The World Health Organization defines a perivascular epithelioid cell tumor (PEComa) as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. Although PEComas have the potential to behave in a malignant fashion, malignant PEComas are extremely rare.An 83-year-old man visited our clinic presented with palpable, painless, and movable mass in the right knee area.Malignant PEComa was diagnosed by incisional biopsy. No metastases was confirmed by radiologic imaging including PET/CT, magnetic resonance imaging, high resolution computed tomography.We performed wide excision of the mass and used an anterolateral thigh free flap to reconstruct the defect on the right knee.The permanent histopathology showed malignant PEComa was totally resected. The flap which was performed to cover the defect was survived and the patient discharge without any complications.PEComa can metastasize to various anatomical regions. Although there is no established standardized treatment, radical resection is still considered the cornerstone of treatment. Rapid and appropriate defect coverage is important to improve the patient’s prognosis. Classification of Tumors of Soft Tissue and Bone.A perivascular epithelioid cell tumor (PEComa) is defined as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features in the fourth edition of the World Health Organization’s  PEComa occurs in various anatomical regions, including the colon, pancreas, retroperitoneum, heart, adrenal gland, breast, eye, biliary tract, bone, urinary bladder, skull base, liver, uterus, cervix, skin, nasopharynx, upper airway, and soft tissue. Cases occurring in soft tissue are even rarer than those in other areas.PEComa is a rare disease, with approximately 100 cases reported worldwide.Herein, we report a rare case of an 83-year-old male patient who presented with malignant PEComa on the right knee. We performed wide excision of the mass and used an anterolateral thigh free flap to reconstruct the defect on the right knee. This study received approval from the Jeonbuk National University Hospital’s institutional review board. The study was conducted in accordance with the principles of the Declaration of Helsinki, and the patient, being elderly and considered as vulnerable population, written informed consent was received from the patient’s legal guardian prior to inclusion in the study.An 83-year-old man visited the outpatient department with a palpable, painless, and movable mass in the right knee area. The patient had a history of gastric cancer that was completely cured after surgery. A biopsy was performed for evaluation, and PEComa was diagnosed. Subsequently, a bone scan, positron emission tomography-computed tomography, magnetic resonance imaging (MRI), and high-resolution computed tomography were performed to evaluate the lesion and determine whether metastasis had occurred. On MRI, multiple subcutaneous soft tissue masses with high density and enhancement were observed in the right prepatellar region, but no metastasis was observed Fig. .Wide resection was performed with a safety margin of 3 cm, and the lateral third of the patella was resected. After resection, there was a large skin and soft tissue defect measuring about 20 × 10 cm, and an anterolateral thigh free flap was planned to cover the defect. An elliptical flap was designed for the contralateral thigh, and a myocutaneous perforator originating from the descending branch of the lateral circumflex femoral artery was found and dissected using Doppler ultrasound.The elevated flap was inserted into the defect site, and end-to-end anastomosis was performed between the descending branch of the lateral femoral circumflex artery and the medial genicular artery. An end-to-end anastomosis was performed between the vena comitans and the medial genicular vein Fig. .The permanent histopathology showed malignant PEComa which is composed of epithelioid cells, with clear cytoplasm and significant cytologic atypia. Also, the tumor cells showed strongly positive for Transcription Factor Binding to IGHM (immunoglobulin heavy constant Mu) Enhancer TFE3) and weakly positive for HMB45  than in men. The PEComa family includes angiomyolipoma, clear-cell sugar tumors, lymphangiomyomatosis, and clear cell myomelanocytic tumors, which are characterized by immunoreactivity with melanocytic and muscle markers.PEComas are a rare tumors defined by the World Health Organization as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. The pathological findings are characterized by perivascular proliferation of epithelioid and fusiform cells with light eosinophilic cytoplasm with granularities and visible nucleoli. On immunohistochemical staining, about 92% to 100% of melanocytic markers are positive for HMB-45, 23% to 88% are positive for Melan A/Mart1, 50% to 92% are positive for the micropthalmia transcription factor, and 8% to 33% show positive findings for S100. As for the smooth muscle markers, about 36% to 100% are positive for desmin, 59% to 93% are positive for smooth muscle actin, and 75% to 92% are positive for caldesmon. In addition, regardless of the rearrangement of the TFE3 gene, 29% to 38% of PEComas can show positive findings for the TFE3 transcription factor. PEComas can metastasize to adjacent lymph nodes, bones, and organs, and the most common organ for metastasis is the lung. Since metastasis may occur up to 10 years after surgical resection, regular follow-up is necessary.–7PEComas may be discovered by examining painful areas or palpable masses, but about 20% of cases are found incidentally on radiological imaging such as computed tomography or MRI taken for the purpose of diagnosing other diseases. Since PEComa can occur in various anatomical regions, it is important to perform a complete diagnostic evaluation, and a biopsy is essential for the diagnosis. According to Bajaj et al, the activation of mammalian target of rapamycin signaling as a result of mutations or deletions of tuberous sclerosis genes (TSC1 or TSC2) is a key molecular driver of PEComa. Their study introduced a case with an improved clinical response in a patient with metastatic malignant PEComa who received concurrent administration of a mammalian target of rapamycin inhibitor and radiation therapy. Therefore, those researchers pointed out that an appropriate treatment plan should be established after weighing the benefits and risks of cytotoxic chemotherapy in consideration of the patient’s age and underlying disease.–11As PEComa is a rare disease, there is no established standardized treatment, but the main treatment is currently radical resection. Radical resection is recommended for metastatic tumors, but a poor prognosis is predicted. According to Machado study, palliative chemotherapy, radiation therapy, and immunotherapy can be considered in patients with metastasis. However, data are limited and this remains an area where no consensus exists, requiring more evidence.PEComa is a rare disease and can metastasize to various anatomical regions. Although further research is required, radical resection is still considered the cornerstone of treatment, and there is no established treatment method due to the low prevalence of this condition. Choosing an appropriate coverage method for a large defect after radical resection can be challenging for surgeons, and free flap surgery can be a good option if the patient’s condition permits. Stabilization of the surgical site can allow rapid initiation of postoperative chemotherapy, radiation therapy, and immunotherapy; thus, rapid and appropriate defect coverage is important since it can affect the patient’s prognosis.Conceptualization: Nae-Ho Lee.Data curation: Beom Jin Lim.Investigation: Beom Jin Lim, Kyu Yoon Jang.Methodology: Kyu Yoon Jang.Resources: Beom Jin Lim.Supervision: Si-Gyun Roh.Writing – original draft: Beom Jin Lim, Si-Gyun Roh.Writing – review & editing: Si-Gyun Roh, Jin Yong Shin, Nae-Ho Lee, Yoon Kyu Chung.


We present here a case of complex uterine anomaly—obstructed hemivagina with ipsilateral renal agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 13-year-old girl with a history of recurrent urinary tract infections (rUTI). In the emergency room, a trans-abdominal sonography revealed an ovarian cyst and renal agenesis, without any suspicion of vaginal obstruction. This led to a delay in the diagnosis of this uncommon anomaly. Finally, MRI findings confirmed the presence of OHVIRA syndrome. As the congenital anomalies of the kidney and urinary tract (CAKUT) are present in almost one third of cases associated with genital malformations, urologists should carefully screen patients with rUTI. The patient underwent simultaneous laparoscopy and vaginoscopy, which was in our opinion the most appropriate therapeutic decision. In this article, we are also going to discuss the role of laparoscopy in the management of OHVIRA syndrome, as well as other surgical techniques described in the literature. Congenital anomalies of the kidney and urinary tract (CAKUT) are present in almost one third of cases associated with genital malformations ,2. The pCurrently, the management of OHVIRA is still debated. Although many ideas for OHVIRA management have been suggested, delayed diagnosis is still an obvious problem. In this article, we present a problem-solving flow chart and other methods that could help professionals in their practice. Several operative techniques for OHVIRA management have been reported, such as puncture of the vaginal wall, single-stage vaginoplasty, mini-laparotomy and even unilateral hysterectomy . HoweverWe report a case of a young woman with OHVIRA associated with CAKUT, the condition of rUTI and pain. This case emphasizes that uterus didelphys with obstructed hemivagina (OHVIRA) should be considered in the differential diagnosis of adolescent girls presenting with acute abdominal pain, rUTI and CAKUT. Furthermore, we reviewed the most used surgical procedures for these conditions.5 colony-forming units (CFU)/mL of Escherichia coli (E. coli). The young woman complained of rUTI despite courses of antibiotics that had been administered (Cefuroxime 2 × 1500 mg iv.).We report the case of a 13-year-old girl who presented with acute abdominal pain and a UTI. Hematuria was not present. The patient had a history of rUTIs that occurred six times in the last year, with urine cultures positive for more than 10The patient attained menarche 6 months previously, reporting of back and lower abdominal pain and UTI; it was of 1 week’s duration. She had regular menstrual cycles, every 30 days, lasting 3 to 4 days.Abdominal ultrasonography (US)  revealedParticular image examination was performed at the Department of Gynecology, Endocrinology and Gynecologic Oncology of University hospital.On admission, because of the patient’s severe pain, the examination was conducted under general anesthesia and the dorsal lithotomy position with a virgin-size speculum revealed a single normal vagina and one cervix. Bulging of the vaginal wall on the right side was also found. Unfortunately, the attempt to puncture the thick vaginal wall to relieve patient’s symptoms failed in providing drainage. MRI  providedAccording to the literature, we considered hemivaginal septal resection and anastomosis between the obstructed hemivagina and the normal vagina. However, escalating abdominal symptoms prompted us to perform laparoscopy simultaneously. The medical, family, and psychosocial history of the patient, including genetic information, revealed no history of renal abnormalities.Pre-operative management included psychological counselling of the patient and her family regarding treatment options, with detailed description of a planned surgical approach. Before completion of the whole examination, the patient was given an oral combined estrogen–progestin pill to block recurrent uterine blood flow, which could intensify abdominal symptoms. The patient underwent combined laparoscopy and vaginoscopy. There was clinical evidence to suspect adnexal torsion and hematosalpinx, hence the laparoscopy was performed simultaneously. Vaginoscopy was completed, and with the help of a resectoscope, a vertical incision was created, the vaginal septum was excised and the vaginal opening was extended. Intraoperative abdominal sonography was done for the appropriate evaluation of the place of resection . CombineThe patient came for a health check after one week and was followed up for prevention of complications like vaginal stenosis and recurrence of obstruction. Hormonal therapy with combined estrogen–progesterone pills was administered to avoid heavy periods and possible inflammation which could accelerate the stenosis process. At 6 months follow up, the patient was free from vaginal stenosis, rUTI