenteral nutrition and prokinetics.MMIHS is a rare cause of neonatal intestinal obstruction with around 230 cases reported in the literature. [1-3] It was first described by Berdon and colleagues in 1976. [2] Females are 3 - 4 times more commonly affected than males. Its association with consanguinity has been found and it is thought to have an autosomal recessive inheritance. [1] Etiology of MMIHS is unclear and different hypothesis have been proposed including neurogenic, myogenic, genetic and hormonal origin. In most of the patients histologically normal ganglion cells are found; however, in some deficient ganglion cells have also been reported. Puri and colleagues had proposed myogenic origin of this disease and found decreased smooth muscle in wall of intestine. [1] In another study deficient interstitial cells of Cajal have also been reported in intestine and urinary bladder. [4] Prenatal diagnosis had been done in few reported cases in literature on account of distended urinary bladder, hydronephrosis and variation of amniotic fluid level. [5] Generally patients with MMIHS have poor outcome and need long term parenteral nutrition and prokinetics. Some of patients need vesicostomy; however, there is no consensus of doing vesicostomy at initial presentation. Source of Support: NilConflict of Interest: None