A retrospective case report.To report a case who developed deteriorated paraplegia by spontaneous spinal epidural hematoma (SSEH) based on calcification of the ligamentum flavum (CLF) at the T10–11 level, achieved full neurological recovery following posterior percutaneous endoscopic surgery (PPES).CLF rarely occurs at the thoracic spine, and the symptom usually progress slowly. SSEH is another rare spinal lesion that might progress rapidly and cause emergent severe spinal cord compression syndrome. Coexistence of SSEH and CLF at the same thoracic level was rarely reported in English literature.via PPES was carried out to realize bilateral decompression through a unilateral approach.A 65-year-old man presented to our hospital with the complaint of sensorimotor loss on the lower limbs and dysfunction of bladder for 1 day after a progressive weakness and numbness of the lower limbs for 3 months. MR examination found a dorsal protruding mass at the T10–11 level, while computed tomography (CT) found the protruding mass contained scattered calcified deposits. The patient was diagnosed with thoracic CLF. Decompression During the operation, the protruding mass was found to be composed of SSEH and CLF together. After the operation, the patient's neurological function recovered quickly. One week later, the patient could walk by himself. After 3 months, complete neurological function had recovered.SSEH could develop based on CLF at thoracic level and cause serious neurological dysfunction. PPES might be an advisable method to remove CLF and evacuate SSEH with good clinical results. Calcification of the ligamentum flavum (CLF) is a rare crystal deposition disease that mainly affects the central portion of the ligamentum flavum (LF) , 2, and via percutaneous posterior endoscopic surgery (PPES) achieved satisfactory recovery.The fact that SSEH and CLF coexisted at the same thoracic level was extremely rare. We managed one such case, who developed subacute SSEH with pre-existing CLF at the T10–11 level. CLF removal and hematoma evacuation A 65-year-old man visited our hospital on 6 May 2019 complaining of sensorimotor loss in two legs and bladder dysfunction for 1 day after experiencing progressive weakness and numbness in two legs for 3 months. About 4 years ago, he was diagnosed with coronary atherosclerosis and unstable angina and has taken aspirin, atorvastatin, and isosorbide mononitrate daily since to reduce thrombotic cardiac risk prophylactically.A neurological examination found hypoesthesia below the right L1 dermatome and sensation loss below left L1. Muscle strength of left/right was grade 0/0 between L1 and L4 myotomes, 1–2/0–1 between L5 and S1. The laboratory tests were normal.MR examination on 2 May 2019 detected extradural protruding mass compressing the spinal cord dorsally at the T10–11 level with intramedullary hypo-intense signals on T1-weighted images (T1WI) and hyperintense signals on T2-weighted images (T2WI) . The susSubsequent computed tomography (CT) taken on 6 May 2019 found the calcified protruding mass did not attach to the lamina . CLF wasAdministration of aspirin was replaced by low molecular weight heparin 3,000 iu injected subcutaneously per day. An intravenous drip of 0.5 g of methylprednisolone succinic acid (MP) for a single time produced temporary neurological improvement.The decompression operation was postponed and performed 7 days later, on May 13, fearing intraoperative bleeding from aspirin administration , 7. A hiNeurological function recovered quickly. CT re-examination on 15 May 2019 confirmed fenestration at left T10–11 interlaminar space with complete removal of CLF . After 1Until 2021, only eight thoracic CLF cases were reported in English literature, among them, only one exhibited rapid progression while others exhibited slow progressive process  above. ASSEH is commonly characterized by the sudden onset of dorsal local pain, progressive neurological deficits usually within minutes or hours . It mighIn 1990, Bracken et al. reported the results of the Second National Acute Spinal Cord Injury Study (NASCIS II protocol) and concluded that patients with acute spinal cord injury (ASCI) treated with MP as a bolus of 30 mg per kilogram of body weight, followed by infusion at 5.4 mg per kilogram per hour for 23 h, improved neurologic recovery when the medication was given in the first 8 h . In 1997Aspirin inhibits platelet aggregation , it may In the late 1990s, Yeung designed the YESS endoscope to perform endoscopic transforaminal discectomy , followeAn intramedullary hyperintense signal on T2WI sequences could be detected preoperatively . The abnSSEH could develop based on CLF and cause serious neurological dysfunctions. PPES technique might be an excellent choice to remove CLF and evacuate SSEH, with minor iatrogenic trauma and good clinical result.


Microcystic pancreatic serous cystadenoma (SCA) can be managed without surgery in selected patients. However, the preoperative diagnosis of microcystic SCA remains challenging, and it is potentially misdiagnosed as other pancreatic cystic neoplasms or solid tumors, especially small microcystic SCA.This was a case of a 27-year-old male patient with microcystic SCA causing difficulty in the differential diagnosis from pancreatic neuroendocrine neoplasm (pNEN). A pancreatic tail mass was incidentally discovered on abdominal ultrasound (US). A contrast-enhanced computed tomography (CT) scan revealed a solid tumor measuring 13 mm with early enhancement in the arterial phase at the pancreatic tail. The tumor showed low intensity on T1-weighted magnetic resonance image, high intensity on T2-weighted image, and a slightly hyperechoic mass on endoscopic US (EUS). EUS-fine needle aspiration (EUS-FNA) did not lead to a definitive diagnosis. The tumor was clinically diagnosed as a pNEN, and a laparoscopic spleen-preserving distal pancreatectomy using the Warshaw technique was performed. The final histopathological diagnosis was microcystic SCA.Small microcystic SCA is difficult to distinguish from a hypervascular pancreatic tumor such as pNEN on imaging studies, and it is necessary to focus on the tumor echogenicity of EUS to differentiate microcystic SCA from pNEN preoperatively. Advancement in imaging techniques has led to an increase in the incidental identification of small pancreatic tumors. The most common pancreatic tumor is ductal adenocarcinoma, and microcystic serous cystadenoma (SCA), a subtype of SCA, is a rare benign epithelial neoplasm . BecauseAlthough there might be pitfalls in the clinical diagnosis of microcystic SCA, only a few case reports have focused on investigating the diagnostic difficulty in distinguishing microcystic SCA from other pancreatic neoplasms .Herein, we present a case of a patient with microcystic SCA that was preoperatively diagnosed as pancreatic neuroendocrine neoplasm (pNEN) and underwent laparoscopic spleen-preserving distal pancreatectomy and a literature review including 15 reported cases of microcystic SCA with a difficulty in the preoperative differential diagnosis of pNEN.A 27-year-old male patient underwent annual health checkup, and a hyperechoic lesion was incidentally discovered in the tail of the pancreas via abdominal ultrasound. He was referred to our hospital for possible surgery with an inconclusive diagnosis of pNEN. He has no family history of diseases including pancreatic disorders. The blood cell count, biochemistry, and coagulation tests showed absence of abnormal findings. The carcinoembryonic antigen (CEA) level was 4.4 ng/ml, while the carcinoembryonic antigen (CA) 19-9 level was 23.1 U/ml, both of which were within normal range.Abdominal computed tomography (CT) examination revealed a well-defined mass measuring 13 mm in the tail of the pancreas. The tumor showed slightly low density compared with the pancreatic parenchyma on plain CT Fig. a–d. MagnAlthough a definitive pathological diagnosis could not be obtained, the tumor was preoperatively diagnosed as non-functioning pNEN larger than 10 mm based on the findings of enhanced CT and MRI and EUS-FNA cytology; thus, minimally invasive surgical resection was indicated. Solid-type SCA was one of the possible preoperative differential diagnoses, since the EUS showed a slightly hyperechoic lesion that was a distinctive ultrasonographic feature of a microcystic SCA. As tumor abutment to the splenic artery and vein was observed and spleen preservation was desirable, laparoscopic spleen-preserving distal pancreatectomy was performed following the Warshaw method. The operation was carried out using the 5-port approach. No extrapancreatic invasion of the tumor was observed. In order to preserve the splenic inflow, the distal part of the splenic artery was divided at the proximal site of the root of the left gastroepiploic artery. The operation was considered complete after confirming the absence of changes in the color tone of the spleen. The operation time was 300 min, and the estimated blood loss was 5 ml. The postoperative course was uneventful, and the patient was discharged from our hospital on postoperative day 18.Macroscopically, the tumor was a well-defined, round, and solid mass without cystic components measuring 15 mm Fig. a arrow. SCA is a relatively rare pancreatic cystic neoplasm that accounts for only 1%–2% of all pancreatic tumors . It freqVarious subclassifications of SCA have been proposed based on the macroscopic and microscopic characteristics. In the 7th edition of the general rules for the study of pancreatic cancer by the Japan Pancreas Society , serous The present case corresponds to type 1b (solid type) based on the macroscopic classification by Hifumi et al., since the imaging findings did not show any cystic morphology but a solid mass with enhancement in the arterial phase on enhanced dynamic CT. Microscopically, the tumor was composed of multiple small cysts, and the pathological diagnosis of microcystic SCA was made based on the WHO classification.Generally, majority of SCAs are benign neoplasms and are most often managed conservatively with a follow-up . HoweverWe searched PubMed and Medline for clinical series in which surgical resection was performed with a preoperative diagnosis of pNEN and a postoperative pathological diagnosis of SCA was obtained using the keywords “serous cystadenoma” and “pancreatic neuroendocrine neoplasm”. To the best of our knowledge, 15 resected cases of SCA with preoperative diagnosis of pNEN were reported from 2001 to 2021, including the present case (Table A review of 15 surgically resected SCAs with preoperative diagnosis of pNEN revealed that most of them were evaluated as a hypervascular tumor with a mass-like appearance on CT scan; none of them, which underwent EUS-FNA, could have led to the definitive pathological diagnosis of SCA. These findings suggest that preoperative differentiation based on EUS-FNA alone can be very difficult and challenging. Preoperative EUS was performed in seven patients including the present case. Of them, five patients presented hypoechoic lesions, which was consistent with a typical well-differentiated pNEN. On the contrary, the present case presented a slightly hyperechoic lesion, which was not a typical ultrasonographic feature of well-differentiated pNEN. Since the tumor was pathologically composed of grossly invisible microcysts and thick fibrous tissue, the slight hyperechoic appearance on EUS was considered to reflect multiple ultrasound reflections from numerous tiny cysts inside the tumor. It was retrospectively considered that a hyperechoic lesion on EUS was a key finding, which might have enabled the preoperative diagnosis of solid-type SCA rather than PNEN. The present case report highlights the usefulness of EUS in differentiating microcystic SCA from pNEN.We experienced a case of a patient with microcystic SCA who was preoperatively diagnosed with pNEN and underwent laparoscopic spleen-preserving distal pancreatectomy and reviewed 15 surgically resected SCAs with a preoperative diagnosis of pNEN. Our results indicate that small microcystic SCA is difficult to distinguish from a hypervascular pancreatic tumor such as pNEN on imaging studies, and it is necessary to focus on the tumor echogenicity of EUS to differentiate microcystic SCA from pNEN preoperatively.


