Contrast-enhanced ultrasound (CEUS) is increasingly being used in children. One of the most common referrals for CEUS performance is characterization of indeterminate focal liver lesions and follow-up of known liver lesions. In this setting, CEUS is performed with intravenous administration of ultrasound contrast agents (UCAs). When injected into a vein, UCA microbubbles remain confined within the vascular network until they dissipate. Therefore, visualization of UCA within the tissues and lesions corresponds to true blood flow. CEUS enables continuous, real-time observation of the enhancement pattern of a focal liver lesion, allowing in most cases for a definite diagnosis and obviating the need for further cross-sectional imaging or other interventional procedures. The recent approval of Lumason  for pediatric liver CEUS applications has spurred the widespread use of CEUS. In this review article we describe the role of CEUS in pediatric liver applications, focusing on the examination technique and interpretation of main imaging findings of the most commonly encountered benign and malignant focal liver lesions. We also compare the diagnostic performance of CEUS with other imaging modalities for accurate characterization of focal liver lesions.The online version contains supplementary material available at 10.1007/s00247-021-04976-2. Common benign solid liver lesions in the pediatric population include infantile and congenital hemangiomas and focal nodular hyperplasia, whereas hepatic adenoma and regenerative nodular hyperplasia occur infrequently . Th. Th60]. The CEUS enhancement pattern of the congenital hemangioma is similar to that of infantile hemangioma, demonstrating typically peripheral nodular enhancement during the arterial phase, with centripetal continuous filling during the late portal venous phase Fig.  57]. In. In57]. Focal nodular hyperplasia (FNH) is a benign tumor that is most frequently found in young to middle-age adults, though it is also encountered in the pediatric population, typically between the ages of 2 years and 5 years. It can also be seen in oncology patients following radiation and chemotherapy , 61. PeoFocal nodular hyperplasia is thought to represent a proliferative hepatocellular response caused by increased blood flow resulting from a dystrophic artery. Histologically, it is composed of benign-appearing hepatocytes arranged in nodules that are usually partially delineated by fibrous bands originating from the central fibrous scar that contains the dystrophic arterial vessels , 63. AtyBecause this lesion is composed of normal hepatocytes, on US it appears well-circumscribed and nearly isoechoic with the adjacent normal liver, although it can also be hypo- or, rarely, hyperechoic . The cenIn a recent pediatric CEUS study, classic FNH showed the presence of a central feeding artery that is visible in the early arterial phase and demonstrates a centrifugal filling pattern, with stellate (spoke-wheel) hyperenhancement in the arterial to early portal venous phases, and iso- or hyperenhancement in the late portal venous phase Fig.  7, 66]., 66.7, 6There are some contradictory results regarding the correlation between the lesion size and the identification of the central stellate scar on CEUS. Some investigators showed that the classic spoke-wheel pattern depended on the size of the lesion and was more frequently seen in larger FNHs, typically those greater than 3 cm , 68. OthHepatic adenomas are benign, typically solitary lesions that are frequently diagnosed in women ages 35–40 years. Although hepatic adenomas are very rare in children, they are encountered more often in young girls who take oral contraception, in children who are on steroid therapy and in those with underlying metabolic disease such as galactosemia and glycogen storage disease , 71. A h, intralesional necrosis and hemorrhage). Uncomplicated hepatic adenomas on US tend to be homogeneous lesions with similar reflectivity to the adjacent normal liver, or they appear relatively hyperechoic if there is high intralesional lipid content. However, on a background of diffuse fatty liver infiltration or glycogen storage disease, adenomas might appear hypoechoic .Fig.Fig76].FOne previous study evaluating the characteristics of focal fatty infiltration of 25 lesions in adults reported a variable frequency pattern in the arterial phase, including isoenhancement (44%), hyperenhancement (12%) and hypoenhancement (44%). In the portal venous phase all lesions (100%) demonstrated isoenhancement, and these areas could not be distinguished from adjacent liver tissue . This vaRegenerative nodules, also known as “nodular regenerative hyperplasia,” occur as a hyperplastic response of hepatocytes to an insult. In the rare cases that they occur in children, they are often associated with conditions leading to chronic liver disease  or to previous use of steroids and antineoplastic medication .The appearance of regenerative nodules on US is variable and depends on the size of the nodules. Small nodules might not be apparent, but if there are many of them the liver appears with diffusely heterogeneous echotexture or distortion of normal architecture. If nodules are visible, they are generally well-circumscribed, homogeneous, and hypoechoic or hyperechoic compared with the normal liver , 81.On CEUS these nodules show similar enhancement with the background liver parenchyma throughout all the enhancement phases. They are distinct from their malignant counterparts in that they do not show washout during the portal venous or late phases .Among the primary and secondary malignant hepatic tumors known in children are hepatoblastoma, hepatocellular carcinoma, undifferentiated  sarcoma, angiosarcoma, embryonal rhabdomyosarcoma, lymphoma and metastasis. Their appearance is variable on gray-scale US and can overlap with that of benign lesions. A recent meta-analysis of 57 studies showed that CEUS has excellent diagnostic accuracy in differentiating malignant from benign focal liver lesions, with a pooled sensitivity and specificity of 0.92  and 0.87 (95% CI: 0.86–0.88), respectively . The aveThe arterial-phase enhancement pattern of malignant lesions can be variable. For example, hypervascular metastasis and hepatocellular carcinoma exhibit hyperenhancement during the arterial phase, while some metastases appear as hypoenhanced or demonstrate a rim-like enhancement in the arterial phase. Larger malignant lesions might show a disorganized internal vasculature, which becomes more visible on CEUS studies than it does on non-contrast ultrasound, CT or MRI.It is important to consider that the enhancement pattern of hepatic inflammatory and infectious lesions on CEUS can be like those of primary liver malignancy and metastatic disease. Because of the hyperemia associated with infection, abscesses have a peripheral rim of hyperenhancement during the arterial phase of CEUS. If the central component is replaced by purulent material, then the center does not enhance. However, depending on the degree of liquefaction the central aspect might also be hypoenhancing or have a well-defined non-enhancing region surrounded by enhancing septations. During the venous phase, inflammatory and infectious lesions show hypoenhancement. These patterns can be indistinguishable from malignant lesions, and biopsy might be required if the history, clinical signs and symptoms are not clear , 85.Hepatoblastoma is the most common primary malignant pediatric liver tumor, accounting for more than 90% of liver cancers in children younger than 5 years. It usually occurs sporadically, but it is sometimes associated with prematurity, Beckwith–Wiedemann syndrome, hemihypertrophy and familial adenomatous polyposis coli , 86. It Hepatoblastoma is a well-circumscribed, encapsulated, large solid mass with a lobulated contour. Epithelial hepatoblastomas typically demonstrate a relatively more homogeneous appearance, while mixed epithelial and mesenchymal tumors appear markedly heterogeneous. Teratoid components appear cystic. Areas of hemorrhage or necrosis within the tumor are common . Coarse On CEUS hepatoblastoma can show early peripheral enhancement during the arterial phase and marked contrast washout during the late portal venous phase Fig.  4]. It . It 4]. Hepatocellular carcinoma (HCC) is the second most common primary malignant liver tumor in children, accounting for 20–30% of primary hepatic malignancies. It is usually found in older children and adolescents (10–14 years) but has also been found in children younger than 5 years. Several diseases in children can lead to cirrhosis, which increases the risk of developing HCC  . HoweverThe enhancement patterns of HCC on CEUS have been detailed in adults using the CEUS Liver Imaging Reporting and Data System (LI-RADS) . This syOn CEUS, HCC typically demonstrates arterial-phase hyperenhancement, which can involve diffusely the whole lesion or a portion of the lesion (in what is known as nodule-in-nodule or mosaic appearance). Another typical feature is washout, which is late in onset (beginning 60 s after contrast injection) and mild in degree (the lesion becomes less enhanced than the surrounding liver but still demonstrates some degree of persistent contrast enhancement within 2 min after contrast injection) . The CEUA common feature of all metastatic liver lesions on CEUS is contrast washout, which is typically rapid (beginning within 60 s after contrast injection) and marked (the lesion is completely devoid of contrast within 2 min after contrast injection), resulting in a “punched-out” appearance  and contrast (left) images. In the arterial phase, the lesion demonstrates a peripheral, nodular, discontinuous enhancement pattern. In the portal venous phase, the lesion appears homogeneously hyperenhancing, whereas in the late phase, the lesion remains hyperenhancing compared to the adjacent liver parenchyma A 2-week-old boy with elevated liver enzymes. Same patient as Fig. Online Supplementary Material 2left) and contrast (right) images. During the early arterial phases, rapid enhancement of the lesion originates from the center and progresses centrifugally in in a spoke-wheel pattern. In the portal venous phase, the lesion is homogeneously hyperenhancing and remains hyperenhancing during the late phase, where a central hypoenhancing scar is evident. This enhancement pattern is in keeping with focal nodular hyperplasiaA 17-year-old girl with abdominal pain. Focal liver lesion was identified on US that was performed for appendicitis. Sagittal contrastenhanced ultrasound (CEUS) cinematic clip of the liver with dual display of gray-scale  and contrast-enhanced (right) images. There is hyperenhancement of the lesion in the early arterial phase followed by isoenhancement and slow washout. These contrast-enhanced US features are typical of HCC A 14-year-old boy with fibrolamellar hepatocellular carcinoma (HCC). Sagittal contrast-enhanced ultrasound (CEUS) cinematic clip of the right liver lobe with dual display of gray-scale  and contrast-enhanced (right) images. There is early washout of the lesion beginning at about 10 s. Early washout is typical of liver metastases on CEUS A 7-year-old girl with Wilms tumor and metastatic liver disease. Sagittal contrast-enhanced ultrasound (CEUS) cinematic clip of the liver with dual display of gray-scale (


