Surgery is an effective treatment for desmoid fibromatosis, but it may be difficult, depending on the location or local spread of the tumor, and the decision to perform surgery must be made carefully. We herein report a case of desmoid fibromatosis of the chest wall in a young woman suspected of having invasion to the 1st, 2nd and 3rd ribs.A 35-year-old woman had been aware of dry cough and right chest pain, so she was referred to our hospital. Chest computed tomography showed a localized pleural tumor mainly at the first rib. Magnetic resonance imaging revealed a 75 × 65 × 27-mm tumor with a smooth surface, with partial contact from the first rib to third rib and partial extension to the 1st intercostal space. The tumor showed growth in the two months after the first visit, so resection was performed. The tumor was completely resected, and adjuvant radiation therapy (50 Gy) was performed for the small margin. The pathological diagnosis was desmoid fibromatosis. The postoperative course has been uneventful, without recurrence at 14 months after surgery.In chest wall tumors located ventral of the pulmonary apex, we suggest that a combination of the Grunenwald method and Masaoka anterior approach may be a useful option. In cases where margin is not enough, adjuvant radiation therapy should be considered. Desmoid fibromatosis are myofibroblastic neoplasms of muscle aponeurosis origin that arise from fascial or deep musculoaponeurotic structures. Surgery is still an effective treatment for desmoid tumors but may be difficult, depending on the location or local spread of the tumor. Furthermore, desmoid fibromatosis is locally aggressive and associated with high recurrence rates after surgical resection, with some studies reporting recurrence rates of 20% to 70% . In the Chest wall resection including the 1st rib requires an ingenious approach due to the complex anatomical characteristics. Cosmetic management is also necessary for young women. We herein report a case of desmoid fibromatosis of the chest wall in a young woman suspected of invasion to the 1st, 2nd and 3rd ribs.A 35-year-old woman who had smoked 5 pack-years had been aware of dry cough and right chest pain for 2–3 years, and pain in the right upper limb had gradually appeared. Her laboratory data revealed anemia Hb 10.0 g/dl). Chest X-ray demonstrated a mass in the right thorax. Chest computed tomography (CT) showed localized pleural tumor mainly at the first rib  were cut at the distal portion  was performed Fig. . The radIn the WHO Classification of Tumors, desmoid tumors are defined as a myofibroblastic proliferative soft tissue tumors and classified as an intermediate malignancy. The incidence in the general population is two to four per million people per year. Desmoid tumors are more common in patients 15–60 years old and more frequent in women than in men. The origin is typically intra- or extra-abdominal, such as the abdominal wall, shoulder, breech, or limbs. Extra-abdominal desmoid tumors have been attributed to β-catenin gene mutations.Surgery with extensive resection has been considered the major treatment, but the extremely high postoperative recurrence rate suggests the feasibility of nonsurgical treatments, as recommended by the European Organization for Research and Treatment of Cancer . The JapRegarding the operative procedure in the present case, the modified Grunenwald method  and TrapSome studies have suggested that adjuvant radiation may be useful for managing desmoids. This approach is particularly effective in cases of microscopically positive resection margins , 7 or reIn resection of chest wall tumors close to the pulmonary apex, due to complex anatomy, the approach requires an ingenuity. In such cases, a combination of the Grunenwald method and Masaoka anterior approach is recommended. When the margin is not enough, adjuvant radiation therapy should be considered.


The presence of prostatic tissue, in addition to uterus and adrenal tumors, is possible in 46XX patients with CAH. Lesions of these organs are usually benign. However, complications including prostate and adrenal cancer were also reported. The presence of prostatic tissue, in addition to uterus and adrenal tumors, is possible in 46XX patients with CAH. Lesions of these organs are usually benign. However, complications including prostate and adrenal cancer were also reported. However, little is known about the dual presence of prostatic tissue and uterus in 46XX patients with this condition. Since such association is extremely rare, it is relevant for clinicians to be aware of, in order to detect complications including cancers early.CYP21A2 gene, located on the short arm of chromosome 6. Although genotype‐phenotype correlation was well studied, it continues to display discordances.Congenital adrenal hyperplasia (CAH) is a group of enzymatic defects in adrenal steroidogenesis resulting in glucocorticoid insufficiency and excess of adrenal androgens.2A 40‐year‐old man presented to the endocrinology department with a history of abdominal pressure and an ultrasound showing the presence of adrenal mass and uterus. The patient is the youngest child of a nonconsanguineous parents. His brother died at the age of 5 for an unknown cause. His sisters are married and have children. The patient, phenotypically male, was raised as a boy. There was no suspicion of genital ambiguity at birth. His past medical history was significant for precocious puberty at the age of 6 and evaluation for nonpalpable testes at the age of 15.The patient identified himself as a male. On physical examination, he had a short stature height = 152 cm), normal blood pressure, 15‐cm abdominal mass in the left upper quadrant, pubic hair Tanner score of 5, empty scrotum, hypospadias, and stretched penis size of 7 cm, and there was no gynecomastia. On biochemical examination, the patient had normal serum potassium, creatinine, and cortisol levels. Total testosterone was 9.1 ng/mL . 17‐Hydroxyprogesterone (17‐OHP), dehydroepiandrosterone sulfate (DHEAS), and androstenedione levels were elevated  based on Sanger sequencing method.Ultrasonography, computed tomography scan (CT scan), and magnetic resonance imaging (MRI) of the abdomen and pelvis revealed left adrenal mass of 16.4 × 11.4 × 18.4 cm Figure . Gonads A diagnosis of classic CAH due to 21‐OHD was made, and the patient was proposed for hormonal treatment, unilateral adrenalectomy, hysterectomy, and gonadectomy (if found intraoperatively). We strongly suggested the need for performing prostate‐specific antigen (PSA) dosage and prostate biopsy since prostate cancers were described in these patients, as well as the necessity to screen parents and his sisters for mutation detection.321‐OHD is the most common cause of 46XX disorder of sex development. It is responsible for androgens excess, resulting in virilization and ambiguous genitalia. Internal genitalia are usually normal in these patients.The presence of prostate tissue in 46XX patients with CAH has been described in a limited number of cases Table . In the Our patient had Prader V external genitalia, which could explain the presence of the prostate gland. Calcifications found inside the prostatic tissue in our case were reported only once in a 64‐year‐old phenotypic male with 46XX karyotype.Adrenal masses are common in CAH, up to 82% in homozygous and 45% in heterozygous patients.A recent meta‐analysis confirmed the high prevalence of adrenal tumors (23.6%) in patients with 21‐OHD, which are most likely to be benign, particularly myelolipoma with a prevalence of 8.6% in those patients.Serum androgens levels were high in our case. In the study of Lee et al, 79% of patients with 21‐OHD had serum DHEAS levels above the normal range, without significant difference between salt wasting (SW) and simple virilizing (SV) forms, and all patients had serum androstenedione and testosterone levels above the normal range.CAH is a heterogeneous group of enzymatic defects with classic and nonclassic types. The SW phenotype is the most severe and common form.In our case, the patient had compound heterozygous mutation (I2G/R356W). R356W is a missense mutation in exon 8, while I2G is a splice site mutation in intron 2. The R356W mutation was associated usually with SW form, and the I2G mutation was associated with both SW and SV forms.In the cases cited in table To our knowledge, this is the first case reporting the presence of prostate in 46XX patient and the identification of I2G/R356W mutation in a Tunisian patient. The most frequent mutation in classical forms of 21‐OHD in Tunisian population was Q318X, with 26%‐35.3%. I2G and R356W mutations were present in 6%‐17.6% and 1%‐2.9% of cases, respectively. This distribution of mutation frequencies in Tunisian patients was reported to be different from other countries.In conclusion, we described a rare case of prostatic tissue in 46XX Tunisian patient with CAH due to 21‐OHD, in addition to reporting similar cases found in the literature. The presence of a prostate gland in females with CAH is well proven. Its frequency may be underestimated due in part to the lack of systematic screening but also to unawareness of physicians of the possible presence of prostatic tissue in those patients.None declared.Hamza ELFEKIH: involved in conception and design of study, literature search, and drafting of article. Asma BEN ABDELKRIM, Hajer MARZOUK, and Ghada SAAD: involved in design of manuscript, literature search, and drafting of article. Ayoub GASMI and Hounaida ZAGHOUANI: involved in radiographic image acquisition and interpretation. Moez GRIBAA: involved in genetic testing. Yosra HASNI and Amel MAAROUFI: involved in design of manuscript, drafting of article, and final approval.The patient consented to the reporting of this case in a scientific publication.