and recurrence of obstruction. We obtained written parental permission to present this case study.OHVIRA syndrome is a rare congenital urogenital anomaly derived from Mullerian tract abnormality . The chaThis case highlights the potential pitfalls and challenges in gynecologic surgery in adolescence. It also emphasizes that uterus didelphys with obstructed hemivagina (OHVIRA) should be considered in the differential diagnosis of adolescent girls presenting with acute abdominal pain, recurrent UTI and CAKUT ,21. We aSometimes, a condition misinterpreted as dysmenorrhea and normal menstrual blood flow through the unobstructed hemivagina can cause delay in reaching a correct diagnosis. Dysmenorrhea is reported by 30–90% of young female patients and “irregular menstrual bleeding” in up to 43% . The prePelvic pain in adolescents is often a puzzle, and suspicion and knowledge about different causes are important for accurate diagnosis. As urogenital abnormalities are rare, they can be mistaken for ovarian cysts . In our Recurrent UTI can be associated with specific women’s anomalies, but if not treated properly it can lead to upper urinary tract infections like glomerulo- or pyelonephritis ,4,24,25.Often forgotten, endometriosis in adolescence can provoke dysmenorrhea, chronic pelvic pain, and, in consequence, develop endometrial cysts, which could imitate large abdominopelvic masses .Such abnormalities like vaginal septum, urogenital sinus, rudimentary horn, and others should be in mind when counselling a female adolescent with nonspecific symptoms. The need for precise follow up is necessary in patients with CAKUT, and it must be noted that renal agenesis and other things could be associated with congenital anomalies of female reproductive organs .MRI imaging is the gold standard for diagnosis with higher sensitivity in detecting the uterine morphology and the continuity of the vagina when compared to ultrasonography . HoweverIn the paediatric and adolescent population, scan techniques are essentially limited to a transabdominal or transperineal approach. Therefore, transperineal scanning could reveal a distended vagina and/or uterus filled with fluid containing low-level echoes . FurtherGynecologic surgeons face many challenges when considering complex gynecologic surgery, especially in children. Anticipating the intraoperative risks and a careful step by step, surgical plan should be an integral part of each procedure in children with congenital anomalies.To date, several surgical techniques for OHVIRA management have been reported, such as puncture of the vaginal wall, single-stage vaginoplasty, mini-laparotomy, even unilateral hysterectomy and using a vaginal mold or stent ,29. SeptIt is technically difficult to puncture the thick fibrotic tissue of the hemivaginal septum with a needle. Due to such a proceeding, we expose the patient to unnecessary interventions, like minilaparotomy. Therefore, we offered our patient intraoperative abdominal sonography to directly evaluate the accurate and safe place of puncture and septal resection.Minimally invasive surgery has acquired more and more space in the surgical panorama. Furthermore, it has many advantages in adolescent gynecology. Some of the authors discussed the role of laparoscopy in the management of OHVIRA ,32,33. IHydrometrocopes and those associated with OHVIRA recurrent blood flow can develop hematosalpinx and, in consequence, endometriosis . During The necessity of the unilateral hysterectomy of one uterus is questionable. First of all, such a procedure is not a simple one for children. Another point is regarding the future family planning in patients with congenital anomalies.In contrast to other authors, we believe that concomitant laparoscopic surgery is necessary in the surgical approach for OHVIRA patients ,32, not Because of the higher risk of vaginal restenosis in patients with OHVIRA syndrome, there is a strong need for long-distance follow up in such patients. Sometimes surgical resection is necessary. Early postoperative management with antibiotics and oral contraceptives is necessary to avoid vaginal infection and future restenosis.Follow up of OHVIRA requires prolonged monitoring of solitary kidney function and guidance enabling satisfactory sexual intercourse and delivery. Girls with renal agenesis should be carefully monitored for possible genital tract anomalies, and, conversely, girls with vaginal or uterine anomalies should be monitored for renal malformations .This study has some limitations. First, most reports related to OHVIRA comprise only individual cases, which are caused by the rarity of this disorder. Only some studies showed a larger number of cases. Also, some data from medical history was missing or the value of medical images was poor.The purpose of this study was to outline the correct management pathway and surgical approach concerning OHVIRA, which could vary not only due to anatomical differences, but also due to medical advances and the possibility of delayed diagnosis.OHVIRA conditions may be associated with rUTI and CAKUT. Our findings show, that a careful evaluation of the urinary tract should be recommended in all OHVIRA patients.However, delayed diagnosis may result in late complications, including retrograde tubal reflux and endometriosis. Unrecognized and improperly treated congenital malformations of the vagina, cervix, and uterus, although rare, may have severe implications for future family planning in OHVIRA patients.In our case, transperineal sonography turned out to be a helpful tool in the early diagnosis of OHVIRA. Combined management of laparoscopy and vaginoscopy should be considered in all female patients with suspicion of congenital abnormalities and acute abdominal symptoms as standard procedure.Vaginal septum resection with drainage seems to be the ideal surgery to restore normal vaginal function. After surgery, adolescent patients should undergo regular follow up visits in order to prevent an adhesion-formation recurrence after vaginal septum resection.


Testicular torsion (TT) is the most common urosurgical emergency in the case of acute scrotal pain. Early diagnosis, both clinically with imaging and emergent surgical exploration, is the key step in the salvage of testis and its management.A 12-year-old male with no known comorbidities came to the emergency department of our center with complaints of left scrotal region pain and swelling for 10 h.Left testicular tenderness and swelling with Phren’s sign negative, Deming’s sign positive, and absent cremasteric reflex. Ultrasonography showed coarse echotexture with a lack of obvious vascularity in the left testicle suggestive of TT and bulky left epididymis with bilateral hydrocele; left side greater than right.The patient underwent emergency left orchidectomy with right orchidopexy. Following this, he was symptomatically better, and the excruciating testicular pain and swelling subsided.Extravaginal TT is a rare presentation in pubertal age groups; however, whatever may be the types and causes, TT is a urological emergency that may lead to permanent ischemic necrosis. Delays in diagnosis should be avoided as this is directly related to the percentage of testicular salvage or loss. Prompt emergent surgical exploration is the cardinal point in management. HighlightsTesticular torsion is the most common urosurgical emergency in a case presenting with acute scrotal pain.Extravaginal testicular torsion is a rare presentation in the pubertal age groups.Early diagnosis by both clinical and Doppler studies is a must.Delay in diagnosis should be avoided as this is directly related to the percentage of testicular salvage or loss.Emergent surgical exploration is the key point in management.Torsion of the testis is a urological emergency, and clinical suspicion of testicular torsion (TT) is a serious indication for prompt surgical explorationHerein we report a rare case of spontaneous nontraumatic idiopathic extravaginal TT and its management with surgical orchidectomy and orchidopexy of the viable testis. This case report has been reported in line with the SCARE 2020 criteriaWe reported this case following the updated consensus-based Surgical CAse REport (SCARE) guidelinesA 12-year-old male with no known comorbidities came to the emergency department of our center with complaints of left scrotal region pain and swelling for 10 h. Pain developed following voiding of urine, which was sudden on the onset, prickling in nature, continuous, progressive, aggravated on movement, and was associated with lower abdominal pain and scrotal swelling, which was sudden and gradually progressive. There was no history of any trauma, fever, vomiting, dysuria, weight loss, or any congenital anomaly. On clinical examination, the patient looked anxious with left testicular tenderness and swelling with positive Deming’s sign, negative Phren’s sign, absent cremasteric reflex, and Testicular Workup for Ischemia and Suspected Torsion (TWIST) score of 5. His past history, family history, and allergic history were nonremarkable, and he had normal bowel and bladder habits.On examination, his vitals parameters were stable. The laboratory analyses were sent, which were nonremarkable. Serology was nonreactive for HIV, hepatitis B, and hepatitis C. Ultrasonography (USG) of the scrotum was ordered, which showed coarse echotexture with a lack of obvious vascularity in the left testicle suggestive of TT and bulky left epididymis with bilateral hydrocele left greater than right.The patient was then rushed to the operation theater with the proper consent for the surgery. He underwent left orchidectomy  with decreased platelets so peripheral blood smeared was repeated which showed platelets count to be 142 000 cells/mmFollow-up was carried out in the outpatient department after 1 month, and he is currently asymptomatic without testicular pain and swelling.TT is a surgical emergency commonly seen in adolescentsPatients usually present with sudden onset scrotal pain associated with nausea and vomitingTT is associated with anatomical deformities like bell clapper, congenital anomalies like cryptorchidism, ectopic testis, and traumatic and environmental factorsThe high index of suspicion helps in early diagnosis and prompt management and thus increases testicular salvage. Proper physical examination is the most important to diagnose and rule out when a patient presents with testicular pain. The TWIST scoring system aids the physician in diagnosis. This scoring system was developed to diagnose TT on clinical grounds, thus decreasing the need for ultrasoundColor Doppler ultrasound is accurate and sensitive for the diagnosis of torsion in the setting of acute scrotumThe ideal treatment is surgical exploration and orchidectomy with contralateral orchidopexy or bilateral orchidopexy, depending on the condition of the affected testisThough timely intervention could not be made in our patient due to the delay in patient presentation and the inability to salvage the affected testis could not be made, however, through clinical and imaging intervention helped in the diagnosis and could successfully relieve the patient’s excruciating pain with prophylactic orchidopexy of the viable testis.TT is a urological emergency. Thus meticulous emergent clinical history and physical examination, and Doppler USG should confirm the diagnosis. TT should always be considered one of the leading causes of acute scrotal pain. Delay in diagnosis should be avoided as this is directly related to the percentage of testicular salvage or loss. There are extravaginal, intravaginal, and mesorchial types according to age groups, and etiology may be different whatever the types of TT results into acute ischemia, and urgent surgical exploration is the key point of management for detorsion, reperfusion, and definitive management.Not required.Written informed consent was obtained from the patient’s guardian (mother) for the publication of this case report and the accompanying images to this report, as the patient is a minor. A copy is available for review by the Editor-in-Chief of this journal on request.There is no funding for our manuscript.B.B.R., S.B., S.K., M.S., S.B., and S.M.: led data collection, contributed to the process of original draft preparation, conceptualization, methodology, and discussion. All the authors approved of the final version of the manuscript and agreed to be accountable for all aspects of the work, ensuring questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.There are no potential conflicts of interest or financial or personal relationships with others that could influence the manuscript.Sunil Basukala.Not applicable.Not commissioned, externally peer-reviewed.


