We report a rare case that was initially diagnosed as an inflammatory lesion and ultimately confirmed as primary central nervous system lymphoma (PCNSL) in an immunocompetent patient who was not treated with corticosteroid prior to the initial biopsy. A 70-year-old female patient presented with numbness in the left side of face, arm, and leg. Brain magnetic resonance imaging (MRI) revealed a lesion with intense gadolinium (Gd)-enhancement in the ventral portion of the midbrain. A stereotactic biopsy demonstrated mixed T-cell and B-cell infiltrating inflammatory lesions without demyelination. Three months after postoperative treatment with steroid, the lesion markedly decreased on follow-up MRI. Twenty-six months after the initial attack, she complained of dysarthria and urinary incontinence. Repetitive MRI showed a lesion with homogeneous enhancement, extensively involving the bilateral cerebral hemisphere, corpus callosum, and the right middle cerebellar peduncle. The confirmed diagnosis was diffuse large B-cell lymphoma on the second biopsy. Despite our best efforts, she died 38 months after disease onset. Based on review of the literature and our case, preceding inflammatory lesions are not always demyelinating and T-cell dominant inflammatory lesions. When the initial biopsy reveals an inflammatory lesion in an old-aged patient, the clinician should keep in mind the development of PCNSL and perform close clinical and radiological observations for a timely diagnosis. Primary central nervous system lymphoma (PCNSL) is a rare brain tumor, accounting for 3% of all brain tumors and 6% of all extra-nodal lymphomas . In receMedian survival of patients has improved since the introduction of methotrexate-based chemotherapy for PCNSL treatment . TherefoA 70-year-old woman without previous medical history presented with a one-week history of numbness in the left side of face, arm, and leg. A neurologic examination revealed no additional neurologic sign except above-mentioned symptoms. There were no abnormal laboratory findings from blood samples. Brain MRI showed a contrast-enhancing lesion in the ventral portion of the right midbrain with perilesional edema A–C. With suspect of glioma or lymphoma, Leksell frame-based stereotactic biopsy was performed without cerebrospinal fluid (CSF) examination. Biopsy of the lesion showed lymphocytic infiltration composed of mixed CD3+ T-cells and CD20+ B-cells with low Ki-67 index in the perivascular space and glial stroma. Demyelinating features such as foamy macrophages engulfing myelin debris were not detected . DescripBrain computed tomography (CT) and MRI were performed after the biopsy procedure. Proper specimens were sampled with minimal hemorrhage D. After Neuro-navigation guided burr hole biopsy targeting the right frontal lobe revealed diffuse large B-cell lymphoma . A subseWe present a patient with a steroid-unaffected inflammatory brain lesion preceding PCNSL. This rare clinical presentation implies that tumefactive inflammatory brain lesions, not only demyelinating lesions, could be omen of PCNSL development and that close clinical observation is mandatory for such patients. PCNSL can be associated with preceding inflammatory lesions as summarized in a few case reports ,7. In raIt is difficult to distinguish imaging features of space-occupying inflammatory lesions and brain tumors like PCNSL and glioma from the beginning. Although several imaging studies have been performed, they have not clearly contributed to differential diagnosis. MRI features such as T1 hypo-intensity, T2 hyper-intensity, and contrast enhancing are common to that disease. With the metabolic data from positron emission tomography (PET), advanced MRI techniques  may be useful for differential diagnosis and disease monitoring ,9. AfterMost previous studies have reported that preceding inflammatory lesions were histopathologically characterized by a predominance of T cell infiltration with few B cell and demyelination ,10. DemyThere may be questions about the accuracy of the biopsy result. Corticosteroid treatment before initial biopsy can mask clinical symptoms, imaging appearance, and even histological findings of PCNSL. Corticosteroids have a cytolytic effect on lymphoma cells, making them disappear and causing inaccurate diagnosis . As we tThe interval between preceding inflammatory lesions and following PCNSL ranged from 6 months to 4 years . The prePCNSL might emerge unexpectedly due to abnormal lymphocyte proliferation after independent preceding inflammatory lesions. It can be triggered by preceding inflammatory lesions in pro-inflammatory patients . PCNSL aAnderson et al. have named inflammatory demyelinating brain lesions preceding PCNSL as “sentinel lesions” . After tIn conclusion, preceding inflammatory lesions are not always demyelinating lesions or T-cell dominant inflammatory lesions. Inflammatory brain lesions in old-aged patients have a possibility to be an “omen” or “prodromal lesion” of PCNSL. Thus, clinicians must carry out close clinical and radiological observations to reach a timely diagnosis. It is important not to hesitate repetitive biopsy for suspicious lesions.


Leiomyosarcoma usually develops in the myometrium and is characterized by a high recurrence rate, frequent hematogenous dissemination, and poor prognosis. Metastasis is usually to lungs, liver, and bone, and occasionally to the brain, but seldom to the head and neck region. Primary leiomyosarcoma very rarely arises in the broad ligament.A 54-year old woman presented to the otolaryngology department with a mass in the right posterior region of the neck 4 years after surgery for a primary leiomyosarcoma of the right broad ligament. The neck mass was removed and found to be a metastatic leiomyosarcoma. Leiomyosarcoma localizations in lungs and liver were absent. Morphological examination showed both the primary and the secondary leiomyosarcomas to have features of low-grade tumors. One year after excision of the neck mass, the patient presented with tachycardia. Echocardiography detected two intracardiac nodules suggestive of metastatic tumors. Chemotherapy was administered; the disease has been stable since then.We report the first case of broad ligament leiomyosarcoma with the neck subcutaneous region being the first site of secondary involvement. We speculate that the Batson venous plexus might have been the pathway of dissemination. Leiomyosarcoma is a malignant tumor derived from smooth muscle . It is mA multiparous 54-year-old woman presented to the department of otolaryngology in February 2018 with a round mass, approximately 5 cm in diameter, in the posterior triangle of the right side of the neck . At follow-up in November 2019, the nodules were found to have increased in size  thus monotherapy with pazopanib was introduced. In February 2020, the nodule at the right ventricle apex had grown further, therefore a chemotherapy regimen with paclitaxel alone was initiated. After the fifth course, in April 2020, no further increase in size of either nodule was observed. Heart rhythm had also returned to normal. The disease has been stable since then. The results of a CT scan, performed in July 2020, are shown in Fig. The postoperative course was uneventful. She continued olaratumab therapy and remained in good condition until 13 months after the surgery, when a chest CT scan detected two intracardiac masses. Positron emission tomography (PET)-CT scan and MRI confirmed the presence of the intracardiac masses. Echocardiography showed two hyperechogenic inhomogeneous intracardiac nodules: one (21 × 33 mm) in the lateral wall of the left ventricle and the other (17 × 15 mm) at the apex of the right ventricle. The patient experienced tachycardiaOur patient was unusual in that the subcutaneous tissue of the neck was the first metastatic site of a leiomyosarcoma of the broad ligament. In a previous review of a large cohort of 25,000 sarcomas, only 65 (0.26%) sarcomas were found to have metastasized to the skin or subcutaneous tissue, and in only 17 patients was it the first manifestation of the disease ; moreoveIt is important to distinguish a primary subcutaneous leiomyosarcoma from a secondary leiomyosarcoma because treatment and prognosis are very different . Only fiThe rarity of spread of a uterine leiomyosarcoma to the neck region, without involvement of lung and liver metastases, led us to speculate on the route of dissemination. One possible route is the Batson vertebral venous plexus, which consists of four valveless networks that surround the vertebral column, connecting the cervical, thoracic, abdominal, and pelvic regions , 20. In Cardiac secondaries from leiomyosarcoma are extremely rare . Their fHistopathology is useful for leiomyosarcoma grading, and some immunohistochemical markers have established prognostic value. Diffuse cellular pleomorphism, extensive necrosis, numerous MF and MIB-1 positive cells, and p53 positivity indicate high-grade tumor and worse prognosis , 29.Leiomyosarcoma has a high recurrence rate and a tendency for hematogenous dissemination. Median survival is 20.6 months . In a laTo conclude, we report for the first time an extremely unusual case of well-differentiated leiomyosarcoma of the broad ligament with the neck subcutaneous tissue as the first site of metastasis, without lung or liver involvement. This unique case shows that even a low-grade leiomyosarcoma of the broad ligament has a concrete metastatic potential and that alternative routes of spread exist.Additional file 1: Timeline care.


Stewart-Treves syndrome (STS) is a rare disorder characterized by the development of angiosarcoma in an area of chronic lymphedema . It was A 70-year-old man presented with a 2-year history of a vegetating friable mass with progressive growth on his left leg and mild local pain .He had a history of a car accident with trauma to the left thigh 21 years before that complicated with chronic lymphedema in the affected limb. Incisional biopsy showed dermal vascular proliferation with positive immunohistochemistry (IHC) for CD31, CD34 and factor VIII, a condition compatible with angiosarcoma. The patient was referred for surgical excision, and the histopathological analysis of the specimen revealed high-grade undifferentiated malignancy, fusocellular and epithelioid cells with significant nuclear atypia, areas of necrosis, and fibromyxoid stroma .IHC was positive for CD31, FLI-1, and factor VIII and negative for human herpesvirus-8. Computed tomography revealed lung metastases , and theCutaneous angiosarcomas are aggressive tumors that emerge from the vascular endothelium, usually related to predisposing factors such as chronic lymphedema and radiation exposure . SST wasThis extremely rare case presented as a growing mass located on the lower limb with no previous history of lymphatic surgery, radiation, or malignancy. The late diagnosis contributed to the bad outcome, as the best management for STS is early diagnosis and surgical removal with amputation or wide local excision . AdjuvanNotwithstanding the aggressiveness, this tumor develops in a predictable and specific clinical context with well-established predisposing factors. Thus, physicians should be alert of new or growing lesions in lymphedema sites, regardless of location, to provide early diagnosis and increase the chances for successful treatment. Furthermore, preventive methods to treat lymphedema, such as weight loss, physiotherapy, and compressive measures, should be encouraged .