Intraplacental choriocarcinoma (IC) is a rare type of gestational choriocarcinoma (GC) occurring within the placenta, and only a small number of cases have been reported so far. Intraplacental choriocarcinoma is usually asymptomatic or may present with aspecific symptoms, including unexplained vaginal bleeding during pregnancy. Early diagnosis and treatment are pivotal for ensuring optimal outcomes. However, intraplacental choriocarcinoma is rarely suspected due to limited knowledge and awareness of the condition. Here, we report the case of a 34-year-old woman diagnosed with intraplacental choriocarcinoma by placental histological examination performed after delivery due to unexplained vaginal bleeding at 29 gestational weeks, requiring hospital admission. Two lines of chemotherapy and surgery were necessary to achieve complete remission. Since unexplained vaginal bleeding during pregnancy can be a clinical manifestation of intraplacental choriocarcinoma, we propose to consider placental histological examination in all pregnancies with this complication. Gestational trophoblastic neoplasia is a group of rare tumors that arise from the cells of conception. The malignant forms include gestational choriocarcinoma (GC), placental site trophoblastic tumor, and epithelioid trophoblastic tumor; these forms can arise from a molar pregnancy or a normal genetic pregnancy .GC has an estimated overall incidence of 1 case per 50,000 pregnancies [Intraplacental choriocarcinoma (IC) is a rare form of GC located within the placenta. Due to its rarity, clinical information about IC is substantially limited. Here, we present a case of IC incidentally diagnosed in a woman by a placental histological examination requested for obstetric reasons after term delivery.2). The patient's past medical history was significant for laparoscopic cholecystectomy in 2010. Her obstetric history included two abortions and two previous cesarean sections, one in 2008 for suspected fetal macrosomia and the other in 2015 as an iterative procedure.The patient is a 34-year-old South American woman who delivered at our hospital in October 2017 with pregestational obesity . The brain MRI, as well as the chest CT, did not show metastatic disease. In turn, an abdominal CT with medium contrast showed a uterus with a hyperdense alteration in the myometrium at the body-neck passage in the right paramedian site. The same uterine finding was also identified at the transvaginal ultrasound scan. The hCG levels of the newborn were negative on both blood and urine specimens.The maternal serum hCG level was raised to 5567 mIU/ml two weeks after the initial evaluation. Thus, the patient was immediately started on chemotherapy with methotrexate (MTX) and folic acid according to ESMO guidelines . Serum hThe uterine histological examination showed a minimal residual focus of pathological tissue without evidence of the infiltrative attitude of 12.6 mm × 7.9 mm at the level of the endometrium in the anterior uterine wall Figure -2B.According to the histological examination, which indicated the persistence of active neoplastic cells at the uterine level, the patient underwent a third consolidation cycle of EMA-CO chemotherapy. The postoperative clinical follow-up was regular. Weekly postoperative serum hCG level monitoring demonstrated a complete response to treatment. The patient is currently disease-free (follow-up of 46 months); she monitors her serum hCG level every four months, and she is clinically assessed once a year at our outpatient clinic. The patient gave written informed consent for publishing this study and its accompanying images.IC is a rare form of GC located within the placenta that arises from the chorionic villous trophoblast. It represents 2% of all gestational trophoblastic tumors. The clinical presentation of this tumor is very variable. It can be clinically silent or cause a metastatic maternal and/or infantile disease. The most common symptom, when present, is vaginal bleeding, either during pregnancy or afterward if uterine or vaginal metastases are present. In 50% of the cases, the diagnosis is incidental, and a subsequent pathological examination of the placenta is requested for obstetric reasons [Feto-maternal hemorrhage, stillbirth, and intrauterine growth restriction have been reported as the most common indications for placental pathological examination among asymptomatic patients . In our About half of IC cases are metastatic at diagnosis because the disease is usually not considered until the mother or the child has symptoms. The most frequent metastatic sites are the lungs, the brain, the uterus, and the vagina. Notwithstanding this, the prognosis for IC is optimal, with a cure rate approaching 100%. All cases are associated with elevated serum and urine human chorionic gonadotropin (hCG) levels, which are useful for both diagnostic and surveillance purposes .Our patient presented with a metastatic uterine lesion in the myometrial context, which was diagnosed early by transvaginal ultrasound and CT scan; thanks to the placental histological examination requested for obstetric reasons. Only two cases of infantile metastases have been reported, with one death and one survival after chemotherapy ,6,7. In Given the rarity of IC, there are no specific guidelines for treatment, and those for GC choriocarcinoma are usually followed . Serum hIC is an extremely rare condition that is likely underdiagnosed. Its natural history varies, and prenatal diagnosis is particularly challenging. Early detection is possible only if a placental pathological examination is performed after delivery. However, placental histology is not routinely assessed in most hospitals. This case illustrates the importance of a detailed examination of the placenta in cases of unexplained vaginal bleeding in pregnancy and its significance in diagnosing IC. In the absence of placental histology, a post-partum serum hCG assay may be useful to raise suspicion and prompt specific investigations. The cost-benefit ratio of routine implementation of such tests should be assessed in future research.


Neuroendocrine tumours (NETs) are rare tumours derived from the neuroendocrine cell system, arising across a wide range of organs, most commonly the gastrointestinal tract and bronchopulmonary symptoms. Although NETs can metastasis widely throughout the body, cardiac metastasis is rare with an incidence of 2–4% and usually presents in the presence of extensive metastasis elsewhere. Suprasternal metastasis to the neck is exceedingly rare with <20 cases reported in the literature. We report the case of a 71-year-old female with concurrent cardiac and suprasternal metastasis at diagnosis of terminal ileal NET. Neuroendocrine tumours (NETs) are rare tumours derived from the neuroendocrine cell system, arising across a wide range of organs such as gastrointestinal (GI) tract, bronchopulmonary system and thymus . Constit2 and Ki67 < 1%.A 71-year-old female underwent colonoscopy for a 12-month history of diarrhoea, which revealed abnormal ileocecal mucosa. Computed tomography (CT) chest abdomen and pelvis was suggestive of primary appendiceal malignancy with soft tissue expansion and a midline soft tissue mass just inferior to the thyroid. She underwent laparoscopic right hemicolectomy that revealed a Stage pT2N2 16-mm Grade 1 NET in the terminal ileum with 5/24 lymph node involvement, mitotic rate < 2/mmPost-operative staging DOTA positron emission tomography (PET)/CT  revealedCardiac magnetic resonance imaging (MRI) revealed a 3-mm focus of increased STIR signal at the proximal inferoseptum corresponding to the location of previous focal DOTATATE uptake, confirming a metastatic interventricular septum lesion. There was otherwise normal left ventricular systolic function with an EF of 70%. The patient remained largely asymptomatic aside from occasional flushing sensation and loose motions for three to four times a day, which was stable since the right hemicolectomy. She did not have any heart failure symptoms. She was commenced on lanreotide treatment at 120-mg monthly, despite being relatively asymptomatic, due to the presence of CM.Surveillance included regular chromogranin A, which decreased rapidly from 418 to 269 post-resection of primary ileal disease and suprasternal metastasis and returned to within normal range (20–102) 7 months post-resection. Yearly cardiac MRI showed stable cardiac disease. Three new hepatic metastases were found on surveillance PET, 18 months post-resection, however, the patient remained well with no carcinoid symptoms. She continues lanreotide and regular 6 monthly PET and yearly cardiac MRI surveillance.CM from NETs is rare, with incidence estimated to be 2–4% , 6. The n = 124, A wide range of clinical presentations is seen with CM, from asymptomatic to fulminant heart failure, obstructive/cardiogenic shock and cardiac arrests . LocatioIt is important to distinguish between carcinoid heart disease (CHD) and CM from NET. CHD can occur in up to 50–60% of patients with carcinoid syndrome and is characterized by the endocardial deposition of plaque-like fibrous tissue commonly on valvular cusps, papillary muscles and cords, with a predilection involving the right side of the heart . It maniet al. who demonstrated an incidence of up to 8.7% [The presence of suprasternal metastasis from confirmed GI NET is exceedingly rare; true incidence is not clear as historically cervical and mediastinal metastases have been regarded as medically insignificant. Less than 20 cases have been reported from 1975 until now, the majority of these in a study by Wang YZ  to 8.7% . This nuTo the best of our knowledge, this is the first reported case of concurrent cardiac and neck metastasis from small bowel NET. Importance of early DOTA PET imaging is highlighted for the detection of uncommon metastatic sites. There is no clear consensus on the management of CM and their response to treatment modalities; regular DOTA PET/CT in combination with cardiac MRI for monitoring appears to be the gold standard. However small and asymptomatic CM may be, its presence can sway management to commence somatostatin analogue despite minimal symptoms and low volume disease post-resection. Early surgical excisional biopsy of neck metastasis, rather than relying on FNA, is key in obtaining an accurate tissue diagnosis, removing the potential source of serotonin secretion and eliminating the potential for developing compressive symptoms later.None declared.None.


