Mesotheliomas are a rare malignancy of the serosal membrane. Mainly it affects the pleural surfaces followed by the second most common location, “peritoneum.” The disease follows an aggressive pattern of spread, and by the time the diagnosis is established, the condition significantly spreads to distant locations. Diagnosis of malignant peritoneal mesothelioma is typically made by tissue biopsy. The standard treatment is radical resection; however, patients have benefited from several other modalities. The current case report describes a unique case of malignant mesothelioma, biphasic peritoneal mesothelioma (BPM), which comprises less than 25% of all peritoneal mesotheliomas. The diagnosis and treatment do not differ from other subtypes; however, the prognosis is poor, and if untreated, the survival is typically less than six months. Malignant mesotheliomas are rare and aggressive tumors arising from serous linings of the pleura (65%-70%), peritoneum 30%), tunica vaginalis testis, and pericardium 1%-2%) [%-2% [1].0%, tunicThe recommended treatment for patients with confirmed malignant peritoneal mesothelioma is radical resection . Other tA 70-year-old female presented to the emergency room complaining of RUQ abdominal pain, nausea, and malaise for several days. She also reported significant weight loss for a couple of months. As a part of her diagnostic workup, computed tomography (CT) abdomen showed a large mass in the region of the hepatic flexure and ascending colon, which was contiguous to a 5-centimeter (cm) mass in the lower portion of segment VI of the liver Figure .Also, a smaller mass measuring 2.5 cm in size in the left lobe of the liver was visible on the CT abdomen. Preoperative laboratory work-up including carcinoembryonic antigen and liver function test findings were within normal limits. A colonoscopy was performed, which demonstrated an extrinsic mass, without any mucosal lesion, in the region of the upper ascending colon and hepatic flexure.Subsequently, the decision was made to proceed with the surgical exploration of the lesion. A right transverse abdominal incision was made several centimeters below the right costal margin, and a large right colon mass was identified. Next, the terminal ileum was divided with a stapler. High ligation of the right colonic mesentery was then performed using suture ligatures. The transverse colon was divided with a stapler to the right of the middle colic artery. The transverse mesentery was taken to join the mesenteric cut from the distal right colon. In order to take this tumor en bloc, segment VI of the liver was taken down using electrocautery, compression, clipping, and suture ligation of vessels. This en bloc resection was then mobilized from the retroperitoneum, taking the involved Gerota fat off the kidney, providing a complete resection Figure .Next, a primary anastomosis was performed between the terminal ileum and the left transverse colon with a stapler, and the suture line was reinforced with interrupted 3-0 silk lambert sutures. The wound was closed in layers using single strand #1 PDS*II. Scarpa’s fascia was closed with an interrupted 3-0 Vicryl suture, and the skin was closed with a running intradermal 3-0 Monocryl suture. The surgical specimens retrieved were sent for histological and immunological examinations, which resulted in postoperative pathologic diagnostic of BPM  3. She had a return of bowel function on POD 6. Following a high leukocytosis of 23.8, an infra-hepatic fluid collection was identified on a CT scan on POD 11. Subsequently, CT-guided drainage was performed, and 15 ccs of bilious fluid were aspirated upon placement of the catheter. On POD 14, her leukocytosis had normalized to 10.1. Following an increase in oral intake, she was cleared for discharge on POD 15. The patient died several weeks later, likely related to the highly aggressive nature of the disease.Of the 3,300 mesothelioma cases per year, less than 20% are peritoneal, with approximately two times as many males as females . ExposurDespite various risk exposures, survival depends on three basic histological patterns: epithelioid type (most frequent and better prognosis), sarcomatoid (most rare and worst prognosis), and biphasic (combination of the two) . Our patBPM is defined as epithelioid based, with at least 10% of the composition being a sarcomatoid pattern . LiteratMoreover, diagnosis may be further delayed if a thorough evaluation is not performed. Although no imaging modality has proven to be sensitive or specific for diagnosing PM, CT abdomen and pelvis (CTAP) with IV contrast, as was used in our patient, provides essential information ,3. The cDespite the poor prognosis, radical resection remains the mainstay of treatment for PM. The combination of CRS and HIPEC is found to extend survival to an average of 36 months ,9,11. OtBPM is a rare disease that is difficult to diagnose or treat. The average survival of untreated peritoneal mesothelioma is six months. Radical resection, CRS, and HIPEC improve survival; however, the disease is eventually fatal. To provide a better understanding of the pathogenesis and factors impacting survival, all patients diagnosed with BPM should be enrolled in a large-scale registry nationwide.


Automatic amputation of the ovary represents a rather uncommon condition. Especially asymptomatic autoamputation is an even more unusual laparoscopic finding. We hereby present a case of a 2-days´-old infant with a prenatal ultrasound (US) diagnosis of a cystic mass, laparoscopically proved as an amputated right adnexa. The female infant was asymptomatic and had normal laboratory exams, including hormone levels, according to her age. The infant was managed surgically, as the size of the cystic lesion, both prenatally and postnatally was indicative of surgical intervention. Careful monitoring is critical for the management of cystic lesions diagnosed prenatally. Although rare, the suspicion of an auto-amputated ovary has to be risen during diagnostic approach of infants with adnexal cysts, especially when these lesions are supposed to “wander” during imaging examinations, and also taking into account the size of the lesion in order for a final approach and management to be established. Regarding ovarian amputation, it is indeed a rare complication of a detorsed adnexa or ovarian cyst prenatally or postnatally. Asymptomatic cases are manifested as incidental findings during laparoscopy for other surgical reasons, or even as floating intra-abdominal mass during routine ultrasound (US) prenatal examination.Patient information: a 2-days-old female was admitted to our Pediatric Surgery Department with a prenatally diagnosed intra-abdominal mass. Regarding the baby´s history, she had a free paternal and maternal medical history. During the 34th week of gestation she was prenatally diagnosed with an abdominal cystic lesion measuring 58x57x54mm in US examination (mination . It is rClinical findings: after a full-term gestation, and aging one day, the neonate had an uneventful clinical condition, was afebrile, with normal development, no signs of pain, and with normal bowel sounds and bowel habits according to her age. On palpation, no abdominal mass and no abdominal distension were noted.Diagnostic assessment: the neonate had a new US exam during her first day of life, which revealed the above mentioned cystic lesion, measuring 50x30x43mm and based at the left lower abdomen, with no signs of vascularization (rization . The cysDiagnosis: after the completion of laboratory and imaging examinations, a preoperative diagnosis of an intra-abdominal cystic lesion was established and postoperative histopathology examinations would indicate the final diagnosis and treatment.Therapeutic intervention: the infant underwent a laparoscopy exploration. It is noteworthy that after induction to anesthesia, a mass seemed to wander inside the abdomen on palpation. Through an umbilical incision, a 5mm port was inserted and a 30o camera through it. Via a direct laparoscopic vision, two other ports, one in the right lower abdomen and the other in the left lower abdomen were inserted. Laparoscopy revealed a spherical floating mass inside the peritoneal cavity, with an attachment to a small portion of the distal part of the omentum ( omentum . Careful omentum , via dirFollow up and outcomes: histopathology revealed a macroscopically cystic lesion with a maximum diameter of 4.8cm (of 4.8cm . Microscof 4.8cm . No signAuto-amputation of the ovary is a rather uncommon condition among pediatric population, predominantly emerging from a cystic lesion of the ovary. It is suggested that a certain factor for ovarian cyst development is the augmented stimulation by maternal and placental hormones. Maternal history of diabetes, rhesus immunization, or increased permeability of placenta to b-HCG are also risk factors for such a pathologic entity . AccordiIn terms of antenatal and postnatal management, it is undoubted that imaging evaluation with US scan remains a gold standard. Antenatal US examination has proved, due to its widespread use, a prompt and reliable tool for diagnosis and management. The fact that it represents a non-interventional and quite inexpensive method is also in favor for the implementation of US in such small ages of patients. The proposed algorithm for management of these lesions depends predominantly on the lesion´s size. Regarding antenatal investigation, ovarian cystic lesions larger than 2cm in a fetus have to be put in observation, because they are considered abnormal , 5. A cuIn our case the cystic lesion was intraoperatively proved as an auto-amputated ovary on the basis of a prenatal torsion. It is also important that a laparoscopic approach was selected, as this allows intraoperative diagnostic capabilities, such as inspection of peritoneal cavity or even biopsy and also offers alternative therapeutic measures, such as aspiration, with preservation of adnexa, if it is proved viable, with the least interventional operative method.peritoneumor omentummay lead to malignant transformation, as reported [However, even if the diagnosis of free-floating adnexa has not been preoperatively established, surgical management should be considered in all cases of adnexal cystic lesions larger than 4cm in postnatal measurements, as these are prone to torsion and hemorrhage. Furthermore, possible reimplantation of auto-amputated adnexa into reported , 6, 7. TManagement of cystic lesions of inner genitalia in neonates and infants remain a challenging topic for the pediatric surgeon. Although rare, even the suspicion of an auto-amputated ovary has to be risen during diagnostic approach of infants with adnexal cysts, especially when these lesions are supposed to “wander” during imaging examinations, and also taking into account the size of the lesion in order for a final management approach to be established.