TFE3 rearrangement have no recommended therapy to date. Here, we report the first case of malignant gastrointestinal PEComa with TFE3 rearrangement which has a response to the targeted therapy of an anti-VEGFR tyrosine kinase inhibitor (TKI), apatinib. A 31-year-old female was diagnosed with malignant gastrointestinal PEComa with TFE3 rearrangement and hepatic metastases. A resection of the giant retroperitoneal mass was performed. The patient received the anti-VEGFR TKI apatinib to treat the hepatic metastasis. The tumor remained stable during apatinib treatment and the progression-free survival (PFS) lasted about 7 months. This case suggests that targeting the VEGF/VEGFR signaling pathway may be an essential new therapeutic choice for TFE3-associated malignant PEComas.Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Unresectable malignant PEComas with  TFE3) gene rearrangement has been reported in various sites of human body , apatinib.Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal tumors. They are composed of perivascular epithelioid cells (PECs) with distinctive histological and immunohistochemical characteristics . PECs arman body . Generalman body . PEComasman body . As an oman body . Howeverangement , which cA 31-year-old woman presented with 3-month history of increased abdominal circumference and intermittent fever. The patient complained no other discomforts. She also denied any anomalies in the past and in the family history. In February 2018 the computed tomography (CT) scans showed a large mass in the upper left abdominal cavity. The mass measured about 16.8×12.5cm, receiving blood supply from celiac trunk, splenic artery and branches of left gastric artery. Splenic vein drained blood flow of the tumor into portal vein. Other abnormalities include retroperitoneal lymphadenopathy, multiple hepatic metastases, enlarged left adrenal gland, and multiple small lymph nodes in the mesentery, pelvis and bilateral groin regions. The somatostatin receptor imaging and tomography showed increased expression of somatostatin receptor with necrosis in the left upper abdominal cavity and multiple hepatic space-occupying lesions.TFE3 (modest and diffuse), and negative for PAX-8, S-100, Syn, CK7, desmin, Myo-D1, SMA, and calponin. Ki67 labeling index in the tumor cells was 30%. RNA-based next-generation sequencing identified SFPQ-TFE3 fusion in tumor cells  in China. The tumor remained stable ” to identify a novel cell type characterized by immunoreactivity with melanocyte markers, an epithelioid appearance, a clear or granular eosinophilic cytoplasm, and a perivascular distribution . In 1996PEComas express both melanocytic markers  and muscle markers  . OccasioTSC1 and TSC2, whose gene products play a role in inhibiting mTOR. mTOR regulates cell division, therefore its hyperactivation contributes to TSC, resulting from deficiency of TSC1 and TSC2  instead of conventional PEComas with TSC mutations  . The mutand TSC2 . This meand TSC2 . About 1angement . These i3 fusion , which a3 fusion , 16. Difangement , 17, we utations . Therefo therapy . Our patTFE3 rearrangement. Apatinib is a newly developed anti-angiogenetic agent in China used in third-line treatment of GC, which is an anti-VEGFR2 TKI and has shown efficacy in GC, hepatocellular carcinoma (HCC), and non-small cell lung cancer (NSCLC)  . The ant (NSCLC) . After r (NSCLC) . In our TFE3-associated malignant PEComas needs more investigation.When the tumor finally progressed, we chose the combination therapy of the anti-VEGFR TKI apatinib and an mTOR inhibitor everolimus. However, the outcome was not satisfactory and the tumor kept on progressing. By contrast, a recent study reported that a patient with malignant uterine PEComa receiving combination targeted therapy of a VEGFR inhibitor (sorafenib) with an mTOR inhibitor (sirolimus) has shown a complete response . TherefoTFE3-associated PEComas. We consider that it’s an innovative perspective.Recently the largest international retrospective series analyzed the therapeutic effects of VEGFR inhibitors  in advanced PEComas. In the retrospective series, the ORR for VEGFR inhibitors was 8.3%, and the median PFS was 5.4 months . HoweverTFE3-associated PEComas still needs more exploration. By contrast, in this case report the anti-VEGFR TKI apatinib directly took effect in advanced PEComas with TFE3 rearrangement, which suggested a better therapy for TFE3-associated PEComas than the combination of mTOR inhibitors and exemestane.Another recent retrospective series reported the reversion of the resistance to mTOR inhibitors by adding exemestane in advanced PEComas (with a median PFS of 7 months and a median DOR of 11 months) , which iTFE3 rearrangement. Therefore, targeting the VEGF/VEGFR signaling pathway may be a useful strategy for unresectable PEComas, especially those with TFE3 rearrangement and a limited response to mTOR inhibitors, but to what extent the tumor can be restrained needs more investigation. Future studies should explore the mechanism of apatinib inducing the clinical effects, and whether the combination therapy is useful in TFE3-associated malignant PEComas.Through our exploration, we consider that the anti-VEGFR effect may play a more important role in the treatment of PEComa with TFE3 rearrangement, the mTOR inhibitor therapy shows limited effect, calling for new therapies. One of the strategies is to target another VEGF/VEGFR signaling pathway. Here we are the first to report that VEGFR inhibitor apatinib had a 7 months duration of response in a patient with advanced malignant PEComa with TFE3 rearrangement. This case suggests that targeting the VEGF/VEGFR signaling pathway may be an essential new therapeutic choice for TFE3-associated malignant PEComas.For malignant PEComas with The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Informed consent was obtained from the patient for publication of this case report and any accompanying images. The patient gave consent to publish this case report, and read the article and confirmed its content.JX, X-LG and LZ designed the study, conducted the literature review, collected the data and wrote and revised the draft manuscript and subsequent manuscripts. HW conducted the experiments. JX and X-LG contributed equally to the manuscript. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Hepatobiliary MRI contrast agents are increasingly being used for liver imaging. In clinical practice, most focal liver lesions do not uptake hepatobiliary contrast agents. Less commonly, hepatic lesions may show variable signal characteristics on hepatobiliary phase. This pictorial essay reviews a broad spectrum of benign and malignant focal hepatic observations that may show hyperintensity on hepatobiliary phase in various clinical settings. In non-cirrhotic patients, focal hepatic observations that show hyperintensity in the hepatobiliary phase are usually benign and typically include focal nodular hyperplasia. In patients with primary or secondary vascular disorders, focal nodular hyperplasia-like lesions arise as a local hyperplastic response to vascular alterations and tend to be iso- or hyperintense in the hepatobiliary phase. In oncologic patients, metastases and cholangiocarcinoma are hypointense lesions in the hepatobiliary phase; however, occasionally they may show a diffuse, central and inhomogeneous hepatobiliary paradoxical uptake with peripheral rim hypointensity. Post-chemotherapy focal nodular hyperplasia-like lesions may be tricky, and their typical hyperintense rim in the hepatobiliary phase is very helpful for the differential diagnosis with metastases. In cirrhotic patients, hepatocellular carcinoma may occasionally appear hyperintense on hepatobiliary phase. In non-cirrhotic patients, FNH and FNH-like lesions are likely the most common lesions showing hyperintensity in the HBP.Cholangiocarcinoma and some metastases may demonstrate central contrast retention in the HBP due to fibrotic stroma.In cirrhotic patients, well-differentiated HCC may show contrast uptake in the HBP in 9–14% of the cases.Hepatobiliary MRI contrast agents—i.e., gadobenate dimeglumine  and gadoxetate disodium —are increasingly being used for liver imaging. These contrast agents are taken up by normal hepatocytes through the organic anion transporting polypeptide 1 (OATP1B3) on the sinusoidal surface and excreted into bile by multidrug-resistance-associated proteins MRP2) on the canalicular surface  on the c.The lack of normal hepatocytes in most focal liver lesions results in the lack of hepatobiliary contrast uptake and, therefore, hypointensity of these lesions relatively to normal background liver parenchyma in the HBP. Less commonly, hepatic lesions may show variable signal characteristics Table  on HBP dThis pictorial essay reviews a broad spectrum of benign and malignant focal hepatic observations that may show hyperintense signal intensity on HBP on MRI in non-cirrhotic patients, in patients with vascular disorders, in oncologic and cirrhotic patients.An incidental liver observation detected at imaging in an asymptomatic patient without underlying disease is benign in 96% of the cases –17. CholFNH is the second most common benign liver tumor with a prevalence of 0.03–0.9% in the general adult population, with a peak incidence among women between 30 and 40 years old , 25. FNHFNHs show iso- or hyperintensity in the HBP relatively to liver parenchyma in the vast majority (97%) of cases  Fig. 2)2), and tThis typical iso- or hyperintensity of FNH relatively to liver parenchyma in the HBP allows the differential diagnosis between FNH and HCA—which is hypointense relatively to liver parenchyma most of the time—with a specificity of 91–100% , 34 and A hyperintense rim on HBP with a peripheral hyperintensity higher than a central iso- or hypointense area is demonstrated in 23–66% of cases  of inflammatory HCAs showing iso-hyperintensity on HBP Fig. , which iFocal fatty sparing is a common finding in patients with diffuse fatty infiltration of the liver . Areas oVascular liver disorders—e.g., Budd–Chiari syndrome, congenital portosystemic shunts, hereditary hemorrhagic telangiectasia, cavernous transformation of the portal vein—are associated with the development of hepatocellular tumors such as FNH-like nodules (more commonly), HCAs and HCC –52. The FNH-like nodules arise as a local hyperplastic response to vascular alterations, occurring in about 36% of patients with Budd–Chiari syndrome , 53.FNH-like nodules may rarely be observed in patients with cirrhosis , 55 or cFNH-like lesions demonstrate enhancement in the arterial phase and persistent enhancement on portal venous or delayed phase in most cases; however, washout may be occasionally detected and, in these cases, the differential diagnosis with HCC is tricky , 58.FNH-like lesions are usually iso- to hyperintense on HBP  due to eIn case of FNH-like nodules related to oxaliplatin, FNH-like nodules are also usually hyper- or isointense to the surrounding liver parenchyma in the HBP, and a ring (or doughnut-like) pattern on HBP is observed in approximately 50% cases Fig.  56]. Th. Th56]. Considering the challenges in the differential diagnosis between FNH-like lesions and HCC, specific diagnosis requires extensive clinical, laboratory and imaging work-up, including follow-up every 6 months if liver lesions have features of FNH-like lesions and alpha 1-fetoprotein levels are low, or liver biopsy if imaging