Neurofibromatosis type 1 is a genetic disorder that follows an autosomal dominant pattern of inheritance. Ocular involvement is not uncommon, but spontaneous dialysis of the retina in the absence of a history of trauma is a rare clinical entity. Rare cases of retinal involvement such as retinal detachment or dialysis of ora serrata could be linked with the abnormal cell-matrix formation in neurofibromatosis type 1. Here, we present a case of a 36-year-old man having Neurofibromatosis Type 1 with spontaneous dialysis of ora serrata without prior history of ocular trauma. A routine fundoscopic examination should be done in addition to the examination of the anterior chamber in patients with neurofibromatosis type 1 despite the absence of ocular complaints. SOB was gradually progressive and of modified Medical Research Council (mMRC) grade 3 at the time of presentation. It was relieved while lying on the left side but aggravated on lying on the right side. The cough was productive, whitish, and non-blood mixed. He also had a history of night sweats and weight loss. On examination, 7-9 dark brown coloured cafe au lait spots of approximately 3x2 cm size each were present along with multiple neurofibromas over the trunk .There were several non-tender cervical lymph nodes palpated of which the largest lymph node measured approximately 20 mm. Following clinical examination, a Chest X-ray (CXR) was done which showed obliteration of left costophrenic and cardiophrenic angles with a homogenous opacity in the left middle and lower lung zones without tracheal or mediastinal shift, suggestive of left-sided pleural effusion .To confirm the diagnosis, a Contrast Enhanced Computed Tomography (CECT) chest was done which showed left-sided moderate pleural effusion with normal lung parenchyma along with features suggestive of plexiform neurofibromatosis. Pleural fluid analysis was done which showed lymphocytic effusion with high pleural fluid adenosine deaminase (55 IU/l). The patient was treated with anti-tubercular treatment with a diagnosis of neurofibromatosis with left-sided tubercular pleural effusion. He had no significant family history related to neurofibromatosis or tuberculosis. He was referred to the Department of Ophthalmology for ocular evaluation for NF 1.On further inquiry in the Department of Ophthalmology, he gave no history of ocular symptoms. There was no history of trauma to the eye and preceding ocular surgeries. On ophthalmological evaluation, the patient had a best-corrected visual acuity of 6/9 on the right eye Oculus Dexter (OD), and 1/60 on the left eye Oculus Sinister (OS) with no error noted. Further evaluation showed left exotropia of an eight-degree prism dioptre. Pupillary examination showed no anisocoria; however, a positive Relative Afferent Pupillary Defect (RAPD) of grade 2 was present on the left eye (OS). There was no evidence of angle recession, iridodialysis, phacodonesis, or choroidal ruptures. Slit-lamp examination revealed no iris Lisch nodules in either eye.Fundoscopy following pupil dilation revealed inferiorretinal detachment with the macula off in the left eye. The right eye was normal. The dialysis of the ora serrata was noted from the 4 o'clock to the 7 o'clock positions .The detailed ocular examination did not yield clinical evidence suggesting ocular trauma, and the patient repeatedly denied the same. Rhegmatogenous retinal detachment with spontaneous dialysis of the ora serrata in the left eye was diagnosed. On follow-up after 3 weeks, he was admitted with a lung abscess, for which chest tube insertion and drainage of the fluid were done. However, he had no ocular complaints, and the ophthalmic evaluation revealed no deterioration of vision. Even so, the ocular findings of Rhegmatogenous Retinal Detachment (RRD) with spontaneous retinal dialysis persisted , raising6 The cutaneous features include cafe-au-lait macules, axillary and groin freckling (Crowe sign), dermal tumors, etc.1 It can also affect the gastrointestinal tract, eye, musculoskeletal and neurovascular systems.1 The ocular manifestations can range from benign growths like iris hamartoma (Lisch nodules) and neurofibromas to malignant tumors like Optic Pathway Glioma (OPG).3Neurofibromatosis type 1 (NF 1) is characterized by abnormal growths of epithelial, mesothelial, and endothelial components.7 Likewise, retinal dialysis also occurs secondary to trauma; however, a few inherited spontaneous retinal dialysis cases have also been reported.8 Retinal detachment with spontaneous dialysis of ora serrata in the absence of a history of ocular injury or tumor has rarely been reported in patients with neurofibromatosis type 1.3Retinal detachment in patients with NF 1 usually occurs following mechanical trauma to the eye or with retinal tumors like hamartomas and capillary hemangioma.3 Fibroblasts migrate and proliferate in this region following vitreous haemorrhage and penetrating ocular trauma and are thought to be responsible for the vitreous base avulsion.9 In NF 1, vitreoretinal pathologies are linked with abnormal cellular proliferation in the vitreous base even in the absence of previous ocular pathology.10 One article hypothesized that there is Matrix Metalloproteinase 1 (MMP1) downregulation in stromal cells in NF 1 leading to disturbance in the collagenolytic process and thereby accumulation of abnormal fibroblast and neo-collagen in the ocular region in NF 1.10 Another study showed that there is decreased receptor binding of Epidermal Growth Factor (EGF) in NF 1, which might lead to membrane defect in the retina.6 These factors alter the cellular matrix of the vitreous base resulting in reduced production of collagen fibers that keep the peripheral neurosensory retina intact.10The vitreous base contains numerous fibroblasts in its cortex, which form collagen fibrils that insert through the basal lamina of the pars plana and peripheral retina.3 Unlike in their case, our patient had diminished visual acuity in the left eye noted after examination along with exotropia and grade 2 RAPD therefore, we performed an MRI of the brain instead of an exploration of the retina to rule out tumors. In their case, the underlying defect was corrected by ophthalmological intervention but this approach was not done in our case since the patient had asymptomatic chronic RRD. We opted to observe the patient with routine follow-up as the risk of progression of the RRD outweighed the benefit of surgical repair.In a pediatric case study, the ocular examination revealed retinal detachment with dialysis of the ora serrata in the left eye, similar to our case, the patient had no ocular symptoms and no history of ocular trauma.Although retinal dialysis is a rare entity in cases with NF 1, with this case, we would like to emphasize performing routine funduscopic examination under mydriatics in all the patients with NF 1 even in the absence of ocular complaints in addition to evaluating the anterior segment. Furthermore, imaging modalities like MRI of the brain should be employed to rule out underlying lesions. This will not only aid in ruling out the presence of ocular lesions but also help in diagnosing any obscured asymptomatic retinal pathologies like spontaneous retinal dialysis, as in our case thereby, preventing vision-threatening complications.


Undescended testicles (UTs) and torsion of the testicle are a rare clinical combination. Symptoms may be misleading and interpreted as signs of other common conditions. Moreover, late identification of an UT may significantly delay the diagnosis and lead to adverse outcomes. Here, we present a case of a 17-year-old boy with cerebral palsy and learning disabilities who presented with painful right-sided inguinal mass. Intraoperatively, he was confirmed to have torsion of an UT and orchidectomy was performed. This article also emphasizes the importance of early diagnosis of testicular ectopia. Sudden onset pain and swelling of the scrotum, defined as the acute scrotum, implies an acute pathology of the scrotum and/or its content. Testicular torsion (TT) represents a differential of utmost importance as misdiagnosis results in organ loss, and therefore, scrotal exploration for exclusion is one of the most common procedures carried out on children and young adults . On the In this paper, we present a case of a 17-year-old boy with cerebral palsy and learning disabilities who presented with a painful inguinal mass, found to be an UT in torsion. Discussed below is the presentation, diagnostic work-up, intraoperative findings and significance of the case.A 17-year-old boy with a history of cerebral palsy, learning disabilities and asthma who is wheelchair-bound (WHO Performance Status 4) presented with a 24-hour history of general malaise and pain in the right groin. On examination, a swollen and painful, right inguinal mass was detected, and the scrotum was found to be empty and hypoplastic on both sides. The family was reportedly unaware of the possibility of undescended or vanished testicles. The patient was urgently evaluated for a complicated right inguinal hernia and computed tomography (CT) scan of the abdomen  demonstrSubsequent ultrasound of the scrotum  showed aThe left testicle was not identified. The case was referred to the urology team and urgent exploration was offered considering possible right orchidopexy and/or orchidectomy. The patient underwent surgery later that day. An inguinal incision was made, and the testicle was found to be attached upon the oblique fascia with the gubernaculum attaching superiorly and laterally to the external inguinal ring. The testicle and spermatic cord were dissected from further attachments and the tunica vaginalis was opened. Despite attempts to resuscitate the testicle with warmed gauze and 100% oxygen, its appearance remained severely ischemic and an orchidectomy was performed. The remainder of the procedure was uneventful, and the patient was discharged the next day. The histopathological examination of the specimen revealed hemorrhagic necrosis secondary to infarction—consistent with the clinical history of torsion. The patient was then referred to a specialist for exploratory laparoscopy of the left testicle and to an endocrinologist regarding testosterone replacement therapy.We consider the above case a key learning point for several reasons. Firstly, the intraoperative findings, the examination of the scrotum and the absence of the left testicle imply that the diagnosis of UT had eluded routine clinical examination for years. If the diagnosis had been achieved earlier, the testicle could have been restored to the scrotal position and this presentation may have been avoided. The above underlines the importance of clinical awareness for the clinicians  who usually perform routine examinations during early years and of course the education of patients and their guardians . UnfortuFurthermore, the importance of the clinical examination needs to be highlighted. The presence of pain and a mass in the groin region along with an empty scrotum should raise immediate suspicion of a suffering, UT. Proper physical examination contributes significantly to the early diagnosis of intra-abdominal torsion . DespiteThe assessment of our patient was made more challenging due to him being non-verbal. The presence of cerebral palsy in combination with learning disabilities has been reported in literature to delay parents seeking help, unfortunately resulting in orchiectomy . In our In conclusion, the clinical context of a torsion in the background of UT is associated with adverse outcomes. Salvage rates of the testicle are ~30% as investigation time often exceeds the treatment window for preservation of the organ . The ear