Background: Haemophilia is an inherited coagulopathy caused by the absence or dysfunction of clotting factor VIII or IX. Clinical manifestations are generally secondary to recurrent bleeding episodes mainly in the musculoskeletal system. Bleeding symptoms appear early in life and, when the disease is severe , joint and muscle bleeding may occur spontaneously. A pseudotumour is a recurrent, chronic, encapsulated, slowly expanding, muscle hematoma. Haemophilic pseudotumour is a rare complication of haemophilia which occurs, as a condition either from repeated spontaneous bleeding or coming from a traumatic origin, in 1–2% of haemophilic patients. Case report: A 32-year-old man with severe haemophilia A referred to our Clinic with a massive right iliac wing pseudotumour complicated by Staphylococcus aureus superinfection and skin fistulisation. In this report we describe the medical management and surgical treatment by the adoption of a novel surgical technique which involves the use of a pedicle-screw and rod system (PSRS), a polyglycolic acid MESH and bone cement in order to build up an artificial ilium-like bony mass. This case report highlights the importance of interdisciplinary approach and the efficacy of eradicating surgery as treatment, especially in the case of large and long-lasting lesions. Haemophilia is a recessive X-linked inherited coagulopathy caused by the absence or dysfunction of clotting factor VIII, for haemophilia A, and factor IX, for haemophilia B. This bleeding disorder has an incidence of 1:5000/10,000 male births .Clinical manifestations are generally secondary to recurrent bleeding episodes mainly in the musculoskeletal system where approximately 80% of all haemorrhages occur, usually starting from childhood .The most common pathophysiological process reported in haemophilic patients is joint destruction caused by intra-articular bleeding, condition known as haemophilic arthropathy. Intramuscular haemorrhage is the second most common site of bleeding in hemophiliacs.Pseudotumour is a rare complication of haemophilia which occurs, as a condition either from repeated spontaneous bleeding or coming from a traumatic origin, in 1–2% of haemophilic patients .A pseudotumour is a recurrent, chronic, encapsulated, slowly expanding, muscle hematoma ,3. This Actually, there is no consensus in terms of therapeutic management which in turn depends on variables such as pseudotumour location of the pseudotumour, its size and the characteristics of the patient.We report a successful resection of a massive right iliac wing pseudotumours in a 32 years-old patient affected by severe haemophilia A treated with a novel surgical technique.Our patient was a 32-year-old man, coming from a country with low availability of factor replacement therapy, height 185 cm, weight 90 kg, professional driver, with severe haemophilia A (F VIII < 1%). Family history positive for severe haemophilia A (his aunt was affected). The diagnosis of severe haemophilia A occurred in his hometown during childhood because he was involved in several atraumatic intra-articular haemorrhages in the elbows, ankles and knees.In March 2017, after an accidental fall skiing, he reported a blunt trauma of the right iliac wing with development of blood collection in that site.Pelvic X-rays and bone biopsy were performed at the local hospital and osteosarcoma was excluded. In July 2017 he went to another Haemophilia Centre for the appearance of swelling, fistulisation, leakage of serum blood material and functional limitation of the right hip. He also reported an evening fever of >39°. The physical examination also revealed severe atraumatic functional limitation of the left elbow and the right ankle. Laboratory tests performed showed F VIII <1%, F VIII inhibitors: 0 BU/mL. A pelvis CT was performed, showing the presence of an abscess and a fistula at the right iliac crest . StaphylSurgical treatment to remove the pseudotumour was proposed and accepted by the patient.Pd-FVIII 20 IU/Kg were administered pre-operatively as a slow intravenous bolus every 12 h for seven days before surgery.® 500–700 of the right superficial gluteal artery and the right lumbar artery , a metal bar and two fixing screws (one in correspondence with the acetabulum and the other on the sacro-iliac synchondrosis). Dead space between the implants and the remaining bone were therefore filled by antibiotic coated bone cement with the aid of two polyglycolic acid MESH in order to build up an artificial ilium-like bony mass, as well as graft compression.Finally, two treble hooks were positioned for repositioning the pelvic muscles. One surgical drain was placed at the end of the surgery. Intraoperatively, a biopsy of the lesion demonstrated the presence of fibrin-haematic organised material with fragments of necro-inflammatory material and scattered microcalcifications. Swabs in fistulous passages were also performed and they showed that there was no superinfection. The patient was transfused intraoperatively with one unit of RBCAt the end of the surgical procedure, pd-FVIII 20 IU/Kg was administered as bolus, then pd-FVIII 20 IU/kg every 12 h on the first postoperative day; from the second post-operative day pd-FVIII 20 UI/Kg at 8 a.m. and 10 UI/Kg at 8 p.m., up to the 4th day postoperatively; from the fifth post-operative day pd-FVIII 30 IU/Kg at 8 a.m. and 10 IU/Kg at 8 p.m. for two weeks, with the aim of maintaining F VIII level > 50%. Every 48 h, before the administration of concentrate of FVIII, blood was taken to check Hb, PTT and FVIII. The variations of FVIII biological activity levels during the hospital stay are summarised in The patient was transfused with one RBC unit the day-4, the day-6 and the day-7 after surgery owing to post-operative anaemia. A 21-days hospital stay was necessary in order to achieve a good clinical condition.3/uL (4.00–10.00), CRP 0.97 mg/dL, ESR 85 mm/h. Preoperatively, pelvic CT was performed, and it showed a voluminous collection of infected blood fluids both on the pelvic side along the planes of the ileo-psoas muscle and on the buttock side. The fluid collection extended to the paravertebral musculature, the quadrate loin muscle, and the external oblique muscle superiorly and posteriorly. In the postero-superior site, the collection went beyond the muscle fascia and extended into the subcutaneous fat layer. Therefore, surgical toilet and abundant irrigation was performed. During the surgical procedure, swabs were carried out for culture tests, later found negative for infection. The patient was discharged with a recommendation to continue antibiotic therapy with Rifampicin and Levofloxacin for 5 days and to continue the factor VIII replacement therapy. In this second hospitalisation a 11-days hospital stay was necessary in order to achieve optimal medical condition.In the immediate post-operative period, X-ray and CT of the pelvis showed emptying of the pseudotumour and correct positioning of screws and metal cerclage . PhysiotPatients were followed at outpatient clinic, at 7, 14 and 21 days. He was always in good general condition with surgical wound healed without signs of inflammation. Right hip ROM was almost complete at one month with control X-ray  unchangeThe patient continued prophylaxis with pd-FVIII 30 IU/Kg three times a week. No further bleeding manifestations occurred.Pseudotumour is an uncommon but potentially severe debilitating complication of bleeding disorders like haemophilia. Pseudotumour is a chronic encapsulated haematoma that is not resorbed and continues to expand due to recurrent haemorrhages  developiGilbert distinguished two types of pseudotumour based on location; the proximal type, seen most commonly in adults, occurs in the pelvis , and long bones extremities; in children there is a predilection for distal pseudotumours that occur in the small bones of the hands and feet .Regarding the involvement of soft tissues, pseudotumours mainly affect those of the thigh, the gluteal region and the iliopsoas muscle, with possible simultaneous bone involvement.Muscular pseudotumours can be classified as intra- or extra-muscular. Usually, intramuscular lesions remain localised, although they can sometimes expand towards the bone component. Pelvic haemophilic pseudotumours that develop after bleeding into the psoas or illiacus muscles may grow quite large and cause significant erosion of adjacent bone , like inUnfortunately, most of the haemophilic pseudotumours remain painless and asymptomatic, until they enlarge and compress adjacent structures (mass effect) causing pathological fractures, skin and soft tissue necrosis, fistula or infection ,9.In addition to the clinic, instrumental examinations such as X-ray, CT, MRI, ultrasound can be used for the diagnosis. On radiographs, intraosseus pseudotumours appear as well defined, uni- or multilocular, expansile lytic lesion of variable size with geographic pattern of bone destruction; a CT scan may differentiate the pseudotumours from the other tumours ; MRI is One limitation of MRI is its inability to allow differentiation between acute and subacute hematomas and abscesses .There is no consensus on the management of haemophilic pseudotumour. Modalities for treatment include replacement therapy, local irradiation alone or combined with factor replacement, surgery and conservative measures such as percutaneous drainage and embolisation ,11.In general, the risk of pathological fracture, intractable pain, skin fistula or infection, huge mass with neurovascular compression had been the indication to implement surgical management first ,13,14,15The best treatment for pseudotumours in patients with congenital coagulopathies is prevention , that isAs a general principle, mild-to-moderate lesions of recent origin, not causing pain or deformity can be managed with deficient factor infusions and immobilisation ,17,18,19Therapeutic arterial embolisation had been used in the management of large haemophilic tumours, especially of the pelvic region . HoweverSurgery is generally curative and it is cited as the most effective treatment for pseudotumour ,23,24.However, due to localisation, size and other factors, not all pseudotumours are amenable to surgery .Surgical excision is indicated for large lesions, imminent rupture of pseudotumour, chronic pseudotumours or failure of conservative measures ,25,26,27Surgical management of pseudotumour involving pelvis bone was previously described ,11,28,29Pennekamp et al.  reportedIn our case, too, pelvis containment was needed, and it was realised by adopting a novel surgical technique which involves the use of metal bar, fixing screws, polyglycolic acid MESH and bone cement in order to build up an artificial ilium-like bony mass. The choice of using fixing screws and a bar was dictated by the necessity of minimise the risk of implant mobilisation.To our knowledge this is the first time that a PSRS has been used for the surgical management of a haemophilic pseudotumour.PSRS with various techniques have been proposed for pelvic ring reconstruction following pelvis malignant tumour resection ,31,32. FA surgical MESH is a sterile woven material designed for permanent implantation within the body during open or laparoscopic procedures. A wide range of mesh implants is available with two main functions: to stabilise and strengthen soft tissue defects and to act as a sling to support prolapsed organs and viscera . In our PMMA, commonly known as bone cement, has proved to be one of the most versatile and lasting biomaterials in orthopaedics having an important role in joint replacement, as well as spinal and tumour surgery ,37. At pSurgery is curative but inherently risky, therefore a careful patients selection is mandatory . SurgicaTreatment is especially challenging in patients with large masses and extensive bone destruction .Infection remains a frequent complication of reconstruction with haemipelvic allografts or autoclaved grafts and prosthesis after internal hemipelvectomy, with reported rates varying from 12% to 47%. The following factors contribute to the high risk of infection: the prolonged surgical time necessary to resect pelvic tumours, the proximity of the rectum and genitourinary tract, the large dead space after pelvic bone resection and the large amount of foreign material implanted in the surgical wound ,44. In oAccording to the review of Austin et al.  surgicalIn our case the infection was controlled by antibiotic therapy and the recidivism fistula surgical revision with fistulectomy was successful.Although there is still no consensus regarding the therapeutic management of pseudotumours, the favourable course of the case described above allows us to suggest eradicating surgery as the first line of treatment, especially in the case of large and long-lasting lesions associated with complications, e.g., infection and fistula. However, in order to have the best results in the long term, a not only surgical, but interdisciplinary approach is necessary.Therefore, early diagnosis and a variety of treatment modality should be initiated to prevent the haemophilic pseudotumours progressing to intractable status. A multicentre study or nationwide population-based study should be performed to explore the treatment efficacy in the future.