features are atypical, if significant changes occur over time or if serum alpha 1-fetoprotein becomes elevated .Oncologic patients are more likely to have metastases as compared to the general population, but benign observations showing HBP hyperintensity  are expected to have the same incidence as in the general population –63. HypeAs discussed above, oncologic patients may show FNH-like nodules after chemotherapy, and the diagnosis of these lesions benefits from the use of hepatobiliary contrast agents.In oncologic patients, malignant focal liver lesions showing variable signal characteristic on HBP include metastases  and intrahepatic cholangiocarcinomas .Metastases are the most common malignant liver tumors . Liver mThe use of hepatobiliary contrast agents is particularly important in the evaluation of liver metastases because it increases sensitivity for detection of metastases compared to CT or extracellular agents providing high tumor-to-lesion contrast on HBP , 67. TheLiver metastases are hypointense on HBP due to their lack of normal hepatocytes. However, metastases may occasionally demonstrate in the HBP central areas of relative hyperintensity—described as “EOB-cloud enhancement” similarly to cholangiocarcinoma—compared to surrounding lesion (rim) hypointensity with a resulting target appearance Fig.  22, 23,,23, 67. Intrahepatic cholangiocarcinoma is the most common primary non-HCC malignancy in non-cirrhotic liver . AlthougIntrahepatic mass-forming cholangiocarcinomas are hypointense on HBP because these lesions lack hepatocytes. In 42–57% of these tumors, a target sign is demonstrated as a peripheral hypointense rim and a diffuse, mainly central and inhomogeneous “EOB-cloud enhancement” Fig.  18–20].–20.18–20p = 0.048) . Pe. Pe91]. The causative mechanism and the clinical relevance of this imaging finding are still unclear. In patients with hepatic disorders such as primary biliary cirrhosis and idiopathic portal hypertension, it has been suggested that the periportal HBP hyperintensity is related to regenerative changes of periportal hepatocytes, which lead to a relatively increased uptake of the hepatobiliary contrast agent compared to the damaged background liver . Onishi In current practice, the use of extracellular contrast agents usually allows to determine the diagnosis of most focal liver lesions and should be favored as first imaging approach for the characterization of focal liver lesions and as baseline and follow-up imaging in oncologic patients. When the lesion is deemed indeterminate in studies with extracellular agents, the adoption of hepatobiliary MRI contrast agents is particularly relevant for the differential diagnosis between FNH and hepatocellular adenoma in the non-cirrhotic liver , 34–36 aWhen a focal liver observation shows iso- or hyperintensity in the HBP, our imaging evaluation should consider the clinical setting, the pattern of iso- or hyperintensity in the HBP and the information provided by extracellular images and T1-, T2-, and diffusion-weighted images.A nodule showing a doughnut-like hyperintense pattern in the HBP usually indicates FNH in healthy patients , 32–39, In case of a nodule showing central uptake of contrast agent in the HBP due to fibrotic content, imaging assessment should be based on extracellular phases: If the lesion shows irregular peripheral enhancement in the hepatic arterial phase and gradual centripetal enhancement on following phases, the diagnosis of intrahepatic cholangiocarcinoma is favored because this entity may show central uptake in 42–57% of cases –20; if tIn case of iso- or hyperintense nodules on HBP lacking a doughnut-like pattern or central uptake, our diagnostic approach should be based on the following three scenarios:if the lesion shows lack of signal drop on opposed phase compared to in-phase images in a steatotic liver and is not visible on T2- and T1- and diffusion-weighted images and extracellular phase, the diagnosis of fat sparing in steatotic liver is favored , 48;in healthy or oncologic patients, if the lesion is highly hypervascular on arterial phase, and nearly isointense to liver parenchyma on T2-, T1- and diffusion-weighted images and extracellular phase, the diagnosis of FNH or FNH-like lesion should be favored, respectively;in cirrhotic patients, our imaging evaluation should be aimed at excluding the presence of the small proportion of HCC that may show hyperintensity in the HBP; therefore, radiologists should first analyze extracellular phases, then should assess if the lesion contains intracellular fat on dual phase images and intensity on T1-, T2- and diffusion-weighted images. In case of lack of worrisome features for HCC  and the presence of hyperintensity on pre-contrast T1-weighted images, and if the lesion is less than 1 cm, the hyperintensity on HBP may indicate the presence of regenerative or low-grade dysplastic nodules.In addition to the above considerations and prior to any decision on patient management, it is important to investigate whether the patient has any prior cross-sectional imaging available and to compare all prior examinations, particularly the oldest available one, with the current examination, in order to assess for lesion stability in size or changes of imaging presentations over time.If the lesion showing iso- or hyperintensity on HBP is suspicious for hepatocellular adenomas, biopsy should be indicated to assess if the lesion has the β-catenin mutation because β-catenin hepatocellular adenomas are indicated to surgery due to their risk of malignant transformation , 43–45. In conclusion, the presence of hyperintensity on HBP may be useful for the diagnosis of numerous benign and malignant hepatic masses based on knowledge of the clinical setting. Indeed, FNH and FNH-like lesions are likely the most common lesions showing hyperintensity on HBP in patients without cirrhosis. In cirrhotic and in oncologic patients, well-differentiated HCC are usually hypointense on HBP but may show hyperintensity in the HBP in about 9–14% of cases, while cholangiocarcinoma and some metastases may demonstrate variable inner signal characteristics with a peripheral rim of hypointensity. In such scenarios, malignancy must be ruled out before considering the diagnosis of a benign condition, and the use of hepatobiliary MRI contrast agents proves to be particularly useful. Finally, a histopathological examination may be required to resolve challenging cases.


An apicomarginal defect can be explained as a total loss of buccal alveolus extending from the original crestal bone to the apex of the tooth. This study presents a case of an apicomarginal defect in a first left molar subjected to periapical surgery with vestibular cortex block replacement and A-PRF + membrane coating approximately one year ago. One-year clinical follow-up was performed, with no evidence of recurrence. This case report discusses periapical surgical treatment and the importance of an interdisciplinary approach to the management of teeth with apicomarginal defects. Key words:Periapical surgery, apicomarginal defect, A-PRF+, bone graft. An apicomarginal defect can be explained as a total loss of buccal alveolus extending from the original crestal bone to the apex of the tooth . ApicomaThe role of platelet-rich fibrin (PRF) has been well documented in regeneration processes for the management of periodontal intrabony defects, gingival recessions and furcation defects . AdvanceA 29-year-old systemically healthy non-smoking female reported to the Department of Oral Surgery of the University of Valencia  with mild pain and purulent discharge in relation to the lower left posterior tooth region during the last month. At intraoral examination, the gingival tissue attached to the vestibular surface of the first lower left molar showed the presence of an abscess Fig. A. The paCone-beam computed tomography (CBCT) revealed the presence of localized periapical bone loss with loss of vestibular bone cortex and marginal alveolar bone around the mesial root, compromising the furrow of molar 3.6. Fig. C,D. BaseThe patient signed the informed consent to treatment and the use of data for scientific purposes. Periapical surgery was performed, with regeneration of the apicomarginal defect with vestibular cortical block autograft and A-PRF + membrane coating.For A-PRF+ preparation, the median cubital vein was punctured to collect three 10-ml glass tubes without additives . The tubes were centrifuged at 1300 rpm for 8 min . The fibrin clots obtained were extracted with special tweezers and scissors, avoiding the red blood component below the buffy coat, and were further processed to obtain a standardized membrane thickness in a sterile container .Periapical surgery began with anesthetic infiltration at the bottom of the vestibule of 3.6. using two 4% articaine carpules with 1: 100,000 epinephrine . A sulcular incision was performed, with mesial release of 3.5 and another distal to 3.7., with raising of a full thickness trapezoidal flap Fig. A.Ostectomy of the vestibular cortex was performed with ultrasound tips , and a vestibular cortex block with was obtained chisel and hammer Fig. B.The margins of the vestibular bone cortex lesion were profiled with a tungsten carbide burr mounted on a handpiece with abundant irrigation of physiological saline solution, allowing visualization of the entire lesion. Then, the periapical inflammatory tissue was removed and the apicoectomies of the two roots of the lower left first molar were performed.Once root integrity of the first lower left molar was checked, the retrograde obturation box was performed with ultrasound tips . After proper control of hemostasis with polytetrafluoroethylene strips Fig. C, it wasRegeneration of the apicomarginal defect was performed with replacement of the vestibular cortex, fixed in the coronal third of the tooth root, which was fixed with a screw  Fig. G, and thSuturing was performed with non-reabsorbable 5/0 multifilament suture  Fig. I. The paOne year after surgery, the patient underwent clinical Fig. A and radOf the present case corresponds to a patient with an extensive apicomarginal bony defect. Treatment included the elimination of endodontic infection with periapical surgery, a vestibular cortex block autograft, and its coating with A-PRF membranes. The occurrence of periapical disease, accompanied by periodontal breakdown, constitutes a complex problem in periradicular surgery - being typically associated with a less favorable prognosis, since disruption of the cortical plate can have a deleterious effect upon regeneration of the lost tissues ,9. DestrThe regeneration of periapical tissues after periapical surgery requires complex interactions among recruited stem cells to secure differentiation into committed osteoblasts, periodontal ligament cells and cementoblasts . Growth The present case report highlights the role of PRF in the healing of apicomarginal defects in the mandibular first molar sector. It has been observed that intrinsic growth factors  are better in promoting wound healing than extrinsic growth factors . PlateleHealing in our case could be attributed to three factors: the immune regulatory action of PRF; the vestibular cortical block autograft repositioned in the coronal third of the mesial root of the lower left first molar; and the removal of infection from the surgical site, promoting connective tissue and bone formation from adjacent healthy periodontium .