Splenic injury is usually caused by blunt trauma to the abdomen. Very rarely, spontaneous rupture can occur in patients with splenomegaly due to various underlying pathological conditions such as hematological, neoplastic, inflammatory, and infectious diseases. Here, we report the case of a 48-year-old gentleman who presented to the emergency department with sudden-onset pain in the abdomen and hypotension. Blood investigation revealed anemia and leukocytosis with blast cells on peripheral smear, suggestive of chronic myeloid leukemia (CML) in the chronic phase. Contrast-enhanced computed tomography revealed splenomegaly with grade three splenic laceration and a subcapsular hematoma with hemoperitoneum. Because of persistent hemodynamic instability, despite resuscitation, he underwent emergency splenectomy. The postoperative period was uneventful. Bone marrow biopsy revealed CML in the chronic phase with World Health Organization grade I reticulin fibrosis. Subsequently, he was started on hydroxyurea and discharged for further follow-up with medical oncology. Spontaneous splenic rupture (SSR) is a rare clinical entity, with life-threatening complications . Prompt A 48-year-old gentleman, a known case of rheumatic heart disease with mitral valve regurgitation and bicuspid aortic valve, presented to the emergency department complaining of pain in the left upper abdomen for two days. The pain was sudden in onset, progressive, not responding to analgesics, and associated with abdominal distension. There was no history of nausea, vomiting, fever, and constipation. Additionally, there was no history of trauma or any surgery. On presentation, he was conscious and oriented, with tachycardia (120 beats/minutes) and hypotension (90/60 mmHg). On examination, his abdomen was distended with tenderness in the left hypochondriac region.Blood investigation showed normocytic normochromic anemia (hemoglobin 6.5 g/dL) and a total leucocyte count of 1.94 lakhs/dL. Peripheral smear revealed 3% blasts cells, myeloid bulge, and basophilia, suggestive of CML in the chronic phase. Ultrasonography of the abdomen revealed free fluid abdomen, splenomegaly, and a subcapsular splenic hematoma. Contrast-enhanced computed tomography (CECT) confirmed splenomegaly (19 cm) with a grade three splenic laceration (3.3 × 2.6 cm) in the lower pole with a subcapsular hematoma (>50% of surface area) with hemoperitoneum  with a 3 cm laceration in the lower pole and a ruptured subcapsular hematoma , in association with fever, nausea, or vomiting . Rarely,SSR, similar to traumatic injury, can be managed either surgically (splenectomy) or conservatively . If the patient is hemodynamically stable, conservative management can be attempted. However, compared to patients with blunt abdominal trauma, the rate of successful non-surgical management is low . Of all SSR is an important differential diagnosis for an acute abdomen, especially in patients with splenomegaly. In a case of atraumatic splenic rupture, similar to traumatic injury, prompt recognition and surgical intervention are the best means of definitive care. Given the relatively high failure rate for non-operative management, total splenectomy is the preferred surgical option.


A 56-year-old right-hand-dominant man with a history of hypertension presented with a left thenar eminence cystic mass that started 2 months earlier. The patient denied symptoms, trauma, drainage, or pain but was concerned as he had had a tumor removed from his left forearm previously. The mass was stable in size. On examination, the mass was 1.0 × 0.5 cm. The digits on the patient's left hand were neurovascularly intact; the patient had full range of motion and normal hand cascade. The differential included schwannoma versus ganglion cyst. The patient underwent operative excision and had an uneventful postoperative course. Pathology showed “acral skin with an encapsulated nodular tumor in the dermis, composed of a few ducts, some adipocytes and sheets of ‘plasmacytoid’ cytoplasm, focally residing in a myxoid stroma…. The appearances are consistent with a hyaline  cell-rich mixed tumor or skin.” The case report was deemed institutional board review exempt.What is chondroid syringoma?How does chondroid syringoma present?What are its histological features?What are the treatment and prognosis?-4Chondroid syringomas are mixed tumors of the skin composed of epithelial and mesenchymal elements.2,1,1,3,Chondroid syringoma usually presents in the head and neck region.1On histology, these tumors appear similar to benign salivary gland mixed tumors.,1,3,3,The treatment of chondroid syringoma is excision. Excision is also diagnostic.1,Chondroid syringoma is a benign mixed tumor of the skin.1


A 12-year-old Japanese boy was referred to our hospital for evaluation of a radiopaque area on the left side of the mandible. Radiographic and computed tomographic examinations revealed a radiopaque lesion located on the lingual side, along with permanent tooth eruption. Several small tooth-like structures were noted within the lesion and the mandibular left second premolar was inclined in a mesial direction. An odontoma was clinically diagnosed and surgical removal by an endoscopic intraoral approach under general anesthesia was planned. Reports of oral surgery using an endoscopic approach have been presented, though none for an odontoma. With the expectation that removal of the odontoma would improve dentition in this case, we planned future management. A minimally invasive surgical removal procedure by an endoscopic intraoral approach from the lingual side was performed and good early recovery was noted. The resected tumor consisted of several small tooth-like structures. Histopathological diagnosis was a compound odontoma. One-year follow-up findings showed that the post-surgical course was good. Odontomas are one of the most common benign odontogenic tumors that occur in the jaw and are composed of enamel, dentin, cementum, and pulp tissue ,2. The 4A 12-year-old boy was referred to the Department of Oral and Maxillofacial Surgery at Osaka University Dental Hospital for evaluation of a radiopaque area on the left side of the mandible. There was no special mention of systemic history and the patient reported no symptoms in the affected area. An intraoral examination revealed no obvious gingival swelling around the left mandibular region A, thoughUnder general anesthesia, minimally invasive surgical removal with an endoscopic intraoral approach from the lingual side was performed A–C. SincAdjacent tooth roots showed no problems A,B. One Odontomas are one of the most common benign odontogenic tumors that occur in the jaw, and can be divided into complex and compound type ,2,3. AmoComplications associated with an odontoma include eruption disturbance and tooth malposition, while these tumors have also been linked to root resorption of adjacent teeth ,15. In tTeeth that erupt in a low position may have increased risk of dental caries or periodontal problems due to difficulties with oral hygiene status, and can also cause various other problems. Such a condition can be prevented by early detection and extraction for avoiding abnormalities in the direction of tooth eruption. Diagnosis based on panoramic radiography results is important when malalignment is observed. However, extraction of an odontoma located between adjacent roots of teeth under formation is risky. Therefore, it is important to detect such a lesion early and consider the timing of extraction while observing the eruption of permanent teeth. In the present case, we planned dentition management with the expectation that removal of the odontoma would improve the condition.A minimally invasive surgical approach is important for function preservation, as well as patient satisfaction and early recovery. In the present case, careful removal of the tumor in a manner to spare bone not damage the premolars under root formation was considered important. Endoscopic technology, typically employed for a minimally invasive procedure, is widely used with various surgical techniques to reduce tissue invasion during surgery and minimize bleeding ,8. In thIn the present case, CT results showed several small tooth-like structures within the lesion and their location on the lingual side. A method performed under direct vision is usually selected for removal of an odontoma, though limitations include difficulty with visualizing the lingual side or near the tooth root, which subsequently increases operative time and risk of iatrogenic damage, such as damage to neurovascular structures or excessive bone removal. Since it was difficult to see the lesion directly from the lingual side or with a dental mirror, we decided to use an endoscope for this procedure. With development of intraoral endoscopy techniques, more conservative bone removal has become possible, and we were able to avoid the risk of root damage in the adjacent tooth and confirm that the lesion had been definitely removed.


