Gastric schwannomas are uncommon among a broad range of possible diagnoses in the work up of a gastric mass. Regional lymphadenopathy associated with gastric schwannoma is an even less common occurrence and one would otherwise suspect a malignant neoplasm.We present two non-consecutive cases from a signle academic center depicting Caucasian females in their 5th and 6th decades of life with gastric schwannoma and adjacent lymphadenopathy. Multiple lymph node excisions were performed without evidence of neoplasia.Lymphadanopathy in the presence of a gastric mass typically represents malignant neoplasm. A less than likely presentation of gastric schwannoma with reactive regional lymph nodes poses a challenge to adequate preoperative diagnosis and increases risk for a more aggressive than necessary surgical approach with lymphadenectomy.While the correlation between gastric schwannoma and lymphadenopathy is uncertain, this ought to be considered. If diagnosis can be confirmed preoperatively, omission of lymphadenectomy is appropriate. It is t22.1A 66 year old Caucasian female was referred to our institute for an incidental 5 cm gastric antral mass identified on an elective laparoscopic sleeve gastrectomy for weight management; elective surgery was aborted. Intraoperative findings were also remarkable for multiple diminutive white nodules on the surface of the stomach, small intestine, and peritoneal wall of the left upper quadrant. Biopsies returned mesothelial hyperplasia. She shared complaints of intermittent but chronic nausea and constipation. Physical exam was unremarkable. Prior surgical history consisted of removal of an ovarian cyst. Medical, social, and family histories were noncontributory. Esophagogastroduodenoscopy (EGD) identified a subepithelial bulge within the gastric antrum along the greater curvature of the stomach 5 cm proximal to the pylorus. On endoscopic ultrasound (EUS), the mass appeared heterogeneous arising from the muscularis propria layer of the stomach. Fine needle aspiration (FNA) revealed atypical spindle cells in a hypocellular specimen most consistent with a spindle cell lesion. Immunostains CD117, C-KIT, CD 34, smooth muscle actin, and S100-DAB were non-contributory due to scant cellularity. Of note, there were two hypoechoic perigastric lymph nodes at the level of the gastric antrum 14 mm and 6 mm in size. EUS guided trans-gastric FNA of the dominant lymph node was negative for malignancy. Contrast enhanced computed tomography (CT) scan of the chest, abdomen, and pelvis demonstrated an exophytic gastric body mass with adjacent mild mesenteric adenopathy, sub- and retro pyloric and gastroduodenal distributions a. SubseqWith a working diagnosis of gastrointestinal stromal tumor (GIST), she was offered partial gastrectomy for definitive diagnosis and therapeutic intent in conjunction with sleeve gastrectomy by the patient’s outside surgeon considering that it was feasible and per her initial wishes. Intraoperatively, thickened and chronically inflamed lymph nodes were appreciated along the lateral aspect of the mass. Multiple lymph node specimens were sent for frozen section and returned negative. Grossly, the well-circumscribed firm gastric subserosal nodule measured 8.2 × 6.8 × 4.7 cm with unremarkable appearing overlying mucosa. Cut surface exhibited a white-tan appearance . Microsc2.259 year old Caucasian female presented with melena and acute anemia. Initial EGD revealed a 3 cm mass and overlying erosions at the junction of the gastric antrum and body along the greater curve. FNA was nondiagnostic. She received a blood transfusion and stabilized on oral iron supplements. She was otherwise asymptomatic with normal physical findings. Medical, surgical, family and social histories were noncontributory. A repeat EGD with EUS was performed which described a round subepithelial antral mass. Sonographically, a 35 × 33 mm hypoechoic and irregular lesion appeared to originate from the muscularis propria. Note, two minimally enlarged perigastric lymph nodes were visualized; the largest measured 7 × 5 mm. FNA of the gastric mass was negative for carcinoma with presence of lymphoid tissue. IHC stains were negative for neuroendocrine tumor, GIST, and leiomyoma. Subsequent contrast enhanced CT of the abdomen and pelvis revealed a homogeneously enhancing rounded predominantly intraluminal mass involving the anterior gastric wall measuring 3.6 × 4.1 cm. Several perigastric lymph nodes were noted measuring up to 1.1 cm a and b. 3Many different types of tumors can be found in the stomach such as epithelial tumors, lymphomas and a wide range of mesenchymal tumors. Gastric mesenchymal tumors can be divided among four categories: the true smooth muscle tumors , neurogenic tumors , fibroblastic tumors , and gastrointestinal stromal tumors . GISTs aGastric Schwannomas are neurogenic tumors and are thought to arise from the sheath of Auerbach’s plexus or the sheath of Meissner’s plexus ,2. The iGastric schwannomas are generally benign and have excellent prognosis after resection . A diagnHowever, evident in the reports above and limited data available in the literature, adenopathy is invariably reactive in this setting. The correlation between gastric schwannoma and enlarged lymph nodes remains unclear. Hou Y et al. report on gastrointestinal schwannomas shows that 97% of these tumors present with peripheral cuff like lymphoid aggregates. Lymphocytes are aggregated around the periphery of the tumor and form the characteristic feature of a lymphoid cuff which is not seen in soft tissue and central nervous system schwannomas. The reason for this infiltration is not clear. Most speculate that the lymphoid cuff might result from cytokines secreted by the tumor cells which induce chemokinesis of lymphocytes ,12.Since this is reactive adenopathy, an aggressive surgical approach with regional lymphadenectomy is not warranted. A thorough preoperative workup including an endoscopic ultrasound guided fine needle aspiration is critical to confirm the diagnosis and a wedge resection of stomach, if feasible, should suffice as a definitive treatment. In the literature there are some reports of formal gastrectomy and regional lymphadenectomy which seems like an aggressive management option for a benign tumor with no case reports in literature showing tumor involvement of lymph nodes ,14. If a4In conclusion, gastric schwannoma should be considered in the differential diagnosis of gastric masses presenting with regional lymphadenopathy. EUS-FNA can establish the diagnosis in most cases and if confirmed, a less aggressive surgical approach including omission of lymphadenectomy serves curative in these patients.None.None.The study is exempt from ethical approval in our institution.Written informed consent was obtained from the patients for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Arnav Wadhawan: Resources, acquisition of data, writing - original draft, final approval of the version to be submitted. Maureen Brady: Resources, acquisition of data, writing - original draft, writing - review and editing, final approval of the version to be submitted. Charles LeVea: Acquisition of data, analysis and interpretation of data, final approval of the version to be submitted. Steven Hochwald: Acquisition of data, analysis and interpretation of data, final approval of the version to be submitted. Moshim Kukar: Project administration, acquisition of data supervision, conceptualization, analysis and interpretation of data, writing original draft, final approval of the version to be submitted.This was not required in previous submissions.Moshim Kukar.Editorially reviewed, not externally peer-reviewed.


Mediastinal venous aneurysm is a very rare disease and can be easily misdiagnosed. Patients are often asymptomatic while venous aneurysm of large size with adjacent structures oppressed can lead to discomfort. The surgical treatment for aneurysm of large vessels is often complex and challenging.We reported a 52-year-old man with mediastinal mass who received operation on July 2019 in our hospital. Left innominate vein aneurysm was diagnosed during operation with superior vena cava involved. The aneurysm was resected and pericardium was taken to repair part wall of superior vena cava and reconstruct left innominate vein. The patient’s postoperative course was uneventful.Venous aneurysm should be considered when mediastinal mass has no clear boundary with large veins or even seems to connect with them. Magnetic resonance imaging, computed tomographic angiography and invasive venography can be performed to further evaluate the mass once diagnosis of venous aneurysm was suspected. Using pericardium to repair large veins is a good choice which is safe and costless. Mediastinal venous aneurysm is a very rare disease  and ofteA 52-year-old man was admitted to our hospital for mediastinal mass found incidentally in health examination. He had no special disease history or any symptoms. No face or upper extremity edema was found in physical examination. Enhanced chest computer tomography (CT) showed a size of 5.6*4.3 cm mass in anterior mediastinum, which was enhanced in the artery phase Fig. , and it The venous aneurysm was resected with the proximal and distal end of the left innominate vein blocked Fig. . HoweverLeft innominate vein aneurysm is very rare and displayed as a mediastinal mass mimicking other tumors such as thymoma , 3. In tAutologous pericardium has already been used in cardiac surgery for reconstruction of heart valve. Quinn et al. reported 62 patients who underwent mitral valve repair with fresh autologous pericardium and obtained excellent long term outcome . B-KhanhThe SVC was blocked twice in this case we reported. It should be blocked as short as possible because clamping too long may cause brain edema and leads to serious neurological complication. The surgical strategy should be appropriately designed to reduce the blocking time. We repaired the part wall of SVC first and unblock it immediately after the repairment was finished. Then we anastomosed pericardium roll to the distal end of the left innominate vein. The SVC was blocked again when anastomosed with the other end of pericardium roll. The arranged sequence of surgical repairment was to reduce the continuous blocking time of SVC. However, the maximum safe clamping time for SVC remains unclear. If SVC clamping time was estimated to be long in inexperienced centers, venovenous shunt technique can be considered. Dai et al. reported two kinds of venovenous shunt, external and internal. They connected internal jugular and femoral vein before surgery with cannula which was called external venovenous shunt. The internal venovenous shunt was performed during surgery between right brachiocephalic vein and right atrium. Venovenous shunt can reduce the central venous pressure and median SVC clamping time reached 75 min without any complications as reported .In summary, for patients with mediastinal mass, magnetic resonance imaging, computed tomographic angiography and invasive venography can be performed for further evaluation once venous aneurysm is suspected or can not be excluded. Median sternotomy is recommended for aneurysms of large vessels because of the potential risk of massive bleeding and the possible challenges faced during operation such as need for reconstruction of large vessels. Using autologous pericardium to repair and reconstruct large veins is a good choice which is safe and costless.