CIC) rearrangements. Recently, an array-based DNA methylation analysis of undifferentiated tumors with small blue round cell histology was shown to provide a highly robust and reproducible approach for precisely classifying this diagnostically challenging group of tumors. We describe the case of an undifferentiated sarcoma of the abdominal wall in a 12-year-old girl. The patient presented with a voluminous mass of the abdominal wall, and multiple micro-nodules in the right lung. The tumor was unclassifiable with current immunohistochemical and molecular approaches. However, DNA methylation profiling allowed us to classify this neoplasia as small blue round cell tumor with CIC alterations. The patient was treated with neoadjuvant chemotherapy followed by complete surgical resection and adjuvant chemotherapy. After 22 months, the patient is disease-free and in good clinical condition. To put our experience in context, we conducted a literature review, analyzing current knowledge and state-of-the-art diagnosis, prognosis, and clinical management of CIC rearranged sarcomas. Our findings further support the use of DNA methylation profiling as an important tool to improve diagnosis of non-Ewing small round cell tumors.Undifferentiated soft tissue sarcomas are a group of diagnostically challenging tumors in the pediatric population. Molecular techniques are instrumental for the categorization and differential diagnosis of these tumors. A subgroup of recently identified soft tissue sarcomas with undifferentiated round cell morphology was characterized by Capicua transcriptional receptor ( EWSR1, and members of the E26 transformation-specific (ETS) gene family. EWSR1–FLI1 or EWSR1–ERG are the most common translocations in ES and are observed in ~85%–90% and 5%–10% of all cases, respectively [TET (FUS) and ETS family members  and rearrangements of EWSR1 with non-ETS family genes , which occur in fewer than 1% of ES [Primitive small blue round cell tumors (SBRCTs) in children and young adults pose a diagnostic challenge. Ewing sarcoma (ES) is the prototype for an undifferentiated sarcoma with such a phenotype, although it is shared by several other sarcoma entities together with the membrane expression of CD99 ,2. An adectively ,4. Far r1% of ES ,8,9,10.CIC and BCL6 Corepressor (BCOR) rearrangements [CIC rearranged sarcomas arise more often in soft tissues rather than in bone, show a myxoid matrix, have prominent nucleoli, and are characterized by a poor prognosis, especially for patients with metastatic or recurrent disease [Molecular techniques, including extended Fluorescent in situ hybridization (FISH) analysis and next generation sequencing, have allowed the separation of ES with canonical translocations from a subset of lesions that remains unclassified. These are termed “Ewing-like sarcomas”, or SBRCTs not otherwise specified in the last World Health Organization classification ,12. Althngements ,15,16,17Diagnosis of such tumors is difficult due to their rarity and unspecific histological features but is possible by combining morphological, immunohistochemical, and molecular techniques ,2. HowevCIC alteration identified with DNA methylation profiling. To further inform management of such cases, we review the available literature and discuss the current knowledge on molecular and clinical features of CIC rearranged sarcomas. We conclude that DNA-methylation-based tumor classification can be an important tool in advancing the diagnosis, pathological analysis, and management of these tumors.Here we report a challenging diagnosis of undifferentiated sarcoma with A 12-year-old female was referred to our hospital emergency department with recent-onset abdominal pain. No other symptoms were recorded; she had no fever, vomiting or diarrhea. Diuresis and menstrual cycle were regular as well. Clinical examination revealed a hard-elastic swelling in the meso-hypogastric region. Complete abdomen ultrasound and magnetic resonance imaging (MRI) showed a voluminous bilobed mass of approximately 13.5 cm × 7 cm, with regular margins, in the left paramedian of the anterior abdominal wall, extending from the umbilical region to the supra-bladder area and, posteriorly reaching the spinal vertebral bodies at the lumbar-sacral level. The cranial portion of the mass appeared capsulated with signal over-intensity in all impulse sequences in relation to myxoid component and high cellularity in diffusion weighted imaging (DWI). The caudal portion appeared more inhomogeneous, predominantly hypo-intense in all sequences, as for bleeding A–G.An incisional biopsy of the tumor was performed. Histological examination showed proliferation of medium-sized elements, with sharp cellular contours, dimly eosinophilic cytoplasm, and polymorphic and polymetric nuclei, with evident nucleoli and atypical mitotic figures A–C. The EWS. PCR testing for CIC-DUX4, BCOR-CCNB3, and for the myxoid chondrosarcoma transcript was also negative. Histopathological diagnosis was determined through examination of the specimen at the Italian pediatric sarcoma central pathology panel whose conclusion was a malignant mesenchymal neoplasm/unclassifiable sarcoma.Molecular evaluation by both FISH and PCR found no evidence of the translocation of To complete the diagnostic work-up, the patient underwent bone scintigraphy, which was negative for areas of abnormal accumulation. A Positron Emission Tomography (PET)/CT scan confirmed the presence of the mass, which was metabolically active  at the left rectus abdominis muscle, with extensive development in the meso- and hypo-gastric site. Compressive phenomena in some loops of the small bowel and on the left psoas muscle were also observed. The tumor was capsulated without infiltration of the surrounding structures. There were no other areas of tracer uptake in the peritoneum, lymph nodes, lungs, and skeletal segments. A CT scan showed millimetric nodules in the latero-basal segment of the lower lobe of the right lung, in the dorsal segment of the upper lobe of the right lung in the sub-pleural area, and in the middle lobe, where metabolic activity could not be assessed due to the small size of the nodules.DNA methylation profiling was performed according to protocols approved by the institutional review board, after obtaining written consent from the patient’s parents. Tumor areas with the highest tumor cell content (≥70%) were selected for DNA extraction. Samples were analyzed using Illumina Infinium Human Methylation EPIC Bead Chip (EPIC)  arrays according to the manufacturer’s instructions, as previously reported ,19,20. ICIC alteration”. Global profiling methylation data were also compared to 32 samples randomly extracted from internal and external datasets [FOXR2 (CNS-NB-FOXR2), CIC rearranged sarcoma (EFT-CIC), high-grade neuroepithelial tumor MN1 (HGNET-MN1), and high-grade neuroepithelial tumor BCOR (HGNET-BCOR) using the Heidelberg brain tumor and sarcoma classifier. Bead Chip data were analyzed by means of R (V. 3.4.4) package minfi (V. 1.24.0) to obtain normalized beta values and to perform multidimensional scaling (MDS) analysis, as previously described [CIC, as evidenced by MDS performed on the 1000 most variable islands in the cohort  and doxorubicin (37.5 mg/m2/day for 2 days) every three weeks, disease reevaluation showed an excellent response, with shrinkage of tumor volume (8.1 × 5.6 × 3.9 cm)  2005 non-Rhabdomyosarcoma protocol (Intergroup Rhabdomyosarcoma Study-IRS stage III). After three cycles of ifosfamide (3 gr/m 3.9 cm) H,I. The A gross total resection of the residual tumor was performed. Histological examination showed a reactive fibrous pseudocapsule surrounding the mass peripherally. Regression areas, alternating with vital areas were observed. The regression areas, which were quantified in about 60% of the total mass, showed evidence of necrosis, hemorrhagic infarction, and histiocytic infiltration by elements with a foamy and/or pigmented aspect due to the presence of abundant hemosiderin in the interstice. The vital areas were irregularly distributed within the mass and quantified in about 40% of the total. They consisted of elements with a morphology and immunohistochemical pattern substantially similar to the one detected in the diagnostic biopsy. The margins of resection were negative.2/day for two days), one of ifosfamide (3 gr/m2/day for three days), and doxorubicin (37.5 mg/m2/day for 2 days) every three weeks. At the 22-month follow up, the patient was in good clinical conditions and disease free.Three cycles of adjuvant chemotherapy were administered: two of ifosfamide  and finally detected the CIC-DUX4 rearrangement: CIC (Exon20)→DUX4 (Exon1), confirming the results obtained by methylation profiling . Total RCIC rearranged sarcomas are rare but represent the most frequent molecular subtype among Ewing-like undifferentiated SBRCTs [EWSR and FUS rearrangements [CIC rearranged tumors are characterized by fusions involving CIC, a human homolog of Drosophila Capicua located on chromosome 19q13.2 and encoding a transcriptional repressor, functioning downstream of tyrosine kinase receptor signaling.d SBRCTs ,22, accongements ,23,24,25CIC rearranged round cell sarcoma with t translocation was reported in 1996 in a 12-year-old male with an ankle mass and pulmonary metastases [DUX4 paralog DUX4L.The first case of a tastases ,27. In 2tastases  identifiDUX4 and DUX4L genes contain a 3.3-kb tandem repeat sequence located in the sub-telomeric regions of the long arms of chromosomes 4 and 10, respectively [DUX4/L, which loses most of its sequence in the fusion [CIC. The deriving chimeric protein is an oncogenic transcription factor that dysregulates the expression of its downstream targets [PEA3) family members of ETS-related transcription factors ETV1, ETV4, and ETV5 [PEA3 belongs to the family of ETS-related transcription factors reported as rare fusion partners of EWSR1 in ES [Both ectively . They enectively ,30,31. Ne fusion . Such ge targets . In partand ETV5 ,16,24,33R1 in ES ,34,35.DUX4 is the most common partner gene of CIC [CIC have also been described. These include FOXO4 [NUT midline carcinoma family member 1 (NUTM1) [CIC gene fusions has prompted the use of the term “CIC rearranged family of tumors” [CIC-DUX4 and CIC-DUX4L rearranged tumors [CIC-DUX4 fusion and other CIC rearranged sarcomas compared with ES family of tumors (ESFTs) with EWSR1-ETS fusions show little overlap in differentially expressed genes, supporting the distinction of these tumors from ESFTs [Even if e of CIC ,22, SBRCde FOXO4 ,37 and N (NUTM1)  and occa (NUTM1) ,31,36,37 tumors”  to includ tumors ,37. ConvCIC rearranged sarcomas as a distinct entity was seen in experiments conducted by Yoshimoto et al. [CIC–DUX4 fusion gene (mCDS) and compared the gene expression profile of the mCDS mutant mice with that of mice with classic ES (mES). They found a large number (1661) of differentially expressed genes in mCDS or mES, suggesting that CIC–DUX4 sarcomas are distinct from ES. Moreover, CIC rearranged sarcomas have been reported to show specific epigenetic signature (as discussed below) [Further evidence to support o et al. . The autd below) .CIC rearranged SRBCTs affect a wide age range (6–81 years), but are diagnosed most frequently in young adults  [From a clinical point of view, g males) ,31,39. Ag males) ,16,31,39g males) .CIC rearranged sarcoma with localized disease with a control group of 45 ES cases, showing a 43% 5-year survival versus 77% of ES [CIC rearranged sarcoma patients were minimal or generally temporary with rapid onset of drug resistance and disease progression [CIC rearranged sarcoma patients refer to the older population, so the discrepancy between our patient response and the literature report could be found in the young age of our patient.Although there are limited clinical data to date, these tumors seem to have a significantly more aggressive course when compared with classic ES ,31,33. I7% of ES ,40,41 wigression ,40,41. ICIC rearranged sarcoma patients remains to be defined [The optimal therapeutic approach for  defined ,2,42, peNo specific radiological and gross features have been reported thus far. Tumors are often extensively hemorrhagic and necrotic. MRI studies of CIC rearranged sarcomas usually show tumor originating from the deep soft tissues of the trunk, pelvis, or proximal extremities with heterogeneous but intense post-contrast enhancement , as in tCIC rearranged sarcomas are characterized by a more heterogeneous appearance than usual ES. They more frequently show “atypical features”, including plasmacytoid and rhabdoid features in approximately 25% and spindling in about 10%. They also show greater nuclear pleomorphism [Microscopically, morphism ,32,39,43morphism . Myxoid morphism . They comorphism . Areas omorphism ,45.CIC rearranged sarcomas have been reported to show a unique expression pattern from ES , but negative among other SBRCTs, including ES [Recently, DUX4 immunostaining has been reported to be diffusely positive in all cases of uding ES . The preuding ES .CIC rearrangement was reported [CIC locus on chromosome 19q, may have an adverse effect on FISH analysis [CIC [CIC–DUX4 fusion could only be detected by manually reviewing the reads of these genes [However, as for our case, decision making might be complicated by false negative results in molecular testing of such tumors ,16,31,37reported ,25,50. Ianalysis . Furthersis [CIC . In thesse genes . While tse genes .CIC–DUX4 fusion have been shown to upregulate ETS transcription factors  [ETV1, ETV4, and ETV5 transcripts was reported as a specific and sensitive ancillary test in cases with limited sample availability [PAX7 was identified as highly differentially expressed between ES and CIC rearranged sarcoma, also at the protein level. Indeed, Charville et al. demonstrated that PAX7 expression evaluated by IHC was detectable in 102/103 ES and 0/27 CIC rearranged sarcomas [Use of a surrogate marker could be a more viable approach for facilitating the diagnosis of SBRCTs. SBRCTs with some 3q) ,43,47,50lability ,51. Recesarcomas .Accurate pathologic diagnosis is essential to make optimal therapeutic choices for patients with cancer, avoiding both over- and under-treatment and allowing appropriate inclusion of patients in clinical trials. In recent years, genome-wide DNA methylation array analysis has allowed identification and characterization of episignatures for an increasing number of diseases ,54.DNA methylation profiling has been shown to be a robust and reproducible approach for the classification of several tumor entities across age groups ,21,38,54DNA methylation and histone modifications are among the most widely characterized epigenetic mechanisms of interest in these cases. CpG islands are variably distributed in the human genome; they are often located in gene promoters and act as key regulators of gene expression . DNA metIn cancer, alteration in DNA methylation patterns can both transcriptionally inactivate tumor suppressors and increase oncogenes expression, sustaining tumorigenesis. Aberration in epigenetics can alter normal developmental programs and cell differentiation, leading to an uncontrolled proliferative state of progenitor cells . CytosinArray-based methods have progressively improved to reach over 850,000 CpG analyses throughout the human genome, such as the Illumina Infinium Human BeadChip arrays (450K and EPIC) that we used for our analysis ,56. ThesParalleling this technical progress, computational methods have been developed to deal the raw data in biologically and clinically applicable output ,21,56. TCIC alteration in 6 (20%), to SBRCTs with BCOR alterations in 4 (13%), to synovial sarcoma and to malignant rhabdoid tumors in 2 cases each, 1 case to mesenchymal chondrosarcoma, and 1 to adamantinoma. For cases assigned to the SBRCTs with CIC alterations, methylation class was verified by additional FISH and total RNA sequencing. Four of the six tumors revealed a CIC break-apart signal in the FISH analysis, indicating a rearrangement of the CIC locus. In two of them, RNA sequencing analysis detected a CIC–DUX4 fusion. RNA sequencing also revealed a CIC–DUX4 fusion in one of the two cases without a CIC break-apart signal [This approach also has been shown to be useful for addressing undifferentiated SBRCTs not otherwise specified . Koelscht signal .This breakthrough tool has allowed us to successfully address a challenging diagnosis. Overall, DNA methylation profiling could have clinical implications in the molecular diagnostics of SBRCT, as already documented in central nervous system tumors ,38.CIC rearranged sarcomas are rare tumors that present substantial diagnostic challenges; morphological diagnosis is difficult. Since the immunohistochemical expression pattern seen in these tumors is unique, this is a useful adjunct in orienting a pathologic definition. Molecular testing is necessary to confirm diagnosis, but FISH and total RNA sequencing may sometimes have pitfalls.In our view, DNA methylation profiles represent a highly useful instrument in the diagnosis and the molecular characterization of SBRCTs, with potential application in almost all pathology fields.