Sarcoidosis-lymphoma syndrome associated with portal hypertension is very rare. A 68-year-old female presented with a 5 kg weight loss in 6 months. Soluble interleukin-2 receptor activity was increased and total platelet count was decreased. Contrast-enhanced computed tomography showed the presence of hepatosplenomegaly and a 3 cm-sized tumor in segment 3 of the liver. The hepatic venous catheterization showed mild portal hypertension. On fluorodeoxyglucose-positron emission tomography/computed tomography, progressive malignant lymphoma was suspected. However, bone marrow biopsy showed multiple noncaseating granulomas. A laparoscopic liver biopsy revealed that the liver tumor had features of Hodgkin lymphoma. There were multiple noncaseating epithelioid granulomas in the portal tracts of the liver. Splenectomy for splenomegaly and partial hepatectomy for the liver tumor were performed. Pathological examination of the resected specimens revealed multiple noncaseating epithelioid granulomas in the liver and spleen. Histopathology of the liver tumor confirmed classic Hodgkin lymphoma with mixed cellularity. We conclude that hepatic venous catheterization, positron emission tomography/computed tomography, and pathological examinations of bone marrow, liver, and spleen are crucial for the diagnosis of sarcoidosis-lymphoma syndrome associated with portal hypertension. Sarcoidosis-lymphoma syndrome is a rare condition. It was described as coexistent sarcoidosis and malignant lymphoproliferative disease A 68-year-old female attended our hospital for a weight loss of 5 kg in half a year. Anamnesis revealed a history of breast cancer for which the patient received surgery 2 years ago. At that time, splenomegaly and thrombocytopenia were noted. However, they were left unattended. She had no history of alcohol consumption and smoking. At the time of outpatient consultation, physical examination revealed a conscious patient with correct respiratory and hemodynamic parameters. The rest was unremarkable.4 /µL (12.3 – 33.1 × 104); total bilirubin 1.5 mg/dL (0.3 – 1.3); albumin 4.5 g/dL (3.8-5.0); prothrombin time 87.4% (70 - 130); C-reactive protein 0.16 mg/dL (<0.16); carcinoembryonic antigen 1.9 ng/mL (0-5.0); α-Fetoprotein (AFP) 1.9 ng/mL (0-20); protein induced by vitamin K absence or antagonist-II (PIVKA-Ⅱ) 39 mAU/mL (<40); soluble interleukin-2 receptor (sIL-2R) 3365 U/mL (122-496); Mac-2 binding protein glycosylated isomers (M2BPGi) 6.29 COI (2+) (<1.00); the retention rate of indocyanine green at 15 minutes (ICG15) 32 % (<10). Antimitochondria antibody and antinuclear antibody were negative. Hepatitis B surface antigen and hepatitis C virus antibody were negative. The Child-Pugh score was 5 and the class was A. From these data, non-alcoholic steatohepatitis (NASH)-related liver fibrosis with thrombocytopenia and lymphoproliferative disease were suspected.Laboratory tests revealed hemoglobin 12.9 g/dL ; total leukocyte count 3320 /µL (3500 - 8000); total platelet count 6.8 × 102, respectively. The splenic venous flow volume was 1013 mL/min and showed a marked increase. The mean blood flow velocity, cross-sectional area, flow volume, and congestion index of the portal vein were 35.5 cm/sec, 0.90 cm2, 1913 mL/min and 0.025 cm*sec, respectively.Abdominal ultrasonography showed the presence of hepatosplenomegaly, 3 -cm-sized homogeneous hypoechoic tumor with irregular margin in segment 3 of the liver and gallbladder stones. The mean blood flow velocity and cross-sectional area of the splenic vein were 21.5 cm/sec and 0.79 cm2, the liver volume (LV) and LV/body surface area were 2461 mL and 1517 mL/m2, respectively. Spleen/LV ratio Abdominal plain CT showed a solitary, hypoattenuating tumor in the left lobe. The arterial phase of dynamic CT showed tumor enhancement with central part sparing. The tumor was demonstrated clearly in the portal phase. The equilibrium phase of dynamic CT showed the lesion showed persistent enhancement A–D. 3D-COn magnetic resonance imaging (MRI) of the liver, the tumor was visible in the left lobe which presents hypointense in T1-weighted image A and sliEarly and late phases of hepatic arteriography showed fine tumor staining in segment 3 A and B. 18F-fluorodeoxyglucose (FDG)-positron emission tomography/computed tomography (PET/CT) was performed. In the liver, there were intensive uptakes in the tumor, with maximum standardized uptake values (SUV max) of 21.0 in segment 3. Accumulation of liver parenchyma other than the tumor was not abnormal. The accumulation of the spleen was equivalent to that of the liver, and it could be said that the accumulation was enhanced. There was diffuse hyperaccumulation in the bone marrow of the spine, pelvic bones, ribs, and upper and lower limbs . Axial fusion image showed lymph nodes (arrowhead) around the hepatic artery and around the aorta with intensive uptake   A–C. Progx, 21.9) A and B. Laboratory investigations for possible causes of granulomas were added. Mycobacterium tuberculosis-specific interferon-γ release assay was negative. Angiotensin-converting enzyme was 29.3 IU/L/37 °C For histological diagnosis of the liver tumor and its extension, laparoscopic liver biopsy A and B wHowever, it was still unclear whether the splenomegaly was due to sarcoidosis, lymphoma, or any other causes. Therefore, splenectomy for splenomegaly and partial hepatectomy for liver tumor were performed. Intraoperative portal venous pressure decreased from 24.5 to 13.5 cmH2O immediately after splenectomy. There were gallbladder stones, and cholecystectomy was performed at the same time.4 /µL. Pathological examination of the resected specimens revealed multiple noncaseating epithelioid granulomas in the liver, spleen, gallbladder, and small lymph nodes near the cystic duct. In the liver and spleen, most noncaseating epithelioid granulomas were present in hepatic portal tracts and splenic white pulps -PET/CT was performed after the surgery. Lymph nodes around the hepatic artery and around the aorta, which showed high accumulation by FDP-PET/CT, showed low accumulation by MET-PET/CT. The findings were consistent with sarcoidosis  and 18F-α-methyltyrosine (FAMT) in malignancy has been developed and clinically established. Accumulation of amino acid radiotracers in malignant tissues is thought to be due to increased amino acid metabolism. Several clinical trials on the evaluation of sarcoid lymphadenopathy using MET and FAMT in comparison with FDG have been published Recently, the use of amino acid analog radiotracers, such as Hepatic venous catheterization is a basic examination for portal hypertension. Use of hepatic venous catheterization for the assessment of hepatic sarcoidosis has also been reported ,8. AlthoIt is estimated that 7% of patients with sarcoidosis have splenic involvement, which is commonly asymptomatic Sarcoidosis-lymphoma syndrome is rare, hepatic sarcoidosis with portal hypertension is uncommon, and primary hepatic Hodgkin lymphoma is infrequent. Our case of primary hepatic Hodgkin lymphoma with sarcoidosis accompanied by portal hypertension is extremely rare. The diagnosis was difficult. Two things could be learned from the current case. One was the diagnostic approach and the other was the pathophysiology. We conclude that hepatic venous catheterization, PET/CT, and pathological examinations of bone marrow, liver, spleen, and lymph nodes are crucial for the diagnosis of primary hepatic Hodgkin lymphoma with sarcoidosis (sarcoidosis-lymphoma syndrome) accompanied by portal hypertension.Written informed consent was obtained from the patient for publication of this case report and accompanying images.


Cemento-ossifying fibroma (COF) is a benign tumor classified under fibro-osseous lesions characterized by the proliferation of fibrous tissue associated with the presence of osteoid or cementicle-like masses. COF of bony origin is highly neoplastic in nature compared to their soft tissue counterparts which are relatively rare. The authors here present a case report of COF arising from the left upper lip in a 10-year-old female patient. The lesion was initially asymptomatic, slow-growing in nature, and later turned painful over a period of eight months. A medical CT was taken to elicit a calcified mass seen at the left subcutaneous plane of the upper lip. The lesion was surgically treated by complete excision under local anaesthesia and sutured. Follow-up was done for a period of one year to assess for recurrence which was not evident in this case. This case report, being a peculiar case of COF arising from the soft tissue of the upper lip, describes the clinical presentation, diagnostic imaging, histopathological evidence, and brief surgical management of the lesion. Cemento-ossifying fibromas (COF) are well-circumscribed, generally slow-growing, benign lesions of the jaws . The tumA ten-year-old female patient reported to the author’s clinic with a complaint of painful swelling on the left upper lip region. The patient had first noticed the swelling eight months back, which was initially asymptomatic and gradually increasing in size. The patient later developed pain in the region of swelling over the last two months. The pain was dull and continuous in nature. The patient had no known underlying medical conditions. Family history revealed no significant finding.Extra-oral examination revealed a marked asymmetry, with a swelling arising from the left side of the upper lip at the region of canine fossa. The overlying skin appeared normal. The swelling was hard in consistency, had a normal temperature, was tender on palpation and the overlying skin was pinchable. There was no paraesthesia or lymphadenopathy.On intra-oral examination, the swelling appeared ovoid in shape, measuring approximately 1.5 cm x 1 cm. The overlying mucosa appeared normal with no secondary changes . The lip was retracted and the lesion was fixed by pressing a finger against the base of the tumor. An incision was placed over the labial mucosa covering the lesion Figure . Once thThe excised lesion was elliptical in shape and was 1 cm x 0.6 cm x 1 cm in size. It was greyish white in color, hard in consistency, and gritty in texture. The tissue was covered entirely by a capsule. Microscopic examination of the Hematoxylin and Eosin stained section revealed a well-circumscribed and fully encapsulated ﬁbro-osseous lesion characterized by abundant cellular ﬁbrous tissue with scattered trabeculae of lamellar bone and spherical ossicles. These ossicles exhibited varying degrees of calcification. These psammoma bodies like ossicles were acellular with a concentric lamination. The lesion is suggestive of a psammomatoid variant of juvenile ossifying fibroma. No cellular atypia or dental tissues were present Figure .Modified Gallego's staining of the decalcified section revealed cementum-like deposits in red color Figure .The patient was recalled after seven days postoperatively. The suture was removed and the surgical site was well irrigated with normal saline. The site appeared to be healing well and the patient did not report any sign of discomfort. The patient was recalled after one month and the surgical site was completely healed. Follow-up was done at periods of three months, six months, and one year. There was no evidence of recurrence of the lesion after one year.Peripheral COF is a focal, reactive, non-neoplastic tumor-like growth of soft tissue. Cementifying fibromas may be clinically and radiographically impossible to separate from ossifying fibromas . It is wThe possible differential diagnosis to this case report includes myositis ossificans, traumatic fibroma, and localized osteosarcoma. Myositis ossificans have a histological feature of central fibroblastic tissue, followed by a zone of trabeculae and a peripheral zone of calcification with lamellar bone which is absent in this case. The possibility of traumatic fibroma was excluded due to the absence of any clinically evident stimulus or injurious habit to confirm the same apart from the histopathological differences. Osteosarcoma is undoubtedly ruled out due to the benign nature of the lesion that was histologically confirmed. COF is a peculiar benign lesion that presents with calcified masses of apparently odontogenic origin nevertheless, it can also arise from ectopic regions.Peripheral COF, per se, is a slow progressing lesion, with limited growth. It has a recurrence rate of 8% to 20%, thereby necessitating the removal of the lesion in toto . A folloThe reported lesion has a definitive diagnosis of peripheral COF with the histopathological examination under H/E staining revealing fibrous stroma with ossicles. Furthermore, Gallego's staining confirmed the presence of cementicles ruling out the possibility of traumatic fibroma or myositis ossificans. Histopathology showed no presence of giant cells in connective tissue stroma, thus ruling out the possibility of peripheral giant cell granuloma.This case report demonstrates the rare occurrence of COF in the upper lip and sequential treatment plan elaborating on the investigations, histopathology, and the management of the lesion with a follow-up for one year revealing no evidence of recurrence.