Adrenal schwannomas are extremely uncommon tumors. We report the case of a 39-year-old male with a right adrenal mass. Laboratory tests were normal and radiological exams revealed the adrenal tumor. Open surgical adrenalectomy was performed. The postoperative course was uneventful. Microscopy and immunohistochemistry revealed a cellular schwannoma. Adrenal schwannomas are rare tumors, very difficult to diagnose in preoperative evaluation. Surgical excision of the tumor, histological and immunohistochemical examination of the specimen provide a definitive diagnosis. Prognosis is generally very good. Recurrence rates are related to positive surgical margins. Schwannomas are infrequent tumors deriving from Schwann cells of peripheral nerves sheaths. They were first described by Jose Verocay in 1908, and in 1920, they were subclassed by Antoni into two histologic patterns . They usA 39-year-old male was referred to the department of endocrinology of our hospital for further investigation of a 12x8.5 cm right adrenal mass, which was found on abdominal ultrasonography performed due to abdominal pain. Past medical history included syncopal episodes, which were investigated by our department of cardiology. A magnetic resonance imaging (MRI) of the retroperitoneal space revealed a large solid adrenal mass of 12x8.5 cm with increased T2 signal intensity and decreased T1 signal intensity. The tumor compressed the liver, the right kidney, and the inferior vena cava without presenting an invasive trend. The tumor was suspicious for pheochromocytoma. However, all endocrinological examinations were within the normal range, and the metaiodobenzylguanidine (MIBG) scan was negative. A fluorodeoxyglucose positron emission tomography (FDG-PET/CT) was also done, indicating high uptake in the right adrenal gland, with a standardized uptake value (SUVmax) of 9.5  and very few areas with low cellularity  or MRI . CT usuaThe appropriate treatment for adrenal schwannoma is complete excision, as these tumors do not present sensitivity neither to chemotherapy nor to radiotherapy . The surAdrenal schwannoma is a rare, benign tumor with a very good prognosis. Its diagnosis is based on histopathology and immunochemistry, and surgical excision remains the treatment of choice. Physicians should be aware of schwannomas so that they may suspect them in any case of a retroperitoneal adrenal mass.


Myoepitheliomas are rare tumors that represent about 1% of the salivary gland tumorsThe present investigation reports a case of a patient with a malignant myoepithelioma on the hard palate, with bone destruction, successfully operated upon.R.A., male, 38 years old, complaining of nasal obstruction for years, associated with running nose and recurrent epistaxis. During exam we noticed a palate tumor extending to the right-side nasal cavity. Computerized tomography (CT) showed a large solid mass occupying part of the right maxillary sinus, palate and nasal cavity .Figure 1He was submitted to a transoral resection of the tumor, which pathology exam showed a tissue neoformation made up of ovoid cells of clear cytoplasm with round nuclei and, sometimes, spindle-shaped cells with areas of stromal hyalinization and cystic formations. Immunohistochemistry analysis was positive for 14 cytokeratin, vimentin and specific muscle actin, which result matches the description of a malignant myoepithelioma. He had two new recurrences, also treated surgically.We made him a palate closure prosthesis as a means for functional reconstruction. He has been under follow up for nine years, without signs of recurrence.Malignant myoepitheliomas are rare tumors made up of atypical myoepithelial cells with high mitotic activity and aggressive growth1. Such tumors may stem from the differentiation of a benign tumor, it can stem from a benign tumor or it may recur, which is the most frequent situationThe parotid gland is the most common tumor location, followed by the palate and the submandibular glandHistologically, the malignant myoepithelioma is characterized by pleomorphism, occasionally with eosinophilic cytoplasm, a high mitotic rate and usually with necrosisDifferential diagnosis includes leiomyosarcoma, peripheral nerve sheath nerve tumor, synovial sarcoma and metastatic melanoma, and immunohistochemistry is fundamental do differentiate them5. It shows constant positiveness for the S100 protein, vimentin and cytokeratin antibodiesThe treatment advocated is tumor surgical resection with margins; however, before such procedure, an image exam must be carried out in order to assess the extension and involvement of neighboring structuresThe malignant soft palate myoepithelioma is an extremely hard tumor. Its treatment continues being broad resection. The long patient follow up described in the present case corroborates literature data.


Hamartoma of mature cardiomyocytes is a rare tumor and the present case shows a complex diagnostic pathway to understand its nature and treatment options in a young patient. The myocardial bridge was also part of the clinical evaluation discovered during the diagnostic workout.18F-fluorodeoxyglucose (18F-FDG) uptake, and evidence of myocardial bridging on coronary angiography. On suspicion of malignancy, coronary unroofing and surgical biopsy was performed. The final diagnosis was hamartoma of mature cardiomyocytes.A 27-year-old woman with atypical chest pain and a normal electrocardiogram received the diagnosis of neoformation of the interventricular septum with 18F-FDG positron emission tomography (PET) was used. A surgical biopsy was performed, and the final diagnosis was completed after the immune-histochemistry study. A myocardial bridge was found during preoperative coronagraphy and was treated accordingly.This case offers great insight into medical reasoning and decision-making process. Given the history of chest pain, the patient was evaluated for possible ischemic, embolic, or vascular causes. Given a left ventricular wall thickness ≥15 mm, hypertrophic cardiomyopathy (HCM) should always be suspected; nuclear magnetic resonance imaging is essential to distinguish between HCM. The magnetic resonance imaging is also critical in distinguishing HCM itself from tumoral phenocopies. To rule out a neoplastic process,  To evaluate the patient with suspected hypertrophic cardiomyopathy (HCM).To review the potential tumoural phenocopies of HCM and to establish a diagnostic pathway for differential diagnosis.To review the usefulness of second-line imaging (MRI and PET) to refine diagnosis along with potential pitfalls.To review the feature of the rare hamartoma of mature cardiomyocytes and the implications for its diagnosis and treatment.To review the incidental finding of myocardial bridging and clinicopathological correlations.1 and its diagnostic workup is a challenging scenario. The multidisciplinary approach has been the key to the final diagnostic findings and treatment option strategy.Hamartoma is broadly defined as a benign abnormal polyclonal proliferation of cells native to the affected organ. Hamartomatous lesions that are composed of mature muscle fibre bundles that occur in cardiac tissue have been called hamartoma of mature cardiac myocytes (HMCMs); HMCMs are rare tumours; only 47 cases have been described to date in both paediatrics and adults,A 27-year-old woman presented to the emergency department for atypical chest pain that had worsened in recent weeks. Past medical and family history was not significant. The electrocardiogram and seriated troponin assay were not suggestive of coronary syndromes. She had also undergone computed tomography with contrast medium (CT angiography) which excluded pulmonary embolism. A transthoracic echocardiogram (TTE) revealed the presence of marked septal hypertrophy (24 mm) without left ventricular outflow tract obstruction (LVOTO). The young woman was then referred to our centre for further characterization of the disease and possible treatment.3(Table 1). A 24-h Holter electrocardiogram, TTE, and nuclear magnetic resonance imaging (MRI) were therefore initially scheduled.While an interventricular septal thickness of at least 15 mm, in the absence of other cardiac or systemic causes capable of leading to such hypertrophy, is considered sufficient for the diagnosis of HCM, there are, however, other clinical conditions that share the same phenotypic expression that must be ruled outFigure 1A). The tissue was isointense in T1 sequences compared with the surrounding myocardium and hypointense in its central core; at T2, short tau inversion recovery (STIR) appeared inhomogeneous and slightly hypointense; it appeared to have an inhomogeneous contrast enhancement in the early phase, along with an intralesional late enhancement. To rule out a tumoural phenocopies of mid-ventricular hypertrophic cardiomyopathy (HCM), the patient underwent a 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) scan, which revealed uptake (SUV 7.9) at the level of the septum ; given the high suspicion of malignancy, a biopsy was deemed mandatory; unfortunately, it was not diagnostic. The patient was then referred for surgical biopsy of the mass. A second MRI performed 1 month later showed no growth of the nodular formation. On pre-operative examinations, coronary angiography revealed a myocardial bridge with a marked ‘milking’ effect at the middle level of the anterior descending artery (LAD); this effect was also present at the level of the first septal branch . The surgical schedule was then aimed at unroofing the muscular bridge on LAD and surgical biopsy of the mass with right trans-atrial approach.The 24-h Holter electrocardiogram showed sinus rhythm with normal atrioventricular (AV) conduction and corrected QT interval. The MRI revealed a localized mass (42 × 32 × 50 mm) in the interventricular septum protruding into the right ventricle, in the context of overall increased septal thickness , sending a specimen for histology. After opening the right atrium, the mass was identified, protruding into the right ventricular outflow and partly involving the chordal apparatus of the tricuspid valve . An extended specimen was then excised and sent for histology. Surgery subsequently proceeded according to routine.A median sternotomy was chosen for the procedure. On cardiopulmonary bypass (CPB) and cardioplegic arrest, the bridge myocardium was removed by muscle dissection , myogenin (MyoD1), S100, and proliferating antigen Ki67.Figure 3 panels A–F) compatible with a hamartoma of mature cardiomyocyte (HMCM), while the myocardial bridge consisted of normal cardiomyocytes.Histopathological investigations showed that the bulging mass had features  with limited expression had been raised.This case offers great insight into medical reasoning and decision-making process. Given the history of chest pain, the patient was evaluated for possible ischemic, embolic, or vascular causes. Although the probability of a patient of this age having coronary artery disease is 4.2%±1.3%,5 The MRI is also critical in distinguishing HCM itself from tumoural phenocopies. In this case, although the nodular formation might have been compatible with an expansive lesion, the late gadolinium enhancement (LGE) might have been suggestive of atypical HCM.2 To rule out a neoplastic process, 18F-FDG PET was used, which showed high radio drug uptake in that area (SUV 7.3)—a potentially dramatic picture since an SUV >4.3 is highly suggestive of primary malignant cardiac tumour.6 The most frequent primary cardiac tumours, such as sarcomas , and forms such as cardiac lymphoma and amyloid light-chain (AL) amyloidosis have all been excluded by tissue characterization with MRI and degree of involvement of myocardial structures.7–10Given the prevalence of HCM (1:500) in the general population, HCM should always be suspected in case of a left ventricular wall thickness ≥15 mm; nuclear MRI is essential to distinguish between HCM, athlete’s hypertrophy, metabolic and storage diseases, infiltrative processes (such as amyloid cardiomyopathy), and primary and secondary cardiac tumours.The occasional finding of ‘milking’ of the first septal branch and the anterior descending artery (LAD) on coronarography allowed the ‘open’ biopsy to be combined with myocardial bridge unroofing.11 while in a case of HCMC localized in the LV, a low uptake of the radioactive tracer was demonstrated.12Macroscopically, both myocardial bridge and ventricular mass were indistinguishable; consistent with the literature, the definitive diagnosis of HMCM was histological. It must be emphasized how little is known about this type hamartoma: in a recent report, an atrial HMCM had a high SUV (max 11),ytad077_Supplementary_DataClick here for additional data file.