Ventricular septal rupture (VSR) due to blunt chest trauma (BCT) is rare in infants. Traumatic VSR should be considered in infants with acute congestive cardiac failure following blunt trauma to the chest. Echocardiography is the method of choice for diagnosis and guiding the management of VSR. In this case report, we present a case of VSR caused by BCT in a 1-year and 9-month-old infant, who was diagnosed by emergency bedside echocardiography. We also provide a mini-review of literatures on BCT-induced VSR in children. Ventricular septal defect (VSD) is the most common congenital heart disease . AcquireNon-invasive imaging of the intra-cardiac structure with high spatial and temporal resolutions can be obtained by echocardiography in children. Therefore, echocardiography is the modality of choice for the diagnosis and management of VSR , 8. BedsA1-year and 9-month-old male infant was presented with a history of being involved in a motor vehicle accident 5 h earlier in which he sustained blunt force chest trauma. From the time of admission to the 7th day, he was not on a ventilator, and the ECG monitor showed that his vital signs were stable. His breath was in a regular rhythm, and a Grade 2–3/6 systolic murmur was detected at the left lower sternal border. On the 7th day after admission, the vital signs of the infant were unstable: he was in respiratory distress (respiratory rate 70/min), and his heart rate was 145–155 times per minute. His blood oxygen saturation decreased to 78%. Scratches can be seen on the skin of the occipital scalp and the left forearm.Myocardial zymogram evaluations revealed that hypersensitive troponin 1 was increased to 10.281 μg/L, and the creatine kinase MB isoenzyme was increased to 7.29 μg/L. The bedside electrocardiogram showed pathological Q wave and ST segment elevation . Chest cEmergency bedside echocardiography demonstrated funnel-shaped muscular ventricular septal defect (M-VSD). The diameters of the left and right ventricular shunt orifice were 10 and 5 mm, respectively . Color DThe infant underwent cardio-surgery to occlude the M-VSD with an occluder  under direct vision, which was monitored by trans-esophageal echocardiogram. After occlusion of the M-VSD, the ventricular aneurysm on the left ventricular posterior wall was repaired surgically by intermittent pad stitching. The mitral valvuloplasty and foramen ovale suture closure were performed under cardiopulmonary bypass. The intraoperative findings were consistent with those of echocardiography. Both immediate trans-esophageal and trans-thoracic echocardiogram showed no residual shunt after operation .2 96–99%). The cardiac functions of the infant were normal (EF 57% FS 29% E/A 1.4 IRT 42 ms). On post-operation day 13, he was weaned off the ventilator and was extubated. On the 16th day after operation, the blood oxygen saturation rate was decreased (the lowest level reached 27%). The infant was in a critical condition, and tracheal intubation was performed on the patient. His parents decided to give up treatment.From post-operation day 1–16 the infant had been in a stable condition (T 36.8°C, R 30/min, HR 131 times per minute, BP 90/54 mmHg, SPOTraumatic VSDs present in 2–10% of BCT cases from motor vehicle accidents, and children are more prone to VSR due to the pliability of the immature chest wall , 9, 10. Congenital VSD usually occurs adjacent to the membranous septum . The mosThe case described herein is similar to the acquired VSR cases due to BCT previously reported in children –7, 10. SWe present the case of a child with VSR following blunt chest trauma due to a motor vehicle accident. The M-VSD was occluded with invasive closure, and ventricular aneurysm was surgically repaired. To our knowledge, similar co-occurrence of these features has never been reported.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Informed consent was obtained from the child' legal guardian for the publication of any potentially identifiable images or data included in this article.XJ, CW, KJ, KB, and YW participated in the diagnosis and treatment of the case. XZ collected clinic data. XZ and HH prepared the manuscript. All authors read and approved the manuscript as submitted.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.


Paragangliomas are rare neuroendocrine tumors originating from chromaffin cells of extra-adrenal origin. Ninety percent of adrenergic tumors originate in the adrenal medulla and are known as pheochromocytomas; the remaining 10% are extra-adrenal and are called paragangliomas. Mediastinum paragangliomas is rare and commonly originate from the posterior mediastinum, while those originating from the middle posterior are quite rare. Some paragangliomas secrete catecholamines, leading to symptoms such as hypertension, tachycardia, and diabetes.A 76-year-old woman visited our hospital for the treatment and further evaluation of diabetes. Her hemoglobin A1c levels had risen to 11.0%. To investigate the cause of her diabetes, a contrast-enhanced chest computed tomography scan was performed, revealing a ring-enhancing tumor (30 × 30 mm) in the middle mediastinum. The surgical resection was performed via video-assisted thoracic surgery. Surgery was performed using a vessel-sealing device; however, bleeding was persistent from the surrounding tissue. Total bleeding was 400 g. Blood pressure fluctuations and arrhythmia did not occur during the operation. The patient’s uncontrolled diabetes was cured after the surgery, and the tumor was diagnosed as a functional paraganglioma.We encountered a rare case of functional paraganglioma located in the middle mediastinum. Functional paragangliomas should be considered as a potential cause of uncontrolled diabetes, and a whole-body CT scan should be performed to investigate this possible cause. Paragangliomas are rare neuroendocrine tumors originating from chromaffin cells of extra-adrenal origin. Ninety percent of adrenergic tumors originate in the adrenal medulla and are known as pheochromocytomas; the remaining 10% are extra-adrenal and are called paragangliomas –4. MediaA 76-year-old woman visited our hospital for the treatment and further evaluation of diabetes. She had complained of malaise for several months and was diagnosed with diabetes in another hospital. She had a history of hyperlipidemia in addition to diabetes, and was treated with oral medication. Her blood tests were within the normal range except for her hemoglobin A1c levels, which had risen to 11.0%. Her performance status was normal, although she had malaise and anorexia. Her blood pressure was 114/72 mmHg and pulse was 80 beats/min. Her body weight was 54.4 kg. Urinary glucose was not detected. Her electrocardiogram was within normal limit. She had not been treated with adrenocorticotropic hormone (ACTH) and cortisol.A contrast-enhanced chest computed tomography (CT) scan was performed for the whole-body examinations, and revealed a ring-enhancing tumor (30 × 30 mm) in the middle mediastinum Fig. . An endoSurgical resection was performed using a left-sided video-assisted thoracic surgery (VATS) approach. The tumor was located between the aorta and the left pulmonary artery. The surgery was performed using a vessel-sealing device; however, bleeding was persistent from the surrounding tissue. Total bleeding was 400 g. Blood pressure fluctuations and arrhythmia did not occur during the procedure, and the tumor was completely resected. Systolic blood pressure remained around 120, although it temporarily rose to 160 immediately after the start of the surgery. In addition, intraoperative blood sugar was stable, and it had been around 130 to 160. The postoperative course was generally good; however, the patient had hoarseness that seemed to be a left recurrent nerve palsy. She was discharged 6 days after surgery.Histopathological examination of the tumor is shown, and it was 3.5 × 3.5 × 1.5 cm in diameter Fig. a, b. HemThe present case illustrates some important findings. First, the patient’s uncontrolled diabetes was completely cured after resecting a functional paraganglioma originating from the middle mediastinum.Beninato et al. reported the outcomes of 153 patients with pheochromocytoma who underwent surgical resection, and diabetes was observed in 36 (23.4%) patients [Paragangliomas originating from the middle mediastinum are rare, but they should be considered if a strongly contrasted tumor is detected in a contrast-enhanced chest CT scan. It is recommended that measurements of plasma-free metanephrines or urinary fractionated metanephrines be tested if a paraganglioma is suspected . LendersWe consider that it is important to perform whole-body examinations in patients with uncontrolled diabetes, because functional paraganglioma may cause diabetes. Paraganglioma should be considered if a ring-enhancing solid tumor is detected, and biochemical testing, such as measurements of plasma-free metanephrines or urinary fractionated metanephrines, should be performed.Some reports indicate that a biopsy, such as EBUS-TBNA, before a surgical resection is dangerous because of the risk of bleeding or of blood-pressure alterations , 9. ThusWe encountered a case of functional paraganglioma located in the middle mediastinum, and after surgical resection, the patient’s uncontrolled diabetes was completely cured. Functional paragangliomas should, therefore, be considered as a potential cause of uncontrolled diabetes, and a whole-body CT scan should be performed to investigate this possible cause.


Bone metastases in cholangiocarcinoma are uncommon. We report the case of a patient with disseminated osteolytic lesions who was admitted to the Neurology Department for progressive paraparesis. On the computed tomography examination, specific features for cholangiocarcinoma were described, confirmed later by the histopathological aspect of the bone lesions. Cholangiocarcinoma (CCA) is a malignant tumor, and its origin can be the liver or the extrahepatic bile ducts . UsuallyA 60-year-old male patient, a previous smoker and alcohol user, was admitted to the Neurology II Clinic exhibiting progressive paraparesis for over six months, ostealgia, muscular pain, and paresthesias in the lower extremities. The symptoms have had a gradual onset and have increased in the past three weeks before the presentation due to an accidental falling episode. The patient underwent right femoral surgery in 1996, and a non-MRI compatible metallic plate was placed. The pelvic, femoral and lumbar spine radiography performed in the emergency department showed multiple osteolytic lesions. The cerebral CT scan  revealedThe first neurosurgical consult recommended a spine MRI exam. However, this was not possible due to the non-MRI compatible right femoral metallic plate. The neurological examination revealed: flaccid paraparesis, and gait was possible within small distances, showing generalized muscular atrophy.An extensive blood panel was performed: PSA, alpha-fetoprotein, carcinoembryonic antigen and carbohydrate antigen 19-9 which had normal values, microscopic blood sample with normal characteristics, alkaline phosphatase and gamma-glutamyl transferase – increased values 169 U/L respectively 126 U/L, serum protein electrophoresis – slightly increased alfa 2 globulins , LDH was slightly increased: 254 U/L, mild hyposideremia. The urine analysis showed increased 24-hour proteinuria - 226,5 mg/24h, but IgA, M and G had normal values. An electroneuromyography examination was performed, and it revealed sensitive axonal polyneuropathy. Due to the radiological characteristics of the osteolytic lesions, the high suspicion of multiple myeloma was raised, and therefore, the patient was referred to the hematology department. The diagnosis of multiple myeloma or POEMS  syndrome was excluded .The contrast-enhanced thoracic-abdominal-pelvic CT scan showed multiple osteolytic lesions: in the right iliopubic bone, right and left iliac bone, left femoral bone, the L4 vertebrae  and the The patient underwent left frontoparietal craniectomy, and a week later, he suffered D7 and L4 vertebrae decompressive hemilaminectomy and removal of the D4 and L4 vertebral osteolytic lesions. However, a few days later, during passive movement, he suffered a left proximal femoral fracture. Closed reduction and osteosynthesis were performed. Postoperatively, he was transferred to our clinic. Upon neurological reevaluation, there was a substantial decrease in the pain level.The histopathological examination of the bone samples revealed the following aspects: macroscopically, several yellow-gray fragments were examined and focally rigid–bone areas, some friable, were noticed; microscopically, various bone trabeculae and cartilage, diffusely infiltrated by a tumoral proliferation composed of small and medium tumoral cells with round or oval, hyperchromatic or vesicular nuclei, with marked desmoplasia, reduced inflammatory infiltrate and decreased areas of necrosis and hemorrhage  were seeCholangiocarcinomas (CCAs) are malignancies of the biliary duct system . BiliarySeveral risk factors for cholangiocarcinoma were identified: cholestatic liver diseases, liver cirrhosis, biliary stone disease, diseases caused by toxins - alcohol, tobacco, chemical toxins, metabolic conditions . In thisGenerally, the evolution of the disease is prolonged; patients may experience weight loss, jaundice, abdominal pain or discomfort, and sometimes night sweats . In thisFew cases of bone metastasis from CCA are described in the literature, and the most commonly reported sites were the humerus, the fibula, the femoral bone , and theConsidering that the path to diagnosis started from secondary injuries, the difficulty of this case was to find the source. Osteolytic lesions occur most often in multiple myeloma, melanoma, renal cell carcinoma, non-small cell lung cancer, thyroid cancer, Non-Hodgkin lymphoma, thyroid cancer, in Langerhans-cell histiocytosis and rarely in cholangiocarcinoma ,13. In tAnother tool to help diagnose CCA are tumor markers, but in this case, the values of these markers were within the normal range, despite the advanced stage of the disease. Taking into account that the patient was in an advanced stage of the disease, it is essential to have a histological or cytological confirmation, which can be obtained through endoscopic ultrasound or percutaneous biopsy, ultrasound-guided, depending on the location of the lesion . For thiThe CCA median survival is about 12 months. The prognosis is unfavorable in CCA because of the lack of response to chemotherapy and radiotherapy , 15. TheTherefore, the approach, in this case, should be multidisciplinary and should include an oncologist, gastroenterologist, psychologist, radiotherapist, neurologist, neurosurgeon, and orthopedic surgeon.Cholangiocarcinoma with bone metastases is a rare condition with an unfavorable prognosis. Unfortunately, most patients present in advanced stages when the tumor is unresectable because of the silent evolution and the nonspecific symptomatology. This case of advanced biliary tract cancer with neurological symptomatology – pain and paraparesis- highlights the unusual site of metastases for cholangiocarcinoma. It also shows the difficulty of obtaining a diagnosis when facing nonspecific symptomatology and also marks the importance of a multidisciplinary approach.The authors declare that there is no conflict of interest.We want to thank the Histopathology Department of the Cluj-Napoca County Emergency Hospital for their involvement.