Adrenocortical oncocytic neoplasm arising in ectopic adrenal tissue is a rare finding and presents as a unique diagnostic challenge. We report a case of a 26-year-old female who presented with vague left-sided abdominal pain and a large left retroperitoneal mass. She underwent exploratory laparotomy and resection of the mass and was diagnosed with extra-adrenal adrenocortical oncocytic carcinoma. Adrenocortical carcinoma is a rare, typically aggressive cancer with an incidence of 0.7 per 2 million cases annually A 26-year-old Hispanic female presented to the Emergency Department (ED) with a 2-day history of abdominal pain. She described her pain as dull, progressive and localised to the left abdomen. She denied any changes to her usual state of health, especially with respect to her weight, appetite, nausea, vomiting or bowel habits. Her past medical history was insignificant. Last menstrual period was 2 weeks prior to presentation. Her vital signs and routine blood workup, including complete blood count and comprehensive metabolic panel, were all within normal limits.She underwent a computerised tomography (CT) scan, which revealed a large 21 × 15 cm mass, which appeared to be abutting the left kidney, left renal vessels, distal pancreas and spleen . She wasGrossly, the mass measured 28 × 18 × 10.5 cm and weighed 2,250 grams . HistopaAdjuvant chemotherapy was recommended based on the pathological diagnosis; however, the patient declined to receive any further treatment. She is under surveillance and has had no evidence of recurrence at 30 months follow-up.AONs are an extremely rare subtype of adrenocortical carcinoma (ACC) that were first described in 1986 . A totalEctopic adrenal tissue is relatively common existing in approximately 6% of the population . Since tThe clinical presentation of AOCs is usually due to mass effect on surrounding structures as only 17% of the oncocytic neoplasms are hormonally active . TherefoUnlike most adrenocortical malignancies, the size of the mass and the imaging findings on CT or MRI are not pathognomonic for AOC. Although the percentage washout on CT has been correlated to the differentiation of benign versus malignant AON , this fiThe workup of these oncocytic tumours is similar to adrenal incidentaloma. The final diagnosis is made after a histopathological assessment of the tumour. On gross examination, AOCs are large, round, encapsulated, brown-yellow and well-circumscribed masses with an average diameter of 8 cm and a thin rim of the normal adrenal gland . MicroscHowever, it should be emphasised that the determination of the oncocytic character of the tumour cells represents another diagnostic hurdle. One of the reasons for this is that the determination of oncocytic character is based on subjective morphological criteria . Our casRegardless of the scoring, patients with AOC have a better prognosis when compared to the conventional adrenocortical carcinomas. Adrenocortical carcinomas are aggressive, with an estimated median survival of 8–32 months. AOC, on the other hand, has a comparatively favourable median survival of 58 months , 2.AOC does not have well-established protocols regarding management given the limited data available on patient outcomes. Frequently, treatment follows the same course as in conventional adrenocortical carcinoma , 5, 10. AONs have a reported recurrence rate of 16%, with most recurrences occurring within first 5 years . There iAOC are a rare clinical entity, with only 32 cases reported to date. The present case emphasises the multidisciplinary diagnostic challenges faced during detection of AOC as they do not have any radiological or clinical pathognomonic features, in addition to the ectopic location of the tumour in this case. Thus, oncocytic variants should be in the differential when dealing with tumours of unknown origin along the path of embryonic migration of the adrenals. LWB scoring or the recently validated Helsinki score is used to elucidate the malignant potential of ACC and AOC. Management is aimed at achieving an R0 surgical resection, followed by adjuvant chemotherapy and/or radiation.None.None.The preparation, design and editing of the original draft were done by N.W. D.M. and D.K. reviewed and edited the manuscript. M.K. planned and supervised the project, editing and critical revision. All authors have read and approved the final version of the manuscript.


Case Description: A 76-year-old lady, with a history of nonvalvular atrial fibrillation, presented with a new onset of progressive left lower and upper extremity weakness. She reported back pain and numbness in the left leg up to the knee along with numbness in the left arm up to the shoulder. A CT scan of the neck was suggestive of an epidural hematoma extending from C2-C3 level to C6-C7. As the patient was on apixaban at the time, surgical treatment was delayed for two days to decrease the risk of intraoperative bleeding. Nine days later, she was discharged. Her physical exam was almost unchanged from that on presentation, except for resolution of pain and minimal improvement in motor power in her left lower extremity from 1/5 to 2/5 distally.  With the rise in the use of direct oral anticoagulants (DOACs), more hemorrhagic complications are being encountered. Since the first description of a case of spontaneous spinal epidural hematoma (SSEH) related to the utilization of DOACs in 2012, there have been few reports describing a similar association. However, no cases so far have reported an association between SSEHs and apixaban.  Spinal hematomas represent surgical emergencies with earlier intervention portending better outcome. Based on the few case reports that point to DOACs as a potential culprit, it appears that a high suspicion index resulting in earlier SSEH diagnosis and intervention is crucial for improved neurological outcome and recovery. Prompt diagnosis remains a challenge, especially that SSEH can mimic cerebrovascular accidents. Since the first description of a case of SH associated with the use of a DOAC (rivaroxaban) in 2012 . As the A 76-year-old African American lady presented to the hospital with new onset weakness and back pain. Her medical history consisted of essential hypertension, atrial fibrillation, abdominal aortic aneurysm, sick sinus syndrome, asthma, and gout. She reported that her back pain started after she came back from the bathroom at 4 : 00 a.m. while trying to sit down on the bed. The patient described the pain as shooting in nature, severe in intensity, and radiating down her left arm with no clear exacerbating or relieving factors. Over the following few minutes, she developed progressive weakness in her left lower and upper extremities and later on became unable to move her left leg which led to her being transferred to the Medical Intensive Care Unit. She also developed numbness in her left leg up to the level of the knee and in her left arm up to the level of the shoulder. There was no history of any urinary or stool incontinence or retention or any previous similar episodes.The patient had been recently diagnosed with atrial fibrillation one month prior to presentation in an outside hospital and had since been maintained on apixaban 1 tablet 5 mg twice daily. Her other home medications included alprazolam, allopurinol, losartan, hydrochlorothiazide, and metoprolol. She reported full compliance with her medications prior to presentation. A dual chamber pacemaker was inserted 11 days before this visit in an outside hospital, and her other surgical history included a hysterectomy several years ago for uterine fibroids. She denied any history of smoking, alcohol abuse, or illicit substances usage. The patient did not have previous bleeding history.The patient's vital signs on presentation were within normal limits, and her neurological exam showed a patient alert and oriented to time, place, and person with intact speech. Her motor power was 5/5 in her right upper extremity proximally and 4/5 distally and 3/5 in her left upper extremity proximally and 1/5 distally. In her lower extremities, the motor power was 5/5 on the right and 1/5 all over on the left. She had decreased sensation to light touch in her left upper and lower extremities. She had no clonus, and Hoffman and Babinski signs were negative bilaterally. Her reflexes were 2+ all over and symmetric.Initial lab tests were significant for hypokalemia (3.1 mmol/L), leukocytosis (12700 WBCs per microliter), and slight anemia with a hemoglobin level of 9.3 g/dl with unknown baseline. Her other lab tests including liver function and renal function were within normal limits.Given her presentation of acute focal neurological deficit, a noncontrast head CT scan was ordered and showed signs of chronic vascular ischemic disease only. The patient continued to have severe back pain, and having a history of abdominal aortic aneurysm, an acute aortic dissection was suspected, and hence, a CT angiogram of the abdomen, pelvis, and thorax was ordered. The CT angiogram did not show any signs of aortic dissection. A CT scan of the neck was performed to investigate the cause of the left upper extremity weakness and numbness. It showed an epidural soft tissue density extending along the left aspect of the spinal canal causing compression and marked rightward deviation of the cord concerning for an epidural hematoma extending from C2-C3 level to C6-C7 .As the patient was on apixaban at the time, a decision was made to delay surgical treatment for 2 days to decrease the risk of intraoperative bleeding. A C2-C7 laminectomy, hematoma exclusion, and spinal fusion were then performed. On discharge, 9 days later, her physical exam was mostly unchanged from that on presentation except for the resolution of pain and improvement in motor power in her left lower extremity from 1/5 to 2/5 distally.Spinal hematomas represent surgical emergencies with earlier intervention portending better outcome . The acuX inhibitors [According to our literature review, 4 patients underwent surgical intervention within 24 hours of symptoms onset, and 3 of these 4 patients 75%) had excellent outcomes with unknown outcome in the third one [% had exchibitors . There whibitors . Among tThe number of cases reported is insufficient to reach any statistically significant result, but seems to be pointing towards the importance of early surgical intervention for neurologic recovery. With the development of new reversal agents and increased experience with the current ones, earlier surgical intervention is most likely to increase in frequency as surgeons become more comfortable operating on these patients leading hopefully to less neurologic deficit. This means that a high suspicion index leading to earlier diagnosis is key for timely intervention, and we hope that this article will help in raising awareness about this condition as a potential complication of DOACs use, especially with the significant and continuous increase in their utilization.


Inflammatory pseudotumors (IPTs) are benign masses arising from nonspecific inflammatory conditions including surgical invasion. We herein report the rare case of an IPT mimicking port-site metastasis in a 69-year-old patient who underwent retroperitoneal robotic partial nephrectomy for stage T1a renal cell carcinoma. Radiological examination performed six months after the surgery revealed the presence of a mass underneath the abdominal wall which coincided with a port site. The tumor was resected by laparoscopic transperitoneal approach, and histological examination led to the diagnosis of an IPT that consists of xanthogranulomatous inflammation. We also discuss the etiology of IPT formation and features distinguishing IPTs from port-site metastasis. Inflammatory pseudotumors (IPTs) which are caused by nonspecific inflammatory conditions can develop at nearly every body site . Albeit A 69-year-old Japanese male with diabetes mellitus underwent right-sided retroperitoneal RPN for an incidental renal tumor . Five po18F]fluorodeoxyglucose- (FDG-) positron emission tomography- (PET-) CT, the mass had a mild FDG uptake with a maximum standardized uptake value of 3.2 (Follow-up computed tomography (CT) scan performed six months after the surgery revealed an emerging small mass below the right side of the abdominal wall coinciding with a 12 mm AirSeal™ assistant port site . By [18Fe of 3.2 . FurtherTumor resection was subsequently performed by laparoscopic transperitoneal approach . AlthougIPTs are benign tumors which mimic malignant neoplasms and comprise cells associated with both acute and chronic inflammation. The pathogenesis and etiology of IPTs are unspecific; therefore, IPTs have been described under various names such as xanthogranuloma, inflammatory myofibroblastic tumor , and lipSchloffer's tumor might be considered the diagnosis in the present case based on the potential etiology because it is possible that Vicryl™ suture or Surgicel™ used in RPN might have provoked a foreign body reaction. Asano et al.  reportedIn the present case, the Surgicel™ bolster placed on the renal parenchymal defects was nearly invisible at the IPT site. This image finding shows that the Surgicel™ bolster was normally diminished with time after surgery . It is uWhile the exact cause of IPT in the present case remains unknown, we speculate that the following two processes were more likely etiologies. The first one is chronic inflammation by microabscess formation underneath the abdominal wall, which might be partially due to diabetes mellitus. The second is the involvement of FN. Fat fragments produced in RPN might migrate to a port site and subsequently develop FN provoking chronic inflammation. Amblee and Ganesh  reportedThe preoperative differentiation between IPT and PSM of RCC is difficult because of the rarity of both conditions, which were reported in very few case reports –4, 9–11.Song et al. , who rep