Testicular tumors are one of the most common solid tumors in young males. Choriocarcinoma usually presents as metastatic disease. Gastrointestinal tract involvement is rare. We report a case of a 40-year-old male presenting to our hospital with a three-day history of dyspnea on exertion and black stool after recent diagnosis of testicular choriocarcinoma. Urgent EGD performed revealed small clean-based fundal ulcer and an antral ulcer without the stigma of recent bleeding. Capsule endoscopy was performed and revealed a bleeding ill-defined mass in the proximal duodenum. A subsequent push enteroscopy showed an ulcerated bleeding mass in the third part of the duodenum that was treated with a hemospray with adequate hemostasis. Pathology was consistent with pure choriocarcinoma. The patient received a cisplatin-based chemotherapy regimen. The patient tolerated the chemotherapy regimen well and was discharged for outpatient follow-up. At the three-month follow-up, the patient did not show evidence of recurrent gastrointestinal bleeding. Testicular tumors are one of the most common solid tumors in males aged 15–35 years, with germ cell tumors being the most common type . GastroiA 40-year-old male presented to the hospital with a three-day history of dyspnea on exertion and black stool. He was recently diagnosed with testicular choriocarcinoma and lung metastasis. He underwent left radical orchiectomy ten days prior to admission. The pathology revealed choriocarcinoma with immunopathological stain positive for human chorionic gonadotropin. The patient was in the process of evaluation for chemotherapy at the time of the presentation. Vital signs upon presentation were notable for a heart rate of 122/minute, otherwise stable. Physical exam was significant for mild epigastric tenderness and melena on the digital rectal exam. Initial blood work revealed acute microcytic anemia with a hemoglobin of 8.4 g/dL. Hemoglobin three weeks before the presentation was 14 g/dL. Upper gastrointestinal bleeding was suspected; the patient was resuscitated and started on pantoprazole infusion. Urgent esophagogastroduodenoscopy (EGD) performed after 10 hours of presentation with the finding of one small clean based fundic ulcer and a 2 cm antral ulcer without the stigma of recent bleeding.Because of persistent melena and a drop in the hemoglobin level requiring transfusion of 2 pRBCs, colonoscopy was performed with unremarkable finding. Capsule endoscopy was pursued to look for other possible etiologies. It revealed a bleeding ill-defined mass within 3 minutes from the first duodenal image consistent with proximal small bowel bleed.A subsequent push enteroscopy revealed a friable ulcerated bleeding mass in the third portion of the duodenum that was treated with a hemospray with adequate hemostasis .The patient received a total of 8 units of packed RBCs during the hospital stay of 10 days. Pathology from enteroscopy was consistent with pure choriocarcinoma, and the immunohistochemical stain is positive for human chorionic gonadotropin Figures  and 3.Laboratory tests revealed serum human chorionic gonadotropin 516139 mIU/ml  and alpha-fetoprotein 1.83 ng/ml [<8.3 ng/ml]. The patient started a chemotherapy regimen with etoposide, ifosfamide, and cisplatin. Bleeding was controlled acutely by hemospray and completely ceased after beginning chemotherapy. The patient tolerated the chemotherapy regimen well and was discharged for outpatient follow-up. At the three-month follow-up, the patient did not show evidence of recurrent gastrointestinal bleeding.Germ cell tumor is the most common testicular cancer. The most aggressive and least common type of germ cell tumor is choriocarcinoma . Less thTesticular tumors usually present as painless testicular mass or swelling. However, in choriocarcinoma, widespread hematogenous dissemination occurs early, and many patients present with metastatic disease . MeasureA definite diagnosis of choriocarcinoma requires a radical inguinal orchiectomy. The determination of metastatic choriocarcinoma can be challenging as extensive tissue sampling and careful microscopic examination are essential. The most important pathologic characteristic of choriocarcinoma is the coexistence of both syncytiotrophoblast and cytotrophoblast cells, which distinguishes this tumor from other germ cell tumors with only scattered syncytiotrophoblast. Tumor markers can be of great help in establishing the diagnosis. GATA3 is a transcription factor emerging as a sensitive immune marker for choriocarcinoma .Gastrointestinal tract metastasis occurred in 5% of germ cell tumors. The small intestine, most commonly the duodenum, is the most frequent site for metastasis 72%) followed by the esophagus, stomach, and colon 2% follow.Treatment of testicular choriocarcinoma depends on the disease stage. Radical orchiectomy of the affected testis and dissection of the affected lymph nodes are the treatment of early-stage disease. Metastatic disease is usually treated with cisplatin-based chemotherapy. The serum level of human chorionic gonadotropin is used to monitor response to treatment .Acute treatment modalities of the bleeding gastrointestinal metastasis are similar to other bleeding etiologies and include endoscopic intervention, embolization, or surgical resection. Bain et al. presented a case of bleeding duodenal choriocarcinoma that was treated with angiography and embolization . Iglesia


Aeroportia is the presence of gas in the portal vein. It is considered an ominous radiological sign with poor outcomes. Historically, it was associated with bowel necrosis, and surgery was mandated in all cases. Herein, we present a challenging case of portal venous gas and its management.An 87-year-old male patient, with multiple co-morbidities, presented with abdominal pain. The computerized tomography (CT) scan showed extensive portal venous gas without evidence of bowel ischemia. Initially, he was managed conservatively, but his clinical condition deteriorated. So, an exploratory laparotomy was performed which revealed multiple superficial splenic abscesses covering the surface of the spleen and a pale segment of jejunum with questionable viability. Splenectomy was performed and second-look laparotomy was planned to assess the small bowel viability. Second-look laparotomy revealed dusky discoloration of 30 cm jejunal segment. The affected segment was resected. The patient improved after surgery and was discharged home.In conclusion, aeroportia  is a radiological entity. The clinical condition of the patient must be kept in consideration to manage the patients optimally. However, if the patient deteriorates, a high index of suspicion for mesenteric ischemia and early surgical intervention are the keys to save the patients’ life. Portal venous gas (PVG) and pneumatosis intestinalis (PI) is considered an ominous radiological finding. In the past, it was associated with poor outcome. Fred et al. 1968) described a mortality of 90% in patients with PVG, however, with a better understanding of etiology and advancements in diagnostic technology and critical care, the prognosis has improved, and recently, Gonda et al. 2020) reported a mortality of 32% [968 descr020 reporDecision making in the management of PVG/PI is still fraught with complications because there are no clear guidelines. Herein, we present a case that elaborates on this dilemma and the challenge faced in the management of the patient.An 87-year-old male of Middle Eastern descent, known to have multiple co-morbidities, presented to the emergency department with complaints of central abdominal, colicky pain which was mild to moderate in severity, non-radiating, and non-migratory. It was associated with loose stools for four days. The stool was yellow, non-bloody, and eight to nine times per day. The patient has a previous history of constipation. There was no history of vomiting, fever, or weight loss. He denies any prior such episodes.His medical history is significant for hypertension, coronary artery disease, and asthma. His home medications included metoprolol, aspirin, valsartan, and salmeterol/fluticasone. He has no history of abdominal surgery.Examination revealed a conscious and oriented elderly male, lying in his bed with a bit of discomfort. He had dry oral mucosa and his urine was concentrated indicating dehydration. His blood pressure was 114/70 mmHg, pulse rate was 84 per minute and regular, breathing rate was 19/ minute, temperature was 37 degree Celsius and his oxygen saturation was 96% on room air.Abdominal examination showed no skin changes, visible scars, ecchymosis, or palpable masses. However, it was distended and mildly tender in the umbilical region without rebound tenderness. Hernial orifices were normal and bowel sounds were normal. Digital rectal examination revealed mucoid stool but there was neither blood/melena nor any palpable mass.3 per microliter (4.00 to 10.00 x 103), lactic acid 1.9 mmol/L (0.5 to 1.6 mmol/L), procalcitonin 3.4 ng/mL (0.0 to 0.5 ng/mL), C-reactive protein 38 mg/L (0 to 5 mg/L), pH 7.2 (7.35 to 7.45), bicarbonate 11 mmol/L (20 to 28 mmol/L), partial pressure of carbon dioxide (PCO2) 25 mmHg (35 to 45 mmHg), creatinine 193 micromoles/L (50 to 98 Micromoles/L), blood urea 11.6 mmol/L (2.5 to 6.7 mmol/L) and albumin 24 gm/L (35 to 50 gm/L). Serum pancreatic amylase and lipase were normal. His electrolytes and liver function tests were within the normal range. A bedside urine dipstick test was negative for nitrites or leukocyte esterase.His lab values at presentation showed hemoglobin 12.4 g/dL (12.0 to 15.0 g/dL), white blood cell (WBC) 4 x 10A chest X-ray showed no pneumoperitoneum. A CT angiogram showed gas within the tributaries of the superior mesenteric vein extending to the portal vein with extensive branching portal venous gas pattern in the liver Figure .Pneumatosis intestinalis was noted predominantly in the jejunal loops Figure .There was dilatation of the jejunum and proximal ileum without transition point. The terminal ileum and colon were collapsed. There was no pneumoperitoneum and the bowel loops were enhancing. The aorta and its major branches showed good opacification and no definite thrombus or occlusion could be demonstrated.The patient was resuscitated with intravenous fluids. He was kept on strict input and output charting and vital signs monitoring. The patient improved clinically after fluids. Since he was not in peritonitis and clinically stable, we chose to observe him with serial examinations every two hourly by a senior surgeon. Later, after a period of 12 hours, he started to deteriorate with rising lactate levels and worsening of acidosis. He became hypotensive and was started on inotropes. However, his abdominal examination remained unchanged.At this point, the decision was made to proceed with emergency exploratory laparotomy. Intraoperatively, the small bowel was dilated. The spleen was covered with white exudate giving the appearance of splenic abscess Figure .There was a 20-25 cm segment of mid-jejunum which was pale