Chondrosarcoma is a unique kind of tumor that originates from the cartilage-producing neoplastic mesenchymal cells and appears in both the appendicular and atlantoaxial skeleton. It is the second most prevalent neoplastic bone tumor, with an occurrence of 0.79/100,000/year. The biological presentation of this cancer fluctuates extensively, depending on the grade and anatomical location. Since chondrosarcoma is predominantly resistant to conventional chemo- and radiation therapy, surgical resection remains the sole curative treatment, although at present new treatment modalities are under investigation. Chondrosarcoma (CS) is the second most frequent primary solid neoplastic tumor of the bone after osteogenic sarcoma . It is aThe patients with CS generally complain of and present signs and symptoms of swelling or palpable mass with increasing pain and fracture due to weakened bone . CS rougHere we report the history, diagnostic evaluation, and management of a patient presenting with anterior chest wall mass pathologically identified as CS of the ribs.A 45-year-old male, with weight of 74.5 kg and height of 178 cm, presented with an enlarged anterior chest wall solid mass just lateral to the sternum. CT imaging confirmed expansile osseous lesion with cortical destruction arising from the costochondral junction involving the fifth rib with extension up to the fourth and sixth ribs. No metastatic disease was found on chest CT, and PET scan confirmed no distant disease. Biopsy revealed CS, and radical resection procedure was performed.Gross pathological examination of the specimen revealed a fragment of the ellipse of skin, soft tissue, and bone measuring 9.0 x 7.5 x 6.0 cm Figure . Cut secMicroscopically, the tumor size is 5 cm, the mitotic rate is 1/10 HPF, and well-differentiated low-grade chondrosarcoma with staging pT1, pNx is present. On microscopic examination, atypical chondrocytes Figure , bone inChondrosarcoma is a combined title for a cluster of heterogeneous, usually slow-growing, primary neoplastic tumors of the bone . More thCSs that arise unrelated with a prior lesion are called primary CSs, whereas the secondary ones develop from a pre-existing benign cartilage tumor such as an osteochondroma or enchondroma. Additionally, they are classified as central when they originate within the medullary cavity and as peripheral when they grow from the bone surface, e.g., the cartilage cap of an exostosis. Primary CS is almost consistently central, whereas secondary CS can be central or peripheral .The neoplastic primary bone tumor is made up of chondrocytes with varying degrees of malignancy Table . CSs areØ Primary CSs, which include the following:· low- and high-grade dedifferentiated CSs· clear cell CSs· mesenchymal CSsØ Secondary CSs, which originate from benign cartilage lesions including the following:· osteochondromas · multiple hereditary exostoses  · enchondromas · Ollier's disease · Maffucci syndrome Variants of chondrosarcomasPeriosteal ChondrosarcomaThis is an uncommon type accounting for 1-2% of all cases of CS . The devMesenchymal ChondrosarcomaThis type, accounting for 2-3% of all CSs, consists of an undifferentiated cell component with well-differentiated cartilaginous regions . Usual sClear Cell ChondrosarcomaAn uncommon type (2%) of CS, these lesions are differentiated by their cytology, epiphyseal area in long bones, and sluggish growth . The mosDedifferentiated ChondrosarcomaThis form accounts for 10-12% of all CSs. It is described by unique histology and a very unfavorable prognosis. The biphasic tumor correlates low-grade CS with a non-chondroid high-grade sarcoma. This tumor is very destructive, especially the metaphyseal or diaphyseal variants. The osteolytic lesion may predominate, but most of the lytic area is associated with calcifications, leading in the bi-morphic pattern. A giant soft tissue mass without calcifications seen on CT or MRI additionally demonstrates this diagnosis. Imaging assists in coordinating biopsy of the lytic area to improve the histological finding. The treatment includes adjuvant chemotherapy or radiotherapy and surgery. The prognosis is very bad, with an overall five-year survival rate of only 8.5-13% .Secondary ChondrosarcomaOf all CSs, 12% are developed in a pre-existing lesion. As its name indicates, it may be secondary to a solitary osteochondroma, Paget’s disease, osteochondromatosis, enchondromatosis (Ollier’s disease), fibrous dysplasia, irradiated bone, or synovial chondromatosis . The preConsiderable new insights into the molecular cell biology have been growing in the ongoing past. Cytogenetics and immunohistopathology contribute to a deeper understanding of the nature of CSs at the molecular level, which will potentially lead to a better clinical understanding and likely to better-targeted care .Although surgery is an effective treatment option for CS, it is essential to prepare a secure surgical field and perform reconstruction by considering the location, resection, onset, and re-occurrence rate. Due to the possible likelihood of late local and systemic relapse, patients who were treated by surgical resection should undergo standard surveillance and screening for their whole life. Surveillance should comprise physical examination and thoracic imaging with either posterior-anterior/lateral chest radiograph or CT scan every three to six months for the first 5 years and afterward annually for a minimum of 10 years.


No complications such as infection, corneal thinning or graft dislocation were seen. The patient was satisfied with his right eye and demanded the same surgery for his left eye. We suggest vertical split simultaneous CAT as a safe and useful surgical method for the treatment of recurrent double-headed pterygium; however, longer follow-up is required to confirm the outcome.Pterygium is a frequent corneal disease characterized by growing of fibrovascular tissue from the bulbar conjunctiva onto the cornea. Although the causes of pterygium are not obvious, sun exposure is closely correlated with its development. Pterygium, especially double-headed pterygium is mostly seen in warm climate and in individuals who work outdoors. A minority of pterygium is double-headed . Bilateral recurrent double-headed pterygium is a very rare condition. Here, we reported a 35-year-old male patient with bilateral recurrent double-headed pterygium. A brief review about the recent literature concerning the etiology, associated risk factors, operation types and management of patients with recurrent pterygium was also discussed. The patient was a field worker, smoker and had a family history of pterygium. There was no ocular surgery history except pterygium surgery 15 years ago in both eyes. A successful pterygium excision was performed under local anesthesia with the vertical split conjunctival autograft transplantation (CAT) in both temporal and nasal parts of the right eye (randomly) for cosmetic disfigurement and avoidance of recurrence. The pathological report was consistent with pterygium. At postoperative first week, first and 1.5 Pterygium is one of the leading causes of blindness and visual disorder , 2. It iIn this manuscript, an interesting rare case of a bilateral double-headed recurrent pterygium and vertical split conjunctival autograft transplantation (CAT) simultaneous surgery  was presented. A brief review about the recent literature published in Pubmed without language and time restrictions concerning the etiology, associated risk factors, operation types and management of patients with recurrent pterygium was also discussed.A 35-year-old male patient, referred to our clinic for his bilateral recurrent double-headed pterygium for 15 years. He was a field worker, smoker and had a family history of pterygium. He told that his mother and sister had also pterygium. His visual acuity was full in both eyes. On slit-lamp examination, he had bilateral recurrent double headed pterygium. Slit photo of the patient was taken . In the We decided to apply surgery randomly to the right eye a week later following consulting the patient. Successful excision for double-headed pterygium was made under local anesthesia in the right eye after receiving a signed informed consent. Following instillation of topical proparacaine hydrochloride 0.5% , the sterile preparation and draping were applied to the right eye, and then a lid speculum was inserted. The bodies of the nasal and temporal pterygium were marked. Temporal pterygium was larger and excised firstly. Subconjunctival lidocaine hydrochloride + 2% epinephrine was injected into the pterygium head. The pterygium was excised from the cornea by using toothed forceps and crescent blade. Complete dissection of the pterygium body from the sclera, subconjunctival Tenon’s tissue and surrounding fibrovascular tissues was performed with the Westcott scissors. Homeostasis was achieved without using cauterizing. The conjunctival edge was trimmed. The corneal surface was gently scraped with a scalpel. After performing the same procedure on the nasal pterygium, a single piece of conjunctival tissue was taken from the superior bulbar conjunctiva of the same eye and divided into two pieces vertically based on the area of the removed pterygium and placed leaving no open scleral tissue starting from the nasal and temporal limbus, with the epithelial side up and the limbal area aligned with the limbus. Drugs to avoid recurrence like mitomycin C or 5-fluorouracil were not used. Both grafts were sutured to the surrounding conjunctiva by interrupted 7/0 vicryl. The excised pterygium specimen was sent for pathological investigation. Photograph of the right eye was taken at the end of operation . FollowiThis patient was presented because of the rare incidence of bilateral, recurrent and double side corneal involvement. A successful pterygium excision was performed under local anesthesia with the vertical split CAT to both temporal and nasal parts of the right eye for cosmetic purposes and avoiding recurrence. Ethnicity is associated with development of pterygium. Chen T et al.  showed tDouble-headed pterygium enforces the surgeon about a simultaneous or two-stage surgery , 4. DiffYeung SN et al.  performeAidenloo NS et al.  suggesteAnti-fibroproliferative application with 5-fluorouracil or mitomycin C may be a useful adjunct in primary pterygium surgery , 29. HowCorneal perforation related with dellen is a rare but important complication of a pterygium excision. A 60-year-old man with these complications was reported by a bare sclera technique without adjunctive therapy .  CAT isPterygium formation was found strongly associated with its location and laterality [36]. our patient had a bilateral pterygium and did not have amblyopia or eccentric fixation. A case of a free cyst of iris, bilateral pterygium and symmetrical nodosity of the auricle has been previously published . There wGreater protrusion degree might be a risk factor for occurrence of pterygium . Here, tTo the best of our knowledge, this was the second case presentation of bilateral double double-headed pterygium with successful short term management. We had some limitations. Firstly, the pterygium excision for the left eye has not been planned yet and post-operative follow-up period is not long enough. It would be great if we could present both eyes after surgery and long follow-up. Secondly, a bilateral pterygium was presented in a 50-year-old male with H syndrome , patientWe suggest vertical split simultaneous CAT as a safe and effective surgical technique for the treatment of recurrent double-headed pterygium. Family history, sunlight exposure, male gender and smoking were the main significant risk factors for pterygium in our patient. Postoperative medication is also important and using sunglasses for protection from ultraviolet should be advised to patients with pterygium to lessen the recurrence probability.Ethical issues have been completely observed by the authors. All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship of this manuscript, take responsibility for the integrity of the work as a whole, and have given final approval for the version to be published. No conflict of interest has been presented. Funding/Support: None. The datasets analyzed during this study are available from the corresponding author on reasonable request.