Isolated lesions of the sphenoid sinus, particularly malignancies, are rarely reported and exist largely within the Otolaryngology literature. Delayed diagnosis may necessitate neurosurgical involvement; therefore, neurosurgeons must be aware of the range of pathologies in this region in order to provide adequate treatment. We present an unusual case of an 89-year-old female with several weeks of worsening headaches, vision loss, and cranial neuropathies. Work-up at an outside hospital was non-diagnostic. After referral, an expansive and erosive lesion within the left sphenoid sinus was identified. A transsphenoidal approach for resection of the lesion yielded a primary non-salivary non-intestinal type sinonasal adenocarcinoma, as well as bacterial sinusitis and probable allergic fungal sinusitis. The patient was treated with antimicrobial medications as well as stereotactic radiosurgery. Her neurological deficits did not improve with treatment, and she ultimately expired 3.5 months post-operatively after transition to hospice. Primary sinonasal adenocarcinoma is a very rare pathology in this location. Surgical intervention is necessary to obtain an accurate diagnosis and proceed with appropriate treatment. Delayed diagnosis likely portends a worse prognosis. Isolated lesions of the sphenoid sinus are rarely reported; most of these few cases have been described in the Otolaryngology literature. Primary malignancies in this location are particularly infrequent but can have irreversible consequences when overlooked in the differential diagnosis. Because of the location and symptoms associated with these lesions, neurosurgeons may not become involved in the patient’s care. On rare occasion, however, neurological deficits may develop late in the disease course, precipitating a request for neurosurgical assessment; therefore, neurosurgeons should be aware of the range of pathologies associated with this location. Here, we report the unusual case of a primary non-salivary gland non-intestinal type sinonasal adenocarcinoma located within the sphenoid sinus associated with ophthalmoplegia and visual loss.The patient was an 89-year-old woman with a recent history of urothelial carcinoma who presented with worsening headaches and left-eye vision loss over the previous four weeks. While her headaches had been present for several months, the vision loss began a couple of weeks following an intravitreal injection for macular degeneration. She had been following with her outside ophthalmologist who suspected temporal arteritis; however, temporal artery biopsy was negative, and steroids failed to improve her symptoms. A CT head obtained at an outside facility was reportedly negative though unavailable for our review, and because of an incompatible pacemaker, she was unable to have an MRI. She was admitted initially to the Neurology service for workup of stroke versus malignancy, with lumbar puncture returning with a normal CSF profile and cytology negative for malignant cells. Ophthalmology evaluation revealed essentially complete left eye ophthalmoplegia with an unreactive left pupil. CT angiography performed on their recommendation revealed a hyperdensity within the left sphenoid sinus, as well as erosion of the supralateral wall, extension into the optic canal, and enhancement of the left cavernous sinus, concerning initially for an infectious process Figure . At thisThe surgical pathology specimen was examined by our neuropathology team and independently reviewed by an outside pathologist specializing in otolaryngic pathology. The left sphenoid lesion was fixed in formalin, embedded in paraffin, and examined on four-micron sections stained with H&E. Immunoperoxidase studies with appropriate controls were performed with the following antibodies: CK7, CK20, CK5/6, CDX2, b-catenin, TTF1, p63, p16, p53, SOX-10, GATA3, S100, synaptophysin, chromogranin, estrogen receptors (ER), AMACR, and Ki67. Microscopically, the tumor was located growing beneath non-ulcerated mucosa, and the complexity of the growth pattern and local invasive growth supported the diagnosis of malignancy. The morphological features were consistent with sinonasal non-salivary gland non-intestinal type adenocarcinoma, although the immunophenotype supported this diagnosis only partially. There was no evidence of SOX10 and S100 expression that is characteristic of this entity. At the same time, the absence of CDX2 and CK20 immunoreactivity helped to rule out sinonasal non-salivary gland intestinal-type adenocarcinoma, and the lack of TTF1 expression ruled out a metastatic lung and thyroid carcinoma. Figures Although no definitive fungal elements were identified on the nasal biopsy, there were features consistent with this additional diagnosis including the presence of eosinophilic mucus, eosinophils, and Charcot-Leyden crystals. Culture results eventually returned positive for methicillin-sensitive Staphylococcus aureus; therefore, she was treated with two weeks of augmentin and posconazole. After a multidisciplinary discussion with our Hematology and Radiation Oncology teams, she received outpatient palliative radiation of 2000cGy in five fractions to the left sphenoid sinus and cavernous sinus. She was not felt to be a good candidate for chemotherapy given her age and evidence of renal and spinal metastases on positron emission tomography (PET) imaging. At the last follow-up, three weeks after surgery, the patient’s neurologic deficits were stable. She chose to transition to hospice shortly after that appointment in order to avoid lengthy travel to our institution and further diagnostic studies, which she did not feel would improve her outcome. The patient died 3.5 months after surgery.Only a handful of reports of primary adenocarcinoma of the sphenoid sinus are described in the literature -5. AccorRadiographic imaging is the diagnostic tool of choice, with key CT findings in malignancy being bony destruction or erosion ,4,7,8. MThis case particularly demonstrates the importance of obtaining a tissue sample for appropriate diagnosis and treatment. Presumptive treatment for sphenoid sinusitis based on the general prevalence of infection in this region and the radiology impression of sinusitis in this patient’s pre-operative imaging would have undoubtedly allowed even further spread of the tumor. The patient ultimately required two different treatments for separate pathologies, which would not have been detected without a tissue diagnosis. Additionally, it was necessary to exclude a more invasive fungal sinusitis such as mucormycosis, which carries significant morbidity and mortality. The authors chose a microscopic approach to the sphenoid sinus based on preferred surgeon experience. The endoscopic approach may have allowed better visualization of the suspected region of erosion into the cavernous sinus, although this would likely not have changed the patient’s outcome, and previous research indicates that microscopic surgery of the paranasal sinuses is a safe and effective alternative to endoscopic surgery . AdditioWhile isolated lesions of the sphenoid sinus are most likely to be infectious, physicians should include malignancy on the list of differential diagnoses, particularly in patients presenting with vision loss and cranial neuropathies. CT imaging should include careful study of the extracranial structures and should evaluate for bony expansion or erosion which could indicate malignancy. Referral to a tertiary care center should be initiated early in the disease course in those with neurologic signs and symptoms. Tissue diagnosis is necessary to confirm the pathology which can be performed through a microscopic or endoscopic approach. Surgery, chemotherapy, and/or radiation are all treatment options based on the stage of the disease, with earlier detection and diagnosis being key to a more favorable prognosis.


A solid pseudopapillary tumor should be included in the differential diagnosis of every pancreatic cystic lesion. A constellation of microscopic morphology and immunohistochemistry, in addition to the clinical history, aids in reaching the correct diagnosis. A solid pseudopapillary tumor should be included in the differential diagnosis of every pancreatic cystic lesion. A constellation of microscopic morphology and immunohistochemistry, in addition to the clinical history, aids in reaching the correct diagnosis. The morphologic features of the cells were similar to the cells seen in neuroendocrine tumors. Immunohistochemistry cleared up the doubts and made the diagnosis of SPTP the definitive diagnosis.Solid Pseudopapillary tumors of the pancreas (SPTPs) are rare neoplasms that occur most commonly in females in their second or third decade and account for about 0.17%‐2.7% of all pancreatic tumors.Abdominal pain is the most frequent clinical manifestation of SPTPs, and in the rest of the cases, there are no specific symptoms, wherein the diagnosis is made incidentally during routine examination.SPTPs are well defined neoplasms with solid and variably cystic areas. Occasionally, the extensive cystic tumor degeneration along with the bland morphology of SPTPs cells make it difficult to be differentiated from the other pancreatic tumors, especially neuroendocrine tumors; therefore, immunohistochemistry (IHC) plays a crucial role in making the accurate diagnosis. Here, we present a difficult‐to‐diagnose SPTP that manifested as a solitary pancreatic cyst.2A 36‐year‐old nonalcoholic female patient with a history of smoking for 15 years, presented with abdominal pain radiating to her back. The pain was not relieved by NSAIDS. The patient mentioned that she had experienced many episodes of nonbilious vomiting, nausea, and intermittent nonbloody diarrhea.On physical examination, a mass was palpated in the epigastric region. Laboratory tests were normal except for a mild anemia (Hemoglobin = 11 g/dL). The radiological findings on ultrasound (US) and noncontrast computed tomography (CT) revealed a unilocuar cystic lesion in the tail of the pancreas attached to the spleen  with almost total cystic degeneration was favored.One year later, β‐catenin and E‐Cadherin were added to the IHC panel in our laboratory. The stains were then performed, and the tumor cells showed a negativity for E‐cadherin and expressed nuclear positivity for β‐catenin Figure ; consequ3Solid pseudopapillary tumors are rare low‐grade malignant neoplasms of uncertain cellular differentiation, commonly located in the tail of pancreas.Clinically, SPTPs are usually nonsymptomatic and are discovered incidentally as abdominal masses during physical examinations.Usually, the ultrasound and CT of SPTPs reveal well‐defined solid masses with cystic components.Typically, the gross inspection of SPTPs shows well‐defined solid masses with variable areas of cystic degeneration.In our case, the tumor cells showed positivity for CD56, NSE, and cyclin D1, and negativity for the epithelial and histocytic markers. It was difficult to distinguish between SPTP and low‐grade NET because of the microscopic and immunohistochemical overlaps. One year later, E‐cadherin and β‐catenin, but not SOX11, became available in our laboratory and performed to make the final diagnosis. The tumor cells showed negativity for E‐cadherin and positivity for β‐catenin, so we redeemed our diagnosis from NET to SPTP.Surgery is the gold standard treatment with curative results if the lesion is completely resected.4Solid pseudopapillary tumors should be included in the differential diagnosis of every pancreatic cyst. A wide immunohistochemical panel, particularly β‐catenin and E‐cadherin, is needed to differentiate solid pseudopapillary tumors from other pancreatic neoplastic lesions, especially from neuroendocrine tumors.None declared.Ebrahim Makhoul and Zeina Alabbas: drafted the article and collected the patient's data. Ali Adra and Alexey Youssef: participated in collecting the data and drafting the article. Emad Ayoub: performed the surgical biopsy and participated in revising the article. Rana Issa: The guarantor and supervisor, critically revised the article, performed the pathologic examination, and approved the final manuscript.Informed consent was obtained from the patient regarding the report of her clinical scenario data in an anonymous way.