with sluggish peristalsis but viable. Splenectomy was performed and this segment of small bowel with questionable viability was kept for re-look after 24-48 hours. The patient was started on heparin infusion after the surgery.At re-look laparotomy, this segment failed to regain its normal color and peristalsis Figure . ResectiAfter surgery, the patient improved hemodynamically and vasopressors were stopped on post-operative day one. The patient was extubated on day two. He was started on sips of water and eventually progressed to a full diet by postoperative day five. He was discharged after 17 days and before discharge, he was vaccinated against encapsulated organisms according to the local post-splenectomy immunization protocol. His histopathology showed the spleen with congested red pulp covered with thick white exudate. The intestinal segment showed extensive hemorrhagic infarction and necrosis of mucosa, consistent with ischemia without any vascular occlusion.He was followed up in the clinic after two weeks and showed remarkable full recovery.Acute mesenteric ischemia (AMI) is a life-threatening surgical emergency that carries significant morbidity and mortality. The usual presentation of AMI is “pain out of proportion” which may not be present in all cases . One of Portal venous gas (PVG) was first reported in neonatal abdominal radiographs by Wolfe et al. in 1955 , whereasPneumatosis intestinalis (PI), was first described by Du Vernoy in 1730. It has two subtypes, primary and secondary. Primary/idiopathic type (15%), which is often termed pneumatosis cystoides intestinalis (PCI), refers to air pockets that imply a chronic and benign idiopathic etiology. On the other hand, secondary type (85%), refers to radiological findings of linear, micro-vesicular, or more circumferential appearing intramural gas has many underlying causes, ranging from benign to life-threatening conditions like bowel ischemia. PI is a rare entity with a reported incidence of 0.03% [The exact mechanism of PVG/PI remains poorly understood. However, certain theories may explain this phenomenon .Intestinal wall permeabilityA disruption of gastrointestinal mucosa allows intraluminal gas to pass into the mesenteric venous system. It is most commonly associated with bowel ischemia or obstruction.Bowel distensionAn increase in luminal gas pressure, secondary to bowel obstruction or endoscopic exams permits passage of luminal gas into the portal venous system.SepsisBacterial contamination of the portal venous system with a production of gas directly in the mesenteric system. It has been described in diverticulitis, perforated gastric ulcer, pancreatitis, and other inflammatory diseases of the bowel.The etiology of PVG/PI can be summarized in Table DiagnosisThe imaging modality of choice is the CT scan, as it may also identify the underlying cause of PVG. It appears as tubular lucencies branching from the porta hepatis to the edge of the liver. These lucencies are caused by the accumulation of gas in the portal vein where the centrifugal flow of blood carries the gas to the hepatic periphery. It must be differentiated from the presence of air in the biliary tree, also known as “pneumobilia” which is characterized by the central presence of gas. Pneumobilia occurs in large bile ducts at the hilum, due to the centripetal flow of bile ,10.Plain x-rays like erect chest and abdominal x-rays are the usual initial diagnostic tests. It may show branching, tubular lucencies extending from the porta hepatis to the edge of the liver and identify free gas under the diaphragm. But it is not always present . The M mManagementThe management of PVG is controversial because it’s a radiological entity and managing such cases without considering the clinical condition can lead to a fatal outcome. Historically, surgery was advocated in all cases of PVG as it was associated with high mortality and the etiology was poorly understood. It was then questioned whether we really need to operate in all cases of PVG. This was first answered by Schulz et al., who described successful non-operative management in PVG in iatrogenic cases .This shows that PVG per se doesn’t determine the decision algorithm. It is however the clinical status and underlying condition leading to PVG, which dictates the treatment. This notion is supported by Wayne et al. (2010), the largest series to date ( n=88), proposing surgical intervention for suspicious cases of mesenteric ischemia and non-operative management in benign causes . HoweverIn our case, the patient had mild abdominal pain and the CT scan of the abdomen although, it showed extensive PVG, the bowel was enhancing, and vessels were patent. That’s why we chose to observe him closely. But as soon as his clinical condition deteriorated, we took him immediately for surgery.Another challenge we faced, was the intraoperative finding when we identified superficial splenic abscess and a questionable segment of ischemic bowel. The question was which one to take out first and which one is contributing to his septic condition! The bowel was pale with sluggish peristalsis, but not obviously ischemic. So, we decided to keep the questionable segment of the small bowel for re-look and to perform the splenectomy. At re-look laparotomy, the segment of bowel remained unchanged, so we decided to do bowel resection and anastomosis. One possible explanation of ischemia of the small bowel is a low flow state as our patient was in sepsis or venous micro thrombosis.Various authors have described a management algorithm, but none seem perfect. Nelson et al. described the aggressive, be careful and conservative (ABC) algorithm, in which operative management (aggressive treatment), close monitoring (be careful), and medical treatment (conservative treatment) are based on the clinical condition of the patient. However, if PVG was detected on x-rays, he suggested for surgical intervention, which is considered obsolete . Yoo S-KWe propose a simple management algorithm that helps in the decision-making process, shown in Figure PVG is a rare radiological entity, However, whenever it is found, bowel ischemia must be kept in the differential diagnosis. This case highlights the importance of clinical correlation in the management of PVG. The learning point is that a surgeon should be vigilant of this entity and in case we choose to observe our patients, frequent assessments should be performed. At any point, If the clinical condition of the patient deteriorates, the threshold for exploration should be low in order to avoid delay in management.


A 1-year-old boy presented with a solitary alopecic atrophic area on the scalp and a history of a fluid-filled lesion at the site that had presented a few days after birth and healed with thinning of skin . ExaminaDermoscopy revealed  absent fAlthough all signs may not be present in a case, the classic dermoscopic findings described are : absence


Hemimegalencephaly (HME) and its more localized form - posterior quadrantic dysplasia (PQD) - are rare malformations of cortical development (MCD) that normally manifest as refractory focal epilepsy and cognitive impairment in children. We report a case study of a 19-year-old woman who presented with seizure-like symptoms to the emergency department after discontinuing her seizure treatment having fled her country. MRI revealed typical signs of PQD. This case study demonstrates how an unusual mild clinical presentation led to the late diagnosis of this rare MCD. Hemimegalencephaly (HME) is a rare congenital malformation of cortical development (MCD) characterized by enlarged and dysplastic hamartomatous overgrowth of one or all parts of a cerebral hemisphere ,2. HME mIn this study, we report the case of a 19-year old woman suffering from hemi-HME who was referred to our institution for neurosurgical counseling after visiting an emergency department after experiencing symptoms of fainting.A 19-year old woman visited the emergency department for the treatment of pre-syncopal symptoms without loss of consciousness and with flu-like symptoms. However, due to the presence of a linguistic barrier, the first language of the patient being Syrian and the physician speaking French, the collection of initial patient history was limited. Moreover, bloodwork did not indicate any significant anomalies; in particular, there were no signs of infection or inflammation. Furthermore, clinical examination was unremarkable and no signs of neurological deficits could be established. Non-enhanced CT, which was performed to rule out a central cause, revealed the presence of a large hypodense “mass-like” lesion in the left parieto-occipital region  showed marked enlargements of the left parieto-occipital lobe and left occipital horn, periventricular astrogliosis, and polymicrogyria with blurred cortical-white matter junction that is consistent with the diagnosis of PQD Figures -8. A translator assisted with the heteroanamnesis and revealed that the patient was born at home and had apparently experienced hypoxia during birth. Her mother had told her that she was blue for two days after birth; however, no further workup or hospitalization was done. Nonetheless, thereafter, her psychomotor development appeared to be normal. However, she then suffered her first seizure at the age of 8. The seizures appeared as bilateral tinnitus, which was followed by loss of consciousness and spasms that lasted for five to seven minutes. For this condition, she was medicated with lamotrigine, which reduced the frequency of seizures from a few times a week to once a year. However, her treatment had to be discontinued, as she had to flee her country because of war at the age of 16.Electroencephalography revealed a highly active epileptogenic focus with a spike-and-wave pattern in the left occipital region, which was suggestive of cortical dysplasia.Subsequently, treatment was reinitiated with a gradual increase in the dose of lamotrigine to 75 mg twice a day. Moreover, no notable adverse effects were reported. The patient still remains seizure-free after one year of treatment.Our case study presents classical MRI findings that are consistent with the diagnosis of PQD or hemi-HME. However, the clinical presentations were mild compared to the extent of the brain malformation. Moreover, seizures were easily controlled by a medical treatment, which led to the late discovery of PQD.Typically, patients with HME and PQD experience early-onset intractable seizures, which are poorly controlled by medical therapy, often requiring surgical interventions such as hemispherectomy ,12.HME is a rare condition, accounting for only 3% of cortical dysplasia diagnosed by imaging . HoweverBest diagnostic clues are obtained with MRI, due to its superior performance in imaging gray and white matter and its non-irradiating nature.Although HME and PQD are rare MCD, characteristic findings on MRI should be known to the radiologist. HME and PQD commonly affect the pediatric population and initial diagnosis in adults are much more infrequently reported.