Sinonasal malignant melanoma is a rare but aggressive tumor of the head and neck area. It has a poor prognosis. Common symptoms are nasal obstruction, epistaxis, or purulent rhinorrhea. Diagnosis relies on histopathology with immunohistochemistry (IHC) studies. Surgery is the essential treatment, most often supplemented by radiotherapy or immunotherapy. A 63-year-old female patient, with a history of right dacryocystorhinostomy and Parkinson's disease, consulted for symptoms of right nasal obstruction with increasing intensity accompanied by two episodes of mild unilateral epistaxis. Rigid optic examination showed a white-pinkish right obstructive supra-centimetric endonasal tumor. CT revealed an extensive tissue process of the right nasal cavity invading the maxillary sinus, the inferior and middle conchas. A biopsy of the lesion was conducted under local anesthesia. The immunohistochemical study has shown undifferentiated tumor with positive antibody anti PS100 and anti-melan A evoking malignant sinonasal melanoma. The patient underwent two surgeries for maxillectomies as she presented a first local recurrence. She was started on adjuvant radiotherapy. At one year of follow-up, she does not present any local or general signs of disease. Sinonasal melanoma is a particular entity of head and neck mucosal melanomas. The highest incidence is described to be in the seventh and eighth decades of life with no sex difference. IHC profiling of different melanoma subtypes showed the importance of alterations in the KIT gene, this genetic data may constitute a therapeutic target. After surgery, the important local recurrence rates and regional failure justify adjuvant radiotherapy also for resections in free margins. Most authors consider that prophylactic neck dissection is not necessary. Preoperative imaging features (CT scan) are characteristic and helpful for diagnosis. IHC is essential, has a high sensitivity for differentiating achromic melanomas from other neoplasms. Sinonasal achromic melanoma is a very uncommon tumor, invasive, and frequently associated with distant metastasis. Paraclinic examinations are essential for staging and guiding therapeutic management. Immunotherapy is a promising ground of research as it comes to metastatic and advanced disease. Malignant melanoma of the head and neck (HNMM) is a rare and aggressive tumor with a poor prognosis (about 30% at five years) . HNMM caMucosal melanomas differ from cutaneous melanomas by their pathobiology and clinical presentation, as the mucosal form of melanoma is not correlated with solar exposure and also exhibits other cytogenetic alterations  . Around It is known that most HNMMs are diagnosed at a late stage (1) because the tumor process evolves insidiously without clinical symptoms. Common symptoms are represented by nasal obstruction, epistaxis or rhinorrhea, and less frequent pain. Mostly, at the time of diagnosis, lesions have developed, and lymph nodes or metastases are present (up to 10%-30%) . HistoloThe treatment is discussed and determined in a multidisciplinary consultation meeting. It is mainly surgical, most often supplemented by radiotherapy or immunotherapy. On the other hand, surgical treatment for advanced mucosal melanomas can be deleterious because of functional prognosis, cosmetic considerations, and non-oncological resection leading to high local and general recurrence rates. We report the case of a rare presentation of an achromic melanoma of the sinonasal cavity, with a review of the previously reported cases of mucosal melanomas of the head and neck area.We report a case of a 63-year-old female patient with a history of osteoarticular tuberculosis declared cured in 2010, Parkinson's disease under medical treatment for one year, and a right dacryocystorhinostomy in 2015. She consulted in March 2021 at the Otorhinolaryngology Department of Cheikh Khalifa Hospital in Casablanca for rhinological symptoms evolving for 02 months. The patient reports symptoms of intermittent and then permanent right nasal obstruction of increasing intensity accompanied by two episodes of mild unilateral epistaxis in a context of altered physical and mental functions.The rigid optic examination showed a white-pinkish right obstructive supra-centimetric endonasal tumor process encroaching onto the nasal septum bleeding when in contact. The right hard palate was infiltrated Figure . There wThe CT examination of the facial mass revealed an extensive tissue process of the right nasal cavity invading the maxillary sinus, the inferior and middle conchas with respect to the ethmoid sinus, and the bony structures Figure . CervicoThe therapeutic protocol decided by the multidisciplinary consultation meeting was to start with embolization of the right sphenopalatine and lingual arteries. The aim was to avoid revision surgery as much as possible due to the aggressive and recurrent nature of the disease. A right medial maxillectomy was performed to allow for complete excision of the tumor. The final pathological examination confirmed the diagnosis of malignant melanoma. The patient received three postoperative radiotherapy sessions of 45 Gy. The patient was not able to complete her chemotherapy sessions due to cardiac arrhythmias. In the follow-up, she came to consult for a right maxillary purulent swelling five months after surgery. The diagnosis of loco-regional recurrence with the invasion of the anterior, posterior, lateral wall, and floor of the right maxillary sinus and the right nasal cavity was posed.A second surgical intervention was performed by a wide right para-lateral nasal approach comprising a right subtotal maxillectomy with preservation of the orbital floor and placing of a palatal prosthesis Figures -4. No neSinonasal melanoma is a particular entity of head and neck mucosal melanomas. It is a rare and aggressive lesion, with an incidence of 0.2-1 case per million . Recent Patients with HNMM present a poor overall survival outcome with five-year overall rates from 20% to 50%. For most authors, this is attributed mainly to concomitant metastasis at the time of diagnosis . HoweverSinonasal melanoma has been described more frequently in areas where skin melanomas are less common, which may be caused by factors associated with the disease, as aforementioned genetic features .AlthougConcerning the site of the lesion, the nasal cavity is the most affected, followed by the maxillary, ethmoid sinus, and other paranasal sinuses . The maiPreoperative imaging features are characteristic and helpful for diagnosis. CT is the most common examination modality, the lesions presenting as heterogeneous hypodense images . Bony erThe sinonasal melanoma is a very uncommon tumor, whereas the amelanotic form tends to be exceptional. On microscopic analysis, the melanotic subtype represents 86.8% of the cases and the amelanotic only 13.2%. For malignant melanoma, most tumor cells are pigmented and thus the diagnosis of melanin-rich melanoma is obvious. On the contrary, the amelanotic presentation is a challenge for histopathologists [To provide the best chance for a long-term cure, large surgical excision with free margins is the cornerstone for treating any HNMM. The size and location of the lesion influence the surgery choice (type and extent) for oncological resection.This objective remains challenging and is not uncommonly impossible. Proximity of the tumor to vital structures  and in some cases locally advanced disease  at presentation has to be considered in the preoperative assessment. It remains unclear whether radical surgery if causing significant esthetic and functional impairments are justified. In all cases, aggressive adjuvant therapy must be used for locally advanced disease and large tumors without consideration of any margin status. Surgery as a single-mode therapy for HNMM patients is exclusively used for localized tumors .The HNMM patients have a moderate rate of clinical lymphadenopathies at initial presentation (10%-30%) . Neck diSeveral studies suggested that definitive RT did not reach as significant local control of the tumor extent as surgery . Doses hThe frequency of KIT gene alterations in mucosal melanomas suggests a potential utility for selective KIT inhibitors in the treatment of these tumors. For patients presenting advanced disease and c-KIT mutations, immunotherapy (tyrosine kinase inhibitor imatinib) had a disease response rate of 54% and an overall control rate of 77% . The oveSinonasal achromic melanoma is a very rare tumor, potentially aggressive. The diagnosis of this pathology relies on clinical examination, CT scan, and IHC after biopsy of the lesion. Extension assessment is essential for staging and guiding therapeutic management. Surgery is the cornerstone for localized tumors and radiotherapy is indicated even for radical resections in free margins. Immunotherapy is a promising ground of research as it comes to metastatic and advanced disease.