Multirooted supernumerary tooth is a rare finding, which predisposes to various malocclusion. The present case report depicts the presence of a multirooted supernumerary tooth in the anterior maxilla just adjacent to the midline in a 24‐year‐old patient. Multirooted supernumerary tooth is a rare finding, which predisposes to various malocclusion. The present case report depicts the presence of a multirooted supernumerary tooth in the anterior maxilla just adjacent to the midline in a 24‐year‐old patient. On examination, an irregular, diffuse white mass was seen protruding from the maxillary gingiva, adjacent to the maxillary right central incisor Figure . The maxAfter addressing his chief complaint, the patient was advised for an intraoral radiograph to get a definitive diagnosis of the white mass. The radiograph showed a tooth‐like mass although not resembling any particular tooth Figure . CorrelaThe calcified mass was approached through an intra‐oral incision and removed in toto. There was no lining associated with the mass. The excised mass showed a multirooted tooth partially resembling a primary molar Figure .Multirooted supernumerary teeth in anterior maxilla is a rare finding and can not only compromise function, and esthetics but can also predispose to an array of malocclusions, dental caries, cysts, and tumors leading to potential complications.All the authors contributed to the writing of the manuscript.None.Ethics approval was not required from the institution, following our country's law, as this was a case report.Written informed consent was obtained from the patient to publish this case image following the journal's patient consent policy.


Adenoid cystic carcinoma of the lung is a distinctive salivary-gland-type malignant epithelial neoplasm that rarely presents as a primary tumor of the respiratory tract. Complete surgical resection remains the treatment of choice for adenoid cystic carcinoma. We present a case of large ACC tumors that caused severe central airway obstruction and were effectively treated with therapeutic bronchoscopy followed by radiotherapy and chemotherapy.A 31-year-old Malay Indonesian female patient who was a nonsmoker and had no family history of cancer was admitted to the emergency ward because of worsening breathlessness accompanied by stridor since 1 week prior. Chest computed tomography revealed segmental atelectasis of the left lung; a mass on the left main bronchus, with infiltrates in segments 1, 2, and 3 of the left lung; and consolidation in the left inferior lobe, with narrowing of the main left bronchus. Lobulated masses obstructing almost the entire distal trachea up to the carina and the entire left main bronchus were found on bronchoscopy. Owing to the large tumors causing severe central airway obstruction, the medical team decided to perform central airway mass removal through rigid bronchoscopy. A neodymium-doped yttrium-aluminum-garnet laser was used first to facilitate mass shrinkage. After the laser treatment, mechanical mass removal using a rigid scope was performed. The tracheal and carinal lumens were opened to > 50% of their diameter, with the left main bronchus lumen opened only slightly. After the treatment, the patient was stable, and no stridor was found. Adjuvant intensity-modulated radiotherapy and chemotherapy were performed after the therapeutic bronchoscopy. At the end of the entire treatment, reevaluation by thoracic computed tomography scan and bronchoscopy revealed no remaining mass.In cases of nonresectable large adenoid cystic carcinoma tumors with life-threatening central airway obstruction, therapeutic bronchoscopy followed by sequential radiochemotherapy might achieve a complete response outcome. Adenoid cystic carcinoma (ACC) of the lung is a distinctive salivary-gland-type malignant epithelial neoplasm that rarely presents as a primary tumor of the respiratory tract. It accounts for 10% of all salivary gland tumors, 1–2% of all head and neck malignancies, and < 1% of all bronchopulmonary cancers . Owing tAccording to current evidence, complete surgical resection is still the treatment of choice for patients with ACC . DespiteA 31-year-old Malay Indonesian female patient who was a nonsmoker and had no family history of cancer was admitted to the emergency ward with worsening breathlessness accompanied by wheezing since 1 week prior. The patient also had recurring hemoptysis, which started 2 years before and worsened in the past 6 months. Previously, she received antituberculosis treatment for 6 months, along with inhaled long-acting bronchodilator and steroid therapies. However, no clinical improvement was observed.Upon physical examination, severe dyspnea with stridor and decreasing vesicular breath sounds in the left lung were found. Chest radiography and computed tomography (CT) performed 1 week earlier revealed a mass on the left main bronchus  targeting the tracheobronchial mass, including the distal trachea, carina, and left main bronchus as gross tumor volume (GTV), was commenced 2 weeks after the therapeutic bronchoscopy. The clinical target volume (CTV) was GTV + 1 cm, and the planning target volume was CTV + 0.5 cm. The total dose plan amounted to 60 Gy, but the radiation dose could be delivered at 40 Gy and administered in 20 fractions of 2 Gy. After 20 fractions of radiation over 4 weeks, the patient developed esophagitis and dysphagia. Positioning the patient with thermoplastic masks was difficult because of mucous hypersecretion and persistent coughing. Two weeks after the radiotherapy series, the patient received a chemotherapy regimen consisting of carboplatin with an area under the curve of 5 and paclitaxel 175 mg/mTwo months after completion of the treatment, the patient had no dyspnea. In reevaluation by chest CT scan, no mass in either lung or the mediastinum and no lymph node enlargement were visible Fig. D. The trMalignant endoluminal central airway obstruction still proves challenging for clinicians and thus requires individualized strategies with specific interventions that can be performed in a particular healthcare center. In the present case, surgery was not performed because of the tumor location and size. Previous studies have reported that surgical management of ACC tracheobronchial cases was only indicated for locoregional tumors. Complete tumor resection and negative airway margins were considered indicators of successful surgery . On the In life-threatening cases, immediate-effect bronchoscopy is the modality of choice. We chose a combination of rigid scope, laser, and argon plasma coagulation in the present case. Lasers are capable of resecting and vaporizing tissues and therefore produce an excellent coagulation effect , 8. The ACC has been reported to exhibit low radiation sensitivity, but some studies have reported good radiotherapy response , 16. AftSurgical resection is a widely recommended treatment for localized tracheal ACC. In nonresectable large ACC tumors with life-threatening central airway obstruction, therapeutic bronchoscopy may be lifesaving, and sequential radiochemotherapy after mass removal might achieve a complete response outcome.


The Morel-Lavallée lesion (MLL) is a closed soft-tissue injury that is frequently associated with high-intensity trauma. The thigh, hip, and pelvic regions are the most typically affected regions. It is critical to recognize and treat an MLL as soon as possible because it is often neglected or its identification is delayed because of other distracting injuries in a polytrauma patient. Bacterial colonization of these closed soft-tissue wounds can result in an increased risk of perioperative and postoperative infection. Magnetic resonance imaging has recently been used to define and grade these lesions. To reduce the dangers of these situations, clinical suspicion and on-the-spot identification of these lesions are essential. Here, we report an operated case of fracture shaft femur associated with MLLs and discuss the diagnostic and surgical approaches. The Morel-Lavallée lesion (MLL) is a kind of degloving injury to traumatized soft tissues that are closed externally. In 1863, French physician Victor-Auguste-François Morel-Lavallée described the lesion for the first time . This stThe MLL can either present acutely or can occur days after the injury. Several factors determine its presentation. The clinical diagnosis of MLL is often determined by the rate and degree of hemolymphatic accumulation inside the cavity and the body habitus of the patient. The affected areas can display ecchymosis, swelling of the soft tissue, and fluctuation or hypermobility of the skin. Because the superficial skin discoloration may be delayed for many days, initially, the diagnosis can go unidentified. The affected area can become painful and firm with time, which indicates the formation of capsules. Chronic lesions can imitate other diseases of soft tissue including neoplasms. Due to inappropriate management, the late evolution of the lesion may result in soft-tissue envelope infections or necrosis . IdeallyThe patient reported here presented with swelling and blackish discoloration of the skin on the lateral part of the right distal thigh and the proximal aspect of the right leg with ipsilateral operated fracture of distal one-third shaft femur with retrograde interlock nail in situ. Although the site is not typical of MLL, it occurs in the distal thigh and knee region when associated with high-energy Injuries. Such a combination of injuries has not been previously described. Multiple surgical interventions were done resulting in dramatic improvement.A 36-year-old male presented to the Acharya Vinoba Bhave Rural Hospital (AVBRH) casualty department with swelling and blackish discoloration of the skin on the lateral aspect of the right distal thigh and the proximal part of the right leg Figure  as well He had met with an accident 18 days back while he was driving a motorcycle and a car hit him from the back. He had a fracture in the right distal one-third shaft femur and bruises on the anterior aspect of the right distal thigh. The fracture was managed with closed reduction and internal fixation with retrograde femur interlock nail in a private hospital.On presentation, he gave a history of the development of blisters over the distal lateral aspect of the right thigh three days after the surgery, followed by pus that started coming from this area 10 days later. His routine investigations showed low hemoglobin, and he was given one unit of packed red cells and two units of whole blood. He was taken up for debridement of the involved area, and 200 mL of purulent fluid lying between the fascia of Vasti muscles and the soft tissue overlying it was drained and sent for culture and sensitivity testing  was done. The wound was found to be healthy after five days (Figure All the necrosed tissue was removed and a thorough wash was performed with a mixture of normal saline, betadine, and hydrogen peroxide. Sterile dressing was done. The patient was started on colistin as the culture showed growth of s Figure  and was s Figure  and a prs Figure .The MLL is a sequel of closed degloving injury which involves the separation of the skin and subcutaneous fat from the underlying fascia. This results in the development of potential space between the two layers. This space gets filled with blood, fat, and lymphatic fluid. Often, this fluid gets colonized with a bacterial infection which leads to perioperative infection. In some cases, the granulation tissue that gets collected around the lesion develops into a pseudocapsule and the collected fluid is unable to get reabsorbed which leads to the development of a chronic fluid collection at the local site. This lesion was first described in the hip region. Other common locations for this condition are the pelvis, lower back, buttocks, and the lateral thigh region.In some cases, the lesion is asymptomatic but, in most cases, it is associated with pain and swelling. The patient may or may not recall a history of trauma. Often, the lesion is misdiagnosed as local edema due to trauma. The delay in diagnosis leads to the swelling becoming tense and extensive cellular destruction. Because this lesion is an uncommon occurrence, a localized swelling in a polytrauma patient is often thought to be due to contusion or local edema ,4.MLL is mainly a clinical diagnosis and imaging modalities should be used for confirmation. A localized painful swelling in an emergency setting in a polytrauma patient should raise the suspicion of MLL. An ultrasound can be performed initially that will demonstrate the lesion as an anechoic area. The modality of choice is MRI as it shows excellent soft-tissue construct. Doppler can be used to rule out vascular causes such as deep vein thrombosis. CT scan may also demonstrate fluid collection but is not routinely recommended.This condition can be managed conservatively as well as surgically depending on various factors such as the volume of the cavity, chronicity of the lesion, and pain and discomfort experienced by the patient. In acute conditions, conservative management with compression, rest, and ice fomentation can be helpful. In chronic cases where the lesion is large and non-responsive to conservative management, needle aspiration and drainage should be performed. Needle aspiration should be considered where the fluid collection is less than 50 mL. Cases where the collection is more than 50 mL, the lesion usually recurs. Hence, the collection of more than 50 mL is an indication of operative intervention [Although MLLs are uncommon, doctors should be wary of patients who report chronic pain and fluid collection on advanced imaging following blunt trauma injuries. If identified quickly, conservative therapy can be undertaken; however, if left untreated, surgical intervention may be required, as detailed in this case. This case emphasizes the need of maintaining a high clinical suspicion for an MLL in cases of extensive closed soft-tissue injuries with or without fractures. A thorough follow-up and step-wise treatment help in the early detection, treatment, and prevention of rare but potentially fatal sequelae.