Mediastinal teratomas occasionally rupture into the thoracic cavity, which induces mediastinitis or various other severe complications. Surgical treatment is crucial for ruptured teratomas; however, few literature reviews to date have addressed the characteristics of ruptured mediastinal teratomas.We report a 29-year-old woman with severe mediastinitis owing to a mediastinal mature teratoma that ruptured into the mediastinum and right pleural cavity. Surgical resection by median sternotomy was performed within 24 hours after emergency admission. Intraoperative findings demonstrated the ruptured wall of the tumor with exposure of its white contents, which appeared similar to skin and fat, and necrotic tissue in the anterior mediastinum. The tumor was adhered to the right upper lobe, the ascending aorta, and pericardium. Owing to the severe adhesion of the tumor caused by inflammation in the surrounding tissues, a small portion of the tumor could not be removed, and hence complete resection with a sufficient surgical margin was not achieved. Pathologically, the tumor consisted of a solid mass and a cystic mass with severe adhesion to the resected portion of the lung, which included skin and lipid tissue. The tumor was concluded to be a mature teratoma as neither an immature component nor malignant transformation was observed. The patient had an uneventful postoperative course.To our knowledge, this is the report of successful surgical resection of a ruptured mediastinal teratoma causing severe mediastinitis, with the first literature review of ruptured mediastinal teratomas. We also discuss relevant findings from reports in the literature. Mediastinal teratoma is the most common type of mediastinal germ cell tumor, which occasionally ruptures into the thoracic cavity and induces severe complications. Surgical intervention for ruptured teratomas is required in almost all cases. However, there are few reports to date regarding the indications and surgical procedures for the treatment of ruptured teratomas. Herein, we present a surgical case of a patient with a mediastinal mature teratoma that perforated into the mediastinum and thoracic cavity, resulting in severe mediastinitis and pleurisy.Benign mediastinal germ cell tumors constitute approximately 1.3%  of all mediastinal tumors A 29-year-old woman presented with chest pain that had started 2 months previously. Her right chest pain radiating to the back had suddenly worsened together with the appearance of dyspnea, and she was immediately admitted to our department. On admission, her body temperature, blood pressure, and pulse rate were within the normal range, but her oxygen saturation was 93% at room temperature, and her chest pain continued to worsen. A chest X-ray displayed widening of the upper mediastinum Fig. a. Chest The patient underwent median sternotomy and tumor resection with combined partial resection of the pericardium and right upper lobe. Intraoperative findings demonstrated the ruptured wall of the tumor with exposure of its white contents, which appeared similar to skin and fat, and necrotic tissue in the anterior mediastinum. The tumor was adhered to the right upper lobe, the ascending aorta, and pericardium Fig. a. PleuraMacroscopically, the tumor consisted of an 85 × 55 mm solid mass and a 90 × 50 mm cystic mass with severe adhesion to the resected portion of the lung, which included skin and lipid tissue Fig. b. MicrosThe patient had been treated with antibiotics for 7 days from immediately after admission, and the result of the pleural effusion culture was negative. The patient had an uneventful postoperative course and was discharged on postoperative day 11.Ruptured mediastinal teratomas are relatively rare. There are no standard strategies for their treatment, and physicians make treatment decisions for each individual case. We found that only 56 cases of ruptured mediastinal teratoma have been reported to date, in our literature search of associated or referenced articles for the keywords “mediastinal teratoma” and “rupture” in Pubmed®. To clarify the characteristics of ruptured mediastinal teratoma, we summarized these articles in Table 2. Therefore, the early diagnosis and treatment of ruptured mediastinal teratomas are crucial to reduce mortality3. However, our literature review demonstrated that surgical resection was performed 1 week after onset of the rupture in 48% of the patients (Table Severe chest pain occurred in patients when fluid with digestive enzymes or infectious materials from the ruptured tumor spread into the mediastinum or the thoracic cavity and caused inflammation. Necrosis or ischemia caused by tumor swelling also induces tumor rupture. Emergency surgery should be considered in such cases, because the possible resulting mediastinitis and pleurisy have a high risk of causing mortality4. They are classified as mature teratomas that comprised mature, adult-type tissues, and immature teratomas comprising embryonic or fetal tissues. Immature teratomas account for only 2% of all mediastinal teratomas, and are considered to be malignant neoplasms, which have the potential to metastasize or recur5. Immature teratomas comprise a spectrum of purely mature to predominantly immature tissue from all three germinal layers mixed with embryonic immature tissue. According to our literature review, 2% of patients were found to have malignant ruptured teratoma on postoperative histological analysis, such as an immature teratoma or a germ cell tumor with somatic-type solid malignancy, although most patients were diagnosed as having a mature teratoma (Table Surgical resection with a sufficient margin or complete resection is sometimes impossible because of severe adhesion of the tumor owing to inflammation in the surrounding tissue. A preoperative diagnosis to decide on the surgical strategy is important, even though it is difficult to identify the type of teratoma radiologically. Teratomas histologically consist of germ cell tumors with 2 or 3 germ layers, including the ectoderm, endoderm, and mesodermma Table . In suchWe herein reported a case of a patient in whom surgical resection was performed for a ruptured mediastinal mature teratoma that induced mediastinitis, as well as performed the first literature review of ruptured mediastinal teratomas. Our results demonstrate that thoracic surgeons should make appropriate and prompt decisions to treat ruptured mediastinal teratomas and to minimize their associated complications.Additional file 1. The following articles of Supplementary data were summarized in Table


Hereditary angioedema (HAE) is a rare genetic disease with numerous gastrointestinal manifestations. Intussusception, although rare, has been a reported complication with documentation of bowel wall edema on endoscopy during an acute flare. With the advent of synthetic C1 esterase inhibitors, this disease has become more effectively treatable. This case report shows a HAE flare complicated by colonic intussusception, treated with C1 esterase inhibitor, with complete endoscopic resolution seen on hospital day 5. This case provides evidence that with proper medical treatment, an HAE flare with intussusception has the potential to resolve without any further need for surgical or endoscopic intervention. Patients with hereditary angioedema (HAE) usually present with gastrointestinal symptoms of nausea, vomiting, abdominal pain, and diarrhea. Although mostly self-limiting, rarely these symptoms might suggest underlying colonic intussusception. Advent of C1-inhibitor (C1-INH) therapy in 2012 changed the therapeutic landscape in treatment of symptoms with HAE. Here, we present such a case of young man with HAE who presented with colonic intussusception and was treated with C1-INH with complete resolution.A 27-year-old African American man with a medical history significant for HAE type 2 marked by a qualitative deficiency in endogenous C1-INH protein presented with abdominal pain that had worsened during the past 72 hours. He had been unable to obtain outpatient C1-INH therapy for the past 6 months because of financial constraints. He reported left lower quadrant, crampy pain with loose black stools during the past 2 weeks. He had a similar presentation a month before when computed tomography (CT) imaging showed intestinal edema from the gastric antrum to proximal duodenum. He was then treated with a dose of C1-INH but unfortunately left against medical advice.On presentation, he was afebrile and hemodynamically stable. His examination showed a nondistended abdomen that was tender to palpation in the bilateral lower quadrants without rebound or guarding. Laboratory findings demonstrated a leukocytosis of 13.49k/μL, hemoglobin of 16.4 g/dL, and normal platelet count. The metabolic panel and liver chemistries were unremarkable. Abdominal and pelvic CT with contrast noted significant intestinal angioedema involving the bladder wall and transverse colon, most pronounced at the hepatic flexure, with telescoping of the large bowel at the splenic flexure consistent with nonobstructive intussusception, gastric, and small bowel edema seen on CT the previous month was not visualized  affects 1 in 10,000–50,000 persons across any ethnic group, although because of the rarity, the exact prevalence is unknown.2HAE is a rare autosomal dominant disease that occurs in 3 major types—affecting the C1-INH protein quantitatively , qualitatively , and affecting coagulation factor XII .5 Usually, flares or attacks of HAE are self-limited, lasting from 1 to 4 days before remitting.6 However, as described in the retrospective study above, 1 patient who presented with colonic intussusception required surgery.3 Interestingly, treatment with epinephrine, antihistamines, and corticosteroids are ineffective against HAE because they do not antagonize bradykinin.7 Hence, previous treatment included fresh frozen plasma and plasma kallikrein inhibitor.8 C1-INH proteins have been Food and Drug Administration approved for acute HAE episodes, and the intravenous medication we used on our patient  has been approved since 2012.10 Long-term prophylaxis is mainly achieved with plasma derived C1-INH, given subcutaneously at 60 IU per kg of body weight twice weekly or lanadelumab 300 mg subcutaneously every 2 weeks.The most common gastrointestinal manifestations include nausea and vomiting, colicky abdominal pain, and diarrhea, but intussusception in an adult is exceedingly rare Table .3–5 Usua11 Before the development of C1-INH therapy, such patients underwent invasive procedures including air-contrast enemas and abdominal surgery.3 Now, treatment of the HAE flare with medical management and subsequent air enema is often sufficient to avoid surgical or endoscopic intervention.11Colonic intussusception in adults is worrisome for malignancy because it occurs in 75% of such cases. Surgical adhesions may also be responsible; however, this patient had no previous surgical history. However, HAE is one of the rare benign causes for intussusception in adults.12 To date, no published endoscopic imaging has been reported of an HAE-induced intussusception after treatment with C1-INH inhibitor. Although this case is limited by the fact that endoscopy was performed on day 5 (remembering that attacks spontaneously resolve in 1–4 days), it does show that complete endoscopic resolution is visible within 5 days of treatment. Furthermore, it marks another successful case of medical therapy resolving the intussusception without the need for invasive intervention.Koruth et al in 2005 described the endoscopic appearance of colonic HAE during an attack with the expected edematous bowel wall.Author contributions: J. Roy wrote the manuscript and is the article guarantor. R. Vunnam, GS Venkata, D. Bethards, and T. McGarrity edited the manuscript.Financial disclosure: None to report.Previous presentation: This case was accepted to the Society of General Internal National Meeting; May 6–9, 2020; Birmingham, Alabama.Informed consent was obtained for this case report.