Osteoma cutis (OC) is an aberrant development or deposit of mature lamellar bone within dermis and subcutaneous tissue. We report a case of acquired plate‐like OC on the scalp, focusing on dermoscopic and ultrasound features.A 23‐year‐old man presented with an irregular plaque localized at the left fronto‐temporal area since the age of 14, arising as a small verrucous neoformation and with significant growth over time. The patient had no concomitant disease and was not taking any therapy.On clinical examination, a 7 × 3 cm pinkish plaque with yellowish keratotic plugs and papular satellite lesions extending toward the left eyebrow, was present; the lesion had hard‐ligneous consistency and was associated with alopecia Figure . It was Histology revealed bone deposits surrounded by plasma cells, granulocytes and neutrophils at the medium and deep dermis Figure .Blood tests ruled out metabolic disorders or abnormalities in calcium and phosphorus; a skull X‐ray demonstrated no involvement of the cranial theca.A diagnosis of plate‐like OC was performed, basing on histologic findings and the presence of all diagnostic criteria, except for the early onset.The patient refused complete excision due to possible cosmetic consequences resulting from a wide surgical scar. He decided to remove only a periocular papule because of visual discomfort.Primary OC is a rare benign condition, first described by Wilkens in 1858, as an aberrant presence of bone in the dermis or hypodermis.de novo bone formation, and it can be associated to syndromic diseases such as Albright's hereditary osteodystrophy (AHO), progressive ossificans fibrodysplasia (POF), or progressive osseus heteroplasia (POH).The classification of OC distinguishes two types: primary and secondary. Primary OC is a rare condition, about 15% of all skin ossifications, corresponding to osteoma cutis in plaque was coined by Worret and Burgdorf in 1978.Clinical classification of OC distinguishes four patterns of presentation: single, plate‐like, widespread, or multiple miliary. The term Although histopathology remains the gold standard the diagnosis, we want to draw attention to non‐invasive tools, such as ultrasound and dermoscopy. Only two sonographic reports of OC are present in literature:Dermoscopy features of OC also have been rarely reported: the milkish appearance, corresponding to a yellowish amorphous area with whitish streaks, suggests the ossification.Plate‐like OC is not associated with risk of neoplastic transformation; therefore, the treatment should consider the patient's wishes regarding the cosmetic outcome.The authors declare no conflicts of interest.


Intracranial dermoid cysts are unusual cystic tumors that are often benign, develop slowly, and are present from birth. They are made up of mature squamous epithelium and may house ectodermal features such as glands . Dermoid cysts may be asymptomatic and can be detected incidentally during brain imaging for unrelated causes. Dermoid cysts tend to grow gradually and may eventually exert pressure on the brain and surrounding areas. Unfortunately, they can seldom burst, resulting in an unfavorable prognosis for the patient depending on the size, location, and clinical presentation. Headache, convulsions, cerebral ischemia, and aseptic meningitis are the most frequent symptoms. Magnetic resonance imaging (MRI) and computed tomography (CT) of the brain aid in accurate diagnosis and therapy planning. In some cases, the treatment consists of surgical monitoring with regular surveillance imaging. In other cases, surgery is needed, depending on the symptoms and the location of the cyst in the brain. Intracranial dermoid cysts are rare tumors that develop from ectopic epithelial cells. The entrapment of the surface ectoderm along the lines of embryonic fusion gives rise to congenital dermoid cysts (CDS) and can keep expanding. With this ability for continual development, proper monitoring of its extension to epidural space and a plan for removal should be highly considered . DermoidA 54-year-old female was brought to the emergency department after two episodes of abnormal tonic-clonic seizures, followed by altered sensorium, and frothing from the mouth and incontinence. It was her third episode in the past two days. No significant past medical history was present. No contributory family history was present, and she had no history of substance or alcohol abuse.On physical examination, the patient was afebrile, not oriented to time, place, and person, with a heart rate of 65/min, blood pressure of 140/70 mmHg, and respiratory rate of 15/min. Her systemic examination, including neurological examination, was unremarkable except for a headache. She had no signs of meningeal irritation or involvement of cranial nerves. Initial laboratory investigations were normal. A provisional diagnosis of seizure disorder was made, as evidenced clinically. The patient was started on levetiracetam 500 mg twice times a day and clobazam 10 mg twice a day for new-onset seizures. Electroencephalography (EEG) was unremarkable with most of the waves of 8hz and higher frequencies.CT scan of the brain revealed a hypodense lesion with a cystic component, linear calcifications, and smooth margins Figure . In addiAfter integration, clinical and radiological features do not fit aneurysm or meningioma. However, after further consultation from the neurosurgery department, the diagnosis of the intracranial dermoid cyst was elucidated based on distinguishing features on CT and MRI images. Informed surgical consent was taken from the patient for the neurosurgical procedure and she was taken for craniotomy for surgical removal of the cystic lesions. The cystic components and their capsule were removed successfully and sent for histopathology, which confirmed dermoid cysts with stratified squamous epithelium associated with adipose tissue, keratinous debris, calcific depositions, and cholesterol pockets. Postoperatively, the patient remained neurologically intact. An MRI scan of the brain obtained three months after resection showed minimal persistent intraventricular fat droplets without recurrence or discernible complications of the resection. The patient has resumed most normal activities.Dermoid cysts seldom cause symptoms. The primary presenting complaints are seizures and headaches, which are connected to its mass-effect features . DespiteDermoid cysts arise from ectopic epithelial cells . These aRadiologically, intracranial dermoid cysts can be easily noticed. The hypodense appearance of dermoid cysts on CT is due to their lipid content . DermoidDermoid cysts, when symptomatic, can be surgically excised. Recurrence is uncommon, provided that complete excision is achieved. Sometimes, due to the local adhesion of the capsule to vital structures, incomplete excision must be performed . There iA total resection is a reasonable consideration if the location is favorable, to prevent seizures, and to send the sample for histopathological diagnosis. However, in cases where the tumor is located at either the suprasellar region or cerebellopontine angle, there may be a possibility of severe adhesion occurring between the tumor and the cranial nerves, brain stem, and vessels, and this will make complete resection of the tumor difficult, and in such situations, excessive resection should not be an option ,8. To prIntracranial dermoid cysts are rare, benign, slow-growing cystic lesions but occasionally can cause complications depending on their size and location. Our case report accentuates a rare incidence, which presented emergently with seizures. Clinicians should be aware of the radiological pattern of intracranial dermoid cysts, hypodense on CT and hyperintense on MRI, which along with the location  can aid in its diagnosis. Seizures, especially if they are not controlled, are a good indication of the removal of a tumor.


Urinalysis showed moderate leukocyte esterase with microscopy showing 12 white blood cells and three red blood cells per high-power field. CT of the abdomen and pelvis with intravenous contrast was notable for moderate amounts of left-sided perinephric and periureteric fluid without the presence of an obstructing calculus. Due to the amount of perinephric and periureteric fluid without associated nephrolithiasis, the differential diagnosis was broadened to include spontaneous ureter rupture as well as concern for malignancy. A delayed post-contrast CT scan of the abdomen and pelvis was obtained, which confirmed a spontaneous proximal and mid-ureter rupture. Spontaneous ureter rupture is a rare disease process with significant morbidity and mortality. It often poses a diagnostic dilemma due to a lack of clinical awareness and varied presentation. Diagnosis rests upon obtaining delayed post-contrast CT of the abdomen and pelvis. Currently, there are no standardized treatment guidelines, although most experts utilize minimally invasive endourological approaches in their treatment plans.A 67-year-old female presented to the emergency department with acute-onset severe left flank pain as well as nausea and vomiting. Physical examination was notable for left-sided abdominal, flank tenderness, and costovertebral angle tenderness. Laboratory workup revealed an elevated lactate of 9.2 mmol/L and elevated serum creatinine of 1.14 mg/dL, with an estimated glomerular filtration rate of 53 mL/minute/1.73m Spontaneous ureteral rupture, a rare and emergent urological entity, is associated with increased intraluminal pressures resulting in urinary extravasation from the ureter secondary to a non-traumatic etiology -4. It is2. Urinalysis demonstrated moderate leukocyte esterase with microscopy showing 12 white blood cells and three red blood cells per high-power field. The patient underwent a CT scan of the abdomen and pelvis with intravenous contrast. Imaging revealed an edematous left kidney with dilation of the left collecting system as well as a moderate amount of perinephric and periureteric fluid without the presence of an obstructing calculus (Figure A 67-year-old female with a past medical history of hypertension was brought in by emergency medical services to the emergency department with an acute onset of severe left flank pain. The patient noted that the pain started several hours prior in the setting of no acute inciting factors, traumatic injuries, or changes in her activities of daily living. The pain was not alleviated or exacerbated by anything and was described as a stabbing sensation with radiation from the left flank to the left thoracic paravertebral musculature. The patient additionally endorsed associated nausea and multiple episodes of non-bloody, non-bilious emesis. The patient’s vital signs were noted to be normal, and examination revealed left lower quadrant abdominal tenderness as well as left costovertebral angle tenderness to palpation. Laboratory workup included a complete blood count, compressive metabolic panel, coagulation profile, urinalysis, and serum lactic acid testing, which were found to be unexceptional except for an elevated lactate of 9.2 mmol/L as well as an elevated serum creatinine of 1.14 mg/dL, with an estimated glomerular filtration rate of 53 mL/minute/1.73mDue to the amount of perinephric and periureteric fluid present, the decision was made to obtain delayed CT imaging to exclude ureteral injury. CT with delayed images obtained after contrast injection revealed extravasation of contrast from the left proximal as well as mid-ureter Figure .The patient’s presentation was consistent with spontaneous ureter rupture. The patient received intravenous antibiotics as well as intravenous fluids and underwent cystoscopy and left-sided retrograde pyelogram. This confirmed extravasation of contrast 2 cm distal to the left ureteropelvic junction without the presence of any stones, filling defects, or other etiologies of the ureter rupture. The patient then underwent placement of a double-J stent with Foley catheter placement. The patient was discharged with oral antibiotics and outpatient follow-up for repeat cystoscopy and evaluation for possible stent removal in one month. The patient’s postoperative course was complicated by transient hematuria with outpatient CT urogram showing satisfactory positioning of the left ureteral stent without hydronephrosis, no contrast extravasation, and symmetric excretion of contrast material bilaterally. The patient ultimately underwent a repeat cystoscopy with left ureteroscopy and double-JJ stent removal with no noted stones, tumors within the ureter or within the renal pelvis, and no evidence of extravasation of the ureter.Spontaneous ureteral rupture, with its varied and non-specific presentation, represents a rare but vitally important disease process to promptly identify and treat. While the majority of literature on spontaneous ureter rupture stems from the field of urology, it is a disease entity that can be encountered by many different medical specialties. Although rare, it is important to be aware of this entity as the imaging modalities to diagnose this condition are uniquely different than those used to diagnose the more common and less emergent disease processes that spontaneous ureter rupture mimics. Diagnosis relies upon obtaining delayed CT imaging of the abdomen and pelvis post-intravenous contrast. A delayed post-contrast CT scan is not a commonly ordered imaging study. When this is coupled with a general lack of clinical awareness of spontaneous ureter rupture as well, it is easy to see why spontaneous ureteral rupture often poses a diagnostic problem. Our patient’s initial presentation of severe flank pain mimicked renal colic and our workup was tailored to ruling out nephrolithiasis. The appearance of perinephric fluid and periureteric fluid on the initial contrast-enhanced CT scan is what altered us to the possibility of a potential ureter rupture. Once this disease entity was entertained, we were able to obtain a delayed CT scan post-intravenous contrast to confirm the diagnosis. In this particular case, the repeat CT scan was performed close to two hours following the initial contrast injection, but studies have shown that delayed images obtained as early as 5-20 minutes after contrast injection can confirm this diagnosis . The useSpontaneous ureter rupture is a rare and emergent disease process that may often be misdiagnosed due to a lack of clinical awareness. It is associated with significant rates of morbidity and mortality. Delay in diagnosis and treatment may lead to complications such as renal failure, abscess formation, urinoma, as well as septic shock. Prompt recognition and awareness of this disease state are essential to mitigate the complications and mortality associated with it. This case report should serve to reinforce this rare disease process as well as to reinforce the unique diagnostic modality necessary to confirm the diagnosis.