Human disorganization syndrome (HDS) is an extremely rare congenital syndrome characterized by a seemingly random distribution of multiple developmental anomalies involving all three germinal layers.We report a rare case of a female child whose congenital anomalies are consistent with HDS. The orthopaedic features of this patient include a popliteus pterygium with an associated flexion contracture secondary to an elongated biceps femoris tendon that attached to the gastrocnemius-soleus muscle complex, two finger-like appendages, a tethered cord, a lipomeningomyelocele at the level of L5, and a leglength discrepancy. The patient was treated with a splinting program, release of the biceps femoris tendon at its erroneous insertion from the gastrocs-soleus, and surgical excision of the finger-like appendages. She underwent three subsequent soft-tissue releases to address recurrence of the knee flexion contracture and an anteromedial and lateral distal femoral eight plate procedure for guided growth and slow correction of the remaining flexion deformity.The treatment of HDS can be quite complex and can present with a variety of anomalies with distinctive orthopaedic features correctable with surgical management, including soft-tissue releases, excision of appendages, and growth modulation. The wide range of malformations observed in DS poses a challenge in outlining the diagnostic criteria. The most commonly reported malformations include the following: cranioschisis/exencephaly, unusual hamartomas, appendicular anomalies, eye malformations, pharyngeal defects, and gastroschisis.2 Extremity abnormalities include duplications and reductions, usually involving a single limb, polydactyly, and malformed and small limb girdles.1Disorganization syndrome (DS) was first described in 1958 by Hummel3 proposed a human homologue of DS and described the case of an infant with multiple defects in-line with those observed in DS mice. The infant presented with shortening of the right leg, a web across the popliteus fossa, nine toes on one foot, a finger-like structure arising from the abdomen, and an absent kidney. Since then, few case reports have been published on patients suspected to have Human DS (HDS).5In 1989, Donnai and WinterCurrent case reports have almost exclusively focused on medical genetics despite many HDS-associated anomalies requiring orthopaedic intervention. Here, we report the orthopaedic management of a patient presenting with congenital physical anomalies consistent with HDS.This report describes a female child who was delivered at term via elective caesarean section to healthy, nonconsanguineous parents after an uncomplicated pregnancy. The patient also has an older female sibling who displays no abnormalities. Prenatal chromosomal studies were negative for fetal abnormalities; however, a number of congenital anomalies were observed at birth.The anomalies included a left popliteus pterygium extending from the buttock to the calcaneus, with an associated knee flexion contracture. In addition, a toe-like skin tag was observed on the posterior aspect of the calf and another smaller skin tag on the lower left aspect of the buttock; it appeared that the two appendages had previously been connected Figure . A cutanThe popliteus pterygium caused a 40° knee flexion contracture that was slightly flexible. A full-time splinting program was initiated, and at 3 months of age, her passive range improved to full extension, facilitating subsequent weaning to night-time splinting. An MRI of the left leg revealed that a long biceps femoris tendon was the source of the pterygium. It extended from the ischium to calcaneus and connected the biceps femoris muscle to the gastrocnemius-soleus muscle complex Figure . Owing tAt nine months of age, excision of the distal 5.5 cm of the biceps femoris tendon off of the calcaneus was performed through a posterior approach centered over the Achilles tendon Figure , A. A prSix months postoperatively, the patient had recurrence of a 15° knee flexion contracture despite aggressive stretching and bracing treatment. Regrowth of the fibrous band behind her knee was noted at this time Figure . ConsequBy the age of five, the knee flexion contracture had increased to 30°, and the patient also presented with a 20° equinus contracture of her foot and a 1.5-cm leg length discrepancy. A gastroc recession, hamstring lengthening, and distal tendoachilles lengthening was done. Her postoperative rehabilitation course involved wearing an articulated ankle-foot orthosis during the day and a knee-ankle-foot orthosis at night. After the operation and rehab course, her knee was only 5° short of full extension and she had 10° ankle dorsiflexion and 40° plantar flexion  for HDS remains unknown.Previous published reports of HDS are largely focused on the pathoanatomy and medical genetics of HDS. It is thought that the HDS phenotype is because of a semidominant mutation with incomplete penetrance, which is usually lethal in the homozygous state.26 The indications for the treatment of popliteus pterygium have been functional limitations in ambulation and deformity. Many surgical interventions have been described, including a variety of soft-tissue releases26272829 and bony osteotomies.31 The sciatic nerve is often a limiting factor in complete lengthening of the skin and soft tissues in the affected area because the nerves and vessels are shortened. In addition, the sciatic nerve may be attached to the fibrous band of anomalous muscle, known as the calcaneo ischiadicus.33 Recurrence of the flexion contracture is frequent, and repeat surgical correction is common, as demonstrated in the present case.26This case highlighted the presence of a popliteus pterygium secondary to an elongated biceps femoris tendon that attached to the gastrocnemius-soleus muscle complex. This resulted in a knee flexion contracture that proved difficult to treat with both nonoperative splinting and surgical resection. Popliteus pterygium has been described as one of the most difficult orthopaedic problems.HDS is a complex condition with distinctive orthopaedic features that are at least partly correctable with surgical management. In this case, several orthopaedic surgical interventions were done because of the recurrence of flexion contractures. Further case reports of HDS and discussion in the orthopaedic community to identify similar cases are required to learn more about this extremely rare disorder. This may allow for the identification of possible risk factors and aid accurate diagnosis and management of HDS-related orthopaedic conditions in the future.


Lymphangioma is an abnormal proliferation of lymphatic vessels. Isolated splenic lymphangioma (SL) is rare; in the last 30 years only 22 cases were reported in the literature. Diagnosis is difficult by the absence of typical symptoms and signs. SL is often asymptomatic and occasionally detected through radiological exams or, after the onset of a life-threatening complication, at surgery or pathological examination. Surgery represents the treatment of choice.A 22-year-old Caucasian male was admitted to the Emergency Department after a car accident, complaining of sudden onset, severe, left upper quadrant abdominal pain. Abdominal examination revealed mild abdominal distention and a severe abdominal pain on superficial and deep palpation of left upper abdominal quadrant with obvious muscle guarding and rebound tenderness. Abdominal computed tomography scan showed splenic rupture with hemoperitoneum. Laboratory tests reported severe anemia. The patient received blood transfusions and was taken emergently to the operating room for exploratory laparotomy, evacuation of hemoperitoneum and splenectomy. The postoperative course was uneventful, the patient was discharged on the 7th post-operative day. Diagnosis of microcystic SL was made at pathological examination.Post-traumatic splenic rupture may be favored by the presence of a unknown and asymptomatic isolated SL. Although different types of treatment of SL are reported in the literature, splenectomy represents the treatment of choice to avoid complications.Isolated SL is difficult to diagnose and suspect because of lack of pathognomonic symptoms and specific diagnostic signs. It can be diagnosed after the onset of a life-threatening complication. Splenectomy is the preferable definitive treatment. It is debated whether it is a benign tumor or a congenital malformation of the lymphatic system . Lymphan2A 22-year-old Caucasian male was admitted to the Emergency Department after a car accident, complaining of sudden onset, severe, left upper quadrant abdominal pain. He was found to be hypotensive, pale and diaphoretic. Vital signs on admission were blood pressure 80/40 mm Hg, pulse 125 bpm, respiratory rate 26 per minute and no fever. The patient wasn’t taking any drugs or smoking and his past and familial medical histories were normal. Abdominal examination revealed mild abdominal distention and a severe abdominal pain on superficial and deep palpation of left upper abdominal quadrant with obvious muscle guarding and rebound tenderness. Treatment was initiated with crystalloid fluid bolus with appropriate improvement in blood pressure but persistent tachycardia. Thoracic and abdominal computed tomography (CT) scan showed splenic rupture with massive hemoperitoneum . Laborat3This clinical case describes the post-traumatic rupture of an asymptomatic microcystic SL causing hemorrhagic shock. Although no consensus has yet been reached on whether lymphangioma is a neoplasm or a hamartoma, most researches support the latter opinion. Its formation would be due to abnormal congenital development of lymphatic vessel or attributed to bleeding or inflammation in the lymphatic system, which causes obstruction and consequent lymphangiectasia. Based on the size of the dilated lymphatic channels, lymphangioma can be classified as capillary (supermicrocystic), cavernous (microcystic) or cystic (macrocystic) ; the mic4SL is a rare malformation of the splenic lymphatic vessels, mostly seen in children and rarely in adults, often asymptomatic and incidentally detected through imaging studies. Because of the absence of pathognomonic symptoms and signs, preoperative diagnosis is difficult: in our case diagnosis was favoured by a life-threatening complication (splenic post-traumatic rupture) that represents a possible consequence of SL. Although different types of conservative treatment are reported in literature, total splenectomy represents the preferable definitive treatment to avoid possible lethal complications. Definitive diagnosis is achieved by histopathologic examination after splenectomy.All the authors certify that there is no conflict of interest regarding the material discussed in the manuscript.All the authors declare that this research didn’t receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Ethical approval has been exempted by our institution because this is a case report and no new studies or new techniques were carried out.Written informed consent was obtained from the patient, for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Giuseppe Evola: Drafting the manuscript and literature research.Giovanni Mazzone: Operated on the patient, literature research.Antonino Corsaro: Operated on the patient, literature researchGiovanna Brancato: Drafting the manuscript and literature research.Francesco Roberto Evola: Drafting the manuscript and literature research.Guido Basile: Drafting the manuscript, literature research, revising the manuscript.This case report does not require registration as a research study.The guarantor for this case report is Giuseppe Evola.Not commissioned, externally peer-reviewed.