Intestinal intussusception is uncommon in adults. It occurs more often in the small intestine than in the colon. In adults, when small bowel intussusception occurs, it can be due to a malignant lead point. Malignant etiology is most frequently due to diffuse metastatic disease. We present a rare case of an 18-year-old woman who was diagnosed with jejunojejunal, jejunoileal, and colocolic intussusceptions. She presented with vomiting, abdominal pain, and passage of semisolid stools for 5 days. During emergency exploratory laparotomy, multiple polyps were found in the jejunum, ileum, and sigmoid. Jejunotomy and sigmoidotomy were done to remove the respective polyps. The ileal polyp showed hemorrhagic changes; hence, an intraoperative decision was taken to proceed with resection and anastomosis. On histopathological examination, the resected ileal part showed moderately differentiated adenocarcinoma (grade 2) arising from an adenomatous polyp, while the jejunal polyp and sigmoid polyp were adenomatous polyps with low-grade dysplasia. Patient received six cycles of adjuvant chemotherapy consisting of capecitabine and oxaliplatin (CAPEOX regimen). After 2 years, she is symptom free with a normal colonoscopy. The treatment of intussusception in adults typically involves surgery, often with bowel resection as there is always a pathologic leading cause which may be malignant, like in our case. Intussusception involves the telescoping of a segment of bowel into an adjacent segment. This leads to complications such as obstruction, inflammation, and possible ischemia. Intussusception is the leading cause of intestinal obstruction in children. However, adult intussusception is rare, accounting for less than 5% of all bowel obstructions and 5% of all intussusceptions.Intussusceptions in children are typically primary or idiopathic. In contrast, most adult intussusceptions have a demonstrable etiology in 70 to 90% of the cases.Small bowel neoplasms are exceedingly rare, constituting only 5% of all gastrointestinal neoplasms and only 1 to 2% of all gastrointestinal malignant tumors, despite the fact that they comprise 90% of the length of the alimentary tract.We report a rare case of multiple simultaneous small bowel intussusceptions secondary to adenocarcinoma of the ileum in an 18-year-old woman. The statistics mentioned earlier emphasizes the rarity in reporting of these two conditions occurring together.An 18-year-old woman presented to the OPD with complaints of vomiting, abdominal pain, and passage of semisolid stools for 5 days. There was no history of fever, hematemesis, or malena. She did not have similar episodes in the past. On examination, she was vitally stable. She had diffuse tenderness and distension on per abdominal examination. Rest of abdominal examination was unremarkable. Per rectal examination was normal.On investigating further, plain X-ray of the abdomen showed no abnormalities. Ultrasonography of abdomen and pelvis revealed: ileocolic intussusception in the right iliac fossa with ileum extending into ascending colon up to right half of transverse colon; ileoileal intussusception in left iliac fossa for a length of 8 cm; multiple enlarged mesenteric lymph nodes of average size 10 mm; and mild ascites. A contrast-enhanced computed tomography (CECT) of the whole abdomen confirmed the diagnosis demonstrating three regions of intussusceptions, namely, jejunojejunal, jejunoileal, and colocolic intussusceptions and irregular wall thickening involving the sigmoid colon .After obtaining consent, patient was taken up for emergency exploratory laparotomy. All three intussusceptions were reduced without any complications. Bowel wall was mildly edematous without any obvious signs of ischemia/necrosis. Multiple polyps in the jejunum, ileum, and sigmoid colon were found to be lead points . JejunoHistopathological report revealed: resected ileal segment—moderately differentiated adenocarcinoma (grade II) arising on the background of adenomatous polyp; jejunal polyp—adenomatous polyp with low-grade dysplasia; sigmoid polyp—adenomatous polyp with low-grade dysplasia; and appendix—chronic appendicitis  .On follow-up, patient was started on CAPEOX regimen , 21-day cycles for 6 cycles. Patient responded well to the regimen. Positron emission tomography–CT done 3 months postoperatively showed fluorodeoxyglucose avid small benign polyp in anterior wall of rectum for which polypectomy was done. Patient is symptom free and doing well till date with regular follow-up of 2 years.Intussusception is defined as the invagination of one segment of the bowel into an immediately adjacent segment. It can occur anywhere in the bowel. Intussusception in children usually occurs between 6 and 18 months of age. The incidence of intussusception declines with age accounting for only 1 to 5% of bowel obstructions in adults.In the pediatric population, intussusceptions are typically primary or idiopathic with only 10% of cases having an identifiable precipitating lesion. In contrast, 70 to 90% of the cases of adult intussusceptions are caused by a structural lesion/lead point pathology which can be intraluminal, mural, or extramural.A lead point intussusception involving the small bowel is generally due to a benign condition.Sixty-six per cent of colonic intussusceptions and 30% of intussusceptions in the small intestine occur due to malignant lead points. The most common malignant lead point in colon is adenocarcinoma and in the small intestine, it is diffuse secondary metastasis.Our patient is an 18-year-old woman, who presented to us with multiple intussusceptions of small bowel due to adenocarcinoma of the ileum, both of which are rare entities.Adults rarely present with the classic triad of abdominal pain, bloody currant jelly stools, and palpable tender abdominal mass seen in children. They may present as acute intestinal obstruction/perforation or more commonly chronic intermittent cramping abdominal pain associated with nonspecific signs of bowel obstruction.Preoperative diagnosis ranges from 30 to 70%.Ultrasound is both diagnostic and therapeutic with 100% sensitivity and specificity in children.Abdominal CECT represents the most accurate diagnostic modality with the possibility of demonstrating lead points, vascular compromise, and possible associated complications such as intestinal obstruction or local spread.The approach to management of intussusception is different in pediatric and adult intussusceptions. While in children, treatment is primarily based on radiological reduction, surgical resection is recommended in cases of adult intussusception because it is often associated with a lead point which can be malignant.A selective approach to bowel resection is recommended when patient requires operative management, depending on the age, pathology, and location of lead point. Bowel resection following the appropriate oncologic principles is recommended given the relatively high incidence of malignancy in adults.The suggested first-line adjuvant chemotherapy for adenocarcinoma of ileum is a combination regimen of 5-fluorouracil infusion or oral capecitabine with oxaliplatin.Adult intussusception is a rare entity and challenging to diagnose due to nonspecific symptoms with a vast differential diagnosis. Small bowel tumors are a rare but possible etiology of adult intussusception. Surgical exploration is the only way of making and confirming the diagnosis of small bowel adenocarcinoma despite the availability of sophisticated imaging modalities.


A comprehensive understanding of the anatomical variations of the internal jugular vein (IJV) is essential to prevent inadvertent injuries during neck procedures, particularly neck dissection. In addition, its relationship with the spinal accessory nerve in the upper part of the neck is relatively variable. IJV fenestration refers to bifurcation of the vein with reunion proximal to the subclavian vein, whereas IJV duplication refers to continued branching till joining the subclavian vein separately. We report a case of a fenestrated IJV identified intraoperatively with the spinal accessory nerve passing laterally to both divisions. Neck dissection is a frequent procedure performed in patients with head and neck malignancy. Due to the significant morbidity associated with their injuries, anatomical and functional preservation of the internal jugular vein (IJV), spinal accessory nerve (SAN), and sternocleidomastoid muscle, when oncologically appropriate, has gained more attention. The SAN most commonly crosses superficial to the upper part of the IJV, but its passage medial to the vein has also been reported in up to 3% of patients. Rarely, the SAN passes through the IJV posing a risk of injury to the vein near the skull base, where control of bleeding is surgically challenging . VariatiA 69-year-old male patient presented to the otolaryngology clinic via the rapid access pathway with a painless left neck lump that had existed for one month with no other head and neck or upper aerodigestive symptoms. The patient was otherwise healthy and a lifelong smoker. Examination revealed an approximately 4 cm left level II mass that was soft, mobile, and cystic. Oral cavity examination and office pharyngolaryngoscopy were unremarkable.Radiological workup, including neck ultrasound and magnetic resonance imaging (MRI), revealed a solitary left cystic level II mass with a solid component, suspicious of nodal malignancy. No other cervical lymphadenopathy or evidence of a primary was noted. Ultrasound-guided fine-needle aspiration was repeatedly inconclusive. A positron emission tomography (PET) scan showed intense metabolic activity in the mass and the rest of the examination was normal. A multidisciplinary team (MDT) recommended neck dissection of left level II and III territory along with panendoscopy.Intra-operatively, neck dissection proceeded smoothly, and the SAN was identified and secured. The IJV was found to be splitting from near the skull base to 1 cm above the left of carotid bifurcation  of the neck. Tran-oral robotic tongue base mucosectomy confirmed a microscopic tongue base primary.Injury of the IJV may occur during prophylactic or therapeutic neck dissection, as well as during central venous catheter placement in the neck. Besides associated morbidity from its ligation or occlusion, control of bleeding, especially near the skull base, is surgically difficult and may require drilling of the mastoid and vascular control at the level of sigmoid sinus . To avoiIJV anomalies can be visualized preoperatively by contrast-enhanced imaging such as computed tomography (CT) or MRI of the neck performed for evaluation of the primary neck disease . That saIt is worth mentioning that in our case, the carotid vessels were located posteromedial to both branches of the IJV, and the common facial vein (that had to be divided) drained into the anteromedial branch before the re-joining point. Additionally, the SAN maintained a superficial position to both branches of the IJV.Anomalous IJV could present as an intraoperative finding without prior anticipation by contrast-enhanced cross-sectional imaging. The spinal accessory nerve may cross superficially to both branches of a fenestrated IJV and care must be taken to avoid injury to both.