With chemotherapy, lung computed tomography revealed that the tumor had become much smaller, and the fluid was absorbed. We report a case of extraskeletal Ewing’s sarcoma (EES) in the mediastinum with pleural effusion, which is unusual and challenging. EES is a highly malignant tumor with a poor prognosis. Early diagnosis and treatment can improve the survival rate of patients.Ewing’s sarcoma (ES) is an aggressive cancer in young adults. Primary ES occurring in the chest with pleural effusion is even rarer. We report the case of a 15-year-old girl who presented with intermittent chest pain occurring for more than 2 months and cough and wheezing for 10 days. Radiological imaging showed a large soft tissue mass with multiple small vessel shadows near the left mediastinum and bloody pleural effusion in the left thorax. ES was diagnosed by positive immunostaining for CD99, FLI-1, and NKX2 combined with fluorescence   The tumExtraskeletal Ewing’s sarcoma (EES) accounts for approximately 20% of ES cases and shows a bimodal age distribution among children and adults . The inc2A 15-year-old girl was admitted to our hospital due to intermittent chest pain occurring for more than 2 months and cough and wheezing for 10 days. The patient was a student, and her family denied a history of malignancy. Chest examination revealed solid sounds obtained during percussion and low breath sounds over the left thorax.The laboratory tests showed increased procalcitonin (26.59 ng/ml), elevated interleukin-6 (15.2 pg/ml), and a slightly high erythrocyte sedimentation rate of 28 mm/h. The patient underwent transcatheter thoracic drainage to determine the features of the pleural effusion, and a routine examination showed bloody turbid pleural effusion without tumor cells . HoweverA chest computed tomography (CT) scan showed a large soft tissue mass (15.7 × 11.7 × 10.5 cm) near the left mediastinum with multiple nodules and masses in the left pleura, and the fluid could be seen on the same side of the thorax . Moderatin situ hybridization (FISH) suggested EWSR1 gene breakage and rearrangement . The tumor cells were negative for MPO, GFAP, CD30, EMA, CgA, desmin, and WT-1 . The negangement . Based oConsidering the age, tumor size and properties, and the consensus from the MDT meeting, the patient received treatment with cyclophosphamide (1.8 g), adriamycin (doxorubicin) (75 mg), and vincristine (2 mg) (VDC) for the first cycle. After finishing one cycle of chemotherapy, a CT scan of the chest revealed that the tumor was approximately half its size before treatment, and the pleural effusion had decreased . After aInformed consent: Informed consent has been obtained from all individuals included in this study.Ethical approval: The research related to human use has been complied with all the relevant national regulations, institutional policies, and in accordance with the tenets of the Helsinki Declaration, and has been approved by the authors’ institutional review board or equivalent committee.3EES and ES both belong to the ES family of tumors, which has a common histology and genetic mechanism. ES is the second most common bone cancer in children, and EES only occurs in 20% of ES cases . HoweverES and EES are characterized by monomorphic, small, round blue cells. Small round cell sarcomas (SRCSs) share histological similarities with ES. Capicua transcriptional repressor (CIC)-rearranged sarcoma (CIC sarcoma) and BCOR-rearranged sarcoma (BCOR sarcoma) are two typical SRCSs . CIC sarEarly diagnosis and treatment of EES can improve the survival rate of patients. The diagnosis is mainly based on imaging and histopathology. Imaging plays an important role in the evaluation and treatment management of EES . CT findTreatment for EES is similar to that for ES. The current management of primary ES is a combination of cytotoxic drugs and local management, which includes surgery and radiotherapy . MultiagAlthough pulmonary EES has been reported in previous case reports, primary EES in the mediastinum with massive pleural effusion is rare . We usedIn summary, EES is a highly aggressive tumor with a poor prognosis that can occur in many parts of the body. The diagnosis should involve a combination of clinical manifestations, CT imaging, and multidisciplinary discussion. Image-guided needle biopsies are especially important because they can provide early diagnosis. To improve the long-term survival rate of patients, chemotherapy should be adopted in a timely manner in combination with surgery or radiotherapy.4In this study, we report a rare case of primary mediastinum EES with massive pleural effusion. EES is a highly malignant tumor with a poor prognosis. Reports of this disease are rare, and there is little experience in treatment. The diagnosis and treatment of this disease require the joint discussion and analysis of experts from multiple disciplines . Therefore, we must summarize the characteristics of more cases to provide evidence for further early diagnosis and improve the survival rate of patients.


Oxaliplatin-based regimens are commonly used as adjuvant chemotherapy following surgery for colorectal cancer. Adverse events associated with oxaliplatin include blue liver, which is caused by sinusoidal dilation and diffuse peliosis hepatis. We report herein a case of localized peliosis hepatis closely resembling a metastatic liver tumor.The patient, a 50-year-old male, underwent a robotically assisted colectomy for rectosigmoid colon cancer, which was discovered when hematochezia occurred. The patient received a diagnosis of pStage IIIb and was treated with four courses of CAPOX as adjuvant chemotherapy starting at postoperative month 1. At postoperative month 4, contrast-enhanced computed tomography (CT) of the abdomen revealed a 20-mm, low-density area with heterogeneous internal structure in S6/7 of the liver. Abdominal ultrasound and gadolinium ethoxybenzyl-diethylenetriaminepentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI) findings led to a diagnosis of metastatic liver tumor, for which a laparoscopic partial hepatectomy was performed. The resected lesion was a dark reddish-brown nodule with indistinct margins that appeared to be continuous with the surrounding area. Histopathological analysis revealed severe, localized dilatation of the sinusoids and congestion consistent with the gross nodule. Based on these findings, localized peliosis hepatis associated with oxaliplatin-induced sinusoidal damage was diagnosed.Localized peliosis hepatis associated with oxaliplatin use can be difficult to distinguish from a metastatic liver tumor on imaging studies. When a new liver mass appears after surgery for advanced colorectal cancer, liver metastasis is usually suspected. Early detection by regular imaging studies and accurate diagnosis based on various, detailed examinations are quite important .In recent years, postoperative adjuvant chemotherapy for advanced colorectal cancer has become common. In particular, the introduction of chemotherapy regimens with oxaliplatin has improved the prognosis of advanced colorectal cancer . On the The patient was a 50-year-old male who had undergone a robotically assisted colectomy for rectosigmoid colon cancer. Histopathological analysis confirmed the diagnosis of moderately differentiated adenocarcinoma, pT3, pN1a, pM0, pStage IIIb. Starting at postoperative month 1, the patient received four cycles of CAPOX as adjuvant chemotherapy.At postoperative month 4, contrast-enhanced abdominal computed tomography (CT) revealed a 20-mm, low-density area with heterogeneous internal structure in S6/7 Fig. A suggestBlood tests at the time of the discovery of the liver lesion revealed CEA 2.6 ng/mL, CA19-9 8.2 U/mL, AST 23 IU/L, ALT 21 IU/mL, indicating normal tumor markers and liver enzymes.Based on these findings, liver metastasis of colon cancer was diagnosed, and a laparoscopic partial hepatectomy was performed. Intraoperative findings revealed a solitary lesion at S7 with no obvious abnormalities in the background liver. No blue liver or splenomegaly to the varices suggestive of portal hypertension was observed intraoperatively. The resected specimen was a dark reddish-brown nodule measuring 1.8 × 1.8 × 1.5 cm with indistinct borders Fig. A.Fig. 2RHistopathological analysis revealed severe, localized dilatation and congestion of the sinusoids consistent with the gross nodule Fig. B, C. ThePeliosis hepatis was first described by Wagner in 1861 and named by Schoenlank in 1916. The first description in the English-language literature was published by Zak in 1950 . PeliosiThere are few reports describing the findings typical of peliosis hepatis on imaging studies, such as CT and ultrasound. The characteristics of the condition are not yet well known but may include heterogeneous hypoechoic to isoechoic areas on abdominal ultrasound; low attenuation and internal heterogeneity in the late arterial and portal phases on contrast-enhanced CT ; low sigAlthough peliosis hepatis is definitively diagnosed on the basis of histopathological findings, the risk of bleeding is high even with ultrasound or CT-guided biopsy owing to the vascularity of the lesion, and occurrences of a life-threatening hemorrhagic shock after CT-guided biopsy have been reported . While aDiagnosing localized, solitary peliosis hepatis developing after oxaliplatin administration, as in the present case, is difficult without a hepatectomy . HoweverLocalized peliosis hepatis associated with oxaliplatin treatment can be difficult to distinguish from a metastatic liver tumor on imaging studies.