Periprosthetic osteolysis (PPOL) is a common complication after total knee arthroplasty (TKA) and is most commonly caused by wear-induced particles.We report an unusual case of massive bilateral PPOL in the posterior flanges of the femur and patellae 4 years after bilateral uncemented TKA without patellar resurfacing in a 71-year old female. Bilateral staged revision surgery including polyethylene exchange and allograft morselized bone impaction was performed to treat the osteolytic lesions. There were no signs of implant malalignment, polyethylene wear or component loosening.Several factors are associated with an increased risk on PPOL . Surgical intervention in the context of massive PPOL should include replacement of a potential particle generator (most often polyethylene), correction of potential malalignment, treatment of bone defects and assessment of implant anchorage.This report highlights the available evidence on clinical presentation, associated risk factors and preferred treatment strategy of massive osteolytic lesions after TKA according to available evidence. It is mMassive osteolysis mostly occurs in the posterior flange of the femur and along access tracks of the tibial component . PPOL is2The current case report has been reported in line with the 2020 Consensus Surgical CAse REport (SCARE) guidelines . A 71-ye9/L (range 4–10 × 109/L). Allergy tests for metals by our hospital allergist were negative. Knee aspiration cultures excluded infection.Physical examination revealed warm, swollen knees, flexion/extension of 90/0/0° and pain on palpation of the joint lines. The implanted knees were stable in coronal and sagittal planes. Conventional radiographs and computed tomography scans showed a similar pattern of extensive osteolytic lesions in the posterior femoral condyle and center of the patella in both knees  & 2. BloRevision surgery of both knees, starting with the right knee with an interval of four months, was performed by 2 orthopedic surgeons . After medial parapatellar arthrotomy a caseous synovitis was visible . An exteMultiple tissue cultures from both knees revealed no microorganisms. Auramine colouring and DNA-PCR screening were both negative. Microscopic examination of the samples showed partly amorf eosinophilic material, an inflammatory reaction and birefringent material. At 8 years post revision surgery (12 years post primary TKA), the patient was pain free and able to mobilize normally with an unrestricted range of motion (110° of flexion with no extension lag in both knees). Radiographically, there was no new lesion of PPOL observed during the complete follow-up period . After c3PPOL is a common complication after TKA and is traditionally associated with polymethylmethacrylate (i.e. bone cement) debris  and lateOsteolysis of the patella is quite common in TKA after placement of a patella component . In our Several factors that increase the risk of PPOL have been identified through the years, such as screw fixation , specifiIn our case, PPOL was discovered with conventional radiographs  and a coSurgical intervention due to PPOL should focus on exchange of the particle generator (mostly the polyethylene bearing), implant anchorage and alignment, and  bone defects . There h4We present an unusual case of bilateral PPOL in the femur and patella after uncemented mobile bearing TKA without patellar resurfacing. During revision surgery, both femoral and tibial components were well fixed to the bone despite the large osteolytic lesions that were found. Synovectomy, polyethylene exchange and morselized allograft bone impaction were the surgical interventions that were undertaken, there were no signs of advanced wear on the metal components or the polyethylene insert. Eight years post revision surgery, the patient remains asymptomatic and there are no new osteolytic lesions detected on radiographic follow-up. There are several (patient and implant) factors that are associated with an increased risk of PPOL . Conventional radiographs lack the sensitivity to identify all osteolytic lesions, but implementing CT or MRI in regular TKA follow-up would be too cost-intensive. Surgical intervention in the context of massive PPOL should focus on polyethylene exchange (or exchange of another particle generator), implant anchorage and alignment and bone defects. Dependent on the size and location, these bone defects can be filled up with morselized allograft impaction, metal augments or revision components with porous coated sleeves or cones.N/A.N/A.Not needed for this case report.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. Consent has been obtained in the native language of the patient (Dutch) since the patients does not understand English. A formal translation of a native English speaker is available upon request.Rachid Rassir: Conceptualization; Formal analysis; Writing - original draft; Writing - review & editing.Jorm M. Nellensteijn: Conceptualization; Data curation; Investigation; Writing - original draft; Writing - review & editing.Rachid Saouti: Conceptualization; Data curation; Resources; Supervision; Writing - original draft; Writing - review & editing.Peter A. Nolte: Conceptualization; Data curation; Investigation; Supervision; Writing - original draft; Writing - review & editing.Not applicable.Peter A. Nolte.Not commissioned, externally peer-reviewed.


Fibroepithelial polyps are uncommon benign tumors that mostly occur in skin, oral cavity and genitourinary tract. These lesions have common morphological features with other mesenchymal tumors such as angiomyofibroblastoma, aggressive angiomyxoma, and cellular angiofibroma and present with numerous histological appearances. Benign endobronchial tumors are rare. These neoplasms have a slow growth and usually are related to bronchial obstruction. Only a few cases of bronchial fibroepithelial polyps have been reported. Bronchial fibroepithelial polyps might present with airway stenosis resulting in atelectasis and bronchiectasis and the most frequent manifestations are recurrent infection, refractory asthma, dyspnea and hemoptysis. Chronic inflammation is associated with the pathogenesis of fibroepithelial polyps. Treatment varies according to mainly to the size and symptoms. Small lesions presenting with few symptoms can be treated with corticosteroids and antibiotics while invasive techniques including bronchoscopic resection of the polyp or lobectomy are used for larger lesions. We report a case of a bronchial fibroepithelial polyp with severe hemoptysis as the first manifestation. Physicians should always suspect these lesions in the differential diagnosis of hemoptysis and with initial right diagnosis surgical procedures can be avoided. Fibroepithelial polyps represent rare benign tumors that are most frequently present in skin, oral cavity and genitourinary tract . These lBenign endobronchial tumors are uncommon. These neoplasms present with slow growth and usual manifestation is related to bronchial obstruction . Benign Bronchial fibroepithelial polyps might present with airway stenosis resulting in recurrent respiratory infections, atelectasis and bronchiectasis. The most common reasons for admission are recurrent infection, refractory asthma, dyspnea and hemoptysis . ChronicA 66-year-old female, non-smoker, patient was admitted to our emergency department for severe hemoptysis over the last two days. She had a history of dyslipidemia and atrial fibrillation ablation in the past.A few months ago, she was hospitalised with hemoptysis and during this hospitalisation, she underwent computerized tomography (CT) of the chest examining the bronchial arterial anatomy which did not reveal dilated bronchial arteries. CT showed bronchiectasis in the right lower lobe and thickening of the posterior wall of the left main bronchus. The patient underwent bronchoscopy with flexible bronchoscope and active bleeding from lower left lobe was found. Argon plasma coagulation was performed and mucosal biopsies and bronchial washings for cytological and microbiological examination were obtained, without abnormal findings.On admission, clinical examination revealed crackles on auscultation at both lung bases. Blood pressure was 130/95 mmHg, heart rate was 90 beats per minute, oxygen saturation was 93% on room air and body temperature 36.8°C, without abnormal findings on electrocardiography. Chest X-ray showed a consolidation in the right lower lobe.Laboratory findings, including coagulation tests, were normal. The patient underwent CT of the chest in our hospital, that showed bronchiectasis and ground-glass opacities in the lower lobes and bronchial wall thickening of the left main bronchus, which has already been reported in previous imaging Figure . The patThis is a rare case of the bronchial fibroepithelial polyp. Fibroepithelial polyps were reported for the first time in 1938 when Pollak, Cohen, and Gnassi in a review of the literature for benign bronchial tumors described 27 cases detected at autopsy and 77 encountered during the life of which flbroepithelial polyps were recognised in 11 living patients. [Dinçer et al. described a case of a 55-year-old male patient with recurrent pulmonary infections for 40 years and a chest CT revealed obstruction of the main bronchus at the level of carina. In bronchoscopy, a mobile polypoid pinkish lesion was observed, the patient underwent bilobectomy and the histological examination revealed an endobronchial fibroepithelial polyp . StrachaUshiki et al. reported a case of a 75-year-old female with a productive cough, a solid lobulated nodule in the right main bronchus and subtotal atelectasis of the right lower lobe in CT and bronchoscopy with biopsy confirmed the diagnosis of a bronchial fibroepithelial polyp . WartmanMaskey et al. reported a case of a 39-year-old man, presented with dyspnea, with a fibroepithelial polyp arising from the right upper lobe bronchus and extending into the main stem bronchus, which was successfully removed using an electrocautery snare . Leiro-FSome authors have described cases of bronchial fibroepithelial polyps underlying the role of different specific bronchoscopic techniques in these cases. Saito et al. described a 65-year-old man, who was diagnosed with a bronchial fibroepithelial polyp of the right basal bronchus and underwent bronchoscopic examination, with a videobronchoscope that incorporates two devices for white-light (WL) mode and auto-fluorescence imaging (AFI). Bronchoscopy under WL mode revealed a rounded, whitish, smooth, and glistening polypoid lesion with a lobulated surface and on AFI, the lesion appeared magenta colour (positivity) . The autThe case we describe is original for three reasons. The first reason is that the first manifestation of the lesion was severe hemoptysis. According to a review of cases by Li et al. the most frequent first manifestation of bronchial fibroepithelial polyp was recurrent infection , while iBronchial fibroepithelial polyps are rare clinical entities and are described as a handful of case reports. Medical doctors should always consider the possibility of these tumors in the differential diagnosis of hemoptysis. It is important to recognise fibroepithelial polyps because they may result in symptoms mimicking malignancy or infections and initial right diagnosis may prevent unnecessary surgical procedures. The current case report is unique, as to our knowledge for the first time in literature, bronchial fibroepithelial polyp is presented with severe hemoptysis as first manifestation, thickening of the posterior wall of the left main bronchus in CT of the chest and as mucosa invasion and not as whitish and glistening polypoid lesion with rounded or lobulated borders in bronchoscopic examination.