Schwannoma is a rare benign soft tissue tumor that appears like a neuroma based on its specific location and clinical features. We report a case of a plexiform schwannoma in a middle-aged woman who had a painful bump located in the third webspace on the dorsum of her right foot for the last four years. Initially, the swelling was thought to be Morton's neuroma based on location and clinical feature findings. The mass was resected and was sent for histopathological examination, revealing a plexiform schwannoma, most likely developing from the cutaneous nerves on the dorsum of the foot. She reported improvement in her symptoms after complete and careful excision without any neurological deficit. Investigation of any subcutaneous foot swelling should be coupled with a histopathological examination for comprehensive management. Plexiform schwannoma, also known as neurilemmomas, is a benign and uncommon presentation of peripheral nerve sheath growth. The tumor mainly originates from the slow proliferation of Schwann cells . These nA 37-year-old female without any past medical history presented with a swelling on the dorsum of her right foot for the past four years Figure . The sweSubsequently, an ultrasound of the dorsum of the right foot was done, showing a well-defined lobulated, homogenous hypoechoic lesion of size 4.7 × 3.5 cm is seen between the third and fourth digit of the right foot, along with posterior acoustic enhancement and intralesional flow noted, however, no cystic and calcification changes were present. Considering clinical lesion features, imaging, and location, a provisional diagnosis of a lesion of neural origin, possibly Morton's neuroma, was made. Her biochemical and hematological parameters were nonsignificant. After taking informed consent, the patient was managed operatively by excision of mass while maintaining continuity of the nerve fibers. This was performed under spinal anesthesia. She reported no neural deficit or any neural complication after excision. Post-operatively patients were managed by intravenous fluids, antibiotics, antacids, analgesics, and other symptomatic drugs. The regular aseptic dressing was done with the drain in situ. After two days, the patient was discharged with proper advice for a high-protein balanced diet, adequate bed rest, and regular follow-up.Her microscopic examination of the sections revealed tissue lined by unremarkable to sightly hyperplastic epidermis. The underlying stroma showed a partially well-encapsulated tumor composed of cellular areas alternating with paucicellular myxoid areas showing loosely dispersed spindle cells. The cellular areas showed fascicles of spindle cells arranged primarily on organoid whorls or lying parallel and occasionally also haphazardly. In the cellular areas, there were spindle cell nuclei arranged in a well-defined palisade manner around eosinophilic areas, suggesting the formation of verocay body structures. The spindle cells showed mild anisonucleosis, had an oval to elongated buckled or polypoidal nucleus with vesicular or occasionally slightly darkly stained chromatin, and abundant pale to deeply eosinophilic cytoplasm stretched out at both ends. A few thick and thin-walled blood vessels were also seen. No atypical mitosis is seen in Figure Schwannoma is a rare nerve sheath tumor that grows from Schwann cells. Schwann cell tumors are classified as schwannomas, malignant peripheral sheath tumors, and neurofibromas. Schwannomas make up 5% of all benign soft tissue neoplasms ,7. CliniThe preoperative diagnosis of schwannoma is made by imaging modalities like ultrasonography and MRI. An ultrasound of a schwannoma shows it to be a homogenous, hypoechoic mass and may indicate its proximity to a nearby nerve. Occasionally, posterior acoustic enhancement and internal blood flow patterns can also be observed, as in our case ,9. An MRSchwannoma mainly presents as an asymptomatic solitary nodule with slow progression, which can be histologically categorized into plexiform, cellular, and melanotic variants . The pleSurgical excision is recommended for significant swelling and patients presenting with persistent symptoms. These tumors arising from the nerve sheath are characterized by the splaying of nerve fibers underlying them . TherefoPlexiform schwannoma is a rare benign soft tissue neoplasm. It should be kept in the differential diagnosis while dealing with subcutaneous swelling of the foot. In our case, the clinical presentation of this tumor was misdiagnosed as Morton's neuroma, so for diagnostic accuracy, an excisional biopsy is a crucial step in confirming the diagnosis. Complete surgical excision is recommended to halt recurrence and for good functional outcomes.


Arteriovenous malformations are a structural vascular abnormality caused by the absence of normal capillary beds, which lead to the development of abnormal blood channels that connect the arterial circulation to the venous circulation.A 17-year-old male was referred to our outpatient clinic because of recurrent epistaxis after left nasal polypectomy. He had undergone nasal polypectomy due to nasal congestion at a local clinic 10 days ago. He had neither other symptoms nor any medical history. There was no history of trauma and no sign of allergy. The endoscopic examination revealed bulging of lateral nasal wall and bleeding due to left maxillary sinus mass A. A CompVascular malformations occur as a result of aberrant vessel angiogenesis during embryogenesis.The most common presenting complaint of arteriovenous malformations of the maxillofacial region is chronic intermittent bleeding although some of these tumors have no signs or symptoms.Although many arteriovenous malformations may be suspected clinically, imaging is required for confirmation and treatment planning, especially as incorrect diagnosis may lead to inadequate or inappropriate treatment resulting in life threatening hemorrhage as well as recurrence.Complete surgical resection with or without preoperative intra-arterial embolization is necessary to prevent recurrence. Embolization is indicated for occlusion of unresectable lesions, staged occlusion as preparation for resection, or as an emergent preoperative hemostatic control. Preoperative embolization may help delineate the extent of a lesion, reduce intraoperative bleeding, and lessen the risk of recurrence. Because collateral blood supply becomes established quickly, operation should be within 24–48 h after embolization. Furthermore, no patients with arteriovenous malformations were cured by simple embolization.Diffuse arteriovenous malformations cross and destroy tissue boundaries, and have a high rate of recurrence after surgical or embolic therapy.Although arteriovenous malformation in the maxillary sinus represents a rare pathology, it is important to consider this in the differential diagnosis of patients with recurrent epistaxis from a highly vascular mass of the maxillary sinus. Complete surgical removal via Caldwell-Luc approach may be considered for this lesion if possible, with preservation of cosmesis and function when surgical intervention is indicated.The authors declare no conflicts of interest.