Apocrine Hidrocystoma is a relatively rare benign tumour that begins from the apocrine sweat glands of the head and neck. The Authors present a case series of children with urogenital localization.Two boys (15 years and 9 years) presented with a small mass on the glans. Another 15-year-old boy presented with a cystic lesion in the right side of the scrotum where he had a previous surgery. The last case, a 17-year-old boy, presented because of a penile cyst of 8 mm. All four had surgical operations because of aesthetic discomfort or problems during micturition. Histological examination showed a diagnosis of apocrine hidrocystoma in all cases.This benign tumour rarely affects the urogenital system in children, but when it happens the child can have discomfort and proper treatment is mandatory.Surgery is the preferred treatment with a low risk of recurrence. •Apocrine hidrocystoma is quite rare, benign tumour, beginning from the apocrine sweat glands of the head and neck.•Rarely this tumour involves the uro-genital system.•Is is a rare tumour in the uro-genital system of children.•We present 4 cases who required surgery because of aesthetic discomfort and problem during micturition. The mos2This is a case report from our pediatric surgical department, and has been reported in accordance with the PROCESS criteria .2.1A 15-year-old boy came to our attention reporting a small painless mass on the glans appeared about a year and with a growing trend. The boy complained for several months of aesthetic discomfort and problems while urinating. The personal and family history was not relevant. Clinically, the patient exhibited cystic swelling (approximately 1 cm in diameter) on the glans gland just next and inside the urethral meatus. As a result of reported problems, surgical excision with sedation and local anaesthesia was performed. During surgery, the lesion appeared larger: extending along the urethra to approximately 2 cm. After complete excision, the urethral meatus was reconstructed  and a ur2.2A 9-year-old child came to our hospital for the presence of a cyst approximately 3 mm at the base of the glans, in the ventral region. A further check was carried out at the end of 6 months: the cyst had slightly increased in volume. The child reported discomfort while retracting the foreskin and was recommended for surgery. The surgery was performed with sedation and local anaesthesia. The size of the excised lesion was 4 mm and was limited to the superficial portion of the glans without affecting the urethra. At one-year follow-up, the lesion was not present and the patient was doing well. Histological examination showed a cystic lesion delimited by a single cubic epithelium and a peripheral layer of myoepithelial cells: hidrocystoma apocrine .Fig. 3Hi2.3A 15-year-old boy presented with a cystic lesion in right side of the scrotum. This patient had undergone transcrotal orchidopexy 1 year earlier in another hospital, a month after the surgery he had a dehiscence of the wound which was sutured. Six months later, a cystic lesion appears on the scrotal scar. No pain was reported, just discomfort while wearing clothes. The lesion looked full of liquid, translucent and measured 3 cm in diameter . An ultr2.4A 17-year-old boy came to our attention because of a localized penis pain during erection. During the clinical examination, the patient showed a recurvatum of the glans, a hypertrophy of the frenulum and an 8 mm cystic formation on the penis. The boy complained of pain in the area where the cyst was and increased size. Therefore, the surgical procedure was performed with total removal of the cyst in sedation and local anaesthesia. Histologic analysis led to the diagnosis of Hidrocystoma apocrine. During the six-month follow-up, no pain or cyst was present.3Apocrine hidrocystoma (AH) is a rare benign proliferation of the sweat glands present in the head and neck . It can 4The diagnosis of AH remains clinical and histological. The tumour is benign, but when it affects particular districts, such as genitalia, it can cause problems. The treatment can be surgical or conservative, but in the second case the recurrence seems more common. Based on our experience in childhood, we can say that surgical removal is the preferred treatment for easy surgierremoval and the lower risk of recurrence.Written informed consent was obtained from the patient's parents/legal guardian for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Our University (University of Campania “Luigi Vanvitelli”) exempts the case reports from ethical approval because it is a small group of patients and does not involve any experimental procedures.No funding was received.Noviello Carmine: Study conception and design, Drafting of the manuscriptTrotta Letizia: Data acquisitionRomano Mercedes: Analysis and data interpretationAlfano Roberto: data analysisRonchi Andrea: histology analisysPapparella Alfonso: Critical revision.Noviello Carmine.N/A.The authors declare that there is no conflict of interests.


Surgical separation of conjoined twins remains one of the most unique and rewarding experiences in the field of pediatric surgery, bearing in mind that this decision is their best chance of survival. These are the first reported cases of successfully separating omphalopagus conjoined twins by the liver in Sudan. After an emergency cesarean section, 62-day-old term-conjoined twins were referred to our pediatric surgery center. Examination revealed well-appearing twins fused from the xiphoid to the umbilicus; imaging confirmed a fused liver with a separate portal and caval structures, necessitating surgical separation and closure, which was done successfully on subsequent hours with well tolerance and recovery discharged on day 21. The second case involved 21-day-old term-conjoined female twins who were fused from the xiphoid to the umbilicus and shared the same cord, as well as complete fusion of the liver with separate other vital organs. They were successfully separated and recovered well. Throughout history, the separation of conjoined twins has remained one of the most difficult challenges in the field of pediatric surgery, owing to the technical and ethical complexity of the procedure, which is extremely risky and life-threatening in the majority of cases . This coThe incidence varies between one in 50 000 and one in 20 000 live births with female commonality , 4. The Even though these twins appear theoretically divisible, any attempt to separate them may result in severe hemorrhage and hypovolemic shock, especially if done early in life. These are infrequent in the ischiopagus and pyopagus varieties .Herein we report the first two cases in Sudan of Omphalopagus twins conjoined by the liver referred to our pediatric surgery center, separated successfully by our multidisciplinary team led by pediatric surgeons with a good outcome.A 62 days old, term-conjoined female twins were referred by ambulance to the pediatric-center police hospital, Sudan.The mother was a 21-year-old female with a family history of multiple pregnancies, para three delivered uneventfully vaginally with a good outcome. Her pregnancy passed well with good antenatal care and early use of tonics, on 30 weeks the abdominal ultrasound revealed monochronic conjoined twins. At 40 weeks gestation, an emergency cesarian section was done due to labor pain, outcome was full-term, monochromic conjoined twins, cephalic, cried immediately and passed urine and meconium within 1st 24 h. On examination, both babies appeared well, not pale or jaundiced and not distressed, and weighed 2.4 kg for each one. They were fused from the xiphoid to the umbilicus with the used area covered by skin, and shared the same umbilical cord. Abdominal Computed tomography revealed a fusion of the liver through a large isthmus side by side with normal liver size and texture, no connections at the portal vein, normal vena cava and other structures . other oAfter anesthetic preparation and central line insertion, both twins were placed on sterile sheets and then prepped anteriorly, Incision was made using monopolar and bipolar diathermy in the connecting bridge, skin and muscle were cut, connections within parietal peritoneum were identified, and then carefully divided, followed by the connecting part of the liver , after tThe abdominal wall was closed with Polydioxanone suture (PDS) sutures without mesh, and they were intubated before being transferred to the intensive care unit. Both babies were extubated within 2 days, and antibiotics were administered for another 21 days. On day 4, oral feeding began with good tolerance. Both babies gained weight and were discharged on day 25. They were later seen at the referring clinic after 2 weeks with good healing and well-being.A 21 days old, term-conjoined female twins were referred by ambulance to the pediatric-center police hospital, Sudan.The mother is a primigravida of 24 years old with a positive family history of multiple pregnancies. Her pregnancy was uneventful, with regular antenatal care and tonic use; however, an abdominal ultrasound at week 13 revealed multiple pregnancy. Due to liquor drainage, she had an emergency cesarean section at 38 weeks; the outcome was term monochronic, conjoined twins, breech cried immediately and passed urine and meconium within the first 24 h. On examination, both twins appear healthy, not pale or jaundiced, fused from the xiphoid to the umbilicus and covered by skin, sharing the same cord with a combined weight of 4.5, twin A 2.5 kg and twins B 2 kg.A computed tomography scan revealed ventral omphalopagus-xiphoid union of conjoined twins with separate vital organs and signs of fusion of the anterior aspect of the livers (adhesion), as well as one lower limb arteriovenous malformation (AVM) (AV fistula) seen extending from external iliac major vessels in both with difference in affected side . Both twFollowing the placement of central lines, the two babies were anathesized and stabilized with vital sign monitoring and fluid adjustments. Then, using monopolar and bipolar diathermy, incisions and flaps were made until the peritoneum, where complete fusion of the liver with a sinusoidal connection was discovered. The connected surface area is ⁓8 by 6 cm, primarily in the caudal lobe, whereas twins B has one lobe liver and one gall bladder . The xipThe history of conjoined twins’ dates back to as early as 80 BC, with a stone, craved depicting pygopagus twins discovered in Fiesole Italy. The earliest known attempt to separate conjoined twins took place in Armenia in 945 AD, when a separation of the ischiopagus twin from his dead brother was done, however, he died 3 days later [The first well-reported case of successful surgical separation of conjoined twins was by Farius in 1689 , 6.Conjoined twins are monozygotic, monoamniotic and monochorionic twins. The condition is rare and has an incidence ranging from one in 50 000 to one in 200 000 per live birth, with only 50% being live born . It is eThe conjoined twins are classified into eight types, with five ventral types and three dorsal types. The most common variant is Thoracopagus/thoraco-omphalopagus, with omphalopagus incidence ranging from 18 to 32%, making it the second most common type , 9, 10.According to the literature, there are no known genetic or environmental causes of this condition, with some studies suggesting assisted reproduction techniques and maternal griseofulvin use during pregnancy as possible associated factors , 12.The omphalopagus subtype is characterized by the fusion of the upper abdomen, from the lower chest to the umbilicus, with heart fusion characteristically absent. Over 80% will have fused livers with exomphalos, a conjoined biliary tract in 25%, 16% will have a share upper foregut and up to one-third will have intestines joining usually at Meckel’s diverticulum and share terminal ileum and colon , 13.Prenatal diagnosis can be made as early as 12 weeks using fetal Ultrasound (USS). Other investigations that can be used are Fetal echocardiography for detailed cardiac assessment and magnetic resonance imaging as an alternative technique for overall fetal assessment , 5. PrenThe omphalopagus conjoined twins have the best chance of survival if the surgical separation was done successfully , 13. Howet al. suggested operation at 10 months [The timing for surgery is largely controversial. Most surgeons would prefer the ‘watch and wait approach’ and delay the surgical intervention till after the neonatal period, where it is done in elective setting. O’Neil found that survival rate is at 50% if surgery done after the first 4 weeks, and it increases up to 90% after 4 months of age , 14. Vil0 months . This alDespite this, some cases require emergency surgery, this might be in cases such as when one twin is a threat to the life of the other , presence of severe malformation that threatens the life of twins and require intervention, substantial damage of the connecting bridge and when one of the twins have severe deformity that is incompatible with life and the other one have a good chance of survival , 7. In oVarious methods for closure of abdominal wall, including using a mesh, tissue expander, free flaps and skin grafts. The used method depends on the surgeon’s preference as well as the size of the defect , 14. In Despite limited facilities in Sudan, a multidisciplinary team approach with meticulous pre-operative assessment and post-operative care has led to a success in managing these two cases. The first set of twins were seen 39 days after operation at referral clinic, revealing healthy infants with normal development and growth.None declared.The study was self-funded.Written informed consent was obtained from parents after proper counseling.The data set used and/or analysed during the study are available from the corresponding author on reasonable request.