Treating hernias is one of the oldest challenges in surgery. The gallbladder as content in the case of abdominal hernias has only been reported in a few cases in the current literature. Cholecyst has only been described in the content of an inguinofemoral hernia in one case to date.A 73-year-old female patient was admitted to the Emergency Department due to complaints in the right inguinal area, which had started 1 day earlier. The patient complained of cramp-like abdominal pain and nausea. Physical examination confirmed an apple-sized, irreducible hernia in the right inguinal region. Abdominal ultrasound confirmed an oedematous intestinal loop in a 70-mm-long hernial sac, with no circulation detected. Abdominal X-ray showed no signs of passage disorder. White blood cell count and C-reactive protein level were elevated, and hepatic enzymes were normal in the laboratory findings. Exploration was performed via an inguinal incision on the right side, an uncertain cystic structure was found in the hernial sac, and several small abnormal masses were palpated there. The abdominal cavity was explored from the middle midline laparotomy. During the exploration, the content of the hernial sac was found to be the fundus of the significantly ptotic, large gallbladder. Cholecystectomy and Bassini’s repair of the inguinal hernia were performed safely.Following a review of the literature, it can be concluded that the finding of incarcerated gallbladder in the content of an inguinal hernia is a rare finding. No other similar emergency case and successful surgical intervention have been reported before. Treating hernias is a routine and common surgical intervention and one of the oldest surgical challenges in the world.The majority, 80%, of hernias of the abdominal wall are inguinal hernias . The mosOur case report summarizes the diagnosis, surgical treatment and perioperative care of this rare case in relation to a review of the current literature.In our case report, a 73-year-old female patient was admitted to the Emergency Department for complaints in the right inguinal area, which had started one day earlier. The patient complained of cramp-like abdominal pain and nausea. Bowel movements were normal, and the patient had no fever. From the patient's medical history we should highlight that a right inguinal hernia had been diagnosed years before. At that time surgical consultation had also been involved. However, due to the patient’s severe cardiovascular co-morbidities , the cardiology and preanesthesia consultations determined elective surgical intervention to be very high-risk and the elective operation was therefore contraindicated. In addition the patient had hysterectomy, appendectomy, Alzheimer’s disease and cholelithiasis in her medical history.The physical examination confirmed an apple-sized, irreducible hernia with tenderness to abdominal palpation in the right inguinal region. Skin redness was not detected. Scars from the midline laparotomy and McBurney’s incision were observed. Abdominal ultrasound confirmed an oedematous intestinal loop in a 70-mm-long hernial sac protruding via a 30-mm hernial orifice in the projection of the palpated lesion Fig. . Fluid wExploration was performed via an inguinal incision on the right side. In contrast to the abdominal ultrasound finding, an uncertain cystic structure was found in the hernial sac and several small, solid, abnormal masses were palpated in this structure Fig. a. The leCephalosporin and metronidazole antibiotic therapy and prophylaxis of thrombosis with low molecular weight heparin were administered during the perioperative period. Temporary confusion developed during the postoperative period due to the patient’s Alzheimer’s disease and the stress of the acute surgery; however, no surgical or other organic lesion was found in the background. The drain was removed on the first postoperative day, and oral nutrition was started gradually. Follow-up laboratory findings showed no significant abnormalities. The patient was discharged on the fourth postoperative day, with no signs of inflammation in the wounds, normal passage and no complaints. During the nine-month-long follow-up visits, the sutures were removed and the patient had no significant complaints. Recurrent hernia was not detected. Histology of the gallbladder showed acute cholecystitis with incipient haemorrhagic necrosis in the 210-mm-long sample (Fig. Incarcerated inguinal hernias are among one of the most common emergency problems in general surgery. Incarcerated hernias represent about 5–15% of operated inguinal hernias  and 1.7%Until now, only Rodrigues et al. published a case report where the gallbladder had been described in the content of an inguinofemoral hernia . The galIn the majority of the cases, the surgical method used was laparotomy. Only Paolino et al. and Trotta et al. reported cases of laparoscopies, and, in their cases, the gallbladder was found in the content of hernias of the anterior abdominal wall. In the former study, it was combined with Mirizzi’s syndrome , 18. OurAfter reviewing the literature, it can be concluded that an incarcerated gallbladder in the content of an inguinal hernia is a rare finding, with no other similar case reported beforehand to the best of the authors’ knowledge. The value of our finding is increased by the fact that a curative surgical intervention was successfully performed on our patient despite her multiple co-morbidities.