Pneumorrhachis (PR) is the presence of free air within the spinal canal. It is generally benign and improves with conservative management. Case reports and a literature review exist documenting the existence and potential pathogenesis of this phenomenon, but no evidence-based guidelines exist documenting what treatment, if any, is indicated for this condition. We present a case of a 21-year-old male who developed PR after a preceding upper respiratory tract infection. His symptoms improved with expectant management and administration of high-flow oxygen. The purpose of this case report is to add to the scarce existing literature reporting this condition and to provide a short review of literature detailing the pathogenesis of PR. Pneumorrhachis (PR) is a rare, typically benign condition of air within the spinal canal resulting from iatrogenic, traumatic, or non-traumatic causes. Evidence-based guidelines for the management of this condition are lacking; therefore, treatment for PR is documented through various case reports. The etiology of PR can often be helpful in determining the appropriate course of treatment. In a majority of cases, conservative management with attention paid to cardiorespiratory support and neurologic monitoring is indicated. Surgical intervention is entertained for PR in the situations causing neurologic deterioration and cerebrospinal fluid leaks, or in situations that are secondary to fistulous connections from extra-spinal sources . In thisA 21-year-old male presented with acute onset chest pain following a one-week history of rhinorrhea, nasal congestion, and a non-productive cough. Initial chest radiograph demonstrated linear lucencies in the mediastinum and right supraclavicular region suggestive of pneumomediastinum. A subsequent computed tomography angiogram (CTA) of the chest was obtained, which showed pneumomediastinum with subcutaneous emphysema involving the supraclavicular lower neck region, bilateral axilla, right thoracic paraspinal musculature, and the right chest wall Figure . The patPR is defined by the presence of air in the spinal canal. It is a rare condition that is only sporadically reported in the literature. This condition has previously been referred to as intraspinal pneumocele, spinal epidural emphysema, and aerorachia, until the current term of PR was coined by Newbold et al. in 1987 . PR can PR is generally asymptomatic, but it can present with neurological deficits in the event of air compressing the spinal cord ,5-6. WhiOur case illustrates the occurrence of PR without any associated macro-trauma. The patient’s recent upper respiratory infection preceding admission potentially instilled a high-pressure micro-trauma to his alveoli during coughing events, leading to air escaping in the mechanism described above. No neurological deficits were observed on physical examination, which prompted conservative management. The patient’s symptoms improved during his hospitalization with expectant management and administration of high-flow oxygen. Further imaging would be needed to determine if the PR resolved; however, this would require additional risk associated with radiation that would outweigh the potential benefit in light of the patient’s improving condition.PR is a rare and likely under-diagnosed phenomenon. The underlying etiology and physical examination are critical for management consideration, but this condition is typically treated conservatively. A single-center, large-volume case series would be helpful in guiding management decisions.


Lipomatous tumors herniating through the sciatic foramen are extremely rare with less than 6 cases reported in the existing literature. We report a case of gluteal well differentiated liposarcoma that presented as sciatic hernia.A 66-year-old female presented with persistent pain in the right gluteal region. Physical examination coupled with CT/MRI scans identified a firm mass extending into the right pelvic side via the sciatic foramen. It measured approximately 18.9 cm × 13 cm × 22.8 cm. The tumor was resected via transgluteal approach. Immunohistochemical and microscopic features were consistent with well-differentiated liposarcoma.Although sciatic hernias associated with intraabdominal contents have been recorded less than 100 times in the literature, instances where gluteal lipomatous tumors manifest as sciatic hernias are even more rare. In this case the gluteal liposarcoma was successfully identified utilizing CT/MRI scanning followed by a transgluteal excision of the mass.While sciatic hernias are rare, lipomatous/liposarcomatous tumors presenting as sciatic hernias are extremely rare. This case report highlights a combination of rare phenomena. The information presented adds to the current evidence that will guide accurate diagnosis and appropriate treatment of future cases. HoweverThis work has been reported in line with the SCARE criteria .2A 66-year-old female had complained of intermittent discomfort and pain in the right gluteal area. She had also noticed an alteration in bowel habit with recent constipation. The symptoms persisted, and she was treated with analgesia and laxatives.However, there was no specific diagnosis until she fell onto her back and fractured L2, L3 and L4 vertebrae which necessitated further imaging work-up with CT scan of the thoracolumbar spine. Of note, the fractures were stable and non-displaced. Hence, no need for surgical intervention. The CT scan performed to assess the spine revealed an incidental finding of a large mass extending from the right gluteal region into the pelvis. A CT scan of the pelvis was also performed, and this showed a large fatty intramuscular lesion extending into the pelvis through the greater sciatic notch . The difPhysical examination revealed an area of firm induration/possible mass in the lower aspect of the right gluteal compartment. This was not tender and there was no associated redness or discoloration of overlying skin. Digital rectal examination showed normal anal sphincter tone and no mass was palpated within the anorectum.The patient proceeded to have a CT guided core needle biopsy of the mass. This showed scant fragments of fibroadipose and fibroconnective tissue without features of malignancy. There was focal myxoid change. However, the cytologic atypia that would warrant a diagnosis of atypical lipomatous tumor/well differentiated liposarcoma was absent. Furthermore, FISH for MDM2 amplification was attempted, but could not be performed due to sample inadequacy. The case was presented for discussion at the multidisciplinary tumor conference and the consensus recommendation was to proceed with surgical resection of the tumor. Following this, the patient underwent wide excision of the mass through a transgluteal approach and final pathology showed well-differentiated liposarcoma .Fig. 4HiRegarding follow up, the patient has been undergoing surveillance for 3 years. She was seen every 3 months for the first year after resection. Computerized tomographic scans of the chest, abdomen and pelvis were done every 6 months for the first year of surveillance. The patient was seen twice a year for the following 2 years. Computerized tomographic scans of the chest, abdomen and pelvis were done annually for years 2 and 3 of surveillance. The patient has remained disease-free; that is no evidence of local or distant recurrence of the liposarcoma or the sciatic hernia.3Most sciatic hernias have intraabdominal/intrapelvic contents like intestines, greater omentum, ovaries, fallopian tubes, bladder and ureters. According to Skipworth et al., sciatic hernias are extremely rare and have been documented less than 100 times in current medical literature . Sciatic2. The thickness of the subcutaneous tissue adiposity may have also contributed to decreased prominence of the gluteal mass. Subtle presentation of sciatic hernia with constipation due to compression/ lateral displacement of the rectum attests to the difficulty of diagnosing this condition purely based on clinical signs and symptoms [In this case the patient did not give any history of classic sciatic pain radiating down the lower extremity. Rather, she complained of localized pain in the right gluteal area. It is speculative to consider that her subsequent fall onto her back  may be in some way related to sciatic pain in the right lower extremity. While most cases of sciatic hernia will present with gluteal masses, this patient was found to have gluteal mass on imaging which was done for the spine and subsequently confirmed on physical examination. This is most likely due to the fact that the tumor arose within deep intramuscular space between the gluteus maximus and gluteus medius. The patient was also obese, with BMI greater than 35 kg/mCase reports of lipomatous tumors herniating through the sciatic foramen are extremely rare indeed. To the best of our knowledge the first of such cases was documented by Kerry et al. in 1964 . This caClinical diagnosis can be quite challenging. Hence, the need for high index of suspicion. Closer attention should be paid to pelvic, gluteal and radicular pain to the lower extremity, even in the absence of obvious gluteal mass. The role of imaging in the diagnosis is cardinal. Currently, CT scan and MRI scan of the abdomen and pelvis are the leading diagnostic imaging modalities .Computerized tomographic scans coupled with MRI are useful in identifying underlying tumors/masses. They also help to identify hernia contents. These modalities are also invaluable in defining surrounding or related anatomic structures. This helps to preoperatively evaluate the surgical techniques for repair and oncologic adequate resection (R0) as necessary. That is, transabdominal, transgluteal or combined approach ,12. For Most lipomas are classified by their proliferation of mature adipocytes with a benign structure in contrast to deep seated lipomas of the pelvic region which tend to grow more slowly as their invasive nature would demonstrate . Some au4We report a case of gluteal well-differentiated liposarcoma, which presented as sciatic hernia. While sciatic hernias are rare, lipomatous/ liposarcomatous tumors presenting as sciatic hernias are extremely rare. This case report highlights a combination of rare phenomena. So far, there are only six published cases of lipomatous/atypical lipomatous tumors that presented as sciatic hernias. A systematic approach to diagnosis, adequate oncologic resection of the well differentiated liposarcoma as well as treatment of the sciatic hernia via transgluteal approach are documented in this report. This information adds to the current evidence that will guide accurate diagnosis and appropriate treatment of future cases.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This study is exempt from ethical approval in our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Tolutope Oyasiji MD, MRCSI, MHSA, FACS: Study concept, literature review, drafting of the manuscript, review of the manuscript for important intellectual content and final approval of the version to be submitted.Andrew Gomez-Seoane BA: Study concept, literature review, drafting of the manuscript and final approval of the version to be submitted.This study is a case report.Tolutope Oyasiji MD, MRCSI, MHSA, FACS.Not commissioned, externally peer-reviewed.Nothing to disclose.