Adult Intussusception is an uncommon diagnosis, with one to three cases occurring in a population of 1,000,000 per year, primarily due to underlying pathological lead points, of which 70% are malignant. Lipoma is the most common benign tumour, and primary adenocarcinoma is the most common malignant one. Early diagnosis and treatment are essential to reducing poor outcomes, including ischemia, perforation, and sepsis. Computed tomography imaging is a modality of choice for diagnosis. With a diagnostic accuracy of up to 100% and a specificity of up to 71%. Surgical intervention is the definitive treatment, and the decision is taken according to the situation.A 61-year-old Black African female presented to our surgical casualty with intermittent colicky abdominal pain for 1 month. After that, she started to experience abdominal swelling in the right iliac fossa. A CT scan confirmed the presence of colo-colic Intussusception, cecum-ascending-transverse colon. Laparotomy was scheduled, and a right hemicolectomy was done accordingly. Diagnosis of adenocarcinoma (Dukes stage B2) was made histologically.Intussusception in adults is a challenging diagnosis requiring high clinical suspicion and has a high incidence of fatal complications. Colonic cancer can be worsening by any infection and chronic medical problem. CT imaging is the lifesaving modality of choice for diagnosis. Good patient outcomes depend on timely diagnosis and recruitment of a multi-disciplinary team. Intussusception means telescoping of a proximal segment of the bowel into the lumen of the adjacent distal segment (intussuscipiens). Rarely the opposite could happen, which is known as retrograde Intussusception .Intussusception is very uncommon in adult patients, with one to three cases occurring in a population of 1,000,000 per year, which puts it at the bottom of the list of differential diagnoses of abdominal pain. More often, it may not be counted. Its aetiology differs significantly between children and adults. Adult Intussusception of the colon is most likely secondary to a malignant tumour .In both small- and large-bowel Intussusception, the lipoma is the most common benign tumour, and adenocarcinoma is the common malignant leading point .The diagnosis is commonly surprised at laparotomy, as most patients present as an emergency with intestinal obstruction. Usually, in stable patients, the diagnosis can be challenging as symptoms, mainly intermittent abdominal pain and clinical examination and investigations are often negative, and the patient will probably be labelled with irritable bowel syndrome .Colonic cancer can be worsen by any infection and chronic medical problem .This work has been reported in line with the CARE criteria .A 61-year-old Black African female, medically free, presented to the emergency department of a Military hospital in Khartoum, Sudan, with severe colicky abdominal pain and right iliac fossa swelling for 1 month. The pain was sudden onset, severe, intermittent, and colicky in the right iliac fossa, preventing her from doing her daily activities. It was localized, not radiating to other areas, aggravated by food intake and not relieved by analgesics. On further questioning, it was revealed that there were streaks of blood in her stool. However, there was no constipation, diarrhoea, or vomiting. The patient was experiencing loss of appetite and unintentional weight loss, about 15 kg, 3 months before her illness. Body mass index was 24.2. There was no fever, night sweats, or cardiopulmonary complaints. The patient has no history of a similar condition and had not been diagnosed with colon cancer, inflammatory bowel disease, irritable bowel disease or any recent evidence of pulmonary tuberculosis infection.She has no family history of colon cancer; however, she has a history of breast cancer in the family. At the time, she was not on any medication or had any known allergies. She is a non-smoker and non-alcoholic.On physical examination, the patient looks ill, conscious, vitally stable, not pale nor jaundiced and has no signs of dehydration.Abdominal examination revealed a right iliac fossa mass, oval in shape, 12 × 10 cm, irregular surface, normal skin over it and no scars. The mass has a normal temperature, is tender, and is hard in consistency. It has well-defined edges, is mobile, not compressible or reducible, and not pulsatile. All hernia orifices are intact, and no lymph node enlargement. However, the rest of the abdominal examination was unremarkable, and the digital rectal examination was normal. Abdominal ultrasound was ordered and revealed the presence of colonic mass and multiple gall stones, but it was otherwise not remarkable.Then computed tomography imaging abdomen was ordered and that showed a segment of bowel loop was seen invaginated into its proximal segment with bowel wall thickening in the ascending colon extending to the transverse colon, which confirmed the diagnosis of colo-colic intussusception. Also, multiple gall stones were elicited by the CT scan Fig. .Fig. 1A The patient has low serum sodium, potassium, total bilirubin and albumin levels. Serum CEA tumour marker was within normal ranges. Chest x-ray and echocardiography were normal.So, an elective laparotomy was scheduled 2 days after the presentation. The patient was kept nil per mouth with full fluid maintenance, antibiotic, proton pump inhibitor, and prophylactic anticoagulant. An incision abdomen was opened through a midline, and a large mass was found, including the terminal ileum, the ileocecal valve, the appendix, the cecum and the ascending colon, which were invaginated in the transverse colon. Adhesions were found between the abdominal wall and the mass and were released, as colonic milking was unsuccessful. The surgeon decided to go for the right hemicolectomy and end-to-end anastomosis Anatomical changesPost-surgical adhesionsEndometriosisIdiopathicFibroidsGastrostomy tubeJejunostomy tube .Other risk factors that lead to an intussusception may include:In a previous study, the lesions were found to be varied from 3.5 cm to 8.5 cm in diameter .Inflammatory diseases of the colon or appendix can also play a leading point in Intussusception.Symptoms are sometimes vague, including colicky abdominal pain, intermittent or constant vomiting (can be bilious), bloating, and bloody stool .Inpatient presented with non-emergency nonspecific abdominal pain. CT scan appears to be the most sensitive diagnostic tool for picking Intussusception, with a diagnostic accuracy of 58–100% and a specificity of 57–71% .The CT findings one can illustrate will be a mass-like lesion, including the inner intussusceptum, an eccentric fat density mass that represents the intussuscepted mesenteric fat, and the outer intussuscipiens, and this appears as a “target” or a “sausage” mass according to the imaging plane.Additionally, a CT scan can aid in identifying the pathological lead points, and its extension also could help in anticipating the vascular status of the bowel. In some circumstances, a CT scan can predict the possibility of self-resolution of the condition .In our case, the abdominal CT scan picks up the colo-colic Intussusception and the colonic mass extension that sharply changes the forward workup of the patient.Ultrasound is also a helpful tool but less sensitive than CT scans. The characteristic features that could be revealed by ultrasound include target and doughnut signs in transverse view and pseudo-kidney signs in longitudinal view .The ultra-sonographic findings at the first presentation of our patient raised the colonic mass suspension, so an immediate CT scan was ordered. However, it did not elicit the presence of Intussusception.Surgical interventions are the mainstay of management of adult intussusception as it carries a very high incidence of underlying malignancy. After supportive emergency management, preoperative preparation of the patient for resection according to the appropriate oncological assessment is the plan .Intraoperatively, the location, size, and cause of the Intussusception and the viability of the bowel determine the appropriate decision for the surgical procedure .As the management is purely surgical in adults, there is still controversy about the trail of reduction intraoperatively before resection. There is a debate between two opposing schools. One supports intraoperative reduction as it may minimise unnecessary bowel resection. Another school is fighting intussusception reduction as the risk of dissemination of the malignant cells during the procedure .Adult Intussusception carries a poor prognostic picture due to the delay in diagnosis of its nonspecific presentation and the prevalence of underlying malignancy. This return the complications of vascular supply to be jeopardised and sepsis to manifest earlier with a high mortality rate, especially in developing settings such as Sudan .Intussusception in adults is a challenging diagnosis requiring high clinical suspicion and has a high incidence of fatal complications. CT imaging is the lifesaving modality of choice for diagnosis.Good patient outcomes depend on timely diagnosis and recruitment of an interprofessional team.