Primary yolk sac tumor (YST) is an infrequently-diagnosed malignant extragonadal germ cell tumors. It is likely to recur locally and may present with widespread metastases once diagnosed. Primary YST of the head is uncommon but can cause severe complications, such as loss of vision once the tumor mass invades the optic nerve.A 20-month-old boy presented to the general clinic of the local children's hospital with a complaint of swelling of left face for 1 year and proptosis of the left eye for over 2 weeks as stated by his parents. Initially, he did have some vision, as he could walk by himself, but a special ophthalmologic examination was not performed.Cranial computed tomography and magnetic resonance imaging revealed a large tumor accompanied by peripheral bone destruction in the left pterygopalatine fossa that extended to sphenoid, ethmoid, left maxillary sinuses, left nasoethmoid, and left orbit. The optic nerve was invaded on both sides. Chest and abdominal imaging were normal. A primary diagnosis of Langerhans cell hyperplasia was made. However, blood tests on the second day of hospitalization revealed significantly elevated serum alpha-fetoprotein levels. On the third day, the boy lost his eyesight, with loss of pupillary and no light sensation during flashlight stimulation on both sides.Nasal endoscopy was performed on the fourth day, the vast majority of soft tissue mass was resected for biopsy. Histopathological examination revealed features of endodermal sinus tumor. A final diagnosis of primary YST of pterygopalatine fossa was made. Because the mass could not be resected completely, he received combined chemotherapy with bleomycin, etoposide, and carboplatin for 6 cycles over six months.The patient recovered with significant tumor shrinkage and without secondary metastasis after 18 months but left permanently blind.The worst complication of loss of vision after Primary YST of pterygopalatine fossa alerts us that close physical examination during the initial investigation should be performed, which is especially important in young children who cannot express complaints well. Early detection and treatment with surgical resection and chemotherapy may contribute to satisfactory outcomes and avoidance of visual impairment. In this report, we present a case of YST of pterygopalatine fossa in a child who ultimately developed blindness, although the tumor was satisfactorily treated with combined surgery and chemotherapy. Physicians and parents should learn a lesson from this case.Primary yolk sac tumor (YST) is an infrequent malignant germ cell tumor that mainly originates from the gonads and exhibits a high degree of tissue invasiveness. Alpha-fetoprotein (AFP), imaging methods, and histopathological examination are effective diagnostic and monitoring approaches for the comprehensive management of YST. The incidence of primary YST of head and neck is only 5%.2A 20-month-old boy was referred to the general clinic of the local children's hospital with a complaint of swelling of left face for 1 year and proptosis of the left eye for over two weeks as stated by his parents. Initially, he did have some vision because he was able walk by himself without falling. The patient did not complain of any other symptoms. His symptoms started 1 year ago when his left face exhibited mild swelling that was noticed by his parents. The swelling had worsened recently, and perceived proptosis of the left eye was noted two weeks prior. The patient had unremarkable previous medical history.On examination, he was conscious with evident proptosis of the left eye, and light swelling of the left face was noted without tenderness. Eye movement on both sides was normal. Unfortunately, initial pupillary reflex examinations were not performed, and the vision condition was not assessed. Other physical examinations were globally normal, including normal myodynamia and superficial and deep sensation as well as negative Babinski sign.Cranial computerized tomography (CT) showed a large tumor accompanied by peripheral bone destruction around the left pterygopalatine fossa Fig. A. Brain Blood routine, C-reactive protein, blood biochemistries, liver function, and urine analyses were normal. On the second day of hospitalization, blood tests revealed that the serum AFP level was markedly elevated .On the third day of hospitalization before surgery, the boy lost his eyesight, and eye examination revealed loss of pupillary reflex in both eyes with a bilateral pupil diameter of 4.0 mm. No light sensation was observed during flashlight stimulation on both sides. On the fourth day, the patient underwent exploration, resection and biopsy of the soft tissue mass through nasal endoscopy. The resected specimen appeared as a dark red neoplasm that was brittle and easily bled. Due to the deep location, the mass could not be resected completely. Biopsy confirmed morphologic and immunohistochemical findings of YST, and immunohistochemical stain was diffusely positive for sal-like protein 4, AFP, placental alkaline phosphatase, creatine kinase, and vimentin Fig. . The finThis study was approved by the ethics committee of the Second Affiliated Hospital of Zhejiang University School of Medicine. Written informed consent was obtained from the patient for the publication of this case report and accompanying images.3 upper lip, parapharyngeal and skull base, posterior fossa, floor of the mouth, and right masticator space. To the authors’ knowledge, there has been no previous report of primary YST of pterygopalatine fossa.YST is the leading histologic type among malignant extragonadal germ cell tumors (EGCTs), which occur most commonly in association with teratoma and rarely develop separately. YST occurs much more frequently in female children. Previous studies have reported YST of the left pterygoid region, However, some germ cells will not complete this migration, which results in retention at certain sites near the midline. Second, EGCTs may develop from totipotent cells, which are distributed dispersedly in the entire body during embryonic development and remain dormant under normal conditions. When stimulated, these cells may differentiate to EGCTs.The pathogenesis remains unknown. However, two hypotheses have been proposed regarding the origin of EGCTs. First, EGCTs, which develop from primordial germ cells, are generated as a result of defects in the cell migration process during embryonic development. Primordial germ cells in the 4-week-old embryo present at first in the wall of the yolk sac and migrate through the dorsal mesentery to the genital ridge during embryogenesis.. Immunohistochemically identified AFP is a pivotal biomarker for the diagnosis of YST.YST can be underdiagnosed when imaging examination is exclusively used, but integrating histological and immunohistochemical examinations can improve the diagnosis. CT and MRI images are useful for identifying the location and invasion range of the mass. According to our experience, a soft tissue mass along with bone destruction indicates that it may be a common disease in children, and we initially had a presumptive diagnosis of Langerhans histiocytosis. However, the tumor marker serum AFP was significantly increased. AFP is a glycoprotein that is produced primarily by yolk sac and liver during fetal development. Thus, we suspected that the lesion must be a reproductive tumor. EGCTs secrete basic fetal markers such as AFP and are highly chemo-sensitive. Thus, serum AFP is also a useful tumor biomarker for assessment for the therapeutic effect and predictions of recurrence, residue, and metastasis during the follow-up period in patients with YST. When suspected, the diagnosis can be easily confirmed by histopathology with immunohistochemistry. The major pathologic characteristics of YSTs include Schiller-Duval bodies, a festoon-like pattern, a hepatoid pattern with hyaline globules, and a polyvesicular vitelline pattern However, the prognosis of EGCTs in the head and neck region is uncertain. Thus, Filho et al. advocated surgical resection with radiotherapy for nasal EGCTs, yielding a satisfactory outcome in a 48-year-old man. The patient showed no evidence of metastasis 7 years after treatment. However, not all patients exhibit optimal results. A 3-year-old girl with an EGCT in the orbit/maxillary received chemotherapy and resection of the mass, but the tumor recurred massively 8 weeks following treatment. Gabris et al. demonstrated that radiotherapy might have led to the maldevelopment of teeth. A multidisciplinary approach to treatment including surgical resection and adjuvant chemotherapy has been recommended for most EGCTs of the head and neck region. A multiagent chemotherapeutic cocktail regimen, including bleomycin, etoposide, cisplatin/carboplatin, cyclophosphamide, ifosfamide, vincristine and 5-fluorouracil, has significantly improved the outcome of these patients. In our patient, aggressive surgical excision followed by chemotherapy provided good outcome based on the recent visit.YST generally exhibits poor prognosis due to its tendency for local recurrence and high degree of invasiveness and metastasis. Given that the tumor mass can be absolutely unresectable at diagnosis, multimodal therapy combining surgery, chemotherapy, and radiotherapy have been used in the past.In the current case, the patient developed blindness shortly after hospitalization, which constitutes a severe complication of YST of pterygopalatine fossa that has not been reported previously. Delay in seeking medical care in this circumstance facilitates the growth and invasion of the tumor mass, which should alert parents when a child develops visual impairment and proptosis. In young children, the optimal window for diagnosis may be missed due to the inability to express complaints; thus, physicians should perform a careful and comprehensive physical examination. Nothing is more important than early detection and early treatment in preventing the tragedy of blindness.In summary, primary YST of pterygopalatine fossa occurs rarely but may have poor prognosis due to its high degree of invasiveness and metastasis and the difficulties encountered in obtaining complete resection. The worst complication of loss of vision alerts us of the importance of performing a close physical examination during the initial investigation, which is especially important in small children who cannot express complaints. Early detection of YST of pterygopalatine fossa and treatment with surgical resection and adjuvant chemotherapy may contribute to satisfactory outcomes (tumor shrinkage and restricting tumor metastasis) and avoidance of visual impairment.Conceptualization: Shou-Yin JiangInvestigation: Ye-Hua Shen, Shou-Yin JiangMethodology: Ye-Hua Shen, Shou-Yin JiangSupervision: Ye-Hua Shen, Shou-Yin JiangValidation: Ye-Hua ShenVisualization: Ye-Hua Shen, Shou-Yin JiangWriting – original draft: Ye-Hua ShenWriting – review & editing: Shou-Yin Jiang


Case Presentation. We present the case of an antenatally diagnosed torsion and our subsequent management which included ipsilateral orchiectomy and prophylactic contralateral orchiopexy via a scrotal approach.  Prenatal testicular torsion (PTT) is exceedingly rare in intrauterine development, often diagnosed at the time of birth and very rarely diagnosed in utero during routine gestational ultrasound. As a result, incidence is unknown, and there exists no consensus regarding the pathophysiology of this phenomenon nor universally recognized algorithms and guidelines regarding its diagnosis and management.  While controversy regarding surgical intervention in patients with unilateral PTT exists due to poor salvage rates—estimated to be less than 1%—the risk of anorchia is higher in affected patients due to limitations in the accuracy of detecting bilateral testicular involvement. Risk of misdiagnosis of bilaterality may lead to lasting sequelae such as infertility and devastating psychological consequences for affected patients, supporting the need for surgical exploration, as was performed in our case. Prenatal testicular torsion (PTT) is an exceedingly rare type of neonatal torsion that occurs during the intrauterine period. While the exact pathophysiology remains unknown, it is believed that a hostile intrauterine environment with increased stress—particularly seen in high risk deliveries—leads to extreme cremasteric tension and subsequent torsion . True inFurthermore, complicating the controversy surrounding surgical intervention in PTT is the increased risk of anorchia in patients with unilateral PTT due to inherent limitations in clinical and radiographic diagnoses. Due to lack of universal diagnostic and management guidelines, treatment varies from conservative management to prompt surgical intervention. Furthermore, the decision of surgical intervention varies among physicians—ranging from ipsilateral orchiectomy of the affected testicle to ipsilateral orchiectomy with contralateral fixation and even preservation of the ipsilateral testis plus fixation of the contralateral testis when possible , 3. In wA 30-year-old G2P1 female with a history of loop electrosurgical excision procedure (LEEP) and prior caesarean delivery was monitored in early pregnancy. She received four routine prenatal ultrasonography (US) at 8, 12, 19, and 28 weeks of gestation, during which time, the sonograms demonstrated a male fetus with no apparent anatomical anomalies. At 36 weeks, a prenatal US was performed due to inconsistent fetal size. Initial radiologic interpretation revealed a hemorrhagic incident with thrombosis of the right testicle . The rigThe infant was born at an offsite facility via low, transverse caesarean delivery at 39 weeks of gestation, weighing 3640 g. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. At six hours of life, a scrotal US was again obtained due to concern for the right testicle. This showed an asymmetrically heterogeneous and small testicle. Color Doppler ultrasonography demonstrated absence of blood flow in the right testicle accompanied by chronic changes in the tissue consistent with a subacute or chronic testicular infarct .The patient was then transferred to our facility. Upon arrival, physical exam at bedside was normal with no scrotal edema, erythema, or pain upon palpation. Repeat scrotal US revealed absence of blood flow to the right testicle in addition to accompanying chronic atrophic changes. Scrotal exploration via a midline incision was then performed which revealed right extravaginal testicular torsion with a necrotic right testicle. Subsequent simple orchiectomy was performed . The lefTesticular torsion overall has a bimodal distribution with the highest incidence of torsion occurring after puberty, followed by the neonatal period. Intravaginal torsion occurs in the former, and torsion is almost exclusively extravaginal in the latter . Of thosWhile imaging may aid in diagnosis, the clinical picture and presentation cannot be overlooked. Presenting signs and symptoms can vary widely further muddying the clinical picture. Neonatal torsion in the acute phase may present as an enlarged, hardened testicle often with apparent scrotal inflammation including edema and hyperemia. Other possible differential diagnoses that may mimic acute phase torsion including complete inguinal  hernia, scrotal tumor, epididymoorchitis, and torsion of a testicular-epididymal appendage . ChronicThe lack of consensus and variability in management is perhaps the greatest dilemma urologists face when treating this condition. Due to the poor rate of salvaging a testicle in the perinatal period—as most of these represent irreversible intrauterine events—some argue that there is no advantage to early intervention and that surgery should be deferred until the risks of anesthesia and surgery are better optimized , 2. OtheIn their case series and eloquent meta-analysis, Melcer et al. utilized a different definition of prenatal torsion as that diagnosed in utero or immediately after birth upon newborn examination , 11. Of Surgical approaches vary too. Ipsilateral orchiectomy is not always advised as ipsilateral autotesticular atrophy or leaving an affected testicle in place due to the possibility of some, albeit small, endocrine function , 13. WhiOur case represents a chronic phase extravaginal torsion diagnosed antenatally on routine obstetric ultrasonography. After conferring with the patient's parents, we performed urgent surgical exploration with ipsilateral orchiectomy and contralateral orchiopexy via a scrotal approach. While salvage of the ipsilateral affected testicle was not our intent, surgical intervention was pursued to ensure bilateral synchronous torsion was not present and to also eliminate the risk of anorchia.Current management of PTT ranges from no intervention during the time of diagnosis to possibly even inducing delivery during intrauterine diagnosis and surgical intervention in the neonate. Therapeutic approach is made on a case-by-case basis, with a significant burden placed on weighing the risks and benefits of surgery in a neonate against future infertility, in addition to devastating physiologic, social, and psychiatric consequences for the patient. Further research studies regarding diagnostic intervention and identification of treatment outcomes and prognosis are essential to further the body of evidence to support the development of an evidence-based universal algorithm which would in turn facilitate the most efficacious diagnosis and management approach for this rare clinicopathologic condition.