Alveolar soft part sarcoma (ASPS) is a rare tumor but potentially fatal condition. Understanding the imaging and clinical features of ASPS is of certain value for preoperative qualitative diagnosis and clinical treatment of tumors. Nevertheless, there have been only 11 documented case reports describing the sonographic features in the English literature.Three patients with confirmed ASPS occurring primarily in the limbs were enrolled in this study. Complete surgical excision was performed with conservative limb function. We pay particular attention to the ultrasonographic features and performed a literature review of ASPS cases.With regular surveillance, one patient had no symptom recurrence and two developed lung and/or breast metastasis later. The specific sonographic findings were heterogeneous hypoechoic, well-circumscribed, and lobulated or round contours on grayscale images, abundant flow signals of intratumoral and extratumoral tubular structures on color Doppler images.Its low incidence rate and lack of characteristic clinical manifestations often result in misdiagnosis of ASPS. The specific sonographic findings may add useful diagnostic information. It is known to comprise 0.2% to 0.9% of all soft tissue sarcomas and tends to occur between ages 15 and 35 years.,3 It predominantly occurs in the lower extremities, and it has been described in a variety of unusual locations, including the urinary bladder, breast, larynx, uterine cervix and bone.,5 Understanding the imaging and clinical features of ASPS is of certain value for preoperative qualitative diagnosis and clinical treatment of tumors. Nevertheless, there have been a few reports describing the sonographic features of ASPS. To our knowledge, only 11 cases of the ultrasound features in ASPS were published and available on PubMed. Here, we report 3 cases of ASPS occurring primarily in the limbs, pay particular attention to the ultrasonographic features and review the literature. The written informed consents were obtained from the patients for publication of this case report and accompanying images.Alveolar soft part sarcoma (ASPS) is a rare tumor that was first defined and named by Christopherson et al in 1952.A 30-year-old male was referred to the Department of Orthopedics, for evaluation of painless swelling of the left arm with a period of 1 week. Upon palpation, the patient presented a 10 cm long mass. The mass appears ill-defined, without cutaneous retraction, medial hardness or mobility in the transverse plane.Ultrasonography of the mass was performed using a real-time convex array scanner of 5 MHz and a linear array scanner at a nominal frequency of 12 MHz  with the patient in the sitting position. The lesion was heterogeneous hypoechoic tissue measuring 7.9 × 6.1 × 4.8 cm in the triceps brachii muscle, well limited, and with tube-like echoless in the center and periphery of the tumor. Color Doppler ultrasound showed indiscriminate color flow signals in anechoic area  staining.Metastatic examination included brain MRI and CT of his chest, abdomen and pelvis, and no masses or lesions were found. Complete surgical excision was performed with conservative limb function. Pathological analysis revealed a pseudoalveolar pattern and sinusoidal vessels, and the immunohistochemistry results demonstrated diffuse nuclear immunoreactivity to TFE3 and vimentin, which confirmed the initial hypothesis of ASPS. The clinical course was favorable in the short and medium term with good wound healing.Four months after diagnosis, multiple masses in both lungs had developed. Contrast-enhanced CT imaging of lungs showed mild homogeneously enhancing lobulated lesions in the bilateral lobs (left approximately 0.5 cm and right approximately 0.6 cm) with clear and smooth borders. Right-sided larger lung lesions underwent total gross excision. Histopathology of lung lesions was suggestive of metastatic deposits of ASPS. He was given 3 courses of chemotherapy with vincristine, ifosfamide, and cisplatin. On follow-up ultrasound imaging, there was no evidence of local relapse on the left arm 13 months after surgery.A 30-year-old female presented with a history of swelling in her left thigh for approximately 1 and a half years. She visited her doctor because of the increased size of the lesion and recurrent numbness sensation. Physical examination revealed a well demarcated, non-tender mass on the left thigh.Ultrasonography of the mass was performed using a real-time convex array probe of 6 MHz  with the patient in the supine position. Grayscale ultrasound revealed a heterogeneous hypoechoic tissue measuring 8.2 × 8.2 × 5.5 cm with a clear boundary in the vastus intermedius muscle that was well limited, and had tube-like echoless Fig. A. Color A diagnosis of ASPS was rendered after ultrasound-guided biopsy. Immunohistochemistry then confirmed this because of positive TFE3. CT chest showed multiple bilateral lung metastases before surgery. This patient underwent medial femoral muscle resection under general anesthesia. She was discharged in good condition 12 days after the operation. On follow-up imaging, there was no evidence of local disease on the left thigh. Ultrasonography revealed multiple breast metastases 14 months after surgery. The patient declined chemotherapy and radiation throughout her postoperative course, and she passed away from disease-related complications 38 months later.A 23-year-old female presented to the hospital with a slight pain mass in the left thigh. The mass was first noticed by the patient 2 years earlier and presented with a relatively indolent clinical course. Before 1 month, she presented with intermittent pain and discomfort on the lateral side of the left thigh. Physical examination revealed an ovoid, immobile mass with a longitudinal diameter of 9 cm and some tenderness on palpation.Ultrasonography of the mass was performed using a 9 MHz real-time linear array probe  with the patient in the lateral position. The lesion was heterogeneous hypoechoic tissue measuring 8.3 × 5.1 × 4.0 cm in the vastus lateralis muscle, well limited, and with tube-like echoless , upper extremity (n = 2), lower extremity (n = 4), penis (n = 1), bladder (n = 1) and thyroid (n = 1). ASPS is most commonly found in the extremities, which corresponds with our case series. The remaining 4 cases were diagnosed with breast metastasis by using high-frequency ultrasound. The length-diameter of the ASPS tumor ranged from 15 mm to 150 mm . The measurements of limb tumors were relatively larger than those of primary ASPS in other sites . In our cases, 1 patient had lung metastases at diagnosis and 2 developed lung and/or breast metastasis later. The clinical features of ASPS are slow growth, high rate of early hematogenous metastasis, late local recurrence and fatality. Metastasis may already exist at the initial clinical manifestation.,19Of the 14 patients, including our 3 case reports, ten were female and 4 male, and ages ranged from 3 to 71 years (median 24 years). Patients with ASPS generally range from 20 to 30 years, and women are more likely to develop tumors than men. Therefore, it is usually first diagnosed by symptoms that metastasize to the lungs and brain. The 5-year overall survival rate of patients with metastasis can decline sharply.ASPS usually presents as a slowly growing, painless soft tissue mass and rarely causes functional impairment. Unlike the majority of sarcomas, ASPS has high frequency of metastases, primarily to the lungs, brain and bones.Ultrasound is essential for determining extent of the lesion, which helps in evaluating resectability, and for most tumors complete resection is intricately related to prognosis. After a diagnostic biopsy, all patients but 1 received curative surgical resections. For ASPS, the most common pattern of internal echogenicity includes hypoechoic areas with well-defined boundaries, and these masses may be heterogeneous or homogeneous and vary with size. Two reported cases with a diameter less than 15 mm showed homogeneous echo, and the others with larger sizes were described as heterogeneous. Tumor margins with well-circumscribed and lobulated or round contours were also frequently observed. It has been hypothesized that hypoecho may be attributed to the tumor growth pattern. Histologically, ASPS is characterized by a uniform nest‑like pattern of large, rounded or polygonal tumor cells with eosinophilic granularity, a pseudoalveolar appearance, and often central necrosis. The intratumoral sinusoidal vessels showed dense hyperplasia, fusion and sometimes expansion. The intratumoral vessels could be detected by high-frequency ultrasound. Owing to its hypervascularity, ASPS might be confused with an arteriovenous malformation. In 1 case, color Doppler ultrasound revealed very low RI, approximately 0.24 to 0.58. Low RI indicated the presence of a direct shunt between the artery and dilated vein.Ultrasound also characterizes vascularity. All cases showed hypervascularity with color Doppler ultrasound owing to its abundant blood supply and well vascularized.,14 Color Doppler ultrasound shows that these tubular structures are abundant colored flows. This finding has been reported to be a feature of this tumor and it has been described to exist at both the center and periphery of the tumor. It corresponds to intratumoral and peritumoral flow voids on MRI. The surrounding veins often twisted and dilated, occasionally showing mural attenuation disproportionate to the lumen diameter. This vascular change may represent early sinus vascular remodeling, which may later be incorporated into the tumor.In addition, our cases indicated additional imaging features: the appearance of tubular structures, which is in consistent with 2 other cases describing the vascular nature of this tumor. In our cases, the size of the breast tumors was relatively small, approximately 22.2 mm. The most common ultrasonographic appearance of breast metastasis is 1 or more well-circumscribed, homogeneous or heterogeneous hypoechoic nodules,,15,16 which cannot be differentiated from benign masses from a purely radiological point of view. Although the breast is an unusual metastatic site, ultrasonographic evidence of breast nodules may be the initial or later finding of alveolar soft tissue sarcoma. Therefore, breast nodules should be considered metastatic diseases in patients with extramammary alveolar soft tissue sarcoma. In 1 case, the lesions showed enlarged vessels around the tumor and dilated intratumor vessels at the peripheral portion on grayscale ultrasonic imaging, and there were abundant color flow signals on color Doppler ultrasound. These imaging manifestations are similar to those of primary ASPS but are not common in benign lesions, which indicates the vascular pathological feature of the tumor.Metastases that occur primarily in mammary tissue are considered extremely rare.Despite the rarity of ASPS, we reviewed the sonographic characteristics of a case series. This enables a more comprehensive evaluation of the imaging features of this tumor than before. The sonographic features add useful diagnostic information of ASPS and will aid surgeons in performing wide surgical resection to reduce the risk of local recurrence. A diagnosis of ASPS should be considered when a solid tumor arising in muscle is heterogeneous hypoechoic, well-circumscribed, and lobulated or round contours on grayscale images, hypervascularity on color Doppler images. Additional diagnostic features that we identified are the presence of intratumoral and extratumoral tubular structures, which indicate large vessels at both the center and periphery of the tumor. Prominent intratumoral and extratumoral vessels of a mammary nodule, which are similar to primary tumors, may be a sign of breast metastasis, especially in a young woman with a history of ASPS. In the future, the direct correlation between imaging features and anatomical and pathological results will further deepen our understanding of the imaging manifestations of this rare and unique tumor.Conceptualization: Shanshan Zhang.Data curation: Wenting Fan, Diancheng Li, Hui Tian, Dongdong Che, Lei Yu, Shuang Gao.Formal analysis: Wenxue Li.Funding acquisition: Yiqun Liu.Investigation: Yiqun Liu.Methodology: Yiqun Liu.Supervision: Yiqun Liu.Writing – original draft: Wenxue Li.Writing – review & editing: Yiqun Liu.