EWSR1–AFTF1 rearrangement was detected by fluorescence in situ hybridization and further confirmed by whole-transcriptome sequence analysis. The patient had pulmonary metastasis 8 months later and soon died of the disease. The overall survival of the patient was 20 months. In summary, we reported an extremely rare case of cardiac GNET, indicating that the location of GNET should not be confined to the GI tract as initially defined. Due to the lack of a specific effective treatment and the occurrence of early metastasis, cardiac GNET conferred a poor prognosis. More clinical and experimental studies are warranted to better manage this disease in the future.Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare soft tissue sarcoma and has been designated as a new entity recently. At present, GNET virtually exclusively occurs in the gastrointestinal tract. Here we report a case of extra-GNET that arose in the right heart. A 62-year-old male complained of chest distress and breathing difficulty while lying down at night for over 1 month at admission. The radiological findings revealed an occupying lesion involving the right atrium and the right ventricle without any abdominal abnormalities. The patient then underwent a surgical resection. Microscopically, neoplastic cells proliferated in the pattern of nests and sheets with fibrous separation. Focal areas with cellular dyscohesion imparted a vague pseudopapillary pattern. These tumor cells were small to medium in size with fine chromatin and predominantly pale eosinophilic cytoplasm. The nuclei were typically round to oval with somewhat irregular contours and contained small nucleoli. The mitotic figures were easily found. Immunohistochemically, the neoplastic cells were positive for S100 and SOX-10 but negative for HMB-45, A103, and CD99.  Malignant gastrointestinal neuroectodermal tumor (GNET), a synonym for clear cell sarcoma (CCS)-like tumor of the gastrointestinal (GI) tract, is a very rare mesenchymal tumor first described by Zambrano et al. . It is uvia the coronary sinus, forming another lesion of 4 × 3 × 3 cm that was later removed.A 62-year-old male was admitted to our hospital complaining of chest distress and breathing difficulty while lying down at night for over 1 month. He had a medical history of hypertension and denied any specific family history. On physical examination, no heart murmurs or vascular abnormalities were audible with the heart rate of 66 beats per minute. Pulse deficit was not found as well. The abnormal laboratory findings included a modestly raised level of CA125 (36.10 U/ml) and a remarkably elevated ferritin . The brain natriuretic peptide level was within the normal range. Unfortunately, details on troponin and myohemoglobin levels were unavailable. An electrocardiogram indicated left ventricular hypertrophy and complete right bundle branch block. A computed tomography (CT) scan showed an occupying lesion involving the right atrium and the right ventricle  without in situ hybridization (FISH) preformed on paraffin-embedded mass tissues identified EWSR1–ATF1 rearrangement by EWSR1 break-apart probe  and angiosarcomas . To our EWSR1–ATF1/CREB1 fusions, and it can arise at any location in the GI tract, with the small intestine (particularly ileum) being the most common , as IS often demonstrates MDM2 amplification and a negative expression of S100 or SOX10  should also be considered for differentiation due to a similar expression of Syn and CD56 and morphology to GNET. However, ES/PNET often expresses CD99 protein, which was absent in this case, and frequently harbors EWSR1-Fli1 gene fusion . The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.ZL, XP, and LH analyzed and interpreted the data and contributed to the writing of the manuscript. YF and LL collected all the clinical and pathological data. YX and WG collected and edited the images. XF revised the final manuscript. All the 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.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Agenesis of the left hepatic lobe is an exceedingly rare morphological anomaly. Moreover, agenesis of the left hepatic lobe accompanied by esophagogastric cancer is even rarer, with no reports to date. Agenesis of the hepatic lobe is commonly related to some anatomical variations of the gastrohepatic system. A 76-year-old man was referred to our hospital for surgery for esophagogastric cancer with short Barrett’s esophagus. Multiple preoperative imaging modalities revealed agenesis of the left hepatic lobe accompanied by esophagogastric cancer. Robotic proximal gastrectomy and transhiatal lower esophagectomy were performed. Intraoperative findings showed agenesis of the left hepatic lobe. The patient’s postoperative course was favorable. Today, 16 months after surgery, the patient is alive without recurrence of esophagogastric cancer. We report a case of agenesis of the left hepatic lobe in a patient undergoing robotic proximal gastrectomy and transhiatal lower esophagectomy for esophagogastric cancer. Preoperative comprehension of various visceral anomalies reduces the risk of surgical complications. Agenesis or hypogenesis of the left hepatic lobe is a rare morphological anomaly . It is dA 76-year-old man presented to the clinic with epigastralgia of two weeks duration. Esophagogastroduodenoscopy revealed a type 0-Ⅱa-shaped tumor (30 mm in diameter) at the esophagogastric junction with short-segment Barrett’s esophagus Figure .Histological findings demonstrated a well-differentiated adenocarcinoma in the mass. The patient was referred to our department for surgery. His medical history included angina pectoris and diabetes mellitus, which were well-controlled. Hematological and biochemical findings were all within normal limits. The Child-Pugh classification was A. Serum levels of tumor markers  were also within normal limits. Contrast-enhanced abdominal computed tomography (CT) revealed the absence of the left hepatic lobe without obvious esophagogastric tumor or adjoining lymphadenopathy. In the arterial phase of CT, the left hepatic artery and portal vein were not visualized Figures , 2B. Magnetic resonance cholangiopancreatography (MRCP) also showed agenesis of the left hepatic lobe, with a complete absence of the left biliary system Figures , 3B.The upper gastrointestinal series showed barium translucency at the esophagogastric junction. Gastric volvulus and high positioning of the duodenal bulb were ruled out Figures , 4B.The preoperative diagnosis was Siewert II EGC accompanied by agenesis of the left hepatic lobe. The clinical stage of EGC was T2N0M0, stage I. Robotic proximal gastrectomy and transhiatal lower esophagectomy were also performed. Intraoperative findings revealed agenesis of the left hepatic lobe, but the caudate lobe was also not detected. The right hepatic lobe showed no evidence of cirrhosis. Since there was no need to retract the liver during surgery, a transhiatal procedure for the lower mediastinum was performed with good visibility Figures , 5B, folPathological findings of the tumor showed a well-differentiated adenocarcinoma with short-segment Barrett’s esophagus Figure .The operative time was 613 minutes and the estimated blood loss was 130 mL. 17 lymph nodes were retrieved including one metastatic lymph node at the posterior mediastinal region. The patient’s postoperative course was uneventful. The patient was discharged on a postoperative day 28 without post-surgical complications or gastric reflux symptoms. Today, 16 months after surgery, the patient is alive without recurrence of EGC.Hepatic lobe agenesis is a rare liver anomaly. It is defined as the absence of liver tissue to the left of the gallbladder fossa ,2. In a Agenesis of the liver is clinically asymptomatic, without laboratory signs of liver dysfunction. Multiple imaging modalities play a significant role in the diagnosis . ContrasAgenesis of the left hepatic lobe is commonly linked to visceral abnormalities, such as displacement of the gallbladder, high positioning of the duodenal bulb, sigmoid volvulus, and Chilaiditi syndrome, due to deficiency of gastrointestinal fixation and abnormalities of the biliary system ,8. It isThe etiology of agenesis of the hepatic lobe involves congenital factors . And acquired factors  should be ruled out -11. In tWith reference to previous reports, 35 cases of agenesis of the left hepatic lobe have been reported. There are 24 males and 11 females aged 11-79 years at the time of diagnosis. The median age at diagnosis was 59.5 years . SurgeryProximal gastrectomy and transhiatal lower esophagectomy are standard surgeries for Siewert II-type EGC . In geneThis is the first case of agenesis of the left hepatic lobe accompanied by EGC in a patient undergoing robotic surgery. Multiple imaging modalities enabled confirmation of the anatomy associated with hepatic lobe agenesis. Robotic gastrectomy and transhiatal procedures were performed in a good surgical field. For safe surgical planning, it is beneficial to recognize precise anatomical variations preoperatively.


AKT1 E17K somatic mutation and TP53 C176Y germline mutation in this case. Thus, we presented an extremely rare case of atypical PSP with rapid recurrence and multiply metastases, which can easily be misdiagnosed as primary lung cancer. In addition, PSP-specific AKT1 E17K somatic E17K somatic mutation accompanied with TP53 C176Y germline mutation may contribute to the malignant clinical course of this tumor.Pulmonary Sclerosing Pneumocytoma (PSP) is considered as a benign tumor, although a few cases have been reported to have multiple lesions, recurrence, and even regional lymph nodes (LNs) metastasis. Here, we report a case of PSP with atypical histologic features and malignant biological behavior, and explore its molecular genetic changes. The 23-year-old male showed a 6.5-cm pulmonary nodule in the right middle lobe (RML) and enlarged media stinal LNs. He underwent thoracoscopic RML lobectomy, systematic LNs dissection, and mediastinal lymphadenectomy. The metastases to the cervical LNs and liver were detected in a short period and then resected. Postoperative pathological examination confirmed the diagnosis of PSP in all the lesions, based on the histological characteristics and immune phenotypes. Furthermore, whole-exome sequencing identified both  Pulmonary sclerosing pneumocytoma (PSP), formerly known as pulmonary sclerosing hemangioma, is a rare primary lung tumor originated from incompletely differentiated type II pneumocytes. Although PSP has some clinical and imaging characteristics for differentiation from similar lesions, these are not specific for diagnosis , 2. At pA 23-year-old male was referred to Daping Hospital, Army Medical University for non-productive cough, chest distress, and fever for 1 month. He had no smoking and family disease history. Chest enhanced computed tomography (CT) scans confirmed a 6.5-cm pulmonary nodule in the right middle lobe (RML), with enlarged media stinal LN . PositroA well-demarcated and circular mass, with grayish-white, slightly tough texture cut surface, was found in the RML , 6 cm inThe histopathological and IHC staining of the resected specimens confirmed multiple organ metastasis of hilar, mediastinum , cervicaAKT1 E17K mutations was detected in primary lung tumor, media stinal LN, cervical LN, and liver metastatic lesions . TP53 C176Y mutations were identified in the primary lung lesion, mediastinal LN, cervical LN, and liver metastatic samples, with the frequencies of 42.41, 68.63, 72.84, and 50.52%, respectively. Interestingly, TP53 C176Y mutations were also detected in the paired normal tissue adjacent to the primary lung tumor (frequency 33.02%), suggesting that TP53 C176Y was a germline mutation in this patient  in formalin fixed paraffin-embedded (FFPE) samples from the primary and three metastatic lesions with matched adjacent normal tissue. High frequencies of  patient . This phThe patient received anti-estrogen therapy with medroxyprogesterone after the fourth operation, but the disease progressed rapidly. In May 2019, chest and abdominal enhanced CT scans showed multiple nodules in the right lung and multiple enlarged LNs in neck, jaw and right supraclavicular fossa. In September 2019, the patient felt that the neck mass was significantly larger than before. Chest and abdominal enhanced CT scans showed an enlarged mass in both lung hilum (5.8 × 5.4 cm) and right supraclavicular fossa (5.7 cm in maximum dimension), multiple nodules in the liver and left kidney, and thoracic 8th and 11th vertebral osteolytic bone destruction. The patient was then administered pembrolizumab treatment at 2 mg/kg intravenously every 3 weeks, combined with apatinib at 250 mg/day orally since October 2, 2019. As of January 10, 2021, after multiple cycles of combined treatment, the metastatic lesions were obviously reduced or in a stable state. The patient was in good mental state and had no significant change in weight.Due to the lack of specificity in clinical and imaging features, the diagnosis of PSP mainly depends on postoperative pathological examination. The diagnosis of typical PSP, characterized by two different cell components, four typical histological types, and immune phenotypes with specific features, is not difficult for pathologists . HoweverAKT1 E17K point mutation through WES analysis, which is a relatively specific molecular feature of PSP , aged 38–61 years . The mal.34%, ageAKT1 E17K mutation, identified from all primary and metastatic lesions in this PSP case, were localized to the pleckstrin homology domain (PH domain), which is crucial for membrane localization and downstream activation of AKT1 , a cancer predisposition syndrome associated with high risks for a diverse spectrum of childhood- and adult-onset malignancies . The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.QW and HX designed the study. MJ and XY investigated and provided the clinical data. QW, CL, LZ, and YY performed the molecular experiments. CM, CL, and PF performed the pathologic slides. QW and HX wrote the manuscript. ML, YH, and HX revised and edited the manuscript. All authors contributed to the article and approved the submitted version.This work was supported by The National Natural Science Foundation of China (grant no. 81802781).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.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Leptomeningeal carcinomatosis is the result of metastatic infiltration of the leptomeninges by malignant cells originating from an extra-meningeal primary tumor site. We describe a patient with active breast cancer who presented with thunderclap headaches (THs) and imaging showing multi-segment irregular arterial narrowing of intracranial vasculature.A 58-year-old Caucasian woman with active stage IV estrogen receptor-positive breast adenocarcinoma and migraine presented with THs. Computed tomography and brain magnetic resonance imaging (MRI) without contrast were unremarkable. Over a period of one week, she had recurrent THs. Interval vessel imaging showed multi-segment irregular arterial narrowing. Treatment with verapamil was initiated for suspected reversible cerebral vasoconstriction syndrome (RCVS). She subsequently had two discrete episodes of confusion with aphasia and left upper extremity numbness. Repeat gadolinium-enhanced MRI showed nodular leptomeningeal enhancement. Lumbar puncture revealed malignant cells in the cerebrospinal fluid consistent with leptomeningeal carcinomatosis. She subsequently underwent whole brain radiation treatment and intrathecal chemotherapy and had no further episodes of TH.Our case emphasizes the importance of considering leptomeningeal carcinomatosis in the differential diagnosis of THs and reversible cerebral vasculopathy, especially in patients with known underlying active cancer. The illustration also proves the importance of a complete work-up in patients with known malignancy in the setting of suspected RCVS. Leptomeningeal metastasis (LM) is a result of metastatic infiltration of the leptomeninges by malignant cells originating from the extra-meningeal primary tumor site. It is most commonly a complication of breast adenocarcinoma, occurring in 12-35% of patients [A 58-year-old Caucasian woman presented with recurrent THs associated with nausea and vomiting. She had a history of stage IV breast adenocarcinoma post bilateral mastectomy and reconstruction surgery, with bone and liver metastases, on olaparib and enzalutamide. Other medical co-morbidities included non-Hodgkin's lymphoma in remission, migraine with visual auras, and hypertension. Computed tomography (CT) and brain magnetic resonance imaging (MRI) without contrast were unremarkable. No focal neurological deficits were noted on examination. She received a short course of high-dose oral dexamethasone, butalbital/acetaminophen/caffeine combination, and was started on gabapentin for presumptive diagnosis of an acute migraine attack. She was not started on any other acute migraine abortive medications.Over one week, she had recurrent THs, and interval vessel imaging was completed. Magnetic resonance angiogram (MRA) showed multi-segment irregular arterial narrowing of the proximal and distal branches of the anterior and posterior arterial circulation. Based on her clinical course and imaging, treatment with verapamil was initiated for suspected reversible cerebral vasoconstriction syndrome (RCVS). Headaches transitioned from TH to a persistent headache and she developed visual auras, similar to her typical migraines, as well as binocular diplopia for one week. She subsequently had two discrete episodes of confusion with aphasia and left upper extremity numbness, prompting transfer to our tertiary care center.Upon admission, repeat brain MRA showed similar arterial irregularity and narrowing, more prominent in the anterior than posterior intracranial vessels Figure . Brain MThe pathogenesis of LM is thought to be either hematogenous, due to the growth of the tumor along with nerve roots or arachnoid membrane, or as a direct spread from brain parenchyma . The CSFSeveral mechanisms, often combined, are implicated in the typical symptom-complex characteristic of LM, including hydrocephalus with elevated intracranial pressure, compression and invasion, ischemic injury, metabolic competition, and disruption of the blood-CSF barrier due to tumor angiogenesis . The mosOur patient presented with a recurrent TH, which is pathognomonic of RCVS, with typical reversibility of vasoconstrictive and ischemic changes on MRI and MRA along with the resolution of clinical symptoms . The patOur case emphasizes the importance of having LM in the differential diagnosis of THs that are classically associated with other etiologies like RCVS. The illustration also proves the importance of a complete work-up, including contrast-enhanced MRI and CSF analysis in patients with known malignancy in the setting of suspected RCVS.


