A 42-year-old female presented with complaints of mild occipital headaches of severe months duration. There were no cutaneous lesions or significant family history suggesting any genetic disease. On examination, she did not have any papilloedema, dymetria, dysdiadokokinasia or ataxia. Her cranial nerve examination was normal. On non-enhanced computed tomogram (CT) showed a mildly hyperdense well circumscribed lesion, in the left upper cerebellar hemisphere with no bone involvement (not schown). MRI showed an intra-axial lesion measuring 3.7cm × 3.5cm × 2.5cm in relation to the tentorium cerebelli and lateral sinus which was hypointense on T1-weighted image (T1WI) (A), with the characteristic striated appearance or “tiger-striped” appearance on T2-weighted image (T2WI) (B), there was no apparent contrast enhancement (C). This lesion was hyperintense in diffusion (D) and in fluid attenuation inversion recovery (FLAIR) (E). There was no extension into the internal auditory canal. There was no compression of the brainstem and ventricular system. Proton (¹H) MR spectroscopy study (MRS) revealed normal N-acetyl-aspartate (NAA)/Creatine (Cr) ratio (1.48), moderately decreased choline (cho)/Cr (0.76) (F). An abdominal ultrasound was performed in order to evaluate the intraabdominal organs and found to be normal. She has been under routine follow-up for 3 years. On the last control, the brain MRI showed lesion stability. Given the well-tolerated nature of her headaches and the stability of the lesion, the staff of the neurosurgeons indicated a routine control without surgery.


Spinal epidermoid cysts are benign tumors, which are rarely seen as an intradural extramedullary spinal cord tumor in the conus medullaris region. Acquired spinal epidermoid cysts are mostly caused by iatrogenic procedures, such as lumbar puncture, and the majority of acquired spinal epidermoid cysts have been reported below the L1 level, because lumbar puncture is usually performed around the iliac crest. Here, we report an extremely rare case of an epidermoid cyst that occurred as an intradural and extramedullary spinal cord tumor attached to the conus medullaris after repetitive epidural anesthesia.A 67-year-old female presented with a low back pain and left sciatica. Although the patient had experienced occasional mild low back pain for several years, her low back pain markedly worsened 2 months before her visit, as well as newly developed left sciatica resulting in intermittent claudication. She had a history of several abdominal surgeries. All abdominal procedures were performed under general anesthesia with epidural anesthesia in her thoracolumbar spine. Magnetic resonance imaging of her lumbar spine demonstrated an intradural extramedullary spinal cord tumor at the T12–L1 level. Because her symptoms deteriorated, the tumor excision was performed using microscopy. Histological examination of the specimens demonstrated that the cyst walls lined with stratified squamous keratinizing epithelium surrounded by the outer layer of collagenous tissue with the absence of skin adnexa. A diagnosis of epidermoid cysts was confirmed. Her MRI showed complete resection of the tumor, and there was no recurrence at 2-year follow-up.In this case report, epidermoid cells might be contaminated into the spinal canal during repetitive epidural anesthesia. The patient was successfully treated by complete resection, and there was no recurrence at 2-year follow-up with a good clinical outcome. However, long-term follow-up is required for a potential risk of tumor recurrence. Epidermoid cysts (ECs) are benign tumors, which are commonly observed in the intracranial region. Cruveilhier originally named ECs as “tumeurs perlées (pearly tumors)” in 1829, because they had a pearl-like appearance. Although ECs rarely occur in the intraspinal region accounting for less than 1% of all primary spinal cord tumors [Since Chiari firstly reported an intramedullary EC in 1883 , 5. It hA 67-year-old female presented with a low back pain and left sciatica. Although the patient had experienced occasional mild low back pain for several years, her low back pain markedly worsened 2 months before her first visit to our service. She also complained of newly developed left sciatica during this period, resulting in intermittent claudication. She had not noticed her urinary symptoms specifically, until she was asked at the examination, but she stated that those symptoms had started several months before her first visit. She denied any history of trauma, infectious diseases, or surgeries related to her spine. She had a history of several abdominal surgeries: cholecystectomy for gallstone and Hartmann’s procedure for rectal cancer 10 years ago and Miles’ surgery for anal cancer 9 years ago. She also had a mesh repair surgery for ventral hernia 5 years ago. All abdominal procedures were performed under general anesthesia with epidural anesthesia in her thoracolumbar spine. Radiographs of her lumbar spine showed some degenerative changes including decreased disc height and mild scoliotic changes. Magnetic resonance imaging (MRI) of her lumbar spine demonstrated an intradural extramedullary spinal cord tumor at the T12–L1 level (2.1 cm × 1.2 cm), and her spinal cord was markedly shifted anteriorly by the enlarged tumor Fig. . ComputeSpinal dysraphism or skin abnormalities were not observed in her lumbar/sacral region. On her neurological examination, she showed full strength and an unremarkable sensory deficit in bilateral upper extremities. The patient showed motor weakness 4/5 in the left lower extremity . Decreased sensation in the left L1 to L3 (6/10) and the left L4 to S1 (8/10) distribution was observed. The right lower extremity demonstrated full strength, and her sensation was intact in the right lower extremity. Although sphincter tone was not diminished and perianal sensation was intact, she was found to have urinary symptoms, such as urinary frequency and a feeling of residual urine. Reflexes were normal in the upper extremities bilaterally; however, hyperreflexia was observed in the patellar reflex bilaterally. Babinski sign was negative bilaterally. Because her symptoms deteriorated, a surgical treatment was performed.A laminectomy from T12 to L1 was performed, and the local dura mater was incised in the midline until the tumor was exposed Fig. . The tumSince the clinical symptoms of spinal ECs are variable and nonspecific with slow progression, the diagnosis can be delayed. In this case report, the patient visited our clinic 2 months after she noticed her low back pain and left sciatica; however, the majority of patients with spinal ECs usually have long disease duration due to their slow-growing nature . Roux etSpinal ECs are difficult to diagnose on clinical symptoms alone. Plain X-rays usually show normal or nonspecific findings, especially in acquired spinal ECs. Penisson-Besnier et al. firstly reported an intramedullary EC diagnosed by MRI . MRI is The etiologies of spinal ECs are thought to be both congenital and acquired. Acquired ECs were mostly reported in the region of the cauda equina (below the L1 level) , 11, becSurgical treatment is required when the patients develop neurological deficits. Complete excision of ECs is the essential of surgical treatment . HoweverHere, we report the first case of spinal EC, which occurred as an intradural and extramedullary spinal cord tumor attached to the conus medullaris after repetitive epidural anesthesia. Complete resection of the tumor’s capsule with emptying of the cyst material was achieved by microsurgery, and good clinical outcome was obtained. Long-term follow-up is required for a potential risk of the tumor recurrence.