With this study we provide the most comprehensive series of false-negative DWI findings in ioMRI that were not followed by additional resection. Our study underlines the limitations of intraoperative DWI sequences for the detection and size-estimation of hyperacute infarction. The awareness of this phenomenon is crucial for any neurosurgeon utilizing ioMRI.Intraoperatively acquired diffusion-weighted imaging (DWI) sequences in cranial tumor surgery are used for early detection of ischemic brain injuries, which could result in impaired neurological outcome and their presence might thus influence the neurosurgeon’s decision on further resection. The phenomenon of false-negative DWI findings in intraoperative magnetic resonance imaging (ioMRI) has only been reported in single cases and therefore yet needs to be further analyzed. This retrospective single-center study’s objective was the identification and characterization of false-negative DWI findings in ioMRI with new or enlarged ischemic areas on postoperative MRI (poMRI). Out of 225 cranial tumor surgeries with intraoperative DWI sequences, 16 cases with no additional resection after ioMRI and available in-time poMRI (< 14 days) were identified. Of these, a total of 12 cases showed false-negative DWI in ioMRI (75%). The most frequent tumor types were oligodendrogliomas and glioblastomas (4 each). In 5/12 cases (41.7%), an ischemic area was already present in ioMRI, however, volumetrically increased in poMRI (mean infarct growth + 2.1 cm Besides intraoperative resection control, the updated imaging sets are utilized for further navigation and for the immediate detection of surgical complications6.Intraoperative MRI (ioMRI) is nowadays a commonly used tool providing high quality intraoperative imaging for immediate resection control, especially in brain tumor surgery10. In ioMRI, DWI sequences are employed for the early detection of new surgery related infarcts potentially impairing neurological outcome14. Various studies have shown that the quality of intraoperatively acquired DWI sequences is sufficient to delineate new surgery-related ischemic lesions15. Furthermore, the surgeon's decision whether to continue with further tumor resection after ioMRI control has an impact on the development of new or enlarged ischemic areas around the resection cavity15.Diffusion-weighted imaging (DWI) sequences are routinely used for detection of ischemic infarcts, which can be delineated on DWI sequences within minutes after the ischemic event17, whereas in hyperacute stroke imaging false-negative DWI signals are much more frequently described25.However, little is known about the occurrence of false-negative DWI signals in the context of hyperacute stroke imaging, especially when acquired intraoperatively. The available literature regarding ioMRI with false-negative DWI is limited to a few case studiesTo further analyse and describe the phenomenon of false-negative DWI findings we used our institutional registry to identify cases in which no additional resection was performed after ioMRI and where the postoperative MRI (poMRI) showed new or volumetrically enlarged infarct zones.26. Furthermore, all patients were hospitalized with routine vital parameter monitoring until acquisition of poMRI. To specifically identify cases with false-negative DWI imaging, all cases with additional resection after ioMRI were excluded. This information was extracted from the operation reports or by reviewing video records of the surgeries. Additionally—if not clearly stated in the operation report—the microscope video records were reviewed to rule out cases where extensive electrocautery or placement of agents like FLOSEAL for hemostasis were performed after ioMRI. No carmustin wafers were placed in the resection cavity in any of the procedures, neither any other locally applied cytostatic agent was used.We retrospectively identified patients who underwent brain tumor surgery with ioMRI (including DWI series and apparent diffusion coefficient (ADC) maps) at the Department of Neurosurgery, University Hospital of Zurich, between January 2013 and October 2019. Cases where no follow-up imaging or clinical examination data were available were excluded . Intraoperative MRI images are immediately evaluated interdisciplinary by the operating surgeon and a board certified neuroradiologist to also consider surgery-specific imaging features .Our institution utilizes a 3 Tesla (T) high-field MRI  and a NORAS 8 channel head coil  in a 2-room intraoperative MRI suite concept. After maximum safe tumor resection according to the surgeon's assessment, the dura and skin is closed provisionally, and the patient is prepared for ioMRI transfer. Safety and high quality of intraoperatively acquired MRIs were ensured by following an ioMRI safety checklist that has been published recently27 and by retrospective chart review. The institutional registry and this study have been approved by the local ethical review board , with a waiver for written informed consent. This study was performed in accordance with the ethical standards of the institutional and local ethics review board and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.All data were extracted from our patient registry2, echo time 68 ms, repetition time 6000 ms, slice thickness 4 mm) and ADC image series for each case. Artificial diffusion restrictions due to e.g. microhemorrhage in the resection cavities were ruled out by studying the susceptibility weighted imaging (SWI) sequences. Additionally, immediate surgeon’s feedback was considered by the neuroradiologist to consider any placed hemostatic materials like TABOTAMP or intentionally left tumor remnants which could mimic ischemic-like DWI signal alterations. Cases with insufficient quality to delineate new DWI restrictions in the operation field (e.g. caused by air artifacts) were excluded as well.Infarcts were determined based on DWI  point-shaped infarcts; (2) band-shaped infarcts at the resection cavity; (3) sector-shaped infarcts that involve deeper parts of the parenchyma; (4) territorial infarcts15. In the case of multiple isolated infarcts, only the highest class was considered. Volumetry of all infarct volumes was performed semiautomatically based on DWI b100 images using iPlan Net  “SmartBrush” tool, with manual segmentation adjustments if necessary. No contrast adjustments or other postprocessing methods were used on the original DWI images. The relative infarct volume change . Seven patients (58.3%) harbored new infarcts in poMRI without DWI restrictions in previous ioMRI. Infarct classes ranged from point-shaped to sector-shaped, and volumes ranged from 0.05 to 1.93 cm3 (mean infarct volume 0.77 ± 0.66 cm3). Overall, middle cerebral artery (MCA) vascular territory was affected by ischemia most frequently , followed by posterior cerebral artery (PCA) territory , and anterior cerebral artery territory . Two cases harbored infarcts in both MCA and PCA territory.Five patients (41.7%) showed DWI restrictions already in ioMRI  with a mean infarct volume of 0.75 ± 0.4 cm3) and patient #1  did not exhibit any new neurological deficits in the postoperative course.Five of 12 patients harbored new neurological deficits postoperatively ranging from mild paresis or hypesthesia to aphasia (see Table 3). However, the patient did not exhibit any new neurological deficits postoperatively.71-year old female (patient #1) with right temporal  WHO grade II oligodendroglioma. IoMRI showed only a very slight band-shaped DWI restriction at the resection cavity. Because ioMRI showed no remaining tumor remnants, no further resection was performed after ioMRI. However, poMRI showed a clear enlargement of the infarcted tissue area, ultimately involving deeper parts of the parenchyma  with WHO grade II astrocytoma located in the cingulate gyrus. IoMRI showed no tumor residuals and no obvious signs of DWI restrictions. Discrete DWI hypointensity correlated to blood degradation products in SWI images and was therefore not accounted as an infarct. No additional resection or extensive hemostasis were performed after ioMRI. However, poMRI clearly showed a new sector-shaped infarct of about 1.93 cm3. The patient did not show any new postoperative neurological deficits.68-year old female patient (patient #5) with a recurrent right-sided temporal (fusiform gyrus) glioblastoma who underwent re-craniotomy and tumor re-resection. IoMRI showed a total resection and no signs of any DWI restriction. PoMRI, however, depicted a tiny point-shaped DWI lesion with corresponding ADC signal indicating a circumscribed infarct of 0.38 cm3. Nevertheless, this increased infarct area was not reflected in an impaired neurological status and the patient remained neurologically asymptomatic. A 63-year old male patient (patient #8) was operated on an anaplastic astrocytoma WHO grade III in the right temporal lobe, spanning multiple gyri. Since there was no residual tumor seen in ioMRI, no further resection was performed. On the third postoperative day, the MRI showed a clear infarct growth compared to the small band-shaped DWI restriction seen intraoperatively see Fig. . VolumetIn the present study we could demonstrate the limitations of ioMRI DWI sequences in to detect hyperacute infarction. Our retrospective analysis revealed a high incidence of false negative intraoperative DWI findings. Screening of 225 ioMRI cases identified 16 patients without additional resection after ioMRI of which 12 harbored enlarged or completely new infarcts on poMRI.15. Additionally, numerous studies have shown that thin DWI restrictions, at e.g. the resection cavity margin, can be frequently delineated in 20–64% of cases postoperatively after tumor resection29. While small ischemic areas around the resection cavity are often considered as “normal postoperative changes”, larger surgically induced infarcts harbor the risk of transient or persistent new neurological deficits.Infarction detection based on DWI sequences in an intraoperative setting is a valuable tool for early diagnosis of surgery related complications. Various groups could show that intraoperatively acquired DWI sequences are of sufficient quality to delineate acute ischemic brain injury8, a non-negligible number of articles report cases of hyperacute strokes with initially negative DWI findings25. This raises the question, whether intraoperatively acquired DWI images underestimate or miss hyperacute or not yet apparent ischemic brain lesions, as our data would also suggest. Oppenheim et al.20 retrospectively analyzed 139 stroke patients and found false-negative DWI images in initial MRI scans in 5.8%. The majority of these DWI-negative infarcts were in the vertebrobasilar vascular territory and the initial imaging was performed within 24 h of stroke symptom onset.Despite the reported high sensitivity of DWI sequences to detect ischemic strokes17. Prabhu et al.17 presented three cases with false-negative DWI in ioMRI during tumor resection, although in one patient additional resection was performed after intraoperative resection control, and no information was provided about the other two cases regarding extended resection. In our current report we explicitly filtered cases where no further resection was performed after ioMRI in order to prevent accidental inclusion of new infarcts induced surgically after ioMRI. Saint-Martin et al.16 analyzed hyperacute infarcts delineated in ioMRI during pediatric brain tumor surgery and showed that ioMRI often underestimates the extent of infarcted brain tissue compared to early poMRI. This is in line with the literature30 and is also suggested by our data.In the intraoperative setting, however, the occurrence of false-negative DWI imaging in ioMRI is clearly underreported and includes only reports of single casesIn 5 of our cases intraoperative DWI findings underestimated the size of ischemic lesions, and in 7 cases ioMRI showed no clear DWI restrictions at all, even though new infarcts were identified in poMRI. This demonstrates that DWI imaging in ioMRI harbors the risk of either underestimating the size or missing the presence of infarcts. However, in our case series, the minority of patients suffered of a new neurological deficit postoperatively, and some of the patients with the largest infarct volumes even remained clinically asymptomatic.32. Numerous studies have tried to determine the critical CBF threshold for survival of the penumbra in focal ischemia33.Various possible explanations for the phenomenon of false-negative DWI findings have been postulated. A focal reduction in cerebral blood flow (CBF) below a threshold critical for neuronal function but still sufficient for proper diffusion was suggested35. Mechanical vessel manipulation could cause delayed vasospasms leading to a transformation of the hypoperfused tissue at risk to an actual ischemic infarct area, which could be missed on ioMRI. A reduced cerebral metabolism during anesthesia36 could help collateral vascular supply to maintain a sufficient perfusion during surgery, which might then fail after extubation and normalization of cerebral metabolic needs.Additionally, postoperative cerebral vasospasm has been described in as a possible complication especially after resection of posterior fossa tumors38. This perfusion-diffusion mismatch area might be at high risk for definitive infarction due to varying e.g. blood pressure in the immediate postoperative setting or during surgery. The time between vascular injury during surgery and the ioMRI might be also influential for the here described phenomenon. However, our study cohort did not reveal any correlation between the timepoint of ioMRI relative to surgery start and the size of the missed infarct.Our 5 cases with already present DWI restrictions in ioMRI and enlarged infarcted area in the follow-up MRI are in line with previous reports showing an enlargement of acute ischemic strokes in subsequent MRI studies with a presumed initial perfusion-diffusion mismatch39, our case series demonstrates underestimated and false-negative DWI lesions in all vascular territories.In contrast to reports of false-negative DWI in hyperacute ischemic stroke diagnostics where this phenomenon was predominantly described in lesions of the posterior circulation and the brainstemOur case series indicates that normal DWI findings in ioMRI do not rule out new or enlarged infarcts postoperatively, even when no additional resection was performed. False-negative DWI signals occur in all vascular territories and can range from thin marginal areas around the resection cavities up to more pronounced sector-shaped infarcts with involvement of deeper brain tissue.This is the first large study showing the limitations of ioMRI DWI sequences to detect or volumetrically estimate surgery related ischemic events in brain tumor surgery. However, due to the retrospective design of our analysis and the low case number, generalizable estimates of the actual incidence of false-negative DWI in ioMRI cannot be provided. Additionally, an unequivocal distinction between the etiology for the missed or underestimated infarcts  is not possible based on this study. Nevertheless, the awareness of this phenomenon is important for any neurosurgeon utilizing ioMRI. Since any intraoperative detection of vascular injury can influence the surgeon’s decision regarding the scope of further resection.Supplementary Table 1.