Transmesenteric hernia is a rare cause of small bowel strangulation in adults. Our patient was a 61-year-old previously healthy male, who presented with vomiting and abdominal pain with no surgical history and no trauma in the past. Computed tomography with contrast enhancement was suggestive of superior mesenteric vein (SMV) compression without any obvious cause. The emergency exploratory laparotomy revealed venous congestion of small bowel caused by a transmesenteric hernia with the herniated loop compressing the SMV. On reducing the hernia, complete reversal of the bowel congestion was noted and small bowel resection was averted. A high index of suspicion for a transmesenteric hernia in small bowel obstruction of unknown etiology and a timely surgical intervention are must for a good clinical outcome. Internal hernias are a rare cause of intestinal obstruction. The diagnosis is often elusive even in its acute state and is usually diagnosed intraoperatively. There are many case reports suggestive of a congenital mesenteric defect in the pediatric population. Here we present a case of a congenital internal hernia in a previously healthy 61-year-old male with the herniated bowel loop causing compression of the superior mesenteric vein (SMV). On reducing the hernia, entire dusky small bowel reverted to a normal pink color. The mesenteric defect was closed and the patient was discharged with an uneventful postoperative course.A 61-year-old male patient presented to our emergency with a 15-day history of constipation and multiple episodes of nonbilious vomiting. He had no surgical history, no comorbidities, and denied any abdominal trauma in the past. The patient was evaluated elsewhere and was given a trial of conservative management. He had undergone a contrast-enhanced computed tomography (CECT) scan of abdomen and pelvis that revealed 1.5 L of ascites which was hemorrhagic on tap. The patient was conscious, oriented, and afebrile on admission with a pulse of 100 beats per minute and blood pressure of 120/86 mm Hg. Physical examination revealed a soft but distended abdomen that was not tender on palpation. Per rectal examination revealed an empty rectum and no stains on returning finger.All laboratory parameters were within normal limits. No air fluid levels were seen on an abdominal X-ray in erect position. A repeat CECT of abdomen and pelvis revealed no signs of intestinal obstruction, ischemic bowel disease, ascites, or malignancy. However, compression of SMV was seen . A nasoWe observed 15 cm of dusky bowel herniating through a 10 cm × 6 cm mesenteric defect in the terminal ileum . The enThe postoperative course of the patient was uneventful. Patient was gradually weaned on to full diet by postoperative day 4 and was discharged 8 days after the surgery.Internal hernias, deﬁned as a protrusion of viscus through an intra-abdominal aperture without traversing fascial planes, are a rare cause of intestinal obstructions. These consist of paraduodenal (53%), pericecal 13%), foramen of Winslow (8%), transmesenteric and transmesocolic (8%), intersigmoid (6%), and retroanastomotic (5%) hernias.3%, foramAlthough transmesenteric hernias were previously uncommon, a rise in Roux-en-Y bypass and liver transplant surgeries have led to an increase in their incidence. In the pediatric population, these defects are commonly congenital and in the region of the small bowel (70% of cases), with 53% of these being in the ileocecal area of the mesentery.In adults, these defects are usually acquired as a result of either blunt abdominal trauma or surgical manipulation of the bowel and mesentery. Congenital mesenteric defects are very rare in adults but can cause an internal hernia followed by an incarceration or strangulation of small intestines.An accurate preoperative diagnosis is often challenging because of the paucity of specific clinical signs and poor sensitivity of any radiological investigations. Blachar et al noted that findings of peripherally located small bowel lateral to the colon  and lack of omental fat between the loops and the anterior abdominal wall might be the most helpful CT signs for diagnosing transmesenteric hernias.A lack of a hernia sac allows large length of the bowel to herniate through the mesenteric defect. This along with a small aperture of the defect predisposes transmesenteric hernias to develop volvulus and strangulation or ischemia. A prompt surgical intervention with reduction in the bowel loops, resection/anastomoses of devitalized bowel, and closure of the mesenteric defect may provide good outcomes.In our case, SMV compression secondary to the internal hernia led to venous congestion in entire bowel within the SMV territory that was reversed completely on reducing the hernia. This further highlights the need of a timely surgical intervention to prevent bowel ischemia and infarction.In the absence of a past surgical or trauma history, a congenital transmesenteric hernia is an extremely rare cause of small bowel obstruction in adults. Although a preoperative diagnosis is challenging, a high index of suspicion and a prompt surgical intervention based on clinical findings are required to avert bowel ischemia and to provide a good clinical outcome. The entire mesentery needs to be evaluated and all mesenteric defects need to be sutured to prevent recurrence.


Routine brain imaging with MRI and long‐term follow‐up of Ewing's sarcoma could be the way to move forward by increasing our understanding in this area, as well as improving treatment and long‐term outcome for patients. Routine brain imaging with MRI and long‐term follow‐up of Ewing's sarcoma could be the way to move forward by increasing our understanding in this area, as well as improving treatment and long‐term outcome for patients. He had a right pelvic Ewing's sarcoma surgically resected 6 years ago. MRI of the head demonstrated a lesion in right temporal lobe, extending into infratemporal fossa. He was referred to oncology for further management with chemotherapy.Ewing's sarcoma is a childhood tumor which is rarely seen, occurring in 2.93 children per 1 000 000, more common in male and hardly understood by many clinicians. The definite diagnosis is of Ewing's sarcoma is usually made in tertiary hospital via biopsy and microscopic evaluation of tumor cells and molecular analysis. Ewing's sarcoma usually requires treatment from different specialties including pediatric or adult oncologist, orthopedic surgeons, and other specialists depending on tumor site. Management of Ewing's sarcoma varies greatly among different patients but includes chemotherapy, radiotherapy, and surgical resection. Most of the brain metastases from Ewing's sarcoma usually occur in the range of 20‐30 months from the time of diagnosis of primary tumor. This case report describes the rare presentation of a gentleman who develops single secondary brain metastases of Ewing's sarcoma after 6 years of complete remission.2A 23‐year‐old Caucasian gentleman was brought into the Emergency Department after an episode of collapse with loss of consciousness for 2 minutes. Up‐rolling of both eyeballs was seen throughout the episode.He had been having 2 months history of intermittent pain around left temporomandibular area, not relieved by any analgesics. He had previous history of Ewing's sarcoma found in his right pelvis on May 2013. It was surgically removed via hemipelvectomy on January 2014. Postoperatively, he was continued with 16 courses of chemotherapy and 25 sessions of radiotherapy in total. He was then followed up by oncology department every 6 months for 4 year, followed by annually for 2 years. A chest and pelvic X‐ray was done before every appointment. He was discharged from follow‐up on Jan 2020 disease‐free. Apart from that, he had no other medical or surgical condition.2.1On physical examination, patient was alert and well orientated with Glasgow Coma Scale of 15/15. There was a small bruise to right side of temple with no laceration, possibly due to the collapse. No swelling or tenderness upon palpation was felt on the left side of the face. Upon cranial nerve examination, the only positive sign was a horizontal nystagmus seen in both eyes. There was also no abnormality noted in sensory and motor neurological examination of bilateral upper and lower limbs.2.2Routine blood test including full blood count, urea and electrolyte, and C‐reactive protein was performed. No abnormality or raised inflammatory marker was noted.Computed tomography scan of the head was requested. A space occupying lesion at the left temporal lobe extending through the skull base to the left masticator space was found. This warranted for a further contrast‐enhanced magnetic resonance imaging (MRI) of the head. An aggressive large mass lesion measuring 7 cm by 4 cm was seen in the left temporal location, eroding through the skull base, resulting in extracranial component in the infratemporal fossa. It had solid and cystic components with surrounding edema. Medially, the lesion was seen to extend up to the pterygoid plates and the posterior superior nasopharynx on the left side, and a resultant block of the Eustachian tube and a left mastoid effusion was seen. Midline shift to the right of 6mm as a result of mass effect was also noted. CT‐guided biopsy of the left superficial temporal fossa lesion was carried out. Presence of a CD99 + small round blue cell tumor and detection of an EWSR1 rearrangement at 22q12 in 84% of cells via FISH testing are consistent with diagnosis of recurrent Ewing's sarcoma.After that, patient had a CT thorax, abdomen, and pelvis with contrast, showing no evidence of visceral metastases disease. He then proceeded to have a whole‐body SPECT and whole‐body PET FDG, showing single brain metastases with elevated activity in left skull base region and no evidence to suggest extracranial or bone metastases elsewhere Figures 2.3The patient was then referred back to the initial oncology department for further management. At time of writing this report, the patient has had five courses of chemotherapy, ifosfamide, and etoposide (Doxifos) and had an MRI showing shrinkage of tumor by more than 50% after second course of chemotherapy. He will be continued with more courses of Doxifos and possible surgical resection Figures 3Ewing's sarcoma is a very rare type of bone or soft tissue tumor, predominantly affecting children, adolescents, and young adult. It is the however the second most common type of malignant bone tumors after osteosarcoma. The annual incidence of Ewing's sarcoma is around 1.8 per million for age 0‐14 and 3.7 per million for age 15‐24. The incidence rate is highest among the 15‐19 age group, which is 4.8 per million per year. It is also more commonly seen in male as compared to female with a ratio of 1.89:1.On first discovery of right pelvic Ewing's sarcoma 7 years ago, our patient had undergone a hemipelvectomy, coupled with a regimen of neo‐adjuvant chemotherapy, 25 sessions of radiotherapy, and a regimen of adjuvant chemotherapy. In fact, studies on pelvic Ewing's sarcoma have shown that surgery coupled with neo‐adjuvant chemotherapy and radiotherapy would produce better outcome as compared to radiotherapy coupled with neo‐adjuvant chemotherapy or radiotherapy alone.After 6 years of complete remission, our patient had an episode of collapse and was found to develop secondary brain metastasis. Brain metastasis is rare among children with solid tumors, usually presenting with seizure or hemiparesis.This paper puts forward a proposal for routine imaging such as MRI scan of head on follow‐up of patient with Ewing's sarcoma. Firstly, this might provide a more accurate understanding on the incidence of secondary brain metastases in Ewing's sarcoma. Secondly, this could help in diagnosing any brain metastases before the tumor grows large. Thirdly, this could improve the outcome by enabling surgical resection coupled with chemotherapy or radiotherapy when the tumor is small.Ewing's sarcoma is the most common childhood tumor to cause secondary brain metastasis.Surgical resection coupled with chemotherapy and radiotherapy provides the best long‐term outcome in management of primary or secondary Ewing's sarcoma.Routine brain imaging and long‐term follow‐up of Ewing's sarcoma could be the way to move forward by increasing our understanding in this area, as well as improving treatment and long‐term outcome for patients.This case serves to improve our understanding toward the rare occurrence of secondary brain metastases of Ewing's sarcoma after long period of complete remission, as well as highlighting the importance of further study and research in this field. Routine brain imaging on follow‐up of patient with Ewing's sarcoma could be the next step forward in improving our understanding of this disease as well as improving long‐term outcome of patients.The author of this study has no conflict of interest to declare.JZP: Conception and design, acquisition of data, analysis and interpretation of data, drafting of manuscript, and critical revision for important intellectual content.No approval is required.App S1Click here for additional data file.