A 63-year-old male with multiple co-morbidities presented with a diabetic foot infection which was treated surgically. During admission to the hospital, he developed melena and underwent an endoscopic assessment which revealed an incidental finding of an ampullary mass. The histological analysis of the biopsy revealed ampullary carcinoma with mixed intestinal-type and pancreatobiliary-type features. A magnetic resonance imaging (MRI) of the liver with contrast presented the tumor as an ill-defined small soft tissue lesion measuring 8 x 9 mm in the ampullary region, with multiple lymph nodes in the periportal, peripancreatic, and para-aortic regions. There was no evidence of biliary obstruction. The patient underwent a Whipple procedure with no complications. The final histology report of the specimens taken stated that the tumor is predominantly in the duodenum and focally in the ampulla, and is a well-differentiated neuroendocrine tumor confirmed to be submucosal. The histopathologic and radiologic workup determined the pathological stage classification to be pT3N1, Mx G1. Gastroenteric neuroendocrine tumors (NETs), previously called carcinoids, are rare ,2. They A 63-year-old male patient was admitted as a case of a left diabetic foot infection and underwent debridement and Lisfrank amputation, followed by vacuum-assisted closure of the wound. His past medical history was significant for hypertension, type 2 diabetes mellitus, and dyslipidemia, whereas his surgical history was significant for umbilical hernia repair, cholecystectomy, and right nephrectomy. On postoperative day six, the patient passed black tarry stools and became tachypneic and tachycardic. His hemoglobin level dropped from 8.7 g/dL to 3.8 g/dL and he was given six units of blood transfusion, after which he underwent a gastroscopy to determine the source of the gastrointestinal bleed. The endoscopic assessment revealed multiple duodenal ulcers (Forrest classification: llA and lll) and a suspected ampullary mass from which a biopsy was taken Figure .Contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis with coronal reformats in the early arterial phase showed an ill-defined hypodense prominence of the ampullary region suspicious for an ampullary tumor, with no upstream dilatation of the common bile duct or pancreatic duct. Histological analysis with immunohistochemistry of the biopsy revealed ampullary carcinoma with mixed intestinal-type and pancreatobiliary-type features. A magnetic resonance imaging (MRI) of the liver with contrast showed an ill-defined small soft tissue lesion measuring 8 x 9 mm in the ampullary region. Multiple lymph nodes were noted in the periportal, peripancreatic, and para-aortic regions. The common bile duct (CBD) and main pancreatic duct were clearly traceable up to its confluence with no definite mass lesion and no evidence of biliary obstruction. The patient underwent the Whipple procedure with no complications. A hard mass was felt in the second part of the duodenum with multiple large >2 cms) porta hepatis lymphadenopathy which was excised and sent for histopathological analysis. There were no signs of hepatic or peritoneal deposits of secondaries. The abdomen was closed with two drains inserted, one near the pancreatic and CBD anastomosis and the other near the gastrojejunostomy. The drains had minimal output in the postoperative period. The final histology report of the specimens taken stated that the tumor, which is predominantly in the duodenum and focally in the ampulla, is a well-differentiated neuroendocrine tumor confirmed to be submucosal. Immunohistochemical stains were performed using chromogranin, synaptophysin, CD56, CKAEA/AE3, CK20, CDX2, PAX8, S100, and Ki-67  out of the 20 regional lymph nodes was positive for metastasis. The tumor was unifocally invading the pancreatic parenchyma and peripancreatic soft tissue. There was also lymphovascular and perineural invasion. The pathological stage classification  was pT3N1, Mx G1. His postoperative recovery had been uneventful and he was discharged home in stable condition. The chromogranin A level was found to be 3180 μg postoperatively and he was started on Creon by the oncology team. During follow-up visits in the outpatient setting, the patient denied malaise, weight loss, and other complications of the Whipple procedure.Neuroendocrine tumors are genetically diverse slow-growing tumors characterized by peptides that cause hormonal syndromes. The gastrointestinal (GI) tract accounts for 60% of primary tumors, followed by the tracheobronchial tree for 25%. Within the GI tract, carcinoid tumors most commonly occur in the small intestine. NETs at the ampulla of Vater are extremely rare, with studies showing an incidence of <1% to <0.05% of gastrointestinal NETs . AnnuallPatients with ampullary carcinoids usually present with jaundice. However, other symptoms may include abdominal pain, malaise, steatorrhea, weight loss, or melena due to GI bleed. Pancreatitis was also the chief complaint in some cases ,10. EarlAmpullary NETs that are well-differentiated have a 10-year survival rate of 71% compared to 15% in those that are poorly differentiated . ChromogTreatment of NETs depends on the severity, which is assessed by the symptoms, the patient's general condition, and age, as well as the tumor’s grade, stage, and site. The Whipple procedure along with lymphadenectomy is the cornerstone of management for patients who are able to undergo surgery since aggressive operative extirpation provides the only chance for a cure ,10. BiolThe unusual location of the NET in the ampullary region of our patient made it an interesting and challenging case. However, despite undergoing major surgery such as the Whipple procedure at an advanced age, the patient made an uneventful recovery. Though there have been a few studies on ampullary NETs, the information available is quite limited, hence, there exists a dire need for further research into this topic.


We describe the case of a 3.1-cm BM from CAC in the left parasagittal para-central sulcus region, which was treated using five-fraction SRS with a biologically effective dose (BED) of 81.6 Gy. In the SRS, the gross tumor volume  was defined based on computed tomography (CT)/T1/T2 matching (enhancing lesion 11.66 cm3), and 98.7% of the GTV (CT/T2 mass) was covered with 43.6 Gy (58% isodose) using volumetric-modulated arcs. The maximum tumor response was partial (19.7% of the prior GTV) and sustained for 15.2 months, leaving minor neurological symptoms. However, the patient developed neurological worsening at six months, attributed to adverse radiation effects with a CT/T1/T2 mismatch, for which medical management, including the addition of bevacizumab (BEV), was effective for one year. Multi-fraction SRS with a high marginal and internal BED and sequential systemic therapy, including BEV, can be a minimally invasive, efficacious, and durable treatment option for a large CAC-BM involving the central sulcus. Early co-administration of BEV following SRS, dose escalation to the GTV boundary, and more than five fractions of SRS may be considered to improve the efficacy and safety further.An isolated single brain metastasis (BM) is an extremely rare manifestation of failure in patients with cecal adenocarcinoma (CAC). Total  Brain metastases (BMs) are a rare, hematogenous spread from colorectal cancer (CRC), especially when developing without liver or lung metastases . Howeveren bloc resection (while preserving function) of the BM is preferable for immediate decompression and symptom improvement, a superior cerebral vein crossing directly above the lesion and draining into the superior sagittal sinus and/or poorly demarcated brain-tumor interface with profound microscopic brain invasion can compromise safe total resection [Given the limited survival of patients with CRC BMs, developing efficacious, safe, and minimally invasive treatments for CRC BMs is an important issue. Clinical management of patients harboring a symptomatic CRC BM >2 cm in maximum diameter, localized in the parasagittal para-central sulcus region, is a challenge. Although total esection . Given tesection .We describe a case of a symptomatic 3.1-cm BM located in the parasagittal para-central sulcus region, developing metachronously from cecal adenocarcinoma without any other distant metastases, which was treated by five-fraction SRS with a biologically effective dose (BED) of 81.6 Gy to the gross tumor boundary. The BM showed a partial response, followed by symptomatic local progression six months after the SRS, for which medical management, including changes in chemotherapy, was effective. The gross tumor itself sustained regression during the 15.2-month follow-up period. We discuss ways to further improve the efficacy and safety of this treatment scheme.This report was part of the clinical study approved by the Clinical Research Review Board of Kainan Hospital Aichi Prefectural Welfare Federation of Agricultural Cooperatives (20220727-1).A 68-year-old, right-handed male, with a Karnofsky performance status of 100%, underwent laparoscopic right hemicolectomy for cecal cancer that was detected by screening, which resulted in non-curative resection of the lymph node metastases and disseminated nodules involving the para-aortic major vessels, including ileocolic artery and superior mesenteric artery/vein. The pathological examination revealed moderately differentiated, mainly tubular and partly mucinous, adenocarcinoma, harboring Kirsten rat sarcoma viral oncogene (KRAS) G12D mutation while the other biomarkers, including a serine/threonine-protein kinase B-Raf (BRAF) mutation and microsatellite instability, were negative ,5. Due tThe chemotherapy resulted in complete remission (CR) of the residual lesions and was therefore continued. However, two years after the surgery, the patient noticed progressive motor weakness of the right lower extremity and was clinico-radiologically diagnosed with a single BM of 31 mm maximum diameter, located in the left parasagittal para-central sulcus region  Figure .The patient had been noticing progressive dysesthesia and weakness in the right lower extremity for two months. The patient was nursing a left knee pain that developed while running, and was predominantly using the right lower extremity, and attributed the symptoms to this. Immediately before the BM diagnosis, dysesthesia in the right upper extremity and partial seizure in the right lower extremity appeared, along with difficulties in walking and writing. Steroids were administered immediately, and SRS was selected for the BM, based on multidisciplinary discussions and the patient’s preference Figures .3, the gross tumor volume (GTV) of 7.14 cm3 was defined based mainly on the high-density mass on computed tomography (CT) images and the low-intensity mass visible on T2-weighted images (WIs), excluding the exudation of contrast media into the surrounding brain, under multi-image co-registration and fusion (CT/T1/T2 matching)  was used for the image co-registration, fusion, and contouring. The SRS scheme included sufficient GTV coverage with a BED equivalent to a single fraction of 24 Gy, a concentrically laminated, steep dose increase inside the GTV boundary without any dose constraint to the internal dose, and an appropriate dose gradient outside the GTV [10) , small lung oligo-metastases and new peritoneal dissemination were observed, although asymptomatic. In addition, 6.1 months after SRS, the patient noticed re-deterioration in the movement of his right lower extremity, although milder than that at the time of BM diagnosis. Images showed expansion of the enhancing lesion and perilesional edema, without enlargement of the regressed mass on CT/T2-WIs (CT/T1/T2 mismatch) Figures , 6C, 6G.However, 12.6 months after SRS, the lung metastases and peritoneal dissemination progressed, and lung metastases became miliary in both lungs. Therefore, BEV was continued and a third line of chemotherapy was administered, which resulted in the progression of the extracranial metastases. At 17.3 months after SRS, a change to regorafenib was proposed but systemic therapy was discontinued at the patient’s request. Thereafter, there was no obvious deterioration in the right hemiparesis. One month later, however, gastrointestinal obstruction occurred due to the progression of the peritoneal dissemination and the patient died 18.8 months after SRS (43.9 months after surgery) , was effective for local progression six months after SRS. Integrated diagnosis using conventional CT and magnetic resonance images (CT/T1/T2 matching) was adequate to determine the dominant pathology of local progression. Although the possibility of progression of microscopic tumor invasion into the brain could not be excluded due to the remarkable T1/T2 mismatch, the true progression of the BM was not evident during follow-up . Given tSome chemotherapeutic agents may exacerbate the parenchymal damage by penetrating the disrupted blood-brain barrier around the lesion. After switching from CPT-11 to L-OHP, the radiation effects improved, suggesting that CPT-11 may have exacerbated the radiation injury. Moreover, the addition of BEV was highly effective, facilitating early discontinuation of steroids and leading to minimal perilesional edema ,17,18. I10 of ≥80 Gy [10 87.5 Gy) has been suggested in single-fraction SRS for CRC BM [Considering that the maximum response of the BM was only partial, leaving a 1.4-cc solid mass, dose escalation to the GTV may also have been considered to further enhance the tumor shrinkage . At our f ≥80 Gy ,13,19. Hf ≥80 Gy . Althougr CRC BM . To achir CRC BM . IncreasThe present case also indicates another important caveat. Locally advanced cecal cancer can develop a BM without metastasizing to the liver, lungs, or vertebrae ,8, and dOne of the major limitations of this report was the lack of pathological verification of the brain lesion before and after SRS. Although the high density on CT and the low intensity on T2-WIs are typical findings of CRC BMs, the fact that BM from cecal adenocarcinoma had not been proven and the unknown pathology of the regressed lesion at 15.2 months after SRS greatly hinders the ability to draw definitive conclusions. Nevertheless, the present case forms the basis for further investigation to pursue and establish a more efficacious and safer SRS scheme in appropriate combination with systemic therapy for large CRC BM with eloquent location.An isolated BM without any other distant metastases can develop in patients receiving systemic therapy following non-curative surgery for locally advanced cecal adenocarcinoma. Multi-fraction SRS with a high marginal and internal BED to the GTV and sequential systemic therapy, including BEV, can be an efficacious, durable, and minimally-invasive treatment option for a large para-central sulcus BM from cecal adenocarcinoma. Early administration of BEV-containing regimen following SRS, dose escalation to the GTV (CT/T2 mass), and more than five fractions for ≥3 cm lesion may be considered to further improve the efficacy and safety.