Recurrent respiratory papillomatosis (RRP) is classically described as a benign neoplasm of the larynx. Nevertheless, transformation to dysplasia and invasive carcinoma can occur. Sarcoma of the larynx is rare. Here, we present a case of sarcoma in a patient repeatedly treated for RRP. We report a case of a 73- year old Caucasian male diagnosed with adult-onset recurrent respiratory papillomatosis (AORRP) at the age of 63y. o. During the previous 10 years, he underwent multiple surgeries. In the last therapeutic intervention, he was treated with laser excision of the papilloma and topical mitomycin application. Two months after treatment, papilloma recurred and sarcoma was diagnosed. RRP is a benign lesion.  Affected patients usually require multiple interventions. It rarely degenerates to malignancy. Sarcoma in the larynx in the presence of RRP is a rare case. Extended surgical removal remains the treatment of choice. Adjuvant therapies consist of chemotherapy and radiation and are reserved for unresectable or recurrent cases. Recurrent respiratory papillomatosis (RRP) is characterized by multiple recurring papillomas throughout the respiratory tract, predominantly in the larynx and the trachea. The risk of malignant transformation is approximately 3–7%. Laryngeal sarcoma is an extremely rare malignancy and accounts for <1% of all malignant laryngeal tumors. We present the first clinical report in the literature of sarcoma on a patient with laryngeal papillomatosis. A 73- year old Caucasian male suffering from RRP, initially diagnosed 10 years ago, was subjected to periodical endoscopic examination for his disease. There was no history of smoking and alcohol consumption. In a period of 10 years, he underwent multiple surgeries including laser and cryosurgical removal of the papillomatous lesion.He was also vaccinated with Gardasil HPV vaccine. In his last surgery, he was treated with laser removal of the papillomatous lesion and topically mitomycin application . The hisA month later, the patient turned up at his local Emergency Department with progressive dyspnea. He underwent an urgent tracheotomy. Fiberoptic nasopharyngeal endoscopy in our ENT department showed a large mass occupying the oropharynx and laryngeal vestibule. Microlaryngoscopy and biopsy were carried out under general anesthesia. Histopathological and immunohistochemical examinations showed undifferentiated sarcoma grade 2 . Magnetic resonance imaging (MRI) of the neck , computeThe radiological findings were a large mass, 46.1X35.2X40.2mm in size, arising from the larynx, one right cervical lymph node involvement and no distant metastasis.PET/CT scan showed intense FDG uptake of the lesion with SUVmax of 35.6. After evaluation by the multidisciplinary team (MDT), total laryngectomy, total thyroidectomy, and bilateral elective neck dissection (levels II-III-IV-V) were performed . The histopathological and immunohisto- chemical examinations confirmed the former diagnosis showing a pT3N1M0 sarcoma. The excision appeared to be complete. One month later, the patient had a recurrence of the mass in the oropharynx with a new biopsy confirming sarcoma recurrence. He was treated with four cycles of adjuvant chemotherapy  and palliative radiotherapy. The patient died seven months after the total laryngectomy due to locally extensive disease. Infection by HPV subtypes 6 and 11 accounts for most cases of RRP ,3. It isMitomycin C, the agent administered to this patient, is widely used as an antibacterial, chemotherapeutic agent, and fibroblast inhibitor . It has Head and neck is an uncommon site for soft tissue sarcoma and accounts for <10% of all sarcomas ,8. Smoking, alcohol, and radiation are well-established predisposing factors in carcinosarcoma/sarcomatoid carcinomas of the larynx. Surgery, with either local excision or wide excision (laryngectomy), remains the gold standard treatment for laryngeal sarcoma. Radiation and chemotherapy are adjuvant therapies, usually kept as a salvage therapy after recurrence . In thisSarcoma cannot be directly related to the background of the patient. It is not possible to know whether there was a relationship between RRP/RRP treatment and malignant transformation or whether the sarcoma in this patient was coincidental.The heterogeneity of sarcomas with regard to molecular genetics, histology, clinical characteristics, and response to treatment makes management of this rare and varied neoplasm particularly challenging. Sarcoma of the larynx in a patient with recurrent respiratory papillomatosis is an extremely rare entity and this is the first report in the literature.


Uterine sarcoma accounts for 3–7% of uterine malignant neoplasms. It is more aggressive than epithelial neoplasms, and patients have a poor prognosis. Rhabdomyosarcoma is classified as a heterologous uterine sarcoma. It is the most common soft tissue malignancy in children while rare in adults. In young patients, the majority of genital tract rhabdomyosarcomas occur in vagina; however, the most common site of gynecologic rhabdomyosarcoma is cervix followed by uterine corpus, in adults. Uterine corpus rhabdomyosarcoma is rare in adults. Diagnosis of pure rhabdomyosarcoma in uterus involves widespread and perfect sampling as well as precise histopathological evaluation to uncover any epithelial component.Here we report a case of pure rhabdomyosarcoma of uterine corpus in a 60-year-old Iranian postmenopausal female who had osteopetrosis, presenting with 8-month heavy vaginal bleeding and a protruding cervical mass. She is alive on 18-month follow-up after treatment.Rhabdomyosarcoma of uterine corpus is rare in adults. Diagnosis of pure rhabdomyosarcoma in uterus involves widespread and perfect sampling as well as precise histopathological evaluation to uncover any epithelial component. Treatment options in adult gynecological rhabdomyosarcoma are based on studies in younger patients, and more studies may help us choose the best approach for improving outcome. Rhabdomyosarcoma (RMS) is an aggressive tumor that tends to develop in children and younger patients . A largeA 60-year-old Iranian female patient attended our emergency department with complaints of heavy vaginal bleeding during last 8 months. In her medical history, she had three vaginal deliveries, diabetes mellitus, depression, and osteopetrosis. Her menopause started at 50 years of age. She did not report any history of sexually transmitted infection or receiving hormonal medications. Her medications included metformin (500 mg twice a day), sertraline (50 mg daily), vitamin D (1000 mg daily), and calcium (500 mg daily). She had undergone surgery twice over the last 10 years, due to fracture of lower extremities. Her family history was unremarkable.She was admitted to our hospital. Hemoglobin level was 6 g/dl at the time of admission. The other hematologic and biochemistry tests revealed no significant problems. On pelvic examination, which was performed under anesthesia, we found a remarkably enlarged uterus and a 10-cm soft hemorrhagic mass originating from posterior part of cervix and protruded into vagina. Excisional biopsy was performed. Abdominopelvic computerized tomography (CT) scans revealed a large uterus, a 15-cm pelvic mass in posterior wall of uterus , actinomycin-D (1 g), and cyclophosphamide (1200 mg) (VAC) every 3 weeks for six cycles. Delayed radical hysterectomy, bilateral salpingoophorectomy (BSO), and pelvic lymph node dissection (PLND) were performed due to large bulky tumor and partial response to initial chemotherapy. Her postoperative condition was appropriate, and she was discharged 3 days after surgery. Following discharge, external pelvic irradiation  was performed in 6 weeks. No complications, such as radiation cystitis or proctitis, occurred. She signed an informed consent form and gave us permission to report her disease. She was followed up every 3 months and was free of disease 18 months after treatment  test showed positive desmin Fig. a, MyoD1 Uterine sarcoma accounts for 3–7% of uterine malignant neoplasms and is more aggressive in comparison with epithelial neoplasms , 6.UteriWe hereby presented a case of uterine alveolar RMS in a 60-year-old postmenopausal female patient. We searched PubMed and Scopus for English original articles, letters, and short communications with keywords: “Rhabdomyosarcoma” AND “Uterus” since 1972. Articles that reported rhabdomyosarcoma in corpus of uterus in adults were reviewed if their full text was available. Table Previous pelvic irradiation and long-term use of tamoxifen are among the risk factors for developing uterine RMS . Our patin situ hybridization (FISH) and reverse-transcription polymerase chain reaction (RT-PCR) methods can detect fusion genes such as PAX3_FOXO 1 and PAX7_FOXO1 that are found in alveolar RMS [The histologic differential diagnosis of uterine RMS is endometrial stromal sarcoma, leiomyosarcoma, adenosarcoma/carcinosarcoma. As we mentioned earlier, diagnosis of pure RMS in uterus involves precise histopathological evaluation. Widespread and perfect sampling is necessary to uncover any epithelial component. IHC test can help us to evaluate any suspected areas . IHC staolar RMS . IHC staet al. studied 15 women with gynecologic RMS and showed that the most prevalent site of the gynecologic RMS is uterine cervix, followed by uterine corpus. In this study, the 5-year disease-specific survival (DSS) was 29%, and older age, widespread disease at the time of diagnosis, and unfavorable histologic variants such as pleomorphic and alveolar led to poor outcomes in adults [Ferguson n adults .et al. studied 15 patients with primary cervical RMS . Vaginal bleeding and pelvic mass were the most common symptoms, and Intergroup Rhabdomyosarcoma Study Group (IRS) clinical staging was the best predictor of prognosis [Enzo Ricciardi rognosis . This caThere is limited experience with RMS treatment in adults, and a combined modality is considered based on IRS group studies in younger patients . Completet al. evaluated 137 patients with nonmetastatic gynecologic RMS, in a systematic review. Surgery was the main approach for local control of tumor in all patients [Maha et al. concluded that RMS responds to chemotherapy in adults exactly as it does in children, and there is no reason to select different approaches [In a retrospective analysis of 171 patients (older than 18 years of age), Ferrari proaches .et al. reported 95 adult patients with RMS in a prospective single-center case series. The treatment recommendation was a multidisciplinary approach that included surgery, chemotherapy, and radiotherapy. Chemotherapy was recommended for all patients. It consisted of a multidrug treatment, alternating the ifosfamide, vincristine, and actinomycin-D (IVA) regimen with ifosfamide, vincristine, and adriamycin (IVAd) or ifosfamide, vincristine, and etoposide (IVE). Delayed surgery was performed after three to four courses of chemotherapy. Radiotherapy  was suggested in all cases of alveolar, not otherwise specified (NOS) RMS, and embryonal RMSs that were incompletely resected at diagnosis. In this study, the 5-year event-free and overall survival rates were 33.6% and 40.3%, respectively [Luca Bergamaschi ectively We used neoadjuvant chemotherapy, including VAC regimen, for the patient, and she received external pelvic irradiation with a total dose of 5040 cGy within 6 weeks after surgery.RMS of uterine corpus is rare in adults. It is an aggressive tumor with a poor outcome, despite multidisciplinary approach. Histopathological criteria, IHC staining, and molecular studies differentiate RMS from the other uterine sarcomas. More studies are needed on treatment options in adults.