Intraocular manifestation of hematopoietic tumors is rare and often difficult to distinguish from inflammation. We report a patient with acute lymphoblastic leukemia (ALL) who developed intraocular infiltration during the remission period.A 40-year-old man presented with hypopyon in his right eye. Three months later, extensive subretinal infiltration and the elevation of intraocular pressure were observed. Fourteen months prior to this, he had been diagnosed with Philadelphia chromosome-positive ALL, and had received chemotherapy and bone marrow transplantation that resulted in complete remission.The breakpoint cluster region-Ableson (BCR/ABL) chimera was detected by polymerase chain reaction (PCR) analysis in the patient's aqueous humor. Additionally, a high expression of WT1 (Wilms tumor gene) mRNA in the aqueous humor was discovered. A bone marrow examination yielded a high expression of BCR/ABL fusion gene, and it was determined the patient had experienced a relapse of ALL.The dasatinib was administered orally to the patient.The intraocular infiltration disappeared, and intraocular pressure was normalized.Intraocular infiltration in leukemia patients may be an indication of relapse regardless of systemic conditions. Analyzing mRNA expression of BCR/ABL and WT1 of ocular fluid in patients with hypopyon is beneficial in diagnosing topical relapses in leukemia. The uveitis masquerade syndrome is commonly associated with hematologic malignancies. The major causes of uveitis masquerade syndrome are lymphoma and leukemia.,2 The ocular infiltration of malignancy is mainly diagnosed by cytologic examination followed by immunophenotyping using intraocular fluid such as the vitreous body or aqueous humor. Recently, it has been determined that the Wilms tumor gene (WT1) mRNA expression in blood and bone marrow is a prognostic marker in leukemia. However, the WT1 mRNA of the aqueous humor has never been examined.Hypopyon is the accumulation of infiltrating cells in the anterior chamber, and is a sign of uveitis. The 2 major causes of uveitis are autoimmune disease and infection, in both of which inflammatory cells are infiltrated in intraocular fluid. Occasionally, hypopyon is observed in malignant diseases such as hematologic malignant disease and retinoblastoma. The infiltrated cells in such instances contain malignant cells. Distinguishing whether the signs of hypopyon indicate inflammation or malignancy can be difficult.In this report, we describe a case presenting with unilateral leukemic intraocular infiltration as an initial sign of acute lymphoblastic leukemia (ALL) relapse. The chimeric breakpoint cluster region-Ableson (BCR/ABL) gene in the aqueous humor was detected by polymerase chain reaction (PCR) analysis. Moreover, the aqueous humor showed a high expression of WT1 mRNA. The present findings demonstrate that reveals the chimeric BCR/ABL gene by PCR and detects WT1 mRNA in the aqueous humor.2The medical records of a patient with intraocular infiltration with acute lymphoblastic leukemia were retrospectively reviewed. The present study protocol was approved by the Ethics Committee of the Sapporo Medical University Hospital and conducted in accordance with the Declaration of Helsinki. After a full explanation of the purpose and protocol for this study, patient has provided informed consent for publication of the case.2.1Our patient was a 40-year-old man who had developed systemic pain. He had initially visited another hospital 14 months earlier and had been diagnosed with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ALL). At that time, he underwent chemotherapy and bone marrow transplantation and achieved complete remission. However, a month after termination of the chemotherapy, he developed blurred vision in his right eye.The initial ophthalmic examination disclosed a best-corrected visual acuity (BCVA) of 8/200 in the right eye and 20/12.5 in the left eye. The intraocular pressure (IOP) was 45 mmHg in the right eye and 14 mmHg in the left. A slit-lamp examination demonstrated conjunctival injection, corneal oedema, and hypopyon in the right eye. An ophthalmological examination of his left eye was unremarkable. Results of the laboratory investigations of serum including antinuclear antibodies, anti-double stranded DNA antibodies, rheumatoid factors, and antineutrophil cytoplasmic antibodies were unremarkable. Peripheral blood cell counts were normal. Topical betamethasone and anti-glaucoma medication were applied resulting in normalized IOP to 10 mmHg and improved BCVA to 20/12.5 in his right eye a week later. There was no abnormality in his right eye fundus. Three months following the initial presentation, his right eye developed severe vision loss and ocular pain. Upon examination, the eye showed conjunctival injection, corneal edema, blood-streaked hypopyon, a shallow chamber, and extensive retinal detachment Figs. . The BCV There were no detection of herpes virus species, bacteria, fungi, toxoplasma, toxascaris, or any other pathogens. Next, the chimeric BCR/ABL mRNA and the expression of WT1 mRNA were examined, the details of which are described in the Methods section below.The aqueous humor from his right eye was collected. A multiplex PCR/broad-range PCR was applied to screen the pathogen causing the infection.2.1.1g for 5 minutes to harvest cells. RNA was extracted using the RNeasy Micro Kit . The RNA concentration was measured using a Qubit 3.0 Fluorometer . Complementary DNA was synthesized using the SuperScript IV VILO Master Mix (Thermo Fisher Scientific). Primer sequences for major BCR/ABL1 and WT1 are shown in Table  were used. For detection of major BCR/ABL1, the nested polymerase chain reaction (PCR) was performed using AmpliTaq Gold Fast PCR Master Mix (Thermo Fisher Scientific). SimpliAmp Thermal Cycler (Thermo Fisher Scientific) was used for amplification. PCR products were electrophoresed on a 2% agarose gel and analyzed using ChemiDoc XRS+ with Image Lab Software . For detection of WT1, real-time PCR was performed by LightCycler FastStart DNA Master SYBR Green I . LightCycler 2.0 (Roche Diagnostics GmbH) was used for amplification and analysis.The anterior aqueous humor collected from the patient was centrifuged at 2000 × 5 copies/μg RNA, and that was undetectable in the normal control aqueous humor (data not shown). The bone marrow examination that was performed a week later from aqueous humor paracentesis showed 93,190 copies/μg RNA of BCR/ABL mRNA and 7.1 copies/μg RNA of WT1 mRNA. Neither BCR/ABL nor WT1 mRNA expression was detected in the peripheral blood.The chimeric BCR/ABL was detected in the aqueous humor sample of this case Fig. . The aquIn light of these results, the patient was diagnosed as having a Ph+ALL relapse with intraocular infiltration. Subsequently, dasatinib (100 mg/d) was administered orally, resulting in the hypopyon resolution and IOP normalization Fig.  without 3In this report, we described a case of leukemic intraocular infiltration developing during the period when the patient was believed to be in remission. The ocular manifestation started as unilateral hypopyon that temporarily responded to topical corticoid application. It then extended to intraocular infiltration with retinal detachment without hematological relapse. The molecular biological examination of the aqueous humor resulted in the diagnosis of Ph+ALL relapse. The ocular manifestation was solely resolved with molecular targeted therapeutics.,6 Intraocular infiltration of malignant diseases is thought to occurred through direct infiltration of neoplastic cells, hemorrhage, or by ischemic changes. The phenotype of masqerade syndrome by malignant disease is usually uveitis like but occasionally other phenotypes such as retinal detachment and choroidal thickness are involved. In our case, extensive retinal detachment with high signal lesion in MRI was observed. This suggests the possibility that massive leukemic cells were infiltrated into the subretinal space.The causes of hypopyon vary and include infection, autoimmune diseases, and malignant diseases. The clinical manifestations are similar in resemblance among these causes and it is often difficult to distinguish them solely by ophthalmic examination. The hypopyon is the accumulation of infiltrated inflammatory cells in cases of infection and autoimmune disorders. The intraocular involvement of malignant disease is called masquerade syndrome that resembles uveitis but is resistant to corticosteroid therapy.–12 The etiology of ocular manifestation in leukemia patient is either opportunistic infection following immunosuppressive conditions caused by chemotherapy or leukemic cells infiltration. An accurate diagnosis is essential for precise treatment. In our case, no infectious pathogen was detected by PCR methods. There are several reports indicating intraocular involvement of Ph+ALL.–19 Interestingly, in most of these cases, ocular involvement developed during the remission period with normal blood cell counts. Ocular manifestation is commonly diagnosed with blood tests and characteristic ophthalmic findings. However, when the hematological analysis of blood or bone marrow shows less systemic abnormality, intraocular fluid cytology is required. Although topical corticosteroids were not fully effective to hypopyon in our case, there were no other systemic findings. Our oncologist remained uncertain that hypopyon was the sign of relapse until the results of the molecular analysis of the aqueous humor.The cause of malignant masquerade syndrome in adults is most often malignant lymphoma and leukemia and, more rarely, metastasis of cancer. It has been reported that 0.2% to 2% of leukemia patients develop leukemic infiltration such as hypopyon in the anterior segment of the eye.–18 Although FISH is a popular method to confirm the presence of the chimera gene, it requires a certain amount of cells and false positives can occur through overlap. Therefore, we decided to perform PCR, and were able to determine the BCR/ABL fusion gene consistent with the diagnosis of Ph+ALL. The eye is a small organ, and there are few tissues and samples, including intraocular fluid, that are safe to procure for examination. The PCR method is beneficial for small sample volumes and rapidity in noting results. Similar to our case, when there is no evidence of leukemia in the blood, molecular biological examination of intraocular fluid is favorable. These results led us to perform a bone marrow examination and ultimately, we were able to confirm relapse. There are several additional reports in which ocular involvement was the single sign of leukemic relapse, similar to our case.,16,18–21There are a few reports in which FISH (fluorescence in situ hybridization) was conducted to detect the BCR/ABL fusion gene in the aqueous humor. Additionally, the increase of WT1 expression was concomitant to the progression of chronic myeloid leukemia (CML) and myelodysplastic syndromes (MDS).,23,24 The expression level reflects the activity of leukemia that is independent from chimiric DNA. WT1 is thought to be one of the markers for leukemia, and that can be used for predicting prognosis and monitoring relapse. Recently, WT1 peptide vaccination have demonstrated positive survival efficacy on leukemia patients.–27 We have examined WT1 mRNA expression of the aqueous humor. Surprisingly, the expression was much higher than that of bone marrow during the same period, 2.9 × 105 copies/μg and 7.1 copies/μg of RNA respectively. It was also undetactable in the control aqueous humor. These results suggest that monitoring WT1 expression in intraocular fluid can be a good candidate marker to screen leukemic infiltration in the eye regardless of leukemia subtype. In addition, it appears that the extent of WT1 expression in the eye reflects the leukemic infiltration severity even under silent phenotypic general conditions.The WT1 gene was initially identified as a tumor suppressor gene, and the mutation of the gene led to the development of pediatric renal tumors. It was then determined that WT1 was overexpressed in the majority of acute meloid leukemia (AML) and ALL cases.The present findings suggest the importance of prompt diagnosis of leukemic infiltration in the eye using molecular biological techniques. Furthermore, it raises the possibility of WT1 expression as the marker for topical leukemia relapse.Conceptualization: Miki Hiraoka.Data curation: Miki Hiraoka, Hiroshi Ikeda, Daisuke Furuya.Formal analysis: Daisuke Furuya.Investigation: Miki Hiraoka, Hiroshi Ikeda.Methodology: Daisuke Furuya.Project administration: Satoshi Takahashi.Resources: Miki Hiraoka.Supervision: Hiroshi Ohguro.Writing – original draft: Miki Hiraoka.Writing – review & editing: Hiroshi Ikeda, Daisuke Furuya, Satoshi Takahashi.Miki Hiraoka orcid: 0000-0002-6411-2269.Hiroshi Ohguro orcid: 0000-0003-1052-0280.