Hydatidosis is an anthropozoonosis mainly encountered in pastoral areas. It mostly affects the liver, lung, and rarely the bone and the soft tissues. Skeletal involvement is usually secondary to visceral hydatidosis. We report a case of a 49‐year‐old man presenting with one‐year history of a progressive left hip pain. On local examination, there was tenderness in the left gluteal region with reduction in the hip range of motion. Pelvic X‐ray revealed an expansive bone destruction involving the left hemi pelvis without periosteal reaction. A magnetic resonance imaging showed multiple cystic lesions extending from pelvic bones to the gluteal region. The possibility of hydatid disease was raised, and hydatid serology test was positive. No visceral involvement was found by additional examinations investigations revealed visceral hydatidosis. Thus, the diagnosis of a primary bone hydatid disease was established. No surgical excision was possible, and the patient was put on Albendazole. Echinococcosis should be ruled out while dealing with progressive expansive bony lesions. Surgical management remains a challenge especially if the involvement is very extensive. Echinococcosis should be ruled out while dealing with progressive expansive bony lesions. Surgical management remains a challenge especially if the involvement is very extensive. The patient was living in an urban area with no significant past medical history. He did not report fever, chills, or loss of weight. There was no history of tuberculosis, no prior trauma, and no contact with animals. The patient was limping and walking with the help of crutches. On local examination, there were no signs of skin inflammation. There was tenderness in the left gluteal region, and the mobilization of the left hip was painful with reduction in the hip range of motion.Routine blood investigation did not reveal an elevated leucocyte count nor eosinophilia. C‐reactive protein and erythrocyte sedimentation rate were within normal range.Pelvic X‐ray revealed an expansive bone destruction involving the left ischium, ilium, acetabulum, femoral head, and the trochanteric region without periosteal reaction Figure . Hip ultBased on imaging findings, the possibility of hydatid disease was raised and hydatid serological tests (the enzyme‐linked immunosorbent assay “ELISA” and the immunoblot test) were then performed. They both showed positive results. Clinical examination, and chest and abdominal imaging did not reveal any collection or mass in the liver, lungs, or any other viscera. Thus, the diagnosis of a primary bone hydatid disease was established.The patient was put on Albendazole by oral administration, and he was proposed for surgery but refused by the patient.3Echinococcosis granulosus has definitive hosts dogs and other carnivores where the adult tapeworm lives and those hosts are responsible for the dissemination of the parasite's eggs. The eggs can contaminate intermediate hosts which are sheep and other herbivores and can also contaminate human beings who are considered as incidental hosts.However, bone involvement remains rare accounting for less than 2%. The infestation is primarily via hematogenous seeding, and usually spongy vascularized bones are the most common site of osseous hydatidosis like vertebrae (50%), pelvis (21%), and femur (16%).Hydatid bone disease can be a challenging diagnosis as symptoms are nonspecific.The most frequent symptoms are pain, local swelling, and fracture.Bone hydatidosis was reported to be more prevalent in men with an average age of 50 years.The main basis for diagnosis is still radiological imaging including ultrasonography, plain radiographs, and CT scans with MRI considered as the best imaging modality when soft tissues are involved.In the absence of elements pointing toward echinococcosis in the patient's history, these radiological features often cause misdiagnoses. Imaging differentials include neoplastic lesions, bone metastasis, giant cell tumor, aneurysmal bone cyst, and chronic osteomyelitis tuberculousis of the bone. Solitary lesions can mimic solitary bone cyst, plasmocytoma, and brown tumor of hyperparathyroidism, while chronic hydatidosis can mimic fibrous dysplasia.Those imaging findings might be combined with serological tests to make a more accurate assessment of the disease.It is challenging not only to diagnose echinococcosis of bone but also to treat the lesions. The management of osseous hydatidosis is often complicated as the disease is discovered at the late stage.Even with the best of efforts in treatment, pelvic hydatidosis carries grave prognosis that it has been called “white cancer”.4In areas where hydatid disease is endemic, the diagnosis of pelvic echinococcosis should be kept in mind in case of any bone lesion. We reported this case in order to raise awareness about the nonspecific features of this disease and the importance of adequate radiological and serological investigations to establish the right diagnosis as soon as possible. Surgical management remains a challenge, especially if the involvement is very extensive.Published with the written informed consent of the patient.


Helicobacter infection, intestinal metaplasia, dysplasia, or malignancy was identified histologically.A 63-year-old woman with a history of hypertension, sick sinus syndrome, and Hashimoto's thyroiditis was referred to our facility for evaluation of a mass in segment 8 of the liver. Abdominal computed tomography showed an enhancing mass in segment 8 measuring 5.8 cm. The liver biopsy showed a moderately differentiated adenocarcinoma, most consistent with intrahepatic cholangiocarcinoma. A screening gastrointestinal endoscopy revealed a 7-mm sessile polyp in the antrum Figure . The polFoveolar hyperplastic polyp is a common gastric polyp usually found in the antrum and is considered as reactive changes to chronic gastritis, bile reflux, alcohol, and drugs. Histological features of foveolar hyperplastic polyp include tortuous or dilated gastric foveolae with edema, acute and chronic inflammation, and smooth muscle strands extending from the muscularis mucosae.4 Among these reported 5 cases, 4 patients are men and 1 patient is a woman with ages ranging from 42 to 67 years (average age: 52.8 years). Two of 5 polyps are found in the antrum, and the size of the 5 polyps ranges from 0.3 to 3.0 cm with average size of 1.6 cm. Our current case shows clinicopathologic features similar to those of the previous case reports, including the findings of small-sized polyp found incidentally in middle-aged patients with no clinical history of hypercalcemia or any other abnormalities causing heterotopic bone formation.Osseous metaplasia/heterotopic bone formation is a well-known finding reported in various neoplastic and non-neoplastic conditions. However, osseous metaplasia in foveolar hyperplastic polyps of the stomach is extremely rare. There have been only 4 previous case reports published in the English language, one of which described 2 cases in their report.5The pathogenesis of osseous metaplasia remains unknown. Multiple hypotheses have been formulated, including a recent theory postulating that osseous metaplasia might occur by osteoblasts which differentiate from fibroblasts secondary to inflammation, tissue damage, or substances such as bone morphogenetic proteins.Author contributions: Y. Zhang wrote the manuscript. M. Friedman edited the manuscript. Y. Nakanishi revised the manuscript for intellectual content and is the article guarantor.Financial disclosure: None to report.Informed consent was obtained for this case report.