A retained surgical sponge, also known as a gossypiboma, is a rare cause of serious postoperative complications. Diverse retained surgical materials including instruments such as clamps and sutures have been reported, but surgical sponges are the most common material. We report an unusual case of a gossypiboma mimicking a complicated urachal cyst that led to perforation of the umbilicus.A 38-year-old female patient presented in our facility with a palpable periumbilical mass and discharge of pus from the umbilicus for 7 months after an open appendectomy. Since the onset of symptoms, the patient had been treated conservatively in a peripheral hospital where she had been operated on. As no improvement was seen, an ultrasound scan was performed that suggested an intraperitoneal abscess adjacent to the umbilicus. Consequently, the patient was referred to our specialist outpatient department for surgical intervention. Suspecting a complicated urachal cyst, an exploratory laparotomy was performed but revealed a retained surgical sponge as the underlying cause. The gossypiboma was resected, and the postoperative period was unremarkable.This case demonstrates that gossypibomas, even though rare, continue to occur. They may clinically and radiologically mimic other pathologies, especially abscesses and tumors. Preventive measures as well as the inclusion of gossypibomas in the differential diagnosis of intraabdominal masses or fistulation detected in patients with a history of surgery are of utmost importance to minimize morbidity, mortality, and potential medicolegal implications. A retained surgical sponge, also known as a gossypiboma, is a rare but serious postoperative complication that is a nightmare for every surgeon. A range of retained surgical materials including instruments such as clamps and sutures have been reported, but the surgical sponge, possibly because of its amorphous composition and frequent usage, is the most prevalent material , 2. For Gossypiboma may lead to chronic abdominal pain, an abdominal mass, bowel obstruction, occult infections, fistulae and transmural migration of the foreign body into the small and large bowel or stomach –3. We reA 38-year-old female patient presented to our facility in October 2019 with chronic abdominal pain, swelling in the umbilical region, and pus discharge from the umbilicus for 7 months. The swelling started 3 months after an open appendectomy was performed in a peripheral private hospital using a right paramedian incision. One week after the appearance of swelling, the umbilicus started discharging thick offensive fluid, which was associated with abdominal pain. There was no fever, nausea, or loss of appetite. The patient reported a weight loss of 5 kg in 7 months. Conservative treatment yielded no improvement. As a result of an abdominal ultrasound scan, which suggested an intraperitoneal abscess (Fig. 9/L. Further diagnostic tests and imaging could not be performed due to financial constraints.Upon presentation, the patient was depressed, dehydrated, and pale. The abdominal examination revealed an everted umbilicus with pus discharge and granulation tissue. In the umbilical region, there was a palpable intraabdominal mass that measured 20 × 16 cm, was moderately tender, and was fixed to the umbilicus but was otherwise mobile (Fig. Based on the history and physical findings, a provisional diagnosis of an infected urachal cyst or postoperative intraabdominal abscess was made, and the patient was prepared for an exploratory laparotomy.Intraoperatively, the abdominal cavity was clean. A well-circumscribed 18 × 16 cm adhesive mass was seen around the ileocecal junction. It consisted of an encapsulated lesion at its center, and an ileal segment, the transverse colon, and the umbilicus were all adhered to the mass Fig. b. DuringThe postoperative period was uneventful. Bacteriological examinations were not carried out due to financial constraints. Consequently, empiric antibiotic therapy with intravenous ceftriaxone and metronidazole was administered for 5 days and was then switched to oral antibiotics, which were given for an additional 2 days. Early postoperative enteral feeding was well tolerated with normal bowel activity on the third postoperative day. The laparotomy wound healed by primary intention, and the patient was discharged on postoperative day 9. Follow-up visits in our SOP clinic have also been unremarkable.Gossypiboma is described as a retained surgical sponge or towel after a surgical procedure , 4. OtheA retained surgical sponge is a preventable postoperative complication that usually occurs in emergency surgeries, unexpected surgical procedures, prolonged operations, and surgeries on unstable and obese patients. Furthermore, it is associated with inexperienced staff, poor organization in the operating theatre, and rapid or failed sponge counting during an operation , 6. The Essentially, there are two types of pathological reactions provoked by a retained foreign body: an exudative inflammatory process with the formation of an abscess or an aseptic fibrotic reaction that encapsulates the corpus alienum , 7. In oIntraabdominal gossypibomas are usually reported with signs of an intraabdominal mass, bowel obstruction, intestinal hemorrhage, or fistulation. Rarely, the retained surgical sponge undergoes transmural bowel wall migration without leaving a defect , 2, 8.The diagnosis of gossypiboma is often challenging, as there is generally a wide range of nonspecific manifestations, and most times, the actual diagnosis is not even considered. In line with this phenomenon, neither the peripheral hospital nor our facility included gossypiboma in the differential diagnosis.The presentation of our patient with a periumbilical mass and discharge of pus at the umbilicus prompted the potential differential diagnosis of an infected urachal cyst. Incomplete urachal obliteration during fetal development gives rise to distinct malformations of the median umbilical ligament, such as a urachal cyst. Most anomalies are asymptomatic and resolve during early infancy, but some go unrecognized until adulthood. These rare cases can present with acute abdominal symptomatology secondary to infection of the urachal remnant . The cliIn our patient, a high index of suspicion for gossypiboma should have been raised by the history of surgery associated with persistent abdominal pain, signs of infection, and a palpable mass. A preoperative diagnosis of gossypiboma using the clinical manifestation and recommended imaging modalities was observed by Rajiv et al. in only 75% of cases, highlighting the fact that a significant number of gossypibomas are diagnosed only intraoperatively , 12.In the case of radiopaque impregnation of a foreign body, diagnosis may be facilitated by radiographic and CT images. The sponge we evacuated showed no discernible impregnation, which may have been due to the primary absence of impregnation, as especially seen in resource-poor settings or disintegration , 10 of tRemoval of the retained foreign body by either open or laparoscopic surgery is the mainstay of treatment. However, if the retained foreign body migrates into the stomach, it could be endoscopically removed .Prevention of gossypibomas is essential and can be achieved by “simply” ensuring and insisting on a thorough pack count before closure of the operating cavity. A routine brief but careful postoperative wound and cavity exploration prior to wound closure should be performed by every surgeon. The use of sponges with radiopaque markers is now strictly recommended. Moreover, newer technologies such as radiofrequency chip identification and barcode scanners are helpful for decreasing the incidence of gossypiboma . HoweverThis case shows that the diagnosis of gossypiboma can be complicated by its nonspecific presentation, which clinically and radiologically imitates other common pathologies, such as abscesses and tumors, and rare conditions, such as urachal cysts. Moreover, gossypiboma is associated with significant morbidity, mortality, and potential medicolegal implications. Consequently, prevention of its occurrence and the inclusion of gossypiboma in the repertoire of actively thought differential diagnoses in patients with intraabdominal masses or fistulation concurrent with a history of surgery are of utmost importance.


Among all melanomas, the mucosal type is very rare and may occur in the sino-nasal mucosa, vagina, anus and the oral cavity. At variance with melanomas of the skin, no risk factors, such as familiarity, UV-exposure and skin phenotype, have been clearly identified for such neoplasms. Frequently, the diagnosis is delayed and achieved at advanced stages or when metastases have already occurred. The authors report on a case of mucosal melanoma of the oral cavity presenting as a mass of the hard palate in a 50-year old male, and the corresponding diagnostic-therapeutic pathway. Primary malignant melanoma (MM) supposedly originates from melanocytic precursors via the possible presence of pre-malignant lesions ,2,3. LesSeveral cases of MM of the head and neck have been reported in the literature, but often no clear evidence showed whether such lesions could be considered primary or metastatic. This study reports on the salient clinical and histological features of one case of MM and highlights some peculiar immunohistochemical findings, which may be misleading when not accompanied by extensive sampling at distinct areas of the lesion.A 50-year old Caucasian male was referred to the Department of Odontostomatology of the University of Bari Aldo Moro  with a three-month history of swelling and bleeding of the anterior maxillary area. His medical history was unremarkable, and the patient denied alcohol or smoking addiction. Oral examination revealed a firm and soft nodule, measuring approximately 4 × 6 cm, with pale appearance and focally ulcerated, extending bilaterally to the hard palate. In close proximity to the main nodule, black plaque and multiple pigmented lesions, involving the upper anterior gingiva were evident, which also extended towards and partly covered the non-removable dental prosthesis . No addiPanoramic radiograms  and CT-sAn incisional biopsy of the mass was performed in an area without pigmentation, and the tissue fragment sent for histopathologic examination. Microscopically, the hematoxylin and eosin stained sections revealed a fragment of oral mucosa, lined by keratinized and hypotrophic stratified squamous epithelium , and a dImmunohistochemical stains were performed to further characterize the neoplastic cell population, which highlighted pan-cytokeratins (with a dot-like pattern) and S-100 protein a positivAn additional histopathological examination with much more extensive sampling was performed, showing an intra-mucosal hyper-melanotic lesion a composeThe palatal mucosa healed with residual perforation a, and a MM is very rare and represents 1–8% of all melanomas . Such neAlcohol, cigarette smoking and formaldehyde exposure have been suggested as potential risk factors for oral melanomas ,12, althMucosal MMs usually are immunoreactive for S-100 protein and, less consistently, for HMB45 and Melan-A; also, previous reports highlight positivity for cytokeratin markers, although with variable distribution, diffuse staining being usually absent ,16. MitoThe latter include melanotic macule, amalgam tattoo, oral melanoacanthoma, oral nevus, poorly differentiated carcinoma and spindle cell/epithelioid sarcomas ,21,22,23Oral nevi consist in accumulations of melanocytes either at the junctional area or within the lamina propria; at variance with MM, they display a symmetrical shape, and are composed by compact clusters of tumor cells, displaying minimal or no nuclear atypia and lacking mitotic activity . Also, iPoorly differentiated carcinomas may be more intriguing and difficult to rule out in view of their variable morphologic features and inconstant expression of epithelial markers, such as cytokeratins. Some of them may also show S-100-positivity, particularly those with myoepithelial differentiation; nevertheless, the use of additional epithelial markers, such as Epithelial Membrane Antigen and the lack of HMB45 and Melan-A immunoreactivity may be resolutive in this regard, the latter two antigen being usually absent in the vast majority of carcinomas and sarcomas, with few exceptions ,15,21. IPrimary MMs of the oral cavity are very rare and, usually, their occurrence is asymptomatic in the early stages, thus frequently leading to a delayed diagnosis. Also, as their morphological features are often misleading or non-specific, the diagnosis must rely upon accurate histopathological investigation and extensive immunohistochemical evaluation too. It is worth emphasizing that frequently melanocyte-specific antigens (Melan-A and HMB-45) are unevenly distributed in MMs, and unexpected cytokeratin positivity may occur, which may result in an inaccurate diagnosis.