Teaching point: Granular cell tumors are rare soft tissue tumors that may occur in the breast. While almost always benign, they may mimic a malignant tumor both clinically and on imaging. Figure 1, arrow). Ultrasound demonstrated an irregular delineated hypoechoic lesion. There was a subtle partial hyperechogenic halo  and marked posterior acoustic shadowing . No pathological axillary lymph nodes were found. On magnetic resonance imaging (MRI), the lesion had spicular margins and avid, homogenous contrast enhancement on T1-weighted images (WI) . Histopathology confirmed the diagnosis of a granular cell tumor of the breast.Routine breast screening examination in an asymptomatic 61-year-old female patient revealed a suspicious lesion in the axillary tail of the right breast. There was no history of breast cancer. Mammography showed a dense, spiculated mass at the upper outer quadrant of right left breast (Granular cell tumors (GrCT) are rare tumors of neural origin with Schwannian differentiation. Typically, a GrCT has abundant granular eosinophilic cytoplasm on microscopy, from which the tumor derives its name. A GrCT can occur in all soft tissues and has a prevalence of 6.7:1000 in the population undergoing evaluation for breast cancer. While mostly benign, malignancy has been reported in 0.5%–2.0% of cases.Clinically, a GrCT is a solitary and painless mass that may be identified on palpation. On mammography, a GrCT can present as a benign-looking, well-defined nodule or show features suggestive of malignancy such as irregular margins, spiculation, and architectural distortion. Calcifications are generally absent.Similar to mammography, on ultrasound a GrCT can appear as a well-circumscribed solid nodule or as an ill-defined heterogenic mass with variable vascularization. Sometimes a hyperechogenic halo may be seen, and posterior acoustic shadowing may be present depending upon the degree of reactive fibrosis. The most specific feature is the presence of anisotropy. A degree of variable echogenicity, depending on the angle of the insonating beam, may be seen due to the internal fibrillary composition. On MRI, a GrCT is of low to intermediate signal intensity on T1-WI but is often inconspicuous on T2-WI. Enhancement is variable after administration of gadolinium contrast. Both progressive (type 1) and wash-out (type 3) dynamic curves have been described. Additionally, a GrCT does not have increased metabolic activity on PET-CT; this feature can be helpful to differentiate a GrCT from malignancy.Due to the non-specific imaging findings, tissue analysis is required for definite diagnosis.While the prognosis of a GrCT is generally good, metastatic disease has been described. The recommended treatment is wide surgical excision considering the higher risk of recurrence with positive resection margins .


Gastric masses can be challenging to diagnose pre-operatively due to their heterogeneity in presentation and work-up. One must be cautious that a seemingly benign mass may be malignant and vice versa. Some of the more common gastric masses include peptic ulcer, adenocarcinoma, and gastrointestinal stromal tumour. These diagnoses have vastly different management strategies despite similar presentations. The case presented here is an example of this management, highlighting a patient with a gastric bleeding mass initially thought to be a gastrointestinal stromal tumour. However, on final pathology, the mass was determined to be benign, an ulcerated hematoma. Gastric masses can be difficult to accurately diagnose due to a widespread differential with similar radiologic and endoscopic characteristics ranging from benign to malignant conditions. The most common symptom experienced by patients with gastric masses is gastrointestinal bleeding; however, masses are more commonly asymptomatic. If a mass is identified on initial evaluation for the bleed with an esophagogastroduodenoscopy (EGD), further investigation with computed tomography (CT) scan and surgical intervention may be warranted.Some examples of common diagnoses that cause gastric masses include peptic ulcer disease (PUD), gastric adenocarcinoma, and gastrointestinal stromal tumour (GIST). Peptic ulcer disease is the cause of the majority of upper gastrointestinal bleeding in the United States. The bacteria Helicobacter pylori, non-steroidal anti-inflammatory medications (NSAIDs), and Aspirin are identified as the cause in 80% of cases . H. pyloThe case to follow is an example of such a challenge. In brief, our patient presented with an upper gastrointestinal bleed, underwent endoscopy and imaging, which suggested GIST as the diagnosis and underwent appropriate surgical treatment only to discover that the mass was a large intramural hematoma on the final pathology report.Patient PresentationThe patient is a 43-year-old male with end-stage renal disease who presented to our community hospital with hematemesis. He underwent an EGD with our gastroenterology colleagues, which found an approximately 5cm proximal posterior gastric wall mass with ulceration and central clot Figure . There wEvaluationDue to the large size of the identified mass, the patient was at increased risk for severe recurrent upper gastrointestinal bleeding and malignancy. This prompted a relatively short timeline from diagnosis to surgical intervention. A computed tomography (CT) scan of the chest without intravenous (IV) contrast; and a CT scan of the abdomen and pelvis with oral and IV contrast was obtained to assess for potential metastases Figure . The abdDespite the high likelihood that a gastric ulcer is benign, there is a 5-11% chance that the ulcer is malignant. Both benign and malignant ulcerations have associations with H. pylori. Having a benign biopsy without H. pylori present decreased the chance of this mass being a peptic ulcer or adenocarcinoma ,6. In gaThe gastrointestinal stromal tumour was considered the most likely diagnosis based on the pre-operative endoscopic and CT imaging findings of a single ulcerated submucosal mass with heterogeneous contrast enhancement. A pre-operative submucosal tissue sample via either FNA or core needle was considered low-yield and high risk. Our team, therefore, recommended surgical resection of the mass to diagnose and treat this patient definitively. SurgeryThe pre-operative plan for this patient was to attempt a laparoscopic wedge resection, as the mass was expected to be non-invasive and along the more significant curve of the stomach with plenty of gastric wall remaining. However, we were prepared to change course if our pre-operative assessment was discordant with our direct intra-operative evaluation.During initial laparoscopic visualization, the gastric mass was adherent to both the splenic hilum and the distal tail of the pancreas Figure . This raPathologyFollowing review by multiple pathologists, the final pathology report described the mass as hemorrhagic ulceration Figure . No maliThe case presented here provides an example of the challenges associated with diagnosing a gastric mass in the pre-operative setting. More common diagnoses such as peptic ulcer disease, adenocarcinoma, and GIST have overlapping endoscopic and radiologic findings with rare diseases. Sometimes an ulcerated submucosal mass is not a GIST even when macroscopic appearance seems convincing. Other submucosal tumours can include those originating from any tissue present: lymphatic, muscular, nervous, mesenchymal, or vascular structures .Symptomatic intra-mural gastric hematomas are exceedingly rare, with fewer than 40 other cases available in the literature and fewer than 15 treated with surgery ,9. BecauGastric masses can be challenging to treat because of their diagnostic challenges and may have unexpected intra-operative findings. Deciding which treatment is best for your patient at the time of presentation depends on a thorough evaluation while recognizing that even our current best technology is imperfect. Surgical intervention can still be warranted before a final pathologic diagnosis to palliate a patient’s symptoms. The treating surgeon should be prepared to alter the operative treatment relative to intra-operative findings.


Subgaleal lipoma is a benign tumor of adipose tissue. It should be suspected when a semi-spherical avascular mass with well-defined margins, iso- or hyperechoic in most cases, with thin internal echogenic lines parallel to the long axis of the tumor, is observed between the galea aponeurosis and periosteum of the cranial bone. We report a series of cases of three patients who underwent surgical lesion excision and whose histopathological examination findings confirmed the diagnosis of lipoma.The presence of long continuous echogenic lines within a lens-shaped soft tissue mass located beneath the galea aponeurosis may suggest the diagnosis of subgaleal lipoma. Scalp masses are commonly encountered in clinical practice. Ultrasound is usually the first imaging technique used to investigate soft tissue masses of the scalp due to its accessibility and affordability. Lipoma of the scalp can be easily diagnosed on ultrasound because of its semi-spherical shape and the presence of thin internal echogenic lines parallel to the long axis of the tumor . Subgale2We report the imaging findings of subgaleal lipomas in three patients, reviewing the imaging characteristics of these lesions.Table 1. All patients underwent surgical lesion excision, and histopathological examination confirmed the diagnosis of lipoma. The echogenicity of the lesions, compared to that of the adjacent fat on ultrasound, varied from one patient to the other. However, all lesions showed thin internal echogenic lines, and none showed calcifications or internal Doppler flow.Our three patients were aged between 39 and 64 years at the time of diagnosis. The localization, size, and echogenicity of the lesions are presented in Figure 1. The computed tomography (CT) appearance of patient 1 is presented in Figure 2. The magnetic resonance imaging (MRI) of patient 2 is shown in Figure 3.The ultrasonographic appearance of these subgaleal lipomas is shown in Subgaleal lipoma is a heterotopic tumor of adipose tissue typically occurring between the periosteum and the galea aponeurosis  of the scalp . Most of1Ultrasound is usually the first imaging technique used for soft tissue masses owing to its accessibility and affordability. On ultrasound, the subgaleal lipoma appears as a solid mass, semi-spherical or lens-shaped, with a flat or slightly concave base towards the periosteum of the cranial bone, and a convex surface towards the subcutaneous fat, parallel to the skin surface . Most of1Besides subgaleal lipoma, the differential diagnosis of scalp lumps also includes epidermoid cysts, dermoid cysts, trichilemmal cysts, intraosseous hemangiomas and other vascular malformations, and malignant lesions such as lymphoma, carcinoma, and metastasis .CT scan is typically used to examine the bone alterations . On unen2MRI can be used to evaluate the extent of the lesion . SubgaleOn ultrasound, the diagnosis of subgaleal lipoma should be suspected in the presence of a lens-shaped or semi-spherical soft tissue mass located between the galea aponeurosis and periosteum of the cranial bone that is iso- or hyperechoic, with thin continuous echogenic intralesional lines parallel to the long axis of the lesion.