Blunt scrotal injury represents a diagnostic dilemma for emergency physicians (EP). Consequently, point-of-care ultrasound (POCUS) has emerged as a tool for early investigation of the acute scrotum in the emergency department. We describe a case where an EP used scrotal POCUS to immediately visualize the loss of testicular contour and underlying heterogeneous parenchyma to rapidly make the diagnosis of testicular rupture in a young male presenting with scrotal trauma. The use of POCUS in this case expedited therapy, likely improving the patient’s outcome. To our knowledge, this is the first detailed description of testicular rupture diagnosed with POCUS by an EP Acute scrotal pain is a common complaint in the emergency department (ED).An 18-year-old male presented to the ED with left testicular pain and swelling following blunt scrotal trauma sustained during a wrestling match. The pain was immediate, but did not necessitate removal from play. Post-injury, the pain progressed, prompting presentation to our ED. The patient denied dysuria, hematuria, or any other symptoms. Past medical and surgical histories were unremarkable. Vital signs were normal. On physical examination, the left testis was tender to palpation, grossly swollen, and tense compared to the right. The remaining physical examination, including abdominal exam, was unremarkable.Scrotal POCUS was carried out by the EP using a Sonosite Edge system using an L13–6 linear transducer operating at 10 MHz. Sagittal and transverse images of the affected testis along with comparison views of the unaffected side were obtained. Analysis with color and spectral Doppler was performed. The left testis was enlarged with a grossly heterogeneous texture as seen in The patient was taken to the operating room for scrotal exploration. The left testis was found to be fractured, without viability of the lower segment. All nonviable tissue was removed, and bleeding was controlled. The upper pole was preserved, the tunica albuginea and vaginalis were re-approximated, and the scrotum was closed. The patient was admitted overnight for observation and discharged the following morning. At six-week follow-up, the patient had returned to normal activity.CPC-EM CapsuleWhat do we already know about this clinical entity?Injuries from blunt scrotal trauma are difficult to diagnose. Ultrasound is the preferred imaging modality to visualize scrotal pathologies and differentiate surgical and non-surgical diagnoses.What makes this presentation of disease reportable?To our knowledge, this is the first detailed description of testicular rupture diagnosed with point-of-care ultrasound (POCUS) by an emergency physician (EP).What is the major learning point?The findings of testicular rupture, including loss of tunica albuginea integrity and testicular heterogeneity can be identified sonographically by EPs in a rapid fashion. Rapid recognition of these findings can influence timing of consultation and expedite surgery, which should improve outcomes.How might this improve emergency medicine practice?Rapidly recognizing and/or excluding the findings of testicular rupture can decrease the need for consultative imaging, expedite definitive management and streamline patient care.Blunt trauma to the scrotum may result in a number of injuries, including hematocele, testicular fracture, and testicular rupture. Typically, these patients will present with non-specific symptoms, such as scrotal swelling and severe pain, which often make physical examination difficult to perform. Historically, blunt scrotal traumas were managed non-operatively, with surgical exploration reserved for cases with resulting complications. Higher rates of adverse outcomes  were associated with this delay in care.9US has emerged as the preferred modality for examining the acute scrotum. It can be rapidly employed to provide high quality imaging that can accurately guide clinical decisions and differentiate surgical from non-surgical pathologies. Scrotal POCUS, in the hands of an EP, provides an opportunity to reduce time to diagnosis and reduce dependence on sonographers and radiology departments, which may not be available at all hours. EPs with significant US experience, even in the absence of formal testicular US training, are highly accurate in diagnosing scrotal pathologies.1Ultrasonic imaging of the scrotum should be undertaken with a high-frequency (7–12MHz) linear transducer. The scrotum should be supported by a towel placed between adducted thighs with the penis displaced away from the scrotum. The hand holding the transducer should be supported against the thigh and a generous amount of gel should be used in order to provide adequate acoustic contact between the scrotum and transducer. Each testis should be visualized in multiple planes of the long and short axes with and without color Doppler. If one structure is abnormal, the contralateral side should be used to calibrate the grayscale and color Doppler gain settings for examination of the symptomatic testicle.9Using ultrasonography, a normal post-pubertal testis should appear as a homogeneous ovoid structure of medium echogenicity measuring approximately 5×3×2cm. The tunica albuginea surrounds the testis and appears as a thin echogenic band. The epididymis should appear as a long, tapering tubular structure bordering the posterior aspect of the testis. Typically, the epididymis is isoechoic to hyperechoic and homogeneous.9With scrotal POCUS, hematocele, testicular fracture, and testicular rupture can be quickly identified and treated in patients reporting to the ED with blunt scrotal trauma. Hematoceles appear as extratesticular fluid collections of increased echogenicity acutely after traumas but become hypoechoic and septated over time.7The accuracy of US for the evaluation of these pathologies has been established through retrospective studies that have compared sonographic findings against those found on subsequent scrotal exploration, the traditional gold standard for diagnostic and therapeutic evaluation.In summary, we report a case of testicular rupture diagnosed by an EP using POCUS. To our knowledge, this diagnosis identified with POCUS has not been detailed in the emergency medicine literature. Our case demonstrates an opportunity for EPs to expedite the diagnosis of acute scrotal pathologies, and reduce dependence on radiology departments, which may not be readily available. Furthermore, use of routine scrotal POCUS may be used to stratify patients in need of emergent urologic consultation, help to reduce time to surgical intervention, and reduce the need for additional evaluation, thus improving resource use and patient outcomes.Supplemental VideoThis brief, narrated video reviews the findings in this case which led to the immediate diagnosis of testicular rupture. The findings include enlarged testicle with irregular, heterogeneous echogenicity, as well as loss of integrity of the tunica albuginea.