Synovial sarcoma (SS) is a soft tissue neoplasm that rarely occurs in the vertebral body and should be considered in the differential diagnosis in patients with SS and vertebral lesions. SS often presents as a painless mass in the spine, which may undergo slow enlargement, resulting in sustained symptoms of neurologic deficit and pain. Due to the difficulty in differentiating between SS from other soft tissue tumors and metastatic tumors, careful histological confirmation is required for definite diagnosis. Furthermore, due to its malignancy, the appropriate treatment procedure for SS should be carefully considered.A 56-year-old female patient had low back pain. Radiological examination revealed bony erosion of the L-2 vertebral body, and no soft tissue mass around the lumbar spine.Histopathological and immunohistochemical examination revealed SS.The initial treatment of posterior laminectomy decompression and percutaneous vertebro plasty (PVP) was performed, however, this initial treatment course was inappropriate, but she eventually underwent L-2 complete resection and internal fixation. After the second surgery, she was treated by external beam radiation therapy.operation radiotherapy was finally performed. No local recurrence in L-2 vertebral body or distant metastasis was found at 1-year follow up postoperation; the neurologic symptom gradually relieved, and no other symptom was noted. And no local recurrence in L-2 vertebral body and distant metastasis was found in 1 year follow up postoperation.Solitary spinal SS is extremely rare. Early surgery for total resection and adjuvant radiotherapy/chemotherapy should be emphasized. Here, we report a rare case of SS arising in the spine.Synovial sarcoma (SS) is a rare malignant tumor. The diagnosis of SS is difficult, and is largely dependent on radiological examination and laboratory examination, including genetic and immunohistochemical examination. Complete surgical resection with negative margins is now regarded as the best treatment. Other treatment options, such as radiation therapy and chemotherapy, are also used However, the prognosis of SS is disappointing.2A 56-year-old woman experienced pain in her waist for >3 months. She occasionally experienced bilateral costal paresthesia and lower extremity or dysfunction and pain in the lower extremities and bladder. The most serious symptom was pain in the lumbar vertebrae, and oftentimes, this was the only symptom. She had no previous trauma or a history of similar symptoms. Radiography revealed hypointense bony erosion of the L-2 vertebral body and no soft tissue mass around the lumbar spine. Computed tomography (CT) revealed multiple circular nodes with homogenous density and clear boundaries in both lungs. Similar to the radiography findings, CT also revealed bony erosion of the L-2 vertebral body, and the density of the adjacent spinal canal was higher than that of the remaining spinal canal Fig. . No evid3,4 In previous literature, synovial sarcoma of the spine is rare, it occurs in lumbar spine more than other section. Due to the varying appearance of SS, differentiation of SS from other tumors, such as liposarcoma and fibrosarcoma is challenging. Pathologic diagnosis remains the “gold standard.” However, SS found in the spine is simply paraspinal SS, the primary occurrence of SS in the spinal vertebral body is extremely rare. The imaging features of spinal SS also hardly distinguish spinal metastases. Moreover, no special imaging features can be concluded. Due to SS is a malignant tumor, it also presents the behavior of recurrence and metastasis, especially occurs lung metastasis.,6,7 In the present case, CT and MRI clearly show that the primary tumor occurred in the L-2 vertebral body.The incidence of SS in soft tissue is relatively low, accounting for nearly 5% to 10% of all soft sarcomas and 1% of all malignancies. Theoretically, SS can occur anywhere in the human body, but the most common primary sites are near the large joints of the upper and lower extremities; >85% of SSs occur at these sites.,9 In the present case, during the first surgery, only bone cement was injected into the vertebral body, and completely excision was not performed. This, in combination with the patient's refusal to undergo radiotherapy/chemotherapy, resulted in poor local tumor control. In contrast to the first surgery, complete tumor excision was achieved during the second surgery and adjuvant radiotherapy was administered, which resulted in a better outcome. This result is similar to that of studies showing that age (≤20 years), tumor size (≤5 cm), a negative surgical margin after complete resection, and the administration of adjuvant radiotherapy are good prognostic factors in patients with SS.Consensus on the optimal treatment for SS has not been reached. Complete surgical resection with adjuvant radiotherapy and/or chemotherapy is the currently recommended treatment for SS.In summary, solitary spinal SS is an extremely rare occurrence. Early surgery for total resection and adjuvant radiotherapy should be also emphasized for rare spinal SS to ensure good outcomes.Methodology: Peng Guo.Project administration: Donglai Wang.Writing – original draft: Qi Feng, Peng Guo, Jian Lv.Writing – review & editing: Jiangang Feng.


Pancreatic mucinous cystic neoplasm (MCM) presenting with rupture is extremely rare, and very few studies have followed up patients over the long term after ruptured mucinous cystadenoma (MCA). We report a case of ruptured MCA of the pancreas with recurrence-free survival for 8 years.A 28-year-old Japanese woman was admitted to the emergency department of a local hospital after experiencing acute abdominal pain. Abdominal computed tomography revealed massive ascites and the presence of a cystic tumor measuring 60 mm in diameter in the pancreatic tail. Conservative therapy with antibiotics and abdominal drainage were performed to treat peritonitis that occurred secondary to the ruptured pancreatic cystic tumor, after which the patient’s symptoms improved. The patient was referred to our department for further examination and treatment. We diagnosed a ruptured MCN and performed laparoscopic spleen-preserving distal pancreatectomy. Histopathological findings revealed ovarian-type stroma, which tested positive for estrogen and progesterone receptors by immunohistochemistry. The histopathological diagnosis was MCA. The postoperative course was uneventful, and the patient remains alive without any evidence of recurrence at 8 years postoperatively.A good prognosis is possible even in cases of ruptured MCA. Because of the risk of peritoneal dissemination after ruptured MCA, long-term follow-up is important. Pancreatic mucinous cystic neoplasm (MCN) is characterized by mucin-producing columnar epithelium and ovarian-type stroma. MCN occurs almost exclusively in women aged 40–60 years. MCN is often localized to the pancreatic body or tail without affecting the pancreatic ducts . RuptureA 28-year-old Japanese woman was admitted to the emergency department of a local hospital after experiencing acute abdominal pain. Computed tomography (CT) imaging revealed massive ascites and the presence of a cystic tumor measuring 60 mm in diameter in the pancreatic tail Fig. a. ConserOn admission, her abdomen was flat and soft, and there was no abdominal pain. Laboratory tests showed no anemia, and liver and kidney functions were normal. Serum tumor markers CEA and CA19-9 were 0.8 ng/mL and 3620.9 U/mL, respectively. Enhanced CT imaging showed a tumor measuring 60 mm in diameter with an internal septal formation in the pancreatic tail Fig. . EndoscoAfter induction of general anesthesia, the patient was positioned in the supine position. Complete exploration of the abdominal cavity revealed no ascites or peritoneal dissemination. The soft tumor was identified in the pancreatic tail Fig. . To avoiThe patient remained in hospital for 10 days after surgery with no postoperative complications. The patient remains alive without any evidence of recurrence at 8 years postoperatively.Ruptured MCN is extremely rare because of its thick fibrous capsule . When weWHO defines MCN as “A benign cystic tumour composed of columnar mucin-producing epithelium supported by ovarian-type stroma” . The symThe cause of rupture has been reported to relate to the influence of female sex hormones on the behavior of MCN, especially during pregnancy , 4, 17. Regarding prognosis after rupture, recurrence occurred in the cases of MCC, and a local pattern of recurrence was present. Although no recurrence was observed in the cases of MCA, the follow-up periods were short, and the long-term prognosis remains unclear. Therefore, there is little evidence regarding prognosis after a ruptured MCN. The 5-year survival rates after complete resection of unruptured MCN for adenoma and adenocarcinoma were 98.8% and 86.5%, respectively , 12 and A good prognosis might be expected in patients with MCA even in those with rupture. However, there is a risk of peritoneal dissemination with rupture, and long-term follow-up is thus considered important. To our knowledge, the present patient has survived without recurrence for the longest period among the reported cases of ruptured MCN.