Diagnosis of anomalous intrathoracic lesions may be challenging and require a multidisciplinary approach. We present a case of granulomatosis with polyangiitis (GPA) clinically and radiologically mimicking metastatic lung cancer with a bilateral pulmonary mass, mediastinal and cervical lymph node involvement, and pleural effusion. Surgical biopsy of the thoracic lesion revealed necrotic granulomatous inflammation, and the final diagnosis was subsequently confirmed by kidney biopsy and biochemical parameters. This case illustrates how comprehensive diagnosis secures timely and relevant treatment. Systemic vasculitis may be one of the key differential diagnoses in patients with multiorgan involvement, especially with pattern-mimicking lung cancer. Differential diagnosis of abnormal thoracic lesions constitutes an important part of routine clinical work-up and can include a wide spectrum of diseases. As soon as standard diagnostic procedures fail to recognize a patient’s diagnosis, a collaborative approach is required to secure timely and relevant treatment. Granulomatous diseases can easily mimic a malignant phenotype as in the current case of granulomatosis with polyangiitis . GPA, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are defined as small-vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) . ANCA-as18F) positron emission tomography–computed tomography (FDG-PET/CT) revealed an 8-cm FDG-avid tumor atelectasis complex suggestive of malignancy in the left upper lobe with mediastinal involvement. The FDG-PET/CT scan also revealed FDG-avid enlarged lymph nodes on the left side of the neck, in the mediastinum at positions 4 L and 7 and at both hila, as well as bilateral pulmonary nodules suggestive of metastases, pleural metastasis in the middle right lung’s lobe, left-sided pleural effusion, a small FDG-avid lesion in both parotid glands and a 3-cm FDG-avid lesion in the prostate  and hematuria together with elevated serum creatinine (173 μmol/L). Prostate-specific antigen (PSA), calcitonin, anti-neutrophil cytoplasmic antibodies (ANCA), myeloperoxidase (MPO)-ANCA, anti-glomerular basal membrane (GBM), and immunoglobulins were all normal, but C-reactive protein (CRP) (70 mg/L), proteinase 3 (PR3)-ANCA (44 kU/L), urine albumin creatinine ratio (UACR) (2730 U), blood pressure (172/98 mmHg) were elevated. Serum albumin was low, 21 g/L. Standard additional diagnostic procedures including bronchoscopy with endobronchial ultrasound (EBUS), bronchoalveolar lavage (BAL) and cytological examination of pleural effusion did not show any signs of malignancy. Therefore, to finally confirm or refute cancer, video-assisted thoracoscopic surgery (VATS) with biopsy from the pulmonal tumor in the right middle lung’s lobe was performed , and fouHistological examination of the formalin-fixed and paraffin-embedded tissue from the right lung’s middle lobe revealed a nodular lesion consisting of a consolidated alveolar parenchyma with necrosis, granulomatous inflammation, multinucleated giant cells, often with smudged basophilic nuclei marginated at the periphery of the cell, and cholesterol clefts . The necGPA and microscopic polyangiitis (MPA) confine the major subgroups of AAV, which represents a complex autoimmune disease of multifactorial etiology that can affect all organ systems with preferential involvement of the skin, respiratory tract, and kidneys. Pulmonary involvement in AAV is a frequent finding and with varying radiographic presentations, i.e., multiple lung noduli of varying size, solid and often cavitary lesions or diffuse ground-glass opacities and fibrosis ,15. The Differential diagnosis in AAV patients includes malignancies , infectious diseases, e.g., pneumonia and tuberculosis, and autoimmune diseases, such as systemic lupus erythematosus (SLE) and sarcoidosis ,20. AAV Multidisciplinary and cooperative approaches remain paramount in the assessment of abnormal thoracic lesions, including pertinent planning and timing of subsequent diagnostic steps, to facilitate relevant treatment without unnecessary delays. In this case, standard FDG-PET/CT imaging initially gave rise to the suspicion of lung cancer. However, subsequent VATS and kidney biopsy in combination with positive PR3-ANCA refuted malignancy and confirmed the diagnosis of GPA.


Biliary atresia (BA) is a rare disorder that usually presents with cholestatic symptoms such as jaundice, pale stool, and dark urine shortly after birth. Intracranial hemorrhage is a rare manifestation. BA may sometimes be associated with a cyst in the biliary tree. Differentiating choledochal cyst with BA or cystic biliary atresia can be quite challenging yet very important as they differ significantly in their prognosis and treatment. Furthermore, clear mucoid fluid in the extrahepatic cyst is an unusual occurrence. This case report aims to raise awareness about this rare variant to facilitate appropriate and timely diagnosis and treatment.We report a case of an infant who presented with intracranial hemorrhage and conjugated hyperbilirubinemia. The ultrasonographic and magnetic resonance cholangiopancreatography (MRCP) findings suggested Type I choledochal cyst, but it was later diagnosed as biliary atresia with an extrahepatic cyst and was not corrected by Kasai’s operation. Biliary atresia (BA) is a rare disorder with marked variation in incidence ranging from one in 17,000 to 19,000 live births in the United Kingdom and France and most common in East Asian countries, about one in 5000 in Taiwan. According to the level of most proximal biliary obstruction, it is classified into Type I, II, and III. Type I constitutes 5% of cases with luminal patency up to the common bile duct and proximal part of the cystic duct. Type II constitutes 2% of cases with patency up to the common hepatic duct, and more than 90% of cases are Type III in which the most proximal part of the extrahepatic biliary tract (within the porta hepatis) is entirely solidified [BA mainly presents with conjugated hyperbilirubinemia and persistent alcoholic stool. Hemorrhage may occur, but intracranial hemorrhage as presenting symptoms is rare . When BAWe present a 30-day-old exclusively breastfed female baby who was brought to our outpatient department with complaints of scanty reddish spots over bilateral upper limbs, excessive cry, fever, yellowish discoloration of eyes, clay-colored stool, along with one episode of seizure. The baby was born to a 30-year-old primigravida mother via spontaneous vaginal delivery at term with an uneventful antenatal and perinatal period. On presentation, the baby was febrile, icteric with scanty purpuric and ecchymotic rashes over bilateral upper limbs. No obvious findings were present on systemic examination.Laboratory examination  of brain and ventricles was normal; however, USG abdomen showed a cystic structure measuring 13 mm in diameter anterior to the portal vein separate from the gallbladder and was continuous with the common bile duct, findings suggestive of choledochal cyst Type I. The liver function tests and coagulation parameters were deteriorating progressively (Table Plain magnetic resonance cholangiopancreatography (MRCP) was done, which showed approximately 14 mm × 13 mm-sized cystic lesion in hepatic hilar region in the location of common hepatic duct communicating with the intrahepatic bile ducts, likely Type I choledochal cyst  biopsy section showed biliary canaliculi with ill-defined mucosal lining, extensive fibrocollagen tissue in the stroma, hemorrhagic areas and focal collection of round cells, consistent with BA; gallbladder and common hepatic duct biopsy section showed ill-defined mucosa and extensive hemorrhagic areas.The child’s symptoms and laboratory parameters improved significantly on subsequent follow-up visits, but one month after the operation, the child started developing pale stools again. Details of the laboratory reports on follow-up visits are given in Table Typically, BA presents with jaundice, pale stools, and dark urine shortly after birth . IntracrAbdominal ultrasound is the first-line imaging modality in neonatal cholestasis. The triangular cord sign and gallbladder abnormalities in ultrasonography (USG) have a specificity of 97% and 92%, respectively, for BA diagnosis . HoweverLiver biopsy plays a crucial role in the diagnostic work-up of infants with neonatal cholestasis, and histopathological examination of portal tracts holds the key findings in diagnosing BA. Duct/ductal bile plugs, fibroblasts proliferation, and a variable amount of inflammatory cells, especially neutrophils, are usually present . In 5%-1In our case, BA Type III with non-communicating extrahepatic cyst containing mucoid fluid was present. Two similar cases of BA associated with true cystic structure at the common hepatic or common bile duct were reported by Sookpotarom and Vejchapipat, and Kasai's operation was successful in treating the conditions. The author also reports that awareness of this type of biliary atresia may facilitate the surgeon’s decision during the dissection of the hepatoduodenal ligament .Obaidah et al. reported a case with a pre-operative magnetic resonance imaging (MRI) diagnosis of Type I CC with a blind-ending distal common bile duct . The galThus, pediatricians, surgeons, radiologists, and pathologists should be aware of this rare variant to ensure that the condition is not missed and appropriate treatment is given.Clear mucoid fluid in the extrahepatic cyst associated with BA is an unusual finding. Differentiating CC with BA or CBA can be challenging clinically, radiologically, and histopathologically. Considering the differences in treatment and prognosis, we should be aware of this rare occurrence and the differential diagnoses.