Background: Synovial sarcoma, a malignant mesenchymal tumor, commonly involves the extremities and is rarely found in the pelvis. Cytology with a biphasic pattern can suggest the diagnosis of synovial sarcoma.Case Report: A 32-year-old female presented with complaints of abdominal distension. She was initially evaluated in another hospital where she underwent ultrasound-guided fine needle aspiration cytology (FNAC) of the abdominal mass. The mass was diagnosed as ovarian adenocarcinoma, and the patient underwent 3 cycles of chemotherapy. After 3 months, she was referred to our institute for surgical excision of the mass. Contrast-enhanced computed tomography of the abdomen suggested a well-circumscribed, heterogeneous, solid-cystic mass in the left adnexal region measuring 13.9 × 10 × 9.1 cm and compressing the adjacent structures. No previous radiologic investigations were available. We reviewed the FNAC slide from the outside hospital and made a preliminary diagnosis of biphasic synovial sarcoma. The patient underwent debulking surgery consisting of panhysterectomy with excision of the pelvic mass. Microscopic examination of the pelvic mass showed a biphasic tumor composed of epithelial and mesenchymal components, suggestive of synovial sarcoma. The immunohistochemistry profile supported the morphologic diagnosis. Bilateral ovaries were unremarkable. The patient received 4 cycles of adjuvant chemotherapy and is presently asymptomatic.Conclusion: Although primary pelvic synovial sarcoma is a rare occurrence, this case illustrates that synovial sarcoma can be diagnosed or at least suspected on FNAC. Because of the importance of adjuvant chemotherapy, synovial sarcoma must be high on the list of differential diagnoses of high-grade intra-abdominal masses. Histologically, synovial sarcoma has 2 main subtypes: (1) biphasic with both epithelial and spindle cell components, and (2) monophasic with predominantly spindle cells.1 Fine needle aspiration cytology (FNAC) is a well-accepted, minimally invasive procedure and an effective method of diagnosing benign and malignant soft tissue tumors of the extremities and deep body cavities. Precise morphologic diagnosis and grading of sarcomas by FNAC is problematic; however, biphasic synovial sarcomas have been successfully diagnosed on aspiration.13 Synovial sarcomas often display morphologic features that overlap with a variety of other spindle cell sarcomas and carcinomas such as malignant peripheral nerve sheath tumor, leiomyosarcoma, solitary fibrous tumor, Ewing sarcoma/primitive neuroectodermal tumor, fibrosarcoma, sarcomatoid carcinoma, Müllerian adenosarcoma, and sarcomatoid mesothelioma.3Synovial sarcoma is a soft tissue sarcoma with specific clinicopathologic and genetic features that can occur anywhere in the body. The most common site is the extremities near the large joints (70%).We present a case of pelvic synovial sarcoma that was originally diagnosed as an ovarian malignancy.A 32-year-old female presented at an outside hospital with complaints of abdominal distension for 1 month. She underwent ultrasound-guided FNAC of the abdominal mass, and it was diagnosed as adenocarcinoma of the ovary. She received 3 cycles of chemotherapy (paclitaxel 280 mg + carboplatin 550 mg).9/L . The patient was nonreactive for hepatitis B, hepatitis C, HIV-1, and HIV-2.After 3 months, the patient was referred to our institute for surgical excision. At the time of admission, liver and kidney function tests were within normal range. A complete blood count revealed hemoglobin 10.7 g/dL , total leukocyte count 8,870/μL , and platelet count 567 × 10Contrast-enhanced computed tomography (CT) of the abdomen showed a well-circumscribed, heterogeneous, solid-cystic mass in the left adnexal region measuring 13.9 × 10 × 9.1 cm and compressing the adjacent structures.One hematoxylin and eosin stained slide of the FNAC of the abdominal mass from the outside hospital was received for review. The smear was cellular, displaying loosely cohesive clusters, as well as numerous scattered bland-looking spindle cells with fine chromatin, inconspicuous nucleoli, and elongated delicate cytoplasm , and 1C.The patient underwent debulking surgery consisting of panhysterectomy , excisioLight microscopic examination of the pelvic mass showed a biphasic tumor composed of epithelial and mesenchymal components . The epi2 for 1 day, followed 1 week later by ifosfamide 2 g/m2 for 5 days and doxorubicin (Adriamycin) 30 mg/m2 for 3 days) and was disease-free 21 months posttreatment on follow-up. The patient is being followed every 5 to 6 months for the next 3 to 5 years, and the surveillance regimen includes clinical history and CT scan.The patient received 4 cycles of adjuvant chemotherapy .3Descriptions of cytomorphologic features of synovial sarcoma are based on case reports and case series, with only an occasional study investigating the cytologic criteria for forming a reliable diagnosis on FNAC. One such investigation was carried out by the Scandinavian Sarcoma Group that studied 104 synovial sarcomas.22FNAC is an effective diagnostic tool for preoperative evaluation and assessment of recurrence and/or metastasis in soft tissue sarcomas. Sensitivity and specificity >90% are seen with experienced hands, similar to frozen section interpretation rates.21 In the spindle cell group, 7 cases were synovial sarcomas that were diagnosed on histology. Of these 7 cases, 6 were correctly diagnosed on cytology. One case was reported as spindle cell neoplasm that on histology was diagnosed as monophasic synovial sarcoma, suggesting that the presence of an epithelial cell component is necessary for the diagnosis of synovial sarcoma.Kilpatrick et al evaluated the effectiveness of FNAC in subtyping soft tissue sarcomas by analyzing the cytomorphologic features in 73 consecutive aspirates from 67 patients and subgrouping them into pleomorphic cell (19 cases), small round cell (18 cases), spindle cell (18 cases), myxoid tumor (10 cases), epithelioid/polygonal cell (7 cases), and well-differentiated liposarcoma (1 case).23 Fisher et al reviewed 300 cases of synovial sarcoma, of which 3 were pelvic and 8 were retroperitoneal, and concluded that pelvic tumors metastasize distantly.2 In contrast, retroperitoneal tumors remain confined to the abdomen and do not metastasize remotely.2Nakamura et al studied 90 consecutive patients with pelvic soft tissue sarcomas over 23 years and found 6 patients with synovial sarcoma (6.7%), concluding that high-grade tumors and positive surgical margins represent a high-risk group for local recurrence.24 Malignant peripheral nerve sheath tumor is an important differential of monophasic synovial sarcoma on cytology. The 32In the pelvis, synovial sarcomas need to be distinguished from gynecologic malignancies such as carcinosarcoma and soft tissue tumors such as malignant peripheral nerve sheath tumor, leiomyosarcoma, solitary fibrous tumor, fibrosarcoma, sarcomatoid mesothelioma, sarcomatoid carcinoma, Müllerian adenosarcoma, and Ewing sarcoma.24 Hartel et al found that BCL2 and CD56 reach nearly 100% positivity in cases of primary pulmonary and mediastinal synovial sarcoma,25 and approximately 60% of synovial sarcoma are positive for CD99.20 TLE1 is the most sensitive and specific marker for synovial sarcoma.29Immunohistochemically, the spindle cell population of synovial sarcoma can show focal or widespread positivity for epithelial membrane antigen (most sensitive) and for CK7 and CK19, which are nearly 100% specific.24 The use of newer drugs such as receptor tyrosine kinase inhibitors, epigenetic modifiers, and immunotherapy is under evaluation, and according to Desar et al, DNA damage response inhibitors should also be tested in clinical studies.24 Local recurrence and propensity for distant metastasis are seen in approximately 50% of cases.24 The t  translocation, resulting in SS18-SSX  fusion of oncogenes, is seen in 90% of cases.29Surgery is the treatment mainstay; however, marginal clearance is difficult to achieve for tumors in the pelvic region. Synovial sarcoma is relatively chemosensitive to standard first-line treatment (combination treatment with doxorubicin and ifosfamide is a preferred option) and second-line treatment (pazopanib and trabectedin).A multidisciplinary approach combining clinical consideration with careful scrutiny of morphologic features can help make the diagnosis. The present case illustrates that synovial sarcoma can be diagnosed or at least suspected on FNAC.Primary pelvic synovial sarcoma is a rare entity. The importance of adjuvant chemotherapy has been identified, so synovial sarcoma must be high on the list of differential diagnoses of high-grade intra-abdominal masses, and FNAC can be helpful in preoperative diagnosis.