Ambiguous genitalia is an uncommon situation that happens between 1 and 2 per every 1000 live births and falls under the umbrella diagnosis of disorders of sexual development.  In this article, we report a case of male pseudohermaphroditism with ambiguous genitalia. The proband was a 12 yr old girl without any uterus or ovarian tissues. Karyotype of the case is 46, XY. Genes involved in sexual differentiation such as AR, SRD5A2, LH, LHR, FSH, 17 B HSD and SRY genes were sequenced in both directions. No mutations were found in these genes either. It seems advisable to be cautious in similar cases, and revise protocol for tracing the genes involved in the patients. Ambiguous genitalia is a birth fault where the outer genitals do not have the classic aspect of either a girl or a boy. The disturbances of sexual development 46,XY are scarce, and the most frequent etiology is androgen insensitivity , 2. “CopClinical DescriptionThe patient was a 12-years old girl of non-consanguineous Iranian parents with an ambiguous genitalia and 46,XY karyotype. Gestation was normal and at birth the patient had no dysmorphic feature. No history of birth defects, and congenital malformations were seen in the family. This patient had normal female external genitalia at birth. During pubertal development secondary male sexual characteristics were noticed. Her histopathology results showed a small testis covered by tunica albuginea and fibro-connective tissue measuring 1.8x0.9x0.9 cm, epididymis 1.3x1.2x0.7 cm and vasodefrens measuring 4.5 cm in length and 1 cm in diameter.Sections showed fibrous tissue and dilated congested vessels. Seminiferous tubules with the presence of Sertoli cells and hyalinization of them (immature testis) were seen. No uterus or ovarian tissue was identified. Serum FSH, and LH were in normal range but her serum testosterone level was high (1.2 ng/ml). Sampling for this project was down in Iran but cytogenetic and sequencing was performed in molecular medicine of Karolinska Institutet -a medical university in Sweden from April to June 2015.Cytogenetic and Molecular AnalysisSRY gene was designed using the web- based primer-blast program by standard selection criteria. Polymerase Chain Reaction (PCR) reactions were performed with  using the standard protocol. Genes involved in the process of sex determination such as AR, LH receptor, 5 alfa reductase (SRD5A2), 17 B HSD, and SRY gene, were sequenced and analyzed. GTG banded karyotype of the patient showed 46, XY . No chroEthical considerationThe patient has provided written consent for the case report to be published.There is still much to learn about DSD disturbances, this documented by the fact that, although the many processes of genitalia development is under intense research, many directions of sex determination and differentiation in the 46,XX and 46,XY individuals remain unknown . The terPersistent Müllerian duct syndrome is a type of internal male pseudohermaphroditism, which is developed via synthesis of Müllerian-inhibiting factor deficiencies. In such models, duct derivatives are now in 46XY males-this includes the upper vagina, uterus, and fallopian tubes. These persons with a hernia sac and bowel loops were found with duct derivatives also testes . “The paThis case had normal female external genitalia at birth. During pubertal development secondary male sexual characteristics were noticed. On investigation manifested no Mullerian structures. The dysgenetic testes were capable of producing an enough amount of anti-Mullerian hormone for Mullerian regression and testosterone for Wolffian development, although they were incapable of producing enough amount of testosterone for complete masculinisation of external genitalia. “One of the many challenges to understanding the natural history of DSD in people with 46,XY is that, these conditions are heterogenous in nature and only a portion of cases can be attributed to known genetic causes at this time” . In this research, we checked the most important genes as the standard protocol says for sex determination in similar cases, but no mutations were identified. So, it seems, due to limitations in the current protocol instructions to identify the causes of disease, it is necessary to review and upgrade diagnostic protocols of DSDs. New technologies for perception the multiple etiologies of 46,XY DSD, such as array sequencing, array comparative genomic hybridization, and next generation sequencing may result in a super understanding of the genetic causes of DSD for patients who possess a Y chromosome. Albeit, these improvements and developments have not yet been implemented clinically .


Only 5-10% of osteosarcomas arise from the craniofacial bones. We report the caseof a 14-year-old female patient who presented with headache and a mass that hadbeen growing in the left frontoparietal region for six months. We describe thefindings on conventional radiography, computed tomography, and magneticresonance imaging. Theosteoid and bone matrix of an osteosarcoma are composed of malignant connectivetissue cells. Most osteosarcomas are of unknown cause and can therefore bedesignated idiopathic or primary,2,4,6. Osteosarcomasrelated to known predisposing factors for malignancy, such as Paget’s disease,fibrous dysplasia, and external ionizing radiation, are referred to as secondaryosteosarcomas-7. The most common type ofosteosarcoma is conventional osteosarcoma, the incidence of which is highest inpatients in the second decade of life and the prevalence of which is slightly higheramong males than among females,2. In general, patients present withbone pain, occasionally accompanied by a mass or by soft tissue edema,6. The distinct radiological aspects of conventional osteosarcomaare bone marrow lesions, cortical bone destruction, an aggressive periostealreaction, a soft tissue mass, and a tumor matrix in the destructive lesion, as wellas within the soft tissue mass. Although the tumors can present as purely scleroticor purely osteolytic, most are a combination of the two. The borders are generallyindistinct, with a broad zone of transition. The bone destruction is infiltrative,with a "moth-eaten" appearance, and only rarely geographic. The most common forms ofperiosteal reaction seen in osteosarcomas are the spiculated (sunburst) type andCodman’s triangle, the laminated (onion-skin) type being less common,2,5-7.Osteosarcoma accounts for approximately 20% of all primary bone malignancies. Ofthose, only 5-10% are located in the craniofacial bones accounts and most of thoseare found in the maxilla or mandible. Less than 1% are found in the cranial vault,and there have been only a few reported cases of osteosarcoma at the base of theskull, reflecting the low frequency of that locationA 14-year-old female patient presented with a headache and a mass that had beengrowing in the cranial vault for six months. The mass was hardened and wasapproximately 10 cm in diameter. An X-ray of the skull showed a discretely scleroticlesion in the left parietal region, accompanied by an aggressive spiculatedperiosteal reaction . A compu,4,5. The prognosis is worse than is that of osteosarcoma involvingthe bones of the appendicular skeleton, osteosarcoma of the skull presenting a lowerresponse to aggressive multimodal therapy, with a five-year survival rate below10%-6.Here, we report a case of primary osteosarcoma of the skull, an extremely rareneoplasm at that location. Approximately 100 cases have been described in theliterature,6. The typical pattern of contrast uptake by thelesion is one of intense heterogeneous impregnation.In a clinical context in which the patient presents bone pain associated with a softtissue mass, with suspected aggressive lesion, the investigation should begin withconventional radiology, because it can demonstrate important aspects ofosteosarcoma, such as a periosteal reaction and cortical bone destruction. Computedtomography should be performed in order to characterize the dissemination of thetumor into the medullary cavity, as well as to provide images of the calcifiedneoplastic component, as well as of the involvement of the soft tissue and corticalbone, which are fundamental to the surgical planning. Magnetic resonance imaging hasbecome an effective modality for evaluating such tumors, particularly for mappingthe intraosseous/intracranial spread of the tumor, involvement of the soft tissues,and involvement of the neurovascular bundle. On T1-weighted images, the solid,non-mineralized portions of osteosarcoma generally appear as areas of low-to-mediumsignal intensity. On T2-weighted images, the tumor shows high signalintensity,5,7,8.The imaging-based differential diagnoses of osteosarcoma include hemangioma, which ischaracterized by multilocular lytic foci or coarse vertical striations; giant celltumor, which is typically well-circumscribed but can cause thinning of the corticalbone and usually manifests as a lytic lesion; atypical meningioma, in which there iscortical bone destruction and extradural involvement; and metastases from cancer ofthe thyroid or digestive tract, which can present as expansile lesions accompaniedby bone destruction and invading the soft tissues. Despite their similarity toosteosarcoma, all of those diseases are more commonly found in patients who areconsiderably older. The treatment involves complete surgical resection of thelesion, with tumor-free margins, as well as chemotherapy and radiotherapy-7.Because of the rarity of the osteosarcoma of the skull, it is difficult to make thedefinitive diagnosis on the basis of imaging data. However, the knowledge that it isa destructive lesion associated with a soft tissue mass and an aggressive periostealreaction can facilitate the diagnosis


Syringomyelia is a rare condition in which a cyst forms within your spinal cord, secondary syringomyelia following Tuberculousis is very rare. In the present paper, we report a case of tuberculous meningoencephalitis in a 30 year-old Morrocan male complicated six months after under anti-bacillary treatment by progressive quadriplegia due to granulomatous arachnoiditis. The spinal MRI showed an extensive complex syrinx within the cervical and thoracic cord same signal intensity as the cerebrospinal fluid. Arachnoiditis appeared to be the underlying mechanism in these late-onset cases. The availability of MRI has greatly improved our ability to both diagnose and follow these collections. Surgery is the only viable treatment for syringomyelia. The possibility of developing syringomyelia should be always considered in any patient with a history of central nervous system infection.