A novel SS18-POU5F1 fusion gene was recently reported in soft tissue sarcoma occurring in three adolescent and young adult patients. Herein, we firstly reported the treatment response of SS18-POU5F1 sarcoma to immune checkpoint inhibitor, angiogenesis inhibitor, chemotherapy and radiotherapy. Our patient demonstrated no response to various systemic therapies including immune checkpoint inhibitor, angiogenesis inhibitor and chemotherapy. However, we noted that the SS18-POU5F1 sarcoma had a quick, robust but transient clinical response to radiotherapy. Further studies are needed to elucidate the mechanism underlying the different tumor response to radiotherapy and systemic therapy in this kind of tumor. Recent advances in clinical molecular diagnostics have provided greater understanding of soft tissue sarcoma. Herein, we report the clinical course of a sarcoma patient with a novel SS18-POU5F1 fusion gene.An 18-year-old female presented to the local hospital with a painless immovable non-tender 5×5×2 cm cystic-solid mixed mass founded in the soft tissue of the left groin that had been present for 2 months. The mass was widely excised with negative margins. Microscopically, the tumor was composed of two kinds of cells: the undifferentiated small round cells and the epithelioid big cells. The cells arranged in solid sheets and trabeculae. The tumor had sheet necrosis and large thick wall blood vessels, and some areas were rich in blood vessels. The cytoplasm was scant and eosinophilic. The nuclei were small, round and uniform in shape. Nucleoli were generally small but prominent. There was brisk mitotic activity (20 mitoses per 10 high-power fields). Immunohistochemically, an extensive panel revealed expression of E-cadherin, β-catenin, INI-1, and partly expression for panCK, EMA, CD31, Bcl-2, Vimentin. The immunoreactivity for Fli-1 was weak; the expression for CD99 and S-100 was focal. The tumor was negative for TFE3, CD34, HMB45, MyoD1, Myogenin, F8, ERG. The ki67 proliferative index was approximately 60%. The targeted DNA and RNA sequencing of a panel of 1084 genes revealed a novel SS18-POU5F1 fusion. The transcript involved SS18 exon 9, and POU5F1 exon 2. The tumor mutation burden is 0.47 mutations per megabase. Immunohistochemical analysis of programmed death-ligand 1 expression using the murine 22C-3 antibody revealed a tumor proportion score 3%. The microsatellite stability analysis showed that the tumor was microsatellite stable.Four weeks later, the patient presented to the local hospital again with progressively worsening edema of the left lower limb. A contrast-enhanced total-body computed tomography (CT) scan (head to foot) revealed pelvic, mediastinal and bilateral subclavian lymphadenopathy  and sintilimab . Concurrently, she accepted 39 Gy (3Gy×13 fractions) radiotherapy on pelvic, mediastinal and bilateral subclavian lymph node metastases. The edema of the left lower limb gradually disappear during the radiotherapy. A partial response was observed in the lesions within the radiation fields. However, unfortunately, a progressive disease was confirmed in the abdominal regions outside the radiation fields . Concurrently, she accepted chemotherapy with pegylated liposomal doxorubicin, ifosfamide, vinorelbine and etoposide within two months. The abdominal lesions shrank significantly during radiotherapy. The back pain resolved. However, unfortunately, severe edema of both lower limbs and inferior abdominal wall relapsed during the late stage of abdominal radiotherapy. A total-body CT scan (head to foot) showed progression of the left inguinal, pelvic, mediastinal and bilateral subclavian lymph node metastases  for the publication of any potentially identifiable images or data included in this article.ZL, HY, and MH performed image acquisition and completed 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.


Cronobacter sakazakii (C. sakazakii) is a bacterium known to cause severe neonatal infections in premature infants with the consumption of contaminated powdered milk formula. Adult infections are rare, and there have been no reports of pyosalpinx due to C. sakazakii to date.C. sakazakii in a sexually inactive postmenopausal woman. A 70-year-old woman presented to our hospital with left lower abdominal pain and fever. Abdominal computed tomography disclosed a cystic mass continuous with the left edge of the uterus. Urgent laparotomy revealed a ruptured left pyosalpinx with pus-like content. Left salpingo-oophorectomy, resection of the right tube, and washing of the abdominal cavity with saline were performed. Pathological examination of the left adnexa showed tubal tissue with acute inflammation and inflammatory exudate, which were compatible with pyosalpinx, and pus culture yielded C. sakazakii.We report a case of left pyosalpinx due to C. sakazakii. Cronobacter sakazakii infections in adult women might occur in the elderly, whose immunity has weakened. Further accumulation of cases of C. sakazakii infection is needed to clarify the etiology and behavior of C. sakazakii in adults.This is the first case report of pyosalpinx due to  Cronobacter sakazakii (C. sakazakii) is a Gram-negative, rod-shaped bacterium known to cause severe neonatal meningitis and necrotizing enterocolitis in premature infants with the consumption of contaminated powdered milk formula , 5–8C. sNeisseria gonorrhea and Chlamydia trachomatis, those in reported postmenopausal women are Escherichia coli, Clostridium perfringens, Peptostreptcoccus, Group C Streptococcus, and Bacteroides fragilis [C. sakazakii occurred in the hydrosalpinx that existed, and pyosalpinx developed. One of the candidate original infection sites might be the oral cavity [Pyosalpinx and tubo-ovarian abscess are almost always complications of pelvic inflammatory disease and are sexually transmitted infections in many cases. Therefore, pyosalpinx and tubo-ovarian abscess are usually observed in young women; they are rarely found in older women. One hundred and ninety-four cases of pyosalpinx or tubo-ovarian abscess in postmenopausal woman have been reported in the literature –17. Althfragilis , 15. Amofragilis , and thil cavity .C. sakazakii in a postmenopausal elderly woman. Cronobacter sakazakii infections in adult women might be caused to the elderly women whom the immunity decreased. Further accumulation of cases of C. sakazakii infection is needed to clarify the etiology and behavior of C. sakazakii in adults.In summary, we describe the first reported case of pyosalpinx due to


Case Report. We report the case of an 11-year-old boy who was treated symptomatically for a painful toe since 10 months, without a definitive diagnosis. X-ray, MRI, and scintigraphy, along with the typical nocturnal pain and swelling of the toe, suggested an osteoid osteoma, confirmed by histology after excisional biopsy of the lesion.  Osteoid osteoma is an uncommon, small, benign, self-limiting, and usually painful tumor of the skeleton. Diagnosis can be straightforward if seen in the usual locations as the femur and the tibia in young adults, who present with nocturnal pain, alleviated by salicylates. The diagnosis can be more challenging in the spine, pelvis, hand, or feet.  Osteoid osteoma should always be included in the differential diagnosis when it comes to nocturnal pain without systemic signs, even in unusual places in children. The awareness should lead to a prompt diagnosis and treatment. Jaffe  is accreDefinite diagnosis is usually based on the typical clinical presentation, high uptake on Tc-99 m scintigraphy, and the finding of the nidus, surrounded by sclerotic bone on X-ray or CT scan. MRI shows the lesion, surrounded by an inflammatory reaction on T2-weighted images or STIR .Differential diagnosis should include infection such as subacute osteomyelitis, tuberculous spina ventosa, or malignant tumoral disease like Ewing sarcoma –9.Treatment is usually obtained by destroying the cellular components of the nidus by means of heating with a radiofrequency probe under radiologic control in a percutaneous manner . The draInvolvement of the distal phalanx of a lesser toe is exceptional. The few cases in the literature report a painful soft tissue swelling of the distal aspect of the toe with clubbing and overgrowth of the nail. To our knowledge, this is the first case described in a child.An 11-year-old boy was referred to our pediatric rheumatologic reference center with a history of swelling and permanent pain of the distal end of his fourth toe without know trauma since 10 months. He presented no systemic symptoms, but the lab finding showed an increase in antinuclear antibodies without an increase of the general inflammatory parameters.He presented a partial response to ibuprofen and was treated symptomatically with a plaster cast without beneficial result.Psychologic counseling was offered since the boy was very saddened because he could no longer participate in sport activities like soccer.A fine needle biopsy was performed: no germs were found and there was not enough tissue to permit the histologic examination.The Tc-99m scintigraphy showed an increased uptake of the fourth toe, revealing an intense osteoblastic activity.A new X-ray was performed, revealing a partial sclerotic a slightly lobulated mass, replacing two-thirds of the distal phalanx. The mass was delineated with a radiolucent border from the remaining metaphyseal region. The physis remained intact .The MRI showed a hyposignal center of the distal end of the third phalanx of the fourth toe with surrounding inflammatory response and edema . The injWe decided to perform an excisional biopsy. We found a grayish, slightly lobulated, and rather hard mass, which popped out in toto after the incision. The specimen was sent as such for histologic examination and the diagnosis was confirmed .The defect was filled with lyophilized bone and the skin closed.The day after the excision the patient reported immediate relief and three weeks later, he resumed sports activities. After one year, the swelling of the toe subsided totally and the patient remained asymptomatic Figures .This report illustrates the diagnostic difficulties and its consequences when it comes to atypical presentations of uncommon pathologies. The few cases reported in the literature all concern adults and all presented with a nidus, surrounded by sclerotic bone. In our case, no sclerotic bone is found surrounding the large nidus which has replaced the distal two-thirds of the distal phalanx. The MRI findings are in contradiction with the notion of a high vascularity in the nidus reported by several authors. On the contrary, the perinidal tissues appear clearly hypervascular with the presence of inflammatory edema.To our knowledge, an osteoid osteoma in the third phalanx of a lesser toe in a child was never reported in the literature. This fact, together with a high titer of antinuclear bodies, led to a differential diagnosis of arthritis versus infectious disease, even in the absence of a rise in inflammatory markers, and caused a substantial delay in diagnosis a concomitant treatment.We hope that this case report will raise the awareness of the existence of osteoid osteoma in children, even in atypical locations.