Congenital patellar dislocation (CPD) is a rare deformity in children that involves a laterally displaced patella. While potentially identified in early childhood using diagnostic imaging techniques, it is often misdiagnosed at birth, creating pain and mobility issues as the child grows. Dislocation of the patella is permanent and manually irreducible, often manifesting with flexion contracture of the knee, genu valgum, external tibial torsion, and foot deformity. Surgical correction is the treatment of choice in order to prevent future sequelae. We herein present a case of CPD in a four-year-old Asian male who was initially misdiagnosed before undergoing successful surgical repair using the Roux-Goldthwait technique. Congenital patellar dislocation (CPD) is a highly rare condition, presenting as a manually irreducible lateral patellar bone dislocation with a flexion contracture and valgus tibial deviation . It can In this paper, we present an individual case of a young male presenting with CPD after many misdiagnoses. He was eventually treated successfully via using the Roux-Goldthwait surgical technique, which is a very common and efficient surgical method with minimal postoperative complications. Based on a report of prior surgical treatments, all cases showed no signs of recurrence and significant effectiveness in improving knee function . Thus, wWe present a four-year-old Vietnamese male with Down syndrome for chronic pain and inflammation of the left knee for one year. Per the patient's medical record, he was previously diagnosed with juvenile arthritis at multiple hospitals and clinics, for which he had received several pharmacotherapies without symptom relief. Medications included acetaminophen, ibuprofen, corticosteroid, and methotrexate. As the result of persistent, non-resolving pain in the left knee, the patient was then referred to Xuyen A Hospital for further investigation. Physical examination revealed epiphyseal dysplasia, funnel chest, contracture elbow joint, and diastrophic dysplasia. The left knee displayed genu valgum and flexion contracture. Besides the chronic pain, the patient reported no limit in the left knee’s range of motion. X-ray of both knees was performed, yielding the diagnosis of CPD Figure .After an adequate risk-to-benefit assessment, a surgical intervention appeared to be most appropriate, and the Roux-Goldthwait technique was employed. In this distal realignment procedure, the patellar tendon was split vertically. The lateral half of the patellar tendon was then moved inferomedially to the medial half to form a “Roux” pattern. Finally, the lateral half was attached to the tibia. This pulls the dislocated patella over to the center, thus simultaneously preventing excess lateral shift. This technique is similar to other well-known patellofemoral procedures, such as Fulkerson, Maquet, and Trillat, without disrupting open physes in immature muscle-skeletal structures . In addiAt three months follow-up, the incision appeared to heal well without any infection or rupture. A hypertrophic scar was formed along the incision line, which is a normal reaction of damaged skin from surgery cuts Figure . The patCongenital dislocation of the patella, also known as fixed lateral dislocation of the patella, is a very rare deformity. The condition presents at birth with lateral dislocation of the patella that cannot be reduced manually. CPD can have multiple clinical presentations such as habitual dislocation of the patella or juvenile arthritis. CPD typically presents with a flexion contracture at the knee, genu valgum, foot deformity, and external tibial torsion. Although this disorder can be diagnosed at birth, it tends to be misdiagnosed due to low suspicion of the disorder, lack of patellar visibility on plain radiographs, and difficulty in palpating the hypoplastic patella in the lateral position . CPD is When presenting at birth, the dislocation is often misdiagnosed as a neuromuscular disease or muscular dystrophy. It is unknown what the true statistics of CPD are since the confirmed cases are sporadic. Approximately, the incidence is estimated as 1/100,000, with a higher prevalence in girls than boys . HistoriThis case report presents a Vietnamese male with Down syndrome, who was originally misdiagnosed with juvenile arthritis at one year old. The Roux-Goldthwait technique was implemented for correcting the patella’s location. The benefit of this technique is that it allows the surgeon to adjust the position of the patella according to each presented case in order to improve tracking. This technique is similar to other well-known patellofemoral procedures, such as Fulkerson, Maquet, and Trillat, without disrupting the open physes in immature muscle-skeletal structures .In addition, lateral release, V-Y lengthening of the quadriceps, and posterior release of the knee were also performed on the patient during the surgery to relieve any pain and discomfort that may result from the surgery. During the lateral release procedure, a group of lateral tendons is cut in order for the kneecap to move into the proper groove. This helps relieve the patient of pain and pressure from any tightening in the lateral retinaculum tissue on the outside of the kneecap . The V-YAs with most osteopathic diseases, early diagnosis and intervention are critical. The most common treatment is surgery. Though surgery is the ultimate proper treatment for CPD, other non-invasive treatments in the early stages of the disease include casts, braces, and physiotherapy in order to improve flexural contracture . HoweverIn such a rare condition as CPD, diagnosis can be delayed due to low suspicion and invisible patella on X-rays early in life. Flexion contracture of the knee, genu valgum, external tibial torsion, and foot deformity in pediatric patients should raise the clinician’s suspicion of CPD. Once the diagnosis is confirmed, an early surgical operation is an effective treatment for relieving pain and allowing children to continue development. Surgical treatment before one year of age allows for the reconstruction of almost normal knee function and provides the patient with a normal gait without delay at the onset of walking. Neglected cases require more extensive excisions of contractures to ensure reduction of the patellar dislocation and more complicated surgical reconstructions to ensure patellar stability. Early detection will improve the prognosis of the disorder and enhance patients’ quality of life. In this case report, the patient recovered successfully from CPD and benefited from combined surgical techniques and postoperative physical therapy after suffering from multiple misdiagnoses for multiple years.


Sequestered disc fragments do not have indistinctive features and often share the clinical and radiological presentation as spinal neoplasms making their diagnosis and treatment a clinical challenge.We report a rare case of sequestered lumbar disc fragment at the level of L2-L3 in a 70-year-old male who presented to the ER complaining of six years' history of low back pain with acute onset lower extremities weakness for six days, associated with right foot drop. He was admitted for tumor workup as the MRI showed diffuse bone high signal intensity throughout the spine with a soft tissue epidural mass at L2/3, causing severe compression on the cauda equina nerve roots. The patient underwent L2-L3 decompression and fixation, mass excision, multiple open biopsies. Soft tissue biopsy of the mass revealed fibrocartilaginous tissue consistent with the intervertebral disc, while the bone biopsy was diagnostic of acute leukemia. The patient was observed postoperatively with unremarkable complications. He did well with physiotherapy, and there was a remarkable improvement of his right lower extremity power reaching 4/5.Our case presented a rare phenomenon in which sequestered disc fragments manifested clinically and radiologically as a spinal neoplasm. Vigilant history taking and physical examination are paramount; a physician should be watchful for any red flags that may warrant further investigation such as in our case. Migration of herniated disc fragments is considered extremely rare given the contained anatomical barriers. These sequestered disc fragments do not have indistinctive features and often share the clinical and radiological presentation as spinal neoplasms making their diagnosis and treatment a clinical challenge ,2. In thThis is a 70-year-old male, known to have hypertension, diabetes, benign prostatic hyperplasia, myocardial infarction on dual antiplatelets, presented to the ER complaining of six years' history of low back pain with acute onset lower extremities weakness for six days, associated with a new right foot drop. No reported history of traumas, or infections, no change in bowel and bladder habits. His examination revealed right-sided L2, L3, L4 power of 2/5, L5, S1 of 3/5. Sensation and reflexes were normal bilaterally. He was admitted for tumor workup as the MRI showed diffuse bone high signal intensity throughout the spine with a soft tissue epidural mass at L2/3 causing severe compression on the cauda equina nerve roots Figures -1e. The A wide variety of non-neoplastic spinal diseases including sequestered intervertebral disc herniation may present as mass lesions making preoperative diagnosis and treatment a challenge for many surgeons. One of the early cases of sequestrated disc fragments was reported as protruded annulus fibrosus rotated posteriorly into the spinal canal . The patWe performed a PubMed and Google Scholar search of all reported studies of sequestrated lumbar disc herniation published to date. The following keywords were searched: Sequestered disc herniation, lumbar disc fragment, and spinal neoplasm. We identified 38 cases of lumbar disc fragments from 12 studies (Table Contrast injection is used to differentiate between avascular disc fragments from other spinal neoplasms with the presence of rim-like peripheral enhancement. This finding is suggestive more of an inflammatory and non-neoplastic process . Other cOur case presented a rare phenomenon in which sequestered disc fragments manifested clinically and radiologically as a spinal neoplasm. Vigilant history taking and physical examination are paramount; a physician should be watchful for any red flags that may warrant further investigation such as in our case. Sequestrated disc fragments should be included in the differential diagnosis of patients presenting with low back pain, radiculopathy, or cauda equina syndrome.


Subglottic paragangliomas (PGs) are exceptionally rare and unpredictable, occasionally presenting at an atypical location. There are three different clinical forms of subglottic PGs: intraluminal , extraluminal (thyroid PGs) and the mixed type . These tumors are usually misdiagnosed as other relatively common primary thyroid or laryngotracheal tumors, and the treatment is troublesome.A 22-year-old male patient with subglottic PGs has been successively misdiagnosed as thyroid tumors and subglottic hemangiomas, and lastly underwent local extended lumpectomy and laryngotracheal reconstruction with a pedicled thoracoacromial artery perforator flap (PTAPF). The patient was decannulated successfully after the second-stage tracheal reconstruction with a local flap, and no evidence of local recurrence and distant metastasis of the tumor until now.Subglottic PGs can be easily misdiagnosed as laryngotracheal or thyroid tumors when presented at an atypical location. It is essential for otolaryngologists and head and neck surgeons to remain vigilant against these tumors. If the tumor is not diagnosed or removed completely, patients may encounter a risk of lethal paroxysm, which is incredibly troublesome. Paragangliomas (PGs) are slow-growing, well vascularized and rare-neuroendocrine neoplasms that arise from the paraganglion system. The paraganglia are tissues with common embryological origin that widely distributed throughout the body . PGs havLaryngeal PGs, originating in the superior or inferior laryngeal paraganglion, may be either supraglottic, glottic, or subglottic , 4. MoreA 22-year-old man presented with a history of recurrent hemoptysis of small volume for a duration of two years. He also experienced progressive dyspnea for three months. The patient had no other complaints, such as hoarseness, dysphagia, or any other symptoms. Following the development of stridor in April 2017, the patient underwent a tracheostomy in the emergency department at West China Hospital, with dyspnea relieved markedly. The physical examination didn’t reveal any special issues except for a scar on the neck. A review of the patient’s medical history revealed that 7 years prior, he had undergone left thyroid lobectomy without the neck dissection for a palpable 3-cm left thyroid mass in a local hospital. Moreover, the post-operative histopathology was unknown.He was then hospitalized at the respiratory department after tracheostomy. Flexible fiberoptic bronchoscope revealed a smooth and sessile vascularized mass, located 0.5–1.5 cm below the glottis, causing lumen narrowing by approximately 80%  .Therefore, an accurate diagnosis needs to combine light microscopy and immunohistochemical staining Table . The chiManagement option for PGs is accomplished by surgery, radiotherapy, or observation . The choAn accurate survival rate for subglottic PGs is hard to obtain due to their rarity and diagnostic uncertainty. Therefore, long-term clinical follow-up is required to check for recurrence and distant metastases. Recurrence occurs mainly in the mixed type of subglottic PGs and cases with endoscopic surgery . Until nIn conclusion, subglottic PGs are rare, and most otolaryngologists and head and neck surgeons may never encounter them. Patients with subglottic PGs may show up at various medical and surgical specialists in different departments or hospitals. Therefore, it is crucial for otolaryngologists and head and neck surgeons to remain vigilant against these tumors. Presently, surgery remains the mainstay of treatment and offers the best results to these patients. If the tumor is not diagnosed or resected completely, patients may encounter a risk of lethal paroxysm, which is incredibly troublesome.