Deciduosis is the presence of ectopic decidual tissue outside the uterus, pelvic, or abdominal organs usually associated with pregnancy. Cutaneous deciduosis is a highly uncommon manifestation of deciduosis and most commonly is misdiagnosed as a primary malignancy or a metastatic deposit. Typically, it is detected incidentally during operative procedures. It has been rarely documented within a surgical scar; with the incidence of surgically proven deciduosis being approximately 1.6%, and is often difficult to diagnose due to its rarity. Here, we present a case of deciduosis of cesarean scar in a 34-year-old pregnant female. Deciduosis is clinically defined as the presence of ectopic decidual tissue in locations outside the uterus. It has been reported to occur in various pelvic and extra-pelvic sites. It is typically known to present during pregnancy but has also been reported in non-pregnant women. It is considered to be a benign lesion during pregnancy, not associated with any obstetric complications. It does not have any pathological impact on the mother as well as the fetus. It is usually asymptomatic and can remain undetected throughout pregnancy. Total remission is generally known to occur in the postpartum period; however, some cases may require surgical intervention, especially those that often mimic a malignancy.1Cutaneous deciduosis is an uncommon manifestation of cutaneous endometriosis. Cutaneous endometriosis usually occurs within the umbilical region or in abdominal surgical scars, the latter typically occurring after cesarean section, appendectomy, or an inguinal hernia repair. Although deciduosis has been reported in numerous ectopic locations, most lesions are encountered within the cervix or ovary.2,3To date, only a few well-documented instances of cutaneous deciduosis have been reported, with very few occurring within abdominal scars from previous cesarean sections.2We present a case of cutaneous deciduosis of a cesarean scar diagnosed incidentally in a 34-year-old pregnant female, along with some review of the available literature.All the case series and case reports, inclusive of their references, identified by extensively searching the PubMed, Scopus, Medline and Google Scholar databases utilizing the keywords “deciduosis”, “extra- abdominal deciduosis”, “cutaneous deciduosis” and “deciduosis in a cesarean scar” were read and included in this manuscript. A total of 13 cases of cutaneous deciduosis have been reported in literature from 1982; of these, 8 of them have been known to occur in a scar of a previous cesarean section.A 34-year-old pregnant female (G2P1L1A0) presented to this hospital at 38 weeks of gestation with complaints of abdominal pain.Her previous pregnancy was six years ago, which had concluded in a healthy child with breech presentation, delivered through lower segment cesarean section (LSCS). She was a known case of hypothyroidism and was also suffering from Gestational Diabetes Mellitus (GDM) in the current pregnancy and was being managed for the same with Tab Eltroxin 50ug, Inj Glargine, and Tab Metformin 500mg, respectively.Her obstetric examination revealed a uterine fundus height of 36 weeks with a breech presentation and a normal fetal heart rate (FHR). Her preliminary hematological as well as serological investigations were within normal clinical limits.Obstetric ultrasound examination was also carried out, which revealed an adequate Amniotic Fluid Index (AFI) with the placenta placed anteriorly and a breech fetal presentation.Hence, in view of the above clinical and ultrasonographical findings, the patient was taken for elective LSCS as a case of Antenatal Case (ANC) with breech presentation with previous LSCS with Gestational DM and hypothyroidism.A healthy infant was delivered. Additionally, intraoperatively, the scar of previous LSCS presented with features of endometriosis along the left lateral margin, which was excised clinically as endometrioma and sent for histopathological evaluation.Grossly, the sample presented as multiple fragmented tissue bits, with the largest measuring approximately 2cm and the smallest measuring approximately 1cm in its greatest dimension, respectively.On microscopic evaluation, hematoxylin and eosin (H&E) stained sections revealed multiple nodules composed of decidualized stromal cells surrounding a few slit-like endometrial glands with fibroblasts and collagen .These decidual cells were polygonal, with large nuclei, abundant homogenous eosinophilic cytoplasm , and assOccasional dilated endometrial glands were also noted, which showed eosinophilic secretions with adipose tissue present along the periphery . No featOn immunohistochemical evaluation, it was noted that the decidual cells showed reactivity to PR receptors and CD10 antibody .These histopathological features, along with the clinical findings connoted with the diagnosis of deciduosis of cesarean scar.Deciduosis is clinically defined as the presence of decidual tissue at sites other than the uterus.Grossly, deciduosis of cesarean scar may show a varied presentation ranging from an individual geographic pattern, nodular distribution or a polypoid appearance, which may often mimic a neoplasm.Microscopically, decidual cells are commonly found to be associated with endometrial tissue present over a scar. The lesions may frequently present as small cell groups or single-cell clusters, and uncommonly, they are in the form of widespread-diffuse deciduosis that completely occupies the scar along with the underlying adipose stroma. Our case presented with microscopic features depicting the latter. Decidual cells are generally large and polygonal, with homogeneous, eosinophilic cytoplasm associated with varying degrees of vacuolar degeneration. Decidual cell vacuolization is related to the duration of the pregnancy. Stroma may also show myxoid deposit due to vacuole rupture if the decidual cell cytoplasmic vacuolar degeneration is over 50%.The pathogenesis of ectopic decidual reactions is not yet fully understood. It is still not completely deciphered whether it is a physiological reaction or a pathological process. It is said to be the result of the exaggerated response of the endometrium to progesterone during pregnancy.5Zaytsev and Taxy,Histopathologically, it is important to differentiate deciduosis comprising decidual cells showing varying degrees of atypia with foci of hemorrhagic necrosis from deciduoid variant of malignant mesothelioma and metastatic malignant melanoma. Ectopic decidual tissue containing myxoid stroma and vacuolated decidual cells must also be differentiated from metastatic signet ring cell carcinoma. The clinical history of the patient, the lack of active mitosis in decidual cells along with the CD10 and Progesterone Receptor (PR) positivity with non-reactivity to keratin (CK), WT1, calretinin, and HBME-1 antibody on immunohistochemistry support deciduosis.5Deciduosis of cesarean scar also needs to be distinguished from a recently introduced entity called Deciduoma. Deciduoma is a manifestation of ectopic deciduosis; however, it is a large lesion with abundant vascularity and has a high potential for hemorrhagic complications.1Additionally, our patient was a known case of hypothyroidism and GDM. The literature has shown an increased predisposition to endometriosis development in women suffering from hypothyroidism. A study conducted by Peyneau et al.-35Very few cases of cutaneous deciduosis have been reported in literature to date. 27The mainstay of treatment of scar deciduosis is surgical excision if it fails to undergo complete remission in the post-partum period. The patients are known to completely recover following excision of the lesion, and recurrence, if present, is very rare.,37Scar deciduosis is usually an uncomplicated event with an asymptomatic course. Complications, if present, are rare and may manifest in the form of rupture of the scar, with or without uterine rupture, or secondary infection of the lesion resulting in sepsis.36Scar deciduosis is an uncommon but possible manifestation of cutaneous endometriosis and should always be considered in an appropriate clinical setting. Although it may often mimic a neoplasm, the histopathological features of decidual cells along with the utilization of appropriate immunohistochemical techniques help to establish the diagnosis and rule out other neoplastic mimics of deciduosis.


CIC–DUX4 fusion-positive sarcoma is a subtype of undifferentiated small round cell sarcoma that is rarely reported. As far as we know, less than 200 cases have been reported worldwide to date. The clinicopathologic characteristics of this kind of tumor are non-specific, which makes it difficult to be diagnosed. Therefore, more cases are required to enrich the diagnosis and treatment experience. Here, we present a 17-year-old Asian girl diagnosed with CIC–DUX4 fusion-positive sarcoma after targeted next-generation sequencing. Her clinical manifestation was abdominal pain. Furthermore, a mass in the pelvic cavity and massive ascites were found after an imaging examination. After resection, the mass was sent to the pathology department for a definite diagnosis, and the micromorphology showed an undifferentiated sarcoma with massive necrosis. The tumor cells were round to spindle with clear to eosinophilic cytoplasm and vesicular nuclei. Rhabdoid cells and myxoid mesenchyme were focally shown. Immunohistochemical staining showed diffusely positive for vimentin, cyclin D1, Fli-1, and WT-1 and very focally positive for CD99. Moreover, the targeted next-generation sequencing also revealed other genetic changes in this tumor including LongInDel of POLE, copy number variation of CD79, low tumor mutational burden, and microsatellite stability. With a follow-up time of 6 months, the patient survived the disease and received chemotherapy routinely. This report presented a rare primary site CIC–DUX4 fusion-positive sarcoma (CDS) and revealed novel genetic changes that enrich the manifestation, histology, and cytogenetic scales of this rare sarcoma. In addition, we have summarized the clinicopathologic characteristics of this tumor by reviewing the literature to have a better understanding of CIC–DUX4 fusion-positive sarcomas, which may be helpful for diagnosis and treatment. CIC-rearranged sarcoma is one of the new tumor types of the USRCS, which used to be considered Ewing-like sarcoma.Undifferentiated small round cell sarcomas (USRCSs) are a group of tumors with similar morphology, which makes it difficult to identify and diagnose. However, with the rapidly developing molecular pathology, it is possible to clarify them by genetic changes. CIC–DUX4. Afterward, Italiano et al. implicated that CIC was fused to copies of DUX4 gene not only on 4q35 but also on 10q26.3  arising in the pelvic cavity of a 17-year-old girl with targeted next-generation sequencing results, which enriches the manifestation, histology, and cytogenetic scales of this rare sarcoma.To date, more than 200 cases of n fusion –3, 7–32.A 17-year-old Asian girl came to the hospital with complaints of abdominal pain. A computed tomography (CT) scan showed a cystic and solid mass measured 12.1 cm × 8.2 cm × 13.6 cm in the pelvic cavity with heterogeneous enhancement in an enhanced scan Figure 1Grossly, the tumor showed a fused-nodular mass with an appearance of color and a touch of tenderness when being cut. The histological examination demonstrated the tumor consisted of round to spindle malignant cells with clear to eosinophilic cytoplasm and vesicular nuclei. Rhabdoid cells and myxoid mesenchyme were focally shown. Moreover, necrosis and pathologic mitoses were apparent (CIC exon 20 and DUX4 exon 1 (POLE (exon22_exon31del) (CD79A (5.8 copies), 4) low tumor mutational burden (TMB) at 0.5 Muts/Mb, and 5) microsatellite stability (MSS). Finally, this tumor was diagnosed as CDS.For diagnosis and further treatment strategy, the paraffin section of the tumor was sent for targeted next-generation sequencing (NGS) on the Illumina platform. The exons of 672 and 633 genes were detected by DNA sequencing and RNA sequencing, respectively. The results were as follows: 1) fusion between After the surgery, the patient went to another medical institution for further treatment. At a follow-up of 6 months, the patient was still alive.CIC-rearranged sarcomas were categorized as a new entity of USRCS (In the latest (2020) World Health Organization (WHO) classification of tumors of soft tissue and bone, of USRCS . So far,of USRCS , 26, whiof USRCS , cardiacof USRCS , vaginalof USRCS , and masFor morphology, the typical CDS usually consists of undifferentiated small round-to-oval tumor cells with clear to eosinophilic cytoplasm and vesicular nucleus, arranged in sheets and nests. Pathologic mitoses and geographic necrosis are easy to see. Focal myxoid matrix , 26, 30 During immunohistochemistry, almost all CDS showed vimentin positivity, and most showed weak and focal membranous CD99 positivity. Specht et al.  found thCIC–DUX4 fusion is necessary to reach the final diagnosis. Fluorescence in situ hybridization (FISH) and targeted RNA sequencing are the most popular methods to detect CIC rearrangement. However, Yoshida et al. described four cases that showed negative results by CIC break-apart FISH assays but identified CIC–DUX4 and its fusion breakpoint through high-throughput RNA sequencing , which causes the loss of part of the polymerase domain and CysB motif  ’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.QW wrote the manuscript. YH diagnosed the case and revised the manuscript. Both authors contributed to the article and approved the submitted version.