Hemangiopericytomas (HPC) are vascular tumors and can be found at any place where vessels are present. These were previously known as ‘extrapleural Solitary Fibrous Tumour’. These tumors may reoccur and metastasize after surgical excision. We present herein a HPC of the greater omentum, diagnosed as left inguinal hernia preoperatively.A 61-year-old male, presented with a huge painless mass in his left inguinoscrotal region secondary to weigh-lifting associated with malaise and vague abdominal pain. A well-defined, non-tender, and firm mass was found at the left lower abdomen extending to the left inguinoscrotal region. Based on the examination, a diagnosis of indirect inguinal hernia was made. Abdominal ultrasound showed a heterogeneous, hyporeflective, and vascularized mass. Contrast-enhanced computed tomography scan identified a localized, extraperitoneal, heterogeneously hypodense, well-defined, and lobulated mass, with marked contrast enhancement. On exploration, an encapsulated large mass originating from the omentum with enormously dilated blood vessels was excised. On histopathology, a neoplastic lesion, composed of spindle-shaped cells and moderate cytoplasm was identified. The blood vessels appeared thin-walled with a staghorn appearance in hemangiopericytic pattern. Omental sections showed fibro adipose tissue with dilated lymphatics and thick-walled blood vessels. Features were consistent with a malignant HPC of 20 × 14 × 10 cm.We present an unusual presentation of primary omental malignant HPC as an inguinal hernia, treated by complete surgical resection. These tumors are rare therefore, timely diagnosis is important for proper evaluation, diagnosis, and treatment. It also requires long-term follow up for better survival. •Hemangiopericytoma (HPC), previously known as ‘extrapleural Solitary Fibrous Tumor’, is a vascular tumor.•A well-defined, non-tender, and firm mass was found at the left lower abdomen extending to the left inguinoscrotal region.•Based on the examination, a diagnosis of indirect inguinal hernia was made.•CT scan showed a contrast enhancing, localized, extraperitoneal, heterogeneously hypodense, and lobulated mass.•On exploration, an encapsulated large mass originating from the omentum with enormously dilated blood vessels was excised.•On histopathology, features were consistent with a malignant HPC of 20 × 14 × 10 cm. A solitary fibrous tumor (SFT) is a rare primary omental malignant tumor that usually arises from the pleura. Extrapleural SFT, that can be seen in the head and neck, abdomen, pelvis, or skin includes extra-meningeal SFTs, hemangiopericytoma, lipomatous hemangiopericytoma, and giant cell angiofibroma. These can be benign or malignant, with an overall incidence of 0.6%. These tumors are common in the 5th and 6th decade of life .Hemangiopericytoma (HPC), previously defined by the World Health Organization as an extrapleural SFT, is a vascular tumor that is believed to arise from the pericytes of Zimmerman, showing evidence of myoid or myofibroblastic differentiation ,4,5.To the best of our knowledge, malignant hemangiopericytomas presenting as an inguinal hernia has not been reported before. There are 20 case reports about benign HPC but in literature, only four cases of malignant HPC have been reported . Here, a2A 61-year-old male, with no known comorbid, presented with a huge painless mass in his left inguinoscrotal region associated with malaise and vague abdominal pain for one month. He had a history of lifting a Camel after which a lump gradually developed. On abdominal examination, he revealed a well-defined, non-tender, and firm mass of 15 × 10 cm at the left lower abdomen involving the left inguinal region as well as the left hemiscrotum . Based oThe complete blood count and biochemical tests were within the reference values. Tumor markers i.e. Carcinoembryonic antigen, Alpha-fetoprotein, Carbohydrate antigen (CA) 19.9, and CA 125 were within range. Abdominal ultrasound showed a heterogeneous structure with hyporeflective areas and was highly vascularized. Contrast-enhanced computed tomography (CT) scan identified a mass at the left iliac fossa, adjacent to the anterior wall of the abdomen, but extraperitoneal, measuring 15 × 10 × 10 cm. The mass was heterogeneously hypodense, well-defined, and lobulated, with marked enhancement effect after contrast administration, comparable to vascular density, but no evidence of distant metastasis or lymphadenopathy .Fig. 2SaSurgery was planned under general anesthesia and the patient underwent an en-bloc local resection of the tumor via an inguinal approach. An inguinal crease incision was given over the swelling. On exploration, we found an encapsulated large mass originating from the omentum with enormously dilated blood vessels and a 5 × 5 cm hydrocele, which was excised and weighed around 1.5 kg. Testis was unremarkable A and B. On gross examination, an irregular dark brown to grey-white mass with prominent dilated vessels on the surface was seen. On sectioning, the cut surface of the mass was light brown with cystically dilated hemorrhagic spaces and separately lying omental tissue piece with prominent congested and dilated vessels measuring 17 × 17 × 5 cm. Microscopically a neoplastic lesion was visible, composed of spindle-shaped cells having moderate cytoplasm and bland nuclei without necrosis. The blood vessels appeared thin-walled with a staghorn appearance in hemangiopericytic pattern. Few mitotic figures were seen. No testicular parenchyma or epididymal tissue was reported. Omental sections showed fibro adipose tissue with dilated lymphatics and thick-walled blood vessels. Features were consistent with malignant HPC of 20 × 14 × 10 cm C.Immunohistochemical analysis showed strongly positive CD34 and patchy positive STAT 6 biomarkers while it was negative for cytokeratin, S100, ASMA, desmin, TLE-1, ER/PR, and EMA.The patient was discharged on 3rd postoperative day without any complications. His recovery after surgery was satisfactory. At 12-months follow-up, there was no evidence of recurrence or metastasis and he is alive and well.3. There are only four case reports (Primary benign HPC of omentum represents <2% of all soft tissue tumors and malignant HPCs represent <1% of all vascular tumors ,10. Ther reports  of maligImmunochemistry is not always diagnostic of HPC however, cell markers of normal pericytes, such as desmin and actin, are not usually found in HPC cells . They exBesides a detailed physical examination and laboratory testing, detailed imaging investigations should be performed for the diagnosis of HPC . MagnetiHPC is a tumor of vascular origin, for which a combination of surgery, chemotherapy, and radiotherapy is required. En-bloc surgical excision has been the cornerstone of treatment . RadiothFor accurate diagnosis and effectiveness of treatment, CT or ultrasound-guided biopsy and immunohistochemical staining should be performed preoperatively. This not only helps in the definitive diagnosis but also helps in knowing the extent and prognosis of the mass lesion ,17,18. L4We present an unusual presentation of primary omental malignant HPC as an inguinal hernia, treated by complete surgical resection. These tumors are rare therefore, timely diagnosis is important for proper evaluation, diagnosis, and treatment. It also requires long-term follow up for better survival.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.All the available data is presented.None to declare.None.The authors have no financial or proprietary interest in the subject matter of this article.Ethical approval was acquired from the involved institution i.e. Civil Hospital, to collect the patient details, and present it as a publication.None.Since there is no data analysis here, All authors i.e. Sana Shahid, Hina Khan, Muniba Mehmood, Khaled Abdullah Rage, and Summaya Saeed together did the concept and design of the study; data acquisition; and drafting of the manuscript. All authors critically revised the manuscript, approved the final version to be published, and agree to be accountable for all aspects of the work.Not applicable, since this is a case report.Muniba Mehmood.None.


A case of a skull base cholesterol granuloma (CG) of the squamosal temporal bone. This is the first ever reported case of CG in a well-pneumatized squamous temporal bone. Discussion. CG is a cystic mass typically found in the petrous apex and occasionally in the paranasal sinuses and orbit. Experience with the treatment of these expansile and inflammatory processes has largely been garnered from those occurring in the petrous apex, where they are surgically drained, via a transtympanic, transmastoid, or middle fossa approach. We report a case of cholesterol granuloma situated in the temporal fossa presenting as a temporal mass. The accessible location of this particular lesion made it amenable to total excision, avoiding the need for surgical drainage and possibility for recurrence.  Case report and review of the literature.  This case supports the theory of pathogenesis of such lesions typically occurring where pneumatized air spaces interface with bone marrow, in this case, where the reaches of pneumatized cells in the squamous portion of the temporal bone meet diploic bone. First described over 100 years ago, temporal bone CGs are defined pathologically by the presence of cholesterol crystals surrounded by multinucleated giant cells, round cell infiltration, and hemosiderin-laden macrophages .The mainstay of surgical management for the petrous apex CG is achieving and maintaining drainage. While there exists some controversy as to whether the cyst wall should be removed, simple drainage is the current accepted intervention due to the difficulty to access region of the petrous apex and its proximity to vital skull base structures . We descA 67-year-old female presented due to a left temporal swelling of 5 months. She denied history of trauma, discomfort with chewing, audiologic concerns, or history of infection. Physical exam revealed a 2.5 cm fluctuant mass of the left temporal fossa. Subsequent computed tomography (CT) scan of the temporal bone showed a 2.3 cm cystic lesion of the left squamosal temporal bone with disruption of the lateral bony cortex . MRI revCholesterol granulomas are expansile cystic lesions with the potential for osseous erosion that consists of cholesterol crystals within a fibrous capsule . While CCGs are treated with either surgical drainage or marsupialization, as access to these lesions can be difficult. Many occur in the petrous apex and necessitate complex surgical approaches, and a wait and scan approach has become accepted in many instances when tolerable . Complic


Background: About a fifth of all mediastinal masses are primary cysts arising in the absence of other underlying pathology. Bronchogenic cysts, although rare, are the most frequent type responsible for lower airways compression as they often develop in the peripheral branches of the tracheobronchial tree.Case presentation: We report the case of a 6-months-old child admitted for acute respiratory distress and wheezing not responsive to asthma treatment. Digestive and airway endoscopy proved a mild and a marked reduction of the esophageal and tracheal lumen, respectively. The nocturnal polygraphy showed an underlying obstructive disorder and the chest CT scan confirmed the presence of a wide mediastinal cyst compressing the trachea. The mass, later identified as a bronchogenic cyst, was surgically removed with complete resolution of the patient's respiratory symptoms.Discussion: Our case shows that differential diagnosis of wheezing in pre-school aged children should encompass causes others than airway reactivity, thus prompting further evaluation and management. Despite being one of the most common finding in infants and children, wheezing never ceased to be an alarming symptom for both the parents and the physician. It consists of a continuous sound heard during normal expiration or inspiration when airways obstruction is severe . A wheezA 6-months-old child was admitted at our hospital for acute respiratory failure. Her family history was positive for atopy. She was born at term from a vaginal birth and an uncomplicated pregnancy. Weight at birth was 2,790 g and respiratory distress in the immediate perinatal period was not reported. Recurrent asthmatic bronchitis not responsive to short course of inhaler bronchodilator and corticosteroids and growth retardation without clear symptoms of dysphagia  had occurred in the previous months. The parents reported a progressive worsening of the respiratory load. Physical examination showed dyspnoea, polypnea, prolonged expiration and wheezing worsening while eating. Chest X-ray revealed a normal tracheal air column and main stem bronchi with diffuse thickening of the bronchial walls, as for a non-specific inflammation of the bronchi and their surroundings. Blood tests and microbiologic analysis on respiratory secretions were negative. The echocardiography was limited by a poor acoustic window (due to significant air trapping) and was inconclusive for vascular rings. Based on the history of recurrent symptoms partially responsive to inhaler short term β2 agonists, the patient underwent airway and digestive endoscopy, which revealed a severe tracheomalacia at the T2-T3 level. Antero-posterior compression with a 1:1 ratio between the cartilage rings and the pars membranacea was observed. Anteriorly, the trachea appeared to be compressed by a pulsating mass. No abnormal communications between the airways and the digestive tract were found. Given the airway compression, a nocturnal polygraphy with overnight oximetry was carried out and proved the underlying obstructive disorder. In order to define the extrinsic compression and quantify the tracheal collapse, a dynamic chest CT scan with contrast enhancement was carried out and revealed a 4.0 cm wide mediastinal mass closely adherent to the anterior profiles of the first five thoracic vertebra, both compressing and dislocating the trachea and esophagus to the front and to the right, respectively . IntegraAbout 20% of mediastinal masses are primary cysts arising in the absence of other underlying pathology . Unless Despite the usual location of bronchogenic cysts within the mediastinum, the given one was well above the carina, compressing both the trachea and esophagus and leading to wheezing without feeding problems. On the basis of cyst's dimension, the symptoms were expected to appear earlier in life, but in our case no respiratory distress was noted at birth or immediately after. Treatment of mediastinal masses, such as the cyst we encountered, is hazardous and should always be managed by a multidisciplinary team. Along with bronchogenic cysts, there are a variety of causes of extrinsic compression of the trachea that may cause wheezing and mimic an asthmatic bronchitis in children, ranging from the more common mediastinal neoplasms to the less frequent vascular rings . TherefoWe obtained informed written consent from the patient's parent authorizing publication of clinical case and radiologic imagings.MR made data analysis, wrote the paper and approved the final manuscript. FP contributed to the writing of the paper, revised and approved the final manuscript. PF analyzed the bioptical specimen and made the final diagnosis, revised the paper and approved the final manuscript. AC performed the surgical removal of the cyst, revised the paper and approved the final manuscript. RC contributed to design, revised and approved the final manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.