We describe an exceptional case of erythroid sarcoma in a pediatric patient as a growing orbital mass with no evidence of morphologic bone marrow involvement, who was finally diagnosed of pure erythroid sarcoma based on histopathology and flow cytometry criteria. We discuss the contribution of standardized eight-color flow cytometry as a rapid and reliable diagnostic method. The use of normal bone marrow databases allowed us to identify small aberrant populations in bone marrow and later confirm the diagnosis in the neoplastic tissue. Myeloid sarcoma (MS), previously known as granulocytic sarcoma or chloroma, is an uncommon myeloid neoplasm consisting of a malignant proliferation derived from myeloid hematopoietic blasts occurring in an anatomical site other than bone marrow (BM) . MS may Morphologically, the most common forms of MS are proliferations of immature cells, including myeloblasts, monoblasts, and promonocytes, or less commonly promyelocytes, that efface the normal tissue architecture and are identifiable because of their morphological and immunohistochemical (IHQ) characteristics . The IHQWe present the case of a rapidly growing orbital mass in a pediatric female patient with no evidence of morphologic bone marrow involvement, who was finally diagnosed with pure erythroid sarcoma based on histopathology and flow cytometry (FC) criteria. Although the diagnosis of this pathology is usually difficult given the extreme rarity and peculiarity of its presentation, the information obtained by the eight-color standardized FC was key establishing a rapid diagnosis and allowed the prompt start of specific treatment.A 17-month-old female was admitted to our institution in November 2016. She presented a previous history of nonspecific fever, irritability, and swollen red right eye since September 2016, whereby she was initially diagnosed with cellulitis. Empirical treatment with antibiotics and corticosteroids was started, without improvement.Physical examination showed the presence of an evident orbital mass with significant ocular proptosis and swollen right face without any other significant findings. Cranial Magnetic Resonance showed an invasive and destructive soft tissue mass (measured 54 × 40 × 15 mm) involving the right orbit, implicating upper jawbone and extending forward to the intraconal space. This mass showed no uniform texture, being mostly iso-intense during T1 and of mildly hyper intense signal during T2 with moderate enhancement after contrast administration . Initial+, Histocompatibility Leukocyte Antigen-D Related (HLADR+), CD71+, CD45−, CD105a+, CD117 weak with abnormal size and complexity, and aberrant CD33 weak co-expression)  A. These + weak, CD45+, CD105a+, CD71+, CD117+ weak, CD34+ weak, and CD33+ weak in 100% of the cells. To confirm the erythroid nature of the process, glycophorin A expression was studied (not included in the Euroflow protocol) and intense expression of this marker was observed  to obtain a single-cell suspension following the manufacturer’s instructions. Standardized eight-color FC demonstrated a homogeneous population of erythroid lineage, with the expression of CD36observed B. Based observed .++, CD45+ weak, CD105a+, CD71+, CD117+, CD34+ weak, CD33+ weak). After consolidation treatment, resolution of the soft tissue mass component was achieved.Induction treatment was started according to Spanish Association of Pediatric Hematology and Oncology (SEHOP) AML protocol 2007. After induction, morphological absence of disease was evidenced, with a remarkable decrease in erythroid mass. However, minimal residual disease was detected by high sensitivity standardized eight-color FC, showing the presence of 0.13% erythroid blasts with a similar phenotype to diagnosis . A cytogenetic study was performed and showed the presence of a long arm isochromosome of chromosome 7 as well as a balanced translocation between the long arm of chromosome 11 and the long arm of chromosome 20. The obtained karyotype was 46, XX, i (7) (q10), t  .Unfortunately, in March 2017, just before admission to perform allogenic transplantation of hematopoietic stem cell, the ocular lesion progressed with anemia, thrombopenia, and an increase of LDH. The BM aspirate showed a massive infiltration of 90% erythroid blasts  is a very rare subtype of MS defined as an extramedullar collection of exclusively immature erythroid cells, rarely documented ,11,12. OThe few cases have an aggressive evolution as well as certain tropism by the ocular cavity, as happened in our patient. However, this localization is not exclusive. The first documented case was a 3-month-old female with acute erythroid leukemia presenting bilateral ovarian involvement . The secThe major differential diagnosis of myeloid sarcoma is a malignant lymphoma and other solid neoplasms, particularly those that present a diffuse hypercellular histological pattern and poorly differentiated morphology. Given the heterogeneity and rarity of all of these proliferations, it is difficult to establish diagnostic algorithms applicable in the differential diagnosis of these solid neoplasms and many cases are frequently reclassified after an imprecise initial diagnosis .The extraordinary rarity of this pathology and the difficulty of identifying its nature through conventional histopathological and IHQ studies may imply a significant delay in the diagnosis and complicates the patient’s expectations of recovery. In this sense, the BM study using FC and molecular biology are promising diagnostic tools.FC is a fast and reliable method that should be considered in the diagnosis and quickly provides reproducible results in 2 h or less. Nevertheless, advances in the knowledge of the immunophenotype of erythroid acute neoplasms have been lower than in other leukemias due to the relative scarcity of monoclonal antibodies to identify aberrant patterns as well as the non-application of routinely standardized procedures.EuroFlow Consortium provides commonly accepted diagnostic algorithms, fully standardized laboratory procedures, and antibody panels in order to achieve maximally comparable results ,9. The sUsing this methodology, it is very important to carefully investigate the presence of atypical erythroid cellularity, even in apparently normal bone marrow specimens, since varying amounts of atypical erythroid cells can be recognized. On the other hand, the identification of the erythroid lineage in tumor biopsy using eight-color standardized FC has not been previously documented, and is also indispensable for establishing correspondence with BM atypical proliferation and confirming the erythroid nature of a tumor.In conclusion, the standardized eight-color FC quickly provides reproducible results, and is emerging as a tool to quickly and reliably diagnose this type of malignancy.