To demonstrate the effectiveness of the endonasal endoscopic approach in managing a large bilateral frontal mucopyocele causing a mass effect on the brain.A 17 year old chronic sinusitis patient, presented with bilateral nasal obstruction and increasing left sided headache; was found to have a very large bilateral frontal mucopyoceles with extensive intracranial extradural brain involvement on CT scan and MRI of the sinuses and brain which was drained and evacuated completely using the endonasal endoscopic image guided approach with no complications.The endonasal endoscopic image guided approach is the approach of choice in managing large frontal mucopyocele with brain extension.It is a safe, accurate, reliable approach with no complications. This may result in erosion and reabsorption of the bony sinus walls. They develop due to obstruction of the sinus ostium by congenital anomalies or acquired causes such as allergic rhinitis, post-traumatic, post-inflammation and infection . The comMucoceles may presents with variable manifestations that are based on the location of mucocele and its extent. Symptoms might be rhinologic, neurologic or ophthalmologic .CT scan is the imaging modality of choice with MRI being superior in terms of differentiating a mucocele from soft tissue tumors as well as localizing the mucocele in relation to brain and orbit .We hereby report a rare case of bilateral frontal mucopyoceles managed by image guided endoscopic drainage and marseuplization at our hospital in Saudi Arabia.This case report has been reported in line according to Surgical Case Reports (SCARE) Criteria .2A 17-year-old male patient presented to ENT clinic with history of facial heaviness and chronic headache located in the left frontal area. Associated with bilateral nasal obstruction, anosmia and recurrent epistaxis. His medical history includes diabetes mellitus type 2 on insulin regimen. His surgical history includes FESS done one year back. Ophthalmic examination showed displaced left eye downward and outward. ENT examination showed bilateral nasal pale polyps grade 3-4 with white discharge. Otherwise examination was unremarkable.The patient was evaluated by CT scan of the brain and paranasal sinuses which revealed a marked enlargement of the frontal sinuses, more pronounced on the left side with associated dehiscence of the posterior wall of the sinus and intracranial extension of soft tissues within epidural space measuring 5.3 × 4.1 cm on axial image .Figs. 1–MRI of the brain and sinuses  showed sUnder general anesthesia, image guided endoscopic sinus surgery was undertaken. Examination revealed extensive nasal polyposis grade 4 that was removed by microdebrider and frank pus was drained, removal of the obstructing polyps from the ethmoidal cavities was done, frontal recess was identified full of obstructing infected polyps that was removed and drained . Draf ty6 months post operative follow up CT scan of the sinuses showed complete resolution and normal aeration of the sinuses , Fig. 113Mucoceles are slowly growing, mucus containing lesions that develops in the paranasal sinuses. A chronic expanding mucocele with mucous retention can become infected causing mucopyocele . There aFrontal mucocele may presents with a variable spectrum of symptoms based on the location and extent of the mucocele; symptoms may include nasal obstruction or postnasal drip, headache, dizziness, maxillofacial pressure, subcutaneous forehead or periorbital tenderness or swelling. If the mucocele has invaded the orbital cavity, patients may experience diplopia, ptosis, proptosis and globe displacement ,3,6.Surgery is the modality of choice to achieve complete evacuation of the mucocele and reestablishing the normal sinus outflow . In the For fronto-ethmoidal mucoceles in particular, there were many controversies in previous literature regarding recurrence rates, surgical morbidity and preservation of normal sinus outflow .Before the availability of intraoperative image guidance in paranasal surgeries, open and combined surgical approaches were undertaken for frontal lateral mucoceles where they caused extensive scarring. With the advances in technology and the availability of equipment and adequate training, endoscopic marsupialization with image guidance could become the surgical choice for managing complex mucoceles .The concept behind image guidance navigation system is using an infrared camera or radiofrequency to track along the surgical instrument in relation to the patient’s head during operation. A computer set will process this information and delineate the tip of the instrument over the patient’s preoperative sinus CT scan by a 3-dimensional video display. This technology allows the surgeon to confirm the anatomy of the sinus and its location relative to adjacent structures such as skull and orbits .Surgical approach to treat mucuceles is challenging due to many factors, including complex and variable anatomy, possible scarring and stenosis. In determining the surgical approach to mucoceles, two main factors are considered: the patency of the ipsilateral frontal sinus outflow and the accessibility of the sinus via endoscope. When these factors are not met, a more invasive external approach is undertaken such as endoscopic modified Lothrop procedure (EMLP) or an osteoplastic flap surgery (OPF) with or without frontal sinus obliteration . The EMLRecently, endoscopic approach is the mainstay of management for paranasal sinus mucoceles with lower rates of morbidity . In the Endonasal endoscopic mucocele management has the advantage of allowing a less traumatic approach, as well as reducing morbidity rates and operative time to a minimum. Therefore, endoscopic surgery is becoming the surgical technique of choice ,8.Several possible complications can arise due to the close proximity of the paranasal sinuses and the orbital cavity; Inattentive direct trauma to orbital content during surgery is potential. Susceptibility of direct damage or entrapment of the medial rectus near the lamina papyracea; direct damage to optic nerve and intraorbital hemorrhage are all possible ,13. Post4The endonasal endoscopic approach to large frontal mucopyoceles is successful, with low morbidity and low recurrence rates.The image guided Endoscopic drainage and marsupialization is a safe, reliable and accurate in managing large frontal mucopyocele with brain involvements. This approach, when compared with open techniques, is associated with low recurrence rates and low rates of complications.None.The ethical approval is exempted for the case report at our institution.Written informed consent was obtained from the parents for publication of this case report and images provided on behalf of the patient.Zainab Alhassan: Manuscript draft and final edits.Fadel Molani: Data collection and literature review.Ali Almomen: Operating surgeon, data analysis and interpretation and critical revision of the manuscript.Not Applicable.Ali Almomen.Written informed consent was obtained from the parents for publication of this case report on behalf of the patient.The data used to support the findings of this study are included within the article. Also, they are available from the corresponding author upon request.This work has been reported in line with the SCARE criteria.Not commissioned, externally peer-reviewed.The authors declare that there is no conflict of interest regarding the publication of this paper.


Adiposis dolorosa or Dercum’s disease is a rare lipomatous disorder characterized by painful lipomas. In this article, we report a case of rather large exophytic adiposis dolorosa causing difficulties with ambulation, and our surgical management of the disorder. To our knowledge, this is the first reported case of a large exophytic adiposis dolorosa of the upper medial thigh causing problems with mobility. This is also the first reported case of the use of a delayed split-thickness skin graft (STSG) after interval use of wound vacuum-assisted closure (VAC) following dermolipectomy. A 77-year-old female presented with a chronic mass on the medial aspect of her right thigh for over 40-50 years. She had noticed a recent rapid increase in size, causing some discomfort and interference with mobility and activities of daily living. The patient underwent an MRI with finding consistent with adiposis dolorosa. She underwent dermolipectomy and reconstruction of the resulting defect with a combination of partial primary closure, wound VAC, and delayed closure using STSG. Dermolipectomy with interval application of a wound VAC combined with delayed reconstruction with STSG is a feasible option for patients with large lesions of the extremity that causes difficulty with mobility and activities of daily living. Adiposis dolorosa or Dercum’s disease is an extremely rare disease with characteristic painful lipomas. It is classified under rare diseases by the National Organization for Rare Disorder (NORD) . This enMost of the reported cases describe patients with diffuse or localized adiposis dolorosa. We report the surgical management of a patient with a large exophytic adiposis dolorosa of the medial thigh whose main complaint was pain and difficulty with mobility and activities of daily life. To our knowledge, this is the first reported case of a large exophytic adiposis dolorosa of the medial thigh causing problems with mobility, and the first reported case of the use of a delayed split-thickness skin graft (STSG) after interval use of wound vacuum-assisted closure (VAC) following dermolipectomy.A 77-year-old female was seen in our plastic surgery clinic for evaluation of a right medial thigh mass. The patient was referred to us by a general surgeon who had recommended resection and required assistance with the reconstruction of the resulting defect. The patient had had the mass for over 40-50 years. The mass had rapidly enlarged over the previous six months and was interfering with walking, sitting, and the ability to wear clothing. She reported some localized discomfort and pain but denied any paresthesia or signs and symptoms of claudication. She had not sought any intervention for the mass in the past.Her past medical history was significant for hypertension and obesity. Her physical examination revealed a very large mass protruding from the right medial thigh, measuring more than 40 cm in its greatest dimension Figure . The colThe patient underwent a dermolipectomy and delayed reconstruction. The procedure involved resection of the skin and the subcutaneous tissue (dermolipectomy). The mass had not invaded the muscles or the fascia. The patient was noticed to have large fatty tissue within the mass. The resected tissues were sent for pathological analysis Figure . The resAdiposis dolorosa or Dercum’s disease is a rare and predominantly female disease. It is characterized by the presence of painful subcutaneous adipose tissue in overweight or obese individuals. The current management of this condition includes medical as well as surgical management to control the symptoms, mostly pain. Medical management is aimed at controlling the pain associated with the condition. Some of the modalities include corticosteroids, lidocaine infusion, and methotrexate . These pSurgical management including liposuction and dermolipectomy may be of use in the subset of patients who do not benefit from pharmacotherapy or have limitations in their mobility ,11. Our Dermolipectomy as a treatment for this condition has not been extensively studied, and most of the studies are limited to case reports and case series. Some of the reported complications of dermolipectomy include pain recurrence, recurrence of the collection, and lymph fistula ,13-15. OAdiposis dolorosa is a very rare condition. In this article, we reported the surgical management of a single case at our institution. Management is mostly focused on pain control and can be achieved through observation, medications, or surgery. Dermolipectomy is very effective for large lesions of the extremities to improve mobility. However, these resections can leave large defects unamenable to primary closure alone. Resection with interval application of wound VAC combined with delayed closure with STSG is a feasible alternative.


Sister Mary Joseph’s nodule is a metastatic of a primary cancer, usually adenocarcinoma and associated with poor prognosis. Here we report the case of a 48-year-old man, admitted to the hospital with six months history of epigastric pain without vomiting or externalized hemorrhage and without transit disorders in a context of alteration of the general state with weight loss. Clinical examination showed epigastric sensitivity with a painful umbilical nodule, firm and irregular (A). Laboratory tests revealed a cholestasis. His computed tomography (CT) of the abdomen showed a mass lesion at the tail of the pancreas measuring 45 mm enclosing the splenic artery with secondary ganglionic, hepatic, bone and peritoneal localization . The umbilical lesion biopsies revealed metastasis of the pancreatic adenocarcinoma (D). The patient received palliative chemotherapy.


Epithelioid sarcoma (ES) is a rare mesenchymal tumor, accounting for less than 1% of all adult soft-tissue sarcomas. The diagnosis of such malignancy is challenging. We reported a 31-year-old male diagnosed with rhabdoid ES that histologically mimicked epithelioid rhabdomyosarcoma in a 31-year-old male.A 39-year-old male presented with pain in the left thigh. He had planned for open reduction internal fixation due to left femur fracture. During the surgery, the surgeon found abnormal appearance of the bone and the surrounding tissue. The histopathology examination showed spindle-to-polygonal epithelioid cells arranged in nodular pattern with necrotic areas. Immunohistochemistry examination revealed vimentin and CK positive expression, while myogenin was negative. Further immunostaining using CD34 and HMB45 were also negative.The diagnosis of ES based on only the clinical manifestation has proven to be difficult. Thus, the histopathology examination followed by immunohistochemistry is considered as the main modality for the diagnosis. Better understanding of clinical properties of ES will aid in deciding the best treatment for the patient. Generally, ES is considered as a high-grade soft tissue sarcoma . ES occu2A 39-year old male presented with pain and discomfort of the left thigh. One month prior to the admission, the patient complained of discomfortness with intermittent pain in his left thigh and went to a general practitioner. However, the pain did not subside. Six days before the admission, the patient felt sudden pain in the left thigh while walking fast, then he fell down and was unable to walk. He was brought to a private hospital in Yogyakarta and X-ray examination was performed, showing a fracture and soft tissue mass in the left proximal femur . The surLaboratory examination showed normal range of complete blood count, electrolytes, alkaline phosphatase (ALP), and also lactate dehydrogenase (LDH). There was no increased levels in other specific tumor marker examinations of other organs which showed no source of metastatic bone disease. Bone survey was performed and showed no sign of abnormality in other bones. Further, computerized tomography (CT) scan of the chest and abdomen were done and also showed no signs of metastasis in the regions. Magnetic resonance imaging (MRI) was done. It showed the presence of soft tissue lesion in the left proximal femur (subtrochanteric) with the size 12.2 × 9.8 × 13.8 cm, with expansion of the lesion to femoral head, femoral neck, trochanteric area and femoral shaft causing pathologic fracture of the left subtrochanter femur.Histologic section showed mesenchymal tumors with increased cellularity, arranged in solid and alveolar fashion and infiltration to surrounding soft tissue . MoreoveA radical resection was planned, and the patient was taken for surgical hip disarticulation. Following the surgery, adjuvant chemotherapy was done using Doxorubicin and Ifosfamide for 6 cycles. Then, the patient was referred to radiation oncology and underwent intensity-modulated radiation therapy at 6000 cGy to the left hip joint.The patient had a slow yet uneventful postoperative recovery. On the most recent CT imaging of chest and abdomen done 1-year postoperatively, there was no evidence of metastasis. He returned to daily activities using a lower limb prosthesis and remained free of recurrence.3Epithelioid sarcoma (ES) is a rare soft tissue sarcoma mostly affecting the age group of 20–40 years old. Overall peak incidence of ES is 35 years of age. It was first clearly characterized by F.M. Enzinger in 1970. The incidence is only less than 1% of all adult soft tissue sarcoma and between 4–8% of pediatric non-rhabodmyosarcomatous sarcoma . ES is aES is divided into two clinicopathological entities, which are distal-type ES and proximal-type ES. These two subtypes are considered as a continuum of this disease, not as distinct entities. Distal-type ES tends to be found in younger age predominantly male patients, compared to proximal-type . ES alsoOn physical examination, distal-type ES is characterized as a firm, non-tender, slow-growing tumor with predilection towards distal extremities, especially the hands . DiffereDistal-type ES or classic ES is characterized as tumor nodules with central necrosis and surrounded by large polygonal cells and spindle cells in the periphery. This type of ES includes angiomatoid, fibroma-like, and myxoid variants. Whereas, proximal-type ES is characterized by a tumor with multinodular pattern and sheet-like growth of large polygonal cells. This type is often accompanied by a focal or predominant rhabdoid morphology .In this case, the tumor was in the proximal femur as a soft tissue lesion and infiltrating surrounding deep soft tissue. The tumor consisted of spindle to polygonal epithelioid cells arranged in nodules, small to moderate in size, with a wide necrotic area. Also, there was abundant pathological mitosis, which showed an aggressive course. The tumor was more suitable to be considered as proximal type ES based on the physical examination and tumor cell morphology.Due to its wide range of morphology, it is hard to make definitive diagnosis of ES based on histopathology examination alone. Given the epithelioid morphology of the tumor cells, the differential diagnosis of this case was epithelioid rhabdomyosarcoma which can show almost identical histological patterns. To aggravate this challenge, there is no specific ES biomarker that has been identified. Both of these tumors also express epithelial markers such as cytokeratin in immunohistochemistry. But co-expression of vimentin and keratin is thought to be characteristic of ES. CD34 is positively expressed in 50–60% cases of ES while, it is negatively expressed in rhabdomyosarcoma.9 Also, myogenin can help to distinguish ES from rhabdomyosarcoma and HMB45 can help to distinguish ES from melanoma cases .Regarding this case, the immunohistochemistry examination showed that all tumor cells were positively stained with vimentin and CK but negatively stained with myogenin, which excluded the diagnosis of rhabdomyosarcoma. However, further immunostainings using CD34 and HMB45 were also negative, which showed that the tumor was suitable as epithelioid sarcoma of the bone.Currently, there is no optimal management of ES, and it rarely spreads to lymph nodes. Local recurrence can occur within 1–2 years after treatment. Moreover, most of these cases were often accompanied by distant metastasis . It is rThe primary treatment is wide surgical resection for localized disease, and the goal of the treatment is to remove the tumor while maintaining the optimal function of the extremity ,10. In tAdjuvant radiation therapy after resection can be employed to reduce local recurrence ,13. RadiPatients with localized disease have a better 5-year overall survival at about 75%. Metastasis occurs in almost half of the patients with the localized disease even with adequate treatments. The width of the tumor has been reported to be directly proportional to the rate of metastasis and lower 10-year survival. Female gender is linked with a better prognosis, while tumor size, vascular invasion, and necrosis have been inconsistently reported with a poorer prognosi . PatientWide surgical excision remains the mainstay of treatment, and medical management for systemic disease is, to a large extent, undefined. For localized disease, neoadjuvant or adjuvant radiation therapy is often given to reduce local relapse, but the role of adjuvant chemotherapy is unclear. Systemic chemotherapy provides satisfactory palliation, but response is of short duration. As relapse rates remain high despite multimodal therapy, there is an urgent need for development of targeted treatments for patients with regional and distant metastases .4There are no distinct clinical properties of epithelioid tumors to differentiate with other tumors. Clinically, it is essential to distinguish between the primary bone or soft tissue tumor and metastatic tumor. Radiology examination will help to distinguish metastatic carcinoma. Further, histopathology examination followed by immunohistochemistry will help to establish the diagnosis. Better understanding of the clinical properties of ES will facilitate the surgeon with a better choice of treatment. Complete resection while maintaining optimal function of the extremity is considered as the main treatment. Poorly-planned biopsy of a primary malignancy may contaminate the field, leading to loss of options in considering limb salvage surgery.None.None.Ethical approval was received from the Ethical Committee of Faculty of Medicine, Universitas Gadjah Mada.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.Rahadyan Magetsari: performing the procedure, study concept, data collection.Ery Kus Dwianingsih: data collection, data interpretation, writing the paper.Yudha Mathan Sakti: writing the paper.Yuni Artha Prabowo Putro: writing the paper.Irene Araneta: writing the paper.This study has been registered at researchregistry.com (UIN: researchregistry5162).Rahadyan Magetsari.Not commissioned, externally peer-reviewed.