Osteoblastoma is a rare and benign tumor which requires early diagnosis and surgical excision. Scoliosis is a common presentation following osteoblastoma. It is considered due to pain-provoked muscle spasm on the side of the lesion. Few researches about osteoblastoma combined with severe scoliosis have been reported.A 14-year-old girl presents with progressive scoliosis deformity for 3 years, with gradually appeared low back pain and numbness of left leg. Radiographic results showed osteoblastic mass at the left side of L3-L4 with severe scoliosis deformity, pelvic obliquity and spinal imbalance. The patient underwent posterior tumor excision, spinal decompression, scoliosis correction, spinal fusion with auto-graft and instrumentation from T8-S1. The mass was found to be osteoblastoma. The patient had a full neurological recovery with no aggravate of scoliosis or spinal imbalance during the follow-up.This case emphasizes the importance of early diagnosis and surgical treatment of osteoblastoma. Early surgical excision will not only prevent neurological deficit but also the progression of scoliosis. Atypical scoliosis presence without pain requires carefully examination of whether a tumor exists. Osteoblastoma is a rare and benign tumor characterized by increased osteoid tissue formation surrounded by vascular fibrous stroma and perilesional sclerosis. Spine location is observed in 36% of the tumor and mostly in the posterior elements . It is uA 14-year-old girl was referred to our department whose chief complaint was progressive scoliosis deformity for 3 years, low back pain for 2 year and numbness in the left leg for 6 months. Her parents had initially noticed that her body leaned slightly to left while ambulating 3 years ago. No further attention was given until progression with low back pain. The local hospital diagnosed idiopathic scoliosis with a recommendation for brace treatment, but the torso imbalance developed along with left leg numbness.Physical examination of the patient showed right-side spinal scoliosis with a razor back of 2.5 cm high when bending down. The patient could walk with no intermittent claudication. The right-side shoulder is 1 cm higher than the left-side. The left-side ilium is higher than the right-side, and the absolute length of the bilateral lower extremity is equal. A slight percussion pain was found on the left side of the waist. Hypoesthesia was found in the anterolateral thigh and extensor muscle strength of the knee that decreased to Grade 4. No hypoesthesia region or weaken myodynamia of other part was detected. The straight-leg raising test negative. No Babinski’s sign was noted bilaterally.The laboratory studies showed no increase in tumor markers and only a slight increase in B-ALP. Posterior-anterior radiograph showed a 71 ° coronal plane deformity with an apex in the intervertebral space of L1 and L2. No significant reduction was found in side-bending image. The thoracic curve can be compensated with a Cobb’s angle of 43°. There is a 10.3 cm distant from the mid-point of superior border of sacrum to the C7 plumb line. This prompted severe coronal imbalance. Lateral radiograph showed sagittal imbalance (6.3 cm) and a 44° thoracolumbar kyphosis (T10-L2) . A 6-month follow-up radiographic image showed no aggravate of scoliosis or spinal imbalance Fig. .Fig. 5PoOsteoblastoma was first proposed by Lichtenstein and Jaffe in 1956, which was then described as a category of osteoid- and bone-forming benign tumor other than osteoid osteoma . 36% of Surgical resection is the most effective treatment for osteoblastoma in the spine –10. In BScoliosis is a common presentation following osteoblastoma. Saifuddin’s literature review showed that 293 of 465 cases (63%) had scoliosis. They confirmed that asymmetric location of osteoblastoma in the thoracolumbar spine would be most likely to cause scoliosis [To the best of our knowledge, early Lenke V idiopathic scoliosis may not present such severe torso imbalance and rigid scoliosis. We do not know if the tumor was present when scoliosis was diagnosed due to the lack of examination results from the local hospital. Todd et al. reported a case of thoracic spinal osteoblastoma occurring 9-years after idiopathic scoliosis correction surgery. However, tumor lesion of this patient is 4-segments higher than the apex . In SaifThis case emphasizes the importance of early diagnosis and surgical treatment of osteoblastoma. Early surgical excision will not only prevent neurological deficit but also the progression of scoliosis. Atypical scoliosis presence without pain requires carefully examination of whether a tumor exists.


Calcifying pseudoneoplasms of the neuraxis (CAPNONs) are extremely rare tumors that are frequently misdiagnosed and overlooked by clinicians. To date, only 40 intracranial lesions have been reported, and in all instances, they were found as a solitary calcified mass. To our knowledge, the current case report is the first to illustrate the development of multiple intraaxial CAPNONs and shed more light on the origin of these lesions.We discuss the case of a 67-year-old woman who presented with a six-year history of recurrent seizures. Magnetic resonance imaging revealed two similar heterogeneous intracranial masses in the ventral midbrain and left frontal white matter with indications of more aggressive behavior in the supratentorial tumor. The lesion was resected, and histopathological analysis showed tissue containing nodules of chondromyxoid material with a coarsely fibrillar matrix and focal alveolar pattern. Palisading cells were noted around the edges as well as dystrophic calcifications and osseous metaplasia, consistent with CAPNON. Interestingly, the patient had a previous history of multiple brain abscesses and a mycotic aneurysm. At her four-month follow-up visit, the patient remained seizure-free and there were no indications of residual tumor or recurrence.In contrast to previous reports, intracranial CAPNONs may manifest as multiple lesions and clinicians should include these tumors in the differential diagnosis of intra-axial calcified masses. The previous history of brain abscesses raises the suspicion of an abnormal proliferative process following an insult to the brain tissue as the underlying origin of these lesions. Calcifying pseudoneoplasms of the neuraxis (CAPNON), also known as fibro-osseous lesions, are extremely rare tumors that can be located from the extremities to the central nervous system . DespiteA 67-year-old woman presented with a six-year history of recurrent focal seizures with impaired consciousness suggesting a left hemispheric origin. The patient had a recent increase in the frequency of seizures with poor response to medical treatment. Magnetic resonance imaging (MRI) at the onset of seizures revealed multiple heterogeneous intracranial lesions located in the ventral midbrain and in the left frontal white matter. The MRI features of the lesions were fairly similar, demonstrating hypointensity on T1- and T2-weighted images. The lesion in the left frontal lobe showed moderate contrast enhancement as well as a mild adjacent hyperintense signal on T2-weighted images. Minimal to no contrast enhancement was observed around the lesion in the brainstem  demonstrated dense calcifications in the supratentorial lesion and peripheral calcifications in the brainstem mass Figure .Of note, the patient had a remote history of infection with multiple brain abscesses and a mycotic aneurysm of the right posterior cerebral artery. Reportedly, the infection occurred 30 years before the onset of seizures and was attributed to rheumatic heart disease and infective endocarditis. Her physical examination showed a partial right third cranial nerve palsy, which was the sequelae of her aneurysmal subarachnoid hemorrhage, and a new right pronator drift. An MRI scan performed six months later showed interval growth of the left frontal mass and an increased area of T2-weighted hyperintensity in the adjacent brain . After reviewing the management options, the patient elected to proceed with left frontal tumor resection, as it was likely responsible for her worsening symptoms. Informed patient consent was obtained for her treatment.The lesion was approached and resected through a left frontal craniotomy. The arachnoid was noticeably thickened over the mass. The lesion was extremely firm and near entirely calcified with an irregular, tanned/pink appearance as well as minimal internal vascularity. The lesion was dissected from the surrounding brain tissue and “en bloc” resection was achieved with no complications. Postoperative MRI demonstrated complete resection.The specimen consisted of heterogeneous tissue containing nodules of chondromyxoid material with palisading spindle-to-epithelioid cells at the edges. The chondromyxoid matrix was coarsely fibrillar and had a focal alveolar pattern with dystrophic calcifications and osseous metaplasia. Both immature and mature bone with trabeculae and lacunae that contained osteocytes were present. There were sparse chronic inflammatory cells in the fibrovascular stroma between chondromyxoid nodules Figure .Immunohistochemical staining was performed and revealed negative staining for S-100 protein, GFAP, and minimal MIB-1 labeling. The trichrome stain revealed a mesenchymal origin.The clinical course following surgery was unremarkable. The patient was discharged home on postoperative day 5 with no changes in her neurological exam. At her four-month follow-up visit, the patient was clinically stable and remained seizure-free. Follow-up MRI showed postoperative changes along the resection bed without evidence of residual or recurrent tumor. The brainstem lesion showed no change or growth.The first intracranial calcifying pseudoneoplasm was reported in 1978 by Rhodes and Davis when an unusual fibro-osseous component was identified in intracranial masses at autopsy and was described as an aberrant type of osseous metaplasia . Since tThese lesions are typically extra-axial and occur both infra- and supratentorially , 7. The The distinctive histological features of CAPNONs include 1) a chondromyxoid matrix in a nodular pattern; 2) palisading spindle to epithelioid cells; 3) variable amounts of fibrous stroma; 4) osseous structures containing birefringent lamellar bone, calcifications, psammoma bodies; and 5) foreign-body reaction with giant cells . HoweverThe prognosis following complete resection is typically favorable, and complications tend to arise from adhesions to the arachnoid overlying the cranial nerves, blood vessels, or brainstem, rendering complete excision unsafe . In a seIntracranial calcifying pseudoneoplasms are rare and can manifest as extra-axial or intra-axial masses. To our knowledge, the current report is the first to demonstrate the development of multiple intracranial CAPNONs and sheds more light on the origin of these lesions. Although the lack of specific radiographic features makes their diagnosis difficult, CAPNONs should be included in the differential diagnosis of single or multiple intracranial calcified lesions with hypointensity on T1- and T2-weighted MR images and minimal contrast enhancement. The pathogenesis of CAPNONs remains undefined, but the distinctive aspects presented in this case suggest that the eliciting factor is likely an abnormal reaction of the brain following an insult.


Infected schwannoma has been reported, this being one of the four cases published in the literature. Infected schwannoma has proven to be a tough diagnostic challenge to the treating tumor surgeon, mimicking infectious entities and most essentially, a malignant tumor.Streptococcus was detected by culture. After the confirmation of infected schwannoma, enucleation of the tumor was performed.The authors report the case of a 64-year-old male with a soft tissue mass in his right gluteal area that presented initially with right leg pain, then later with signs of inflammation on the tumor area. Magnetic resonance imaging (MRI), computed tomography (CT), and thallium-201 scintigraphy studies confirm the presence of soft tissue mass which had continuity with sciatic nerve, with subsequent serial MRI findings suggesting tumor enlargement with cystic degeneration. Increased level of C-reactive protein (CRP) was observed before surgery. During an open biopsy upon tissue sampling, exudates with necrotic tissue were seen. Increased level of CRP and necrotic change suggested the possibility of malignant tumor. Histopathological diagnosis was schwannoma, and group B The report concludes that establishment of a benign pathology is essential when presented with similar clinical findings prior to definitive enucleation of an infected schwannoma. Schwannoma is an unusual benign tumor arising from the nerve sheath comprising of Schwann cells . Common Schwannomas mostly arise adjacent to a peripheral, or less commonly an intracranial nerve . These tThese benign tumors are occasionally glandular in structure . But hisInfected schwannoma does happen but is extremely rare. After a thorough review and to the best of the authors’ knowledge, this is one of the only four cases of infected schwannoma reported in the literature. The other three were cases of infected gastric , retropeThe authors report the case of a 64-year-old male who received steroid pulse therapy at another hospital and presented with an infected schwannoma in his right gluteal area. This report underscores the importance of establishing a benign tumor diagnosis by final histopathology prior to definitive enucleation of the mass when presented with findings suggestive of an infected schwannoma.A 64-year-old male had suffered from right leg pain accompanying an enlarging mass in his right gluteal area. The patient received steroid pulse therapy because of sudden deafness at another hospital at 5 months prior to consult. Right leg pain appeared 1 month later. There was no neurological deficit.A firm, soft tissue mass with poor mobility was palpated deep to the area of the right gluteal crease. Before surgery, Tinel’s sign was positive. Computed tomography (CT) showed no phleboliths or foci of calcification within the tumor, and thallium-201 scintigraphy showed accumulation on the area of the mass see Fig. . ElevateStreptococcus, and postoperative antibiotics were shifted to intravenous cefazolin, at a dose 500 mg every 8 hours. Taken together, the mass was diagnosed as infected schwannoma.Before excision of the tumor, an open biopsy was performed. During the open biopsy, exudate with necrotic tissue was seen see Fig. . A part After the confirmation of the existence of bacteria and pathological diagnosis released 5 days following the open biopsy, enucleation was imperative because CRP and WBC counts were persistently elevated  in acute bacterial infection, with the influx of PMNs unquestionably observed at the initial phase and their accumulation peaking by the 2nd day following onset of acute bacterial infection . In the ammation . Rajakarammation  have fouStreptococcus (GBS) disease has emerged as a major cause of invasive infections in adults for the past three decades [p < 0.001), mostly among men aged more than 40 years (p < 0.002). One study identified increasing age as being associated with risk even after adjusting for specific chronic illnesses which are more frequent among older persons [Group B  decades –49. In a decades , the inc persons , and the persons .Taken altogether, the patient in the present case, with a history of steroid pulse therapy and possibly of steroid-induced immunosuppression, could have contracted the infection from a silent GBS bacteremia. When presented with an infected tumor in an adult allegedly immunocompromised, it is prudent that GBS disease must be ruled out. Cefdinir, an oral cephalosporin relatively new in the market, has been shown to have broad-spectrum activity including against GBS . The autThe authors conclude that establishment of a benign tumor diagnosis by final histopathological analysis is essential when presented with similar clinical findings prior to definitive tumor enucleation of an infected schwannoma.


G‐CSF)‐producing esophageal squamous cell carcinoma (ESCC) is rare. Esophageal cancer is a highly aggressive disease and often spreads hematogenously; however, choroidal metastases are rarely seen. This report detailed an extremely rare case of G‐CSF‐producing ESCC with choroidal metastasis.Granulocyte colony‐stimulating factor ( Granulocyte colony‐stimulating factor (G‐CSF) is a cytokine that contributes to neutrophil production. G‐CSF‐producing tumors have been reported in a variety of organs, such as the lungs, gallbladder, liver, and pancreas A 50‐year‐old Japanese man was referred to our hospital with a chief complaint of dysphagia. The patient reported a 10‐kg weight loss in a few months. The patient also complained of blurred vision, oppressed feeling, and hyperemia in the left eye. The patient had no history of smoking and drinking alcohol. Moreover, the patient had no personal or family history of illness. Physical examination revealed no swelling of superficial lymph nodes. Laboratory data showed an increased white blood cell (WBC) count, 27,100/μL, with 85.0% neutrophils, and increased C‐reactive protein concentration, 12.3 mg/dL. All tumor markers, including carcinoembryonic antigen, fragment of cytokeratin subunit 19, and squamous cell carcinoma‐associated antigen, were within the normal ranges. The serum G‐CSF level was elevated by 60.2 pg/mL . Bone marrow aspiration revealed no possibility of haematological neoplasms.Contrast‐enhanced computed tomography (CT) from the neck to pelvis revealed thickened wall of the esophagus and several enlarged mediastinal/abdominal lymph nodes. An upper gastrointestinal endoscopy showed a protruding esophageal tumor 30 cm from the incisors extended to the gastric cardia Fig. . The speOphthalmologic examination revealed that corrected visual acuity and intraocular pressure were normal in the left eye. Fundus examination of the left eye revealed a well‐circumscribed yellowish‐white choroidal mass at the inner upper side of the posterior pole Fig. . CT of t2 administered by rapid intravenous infusion on day 1 and 5‐fluorouracil at 700 mg/m2 administered by continuous intravenous infusion on days 1 through 5, which was performed with 60 Gy concurrent irradiation in 30 fractions of 2 Gy. Two courses of chemotherapy were performed, separated by a 4‐week interval. A total dose of 30 Gy was also given in 10 fractions of 3 Gy to the left retina, including the right retina for the prevention of metastasis. The treatment was well tolerated, with no grade 3 adverse events.The patient was diagnosed with G‐CSF‐producing ESCC T3N2M1, stage IV ; therefore, radical resection was not recommended. Chemotherapy consisted of cisplatin at 70 mg/mAfter chemoradiation therapy, the primary tumor of the esophagus decreased, and food intake increased. Moreover, CT showed that thickness at the posterior pole of the left eye became ambiguous Fig. . HoweverDuring the treatment, the WBC count and serum G‐CSF level shifted as shown in Fig. Granulocyte colony‐stimulating factor is a cytokine that contributes to production of neutrophils and inducing leukocytosis. G‐CSF is mainly produced endogenously by monocytes, macrophages, mesothelial cells, fibroblasts, endothelial cells, and bone marrow stromal cells Intraocular metastases of malignant tumors are rare. The choroid is the most common intraocular site for the development of metastases due to the vascular architecture of the choroid and local microenvironmental factors Previous reports showed that radical tumor resection induced a rapid decrease in both WBC count and serum G‐CSF levels Table This report has highlighted an extremely rare case of G‐CSF‐producing ESCC with choroidal metastasis. In the case of an ESCC patient with leukocytosis, the possibility of G‐CSF‐producing cancer should be considered. Moreover, when ESCC patients display ophthalmologic symptoms, we should be aware of potential choroidal metastasis.SF: designed the study and drafted the manuscript. HM: performed the ophthalmic examination. TW and YO: performed the histopathological examination. HH: performed the haematological examination. KO: made the radiation treatment plan. YF, KI, KK, HI, MT, MY, and MI: participated in the manuscript revision process. All authors read and approved the final manuscript.None declared.Written informed consent was obtained from the patient to allow for the publication of this case report and any accompanying images.


Pelvic Ewing sarcoma is associated with a worse prognosis. Thromboembolic events are relatively common in pediatric patients with cancers including sarcomas. We have presented a case of Ewing sarcoma arising from the left iliac bone with tumor thrombus of inferior vena cava (IVC) which was obtained complete response by both chemotherapy and irradiation. Magnetic resonance imaging (MRI) scan demonstrated that the tumor arising from the left iliac bone extended into the left side of sacral bone, suggesting the difficulty of surgical resection. Computed tomography (CT) revealed the existence of the tumor thrombus of IVC. We performed irradiation (31.2 Gy) and chemotherapy . The tumor was controlled successfully, and the tumor thrombus of IVC has completely vanished. Four years after the treatment, coin lesion in the left upper lung appeared. Suspected of metastasis, segmental resection of the left upper lung was performed. Fourteen years after the surgery, the patient has been remained free of recurrence. It is clinically significant for surgeons to treat pelvic Ewing sarcoma with tumor thrombus. Ewing sarcoma family of tumors (ESFTs) are rare, but high-grade malignant tumors of unclear etiology which mainly occurs in childhood . ParticuA 14-year-old male with continuous left low back pain, suspected of malignancy, referred to our hospital. He had been aware of left low back pain for the last 3 months. A clinical examination revealed spontaneous pain of his left low back and the paresthesia of left S1, 2 lesion. Plain radiograph of the pelvic bone showed osteolytic changes of the left iliac bone . CT and The combination of surgery, chemotherapy, and irradiation for pelvic Ewing sarcoma has only resulted in about 40% five-year survival rate . We haveIt is clinically very important that the pelvic Ewing sarcoma with the tumor thrombus of IVC could be controlled successfully by irradiation and chemotherapy without any surgical procedure. Our case report should be taken into consideration for surgeons to decide the treatment of pelvic Ewing sarcomas with tumor thrombus.


Asymptomatic diaphragmatic hernia is generally thought to be rare among adults. We present two different types of asymptomatic diaphragmatic hernia diagnosed with computed tomography (CT) and discuss treatment strategies.Case 1: A 37-year-old woman was diagnosed with catamenial pneumothorax in the right diaphragm. Partial resection of the diaphragm and lung was performed using a linear stapler. She was asymptomatic after the operation and gave birth 2 years later. After delivery, she experienced recurrent pneumothorax, and CT revealed a right diaphragmatic defect with herniation of a part of the liver into the thorax. An iatrogenic diaphragmatic hernia was diagnosed. There has been no change in the size of the hernia and no symptoms due to the diaphragmatic hernia for more than 3 years after it was diagnosed.Case 2: A 75-year-old woman was previously diagnosed with rectal cancer and had undergone surgery after chemoradiotherapy. One year after surgery, herniation of a 3 × 1.3-cm section of retroperitoneal fat tissue into the left thoracic cavity was observed incidentally at a follow-up CT and was diagnosed as an adult Bochdalek hernia (BH). We reviewed the patient’s past CT findings and confirmed that the same finding had been present since the first scan. A wait-and-see approach was chosen because there had been no change in the size of hernia, there were no symptoms, the patient was elderly, and there was a high risk of recurrence of the rectal cancer. She has had no symptoms to date, and careful follow-up has been performed.There are few reports of asymptomatic adult diaphragmatic hernia. Although symptomatic diaphragmatic hernia is generally treated surgically, there are cases in which a wait-and-see approach has been applied, such as our asymptomatic cases. There are two main types of diaphragmatic hernia (DH), namely, congenital and acquired . AlthougHere, we present two cases of asymptomatic adult DH diagnosed with computed tomography (CT).Case 1: A 37-year-old woman was diagnosed with catamenial pneumothorax in the right diaphragm. Video-assisted partial resection of the diaphragm and lung was performed using two cartridges of endo staplers (45-3.5). She was asymptomatic after the operation and gave birth 2 years after the operation. After delivery, she complained of chest pain, and CT revealed a recurrent pneumothorax. CT also revealed a right diaphragmatic defect with herniation of a part of the liver (6.6 × 4.5 cm) into the thorax  was incidentally observed. She was diagnosed with rectal cancer with invasion into the pelvic cavity at 73 years of age and underwent low anterior resection of the rectum after preoperative chemoradiotherapy. She simultaneously underwent colostomy and resection of the internal iliac vessels, piriformis, coccygeus muscle, and the fifth sacrum. She underwent postoperative adjuvant chemotherapy and had an uneventful course. After 1 year, a follow-up CT scan revealed a defect in the left diaphragm and herniation of the retroperitoneal fat tissue (3 × 1.3 cm) into the left thorax , namely, congenital and acquired. Most surgeons associate DH with a disease requiring emergency surgical treatment. Recently, minimally invasive surgery, including thoracoscopic and laparoscopic surgery, has gradually been used to repair adult DH , 3. WithGenerally, acquired DH is rare, most of the cases are traumatic in origin, and the majority occurs on the left side , 6. SurgThe diaphragm develops as part of fetal development, and its development rarely occurs improperly. Some have reported that defects can be caused by certain gene mutations, but the etiology is still unknown , 13. WheMullin et al. previously reviewed 13,138 cases examined with CT, and 22 cases of BH (0.17 %) were found in 1 year; they thus concluded that BH is rare . HoweverWe described two cases of asymptomatic DH that were incidentally found during postoperative CT examination and provided a literature review. Our cases help to illustrate the differences between cases requiring surgery and those for which the wait-and-see approach may be preferable.


Ameloblastic fibrodentinoma is a benign odontogenic tumor belonging to the category of "odontogenic epithelium with odontogenic ectomesenchyme" along with recognition of induction in the form of dentin in atypical or dysplastic forms. The biological behaviour of ameloblastic fibrodentinoma is not very different from ameloblastic fibroma; hence, it is treated similarly by conservative procedures. It is important to understand the histopathogenesis of these rare tumors. Though rare, they are an independent entity awaiting recognition. Here, we report a case of amelobalstic fibrodentinoma in a 14-year-old female patient. Odontogenic tumors are a diverse set of tumors. The recent World Health Organization (WHO) 2017 classification on odontogenic tumors categorizes odontogenic tumors as epithelial, mesenchymal, and mixed [AFD is a benign odontogenic tumor that histomorphologically simulates an ameloblastic fibroma (AF) but can form dysplastic dentin. It predominantly presents as an intraosseous tumor in the gnathic skeleton, more common in the mandible than the maxilla. Though peripheral lesions are reported, they form miniscule numbers. It accounts for a small percentage of odontogenic tumors and only around 64 cases have been reported so far since its first description by Straith in 1936 -3. Here,A 14-year-old female child reported to the clinical department with a complaint of non-erupted posterior teeth. There was no associated pain or paraesthesia. Medical history and family history were noncontributory. On examination, a swelling 3 x 2.5 cm involving the right mandibular alveolus was seen in the region of 43 to 48. The swelling appeared sessile, soft to firm in consistency, was slightly erythematous on the superior surface, bore the indentation marks of the opposing maxillary teeth (Figure An orthopantomogram taken revealed a well-defined unilocular radiolucent lesion in relation to 44 to 48 region. The radiolucency extended from 44 to the retromolar area displacing and impacting 46, 47 and distally displacing developing 48 into the ramus of the mandible. The mandibular right second premolar showed no resorption or displacement but was placed completely in the tumor mass. The displaced 43 was also evident. The alveolar bone was destroyed in the 46 to 48 area, but the lower border of the mandible was guarded Figure . A proviThe biopsied tissue showed highly cellular myxoid stroma made of ectomesenchymal cells consisting of plump fibroblasts with ovoid hyperchromatic/vesicular nucleus with indistinct cell borders. Multiple branching strands of odontogenic epithelium simulating the dental lamina were noticed Figure . The epiA conservative surgical excision was carried out as the treatment for the lesion. The tumor was soft and friable; hence, it was removed in multiple bits. The tumor could be easily separated from the bony walls. The specimen was received in multiple bits and appeared off-white in color and also on cut section Figure .Histopathology of the excisional mass was similar to that of the incisional biopsy. The tumor showed the presence of a capsular structure that was incomplete. Multiple layers of collagen were seen as a capsule along with neurovascular bundles at certain areas. The tumor revealed odontogenic strands in the primitive myxoid ectomesenchymal stroma resembling an ameloblastic fibroma. Dentinoid-like material was evident Figure .Some new findings added enigma to the histopathological findings. Few areas of odontogenic follicles in the ectomesenchyme which had opened showed central cystic degeneration Figure . In the There was no evidence of enamel or enamel matrix formation even after extensive sectioning, ruling out the diagnosis of AFO. The other differentials considered were ameloblastoma. Though, few odontogenic follicles with peripheral columnar cells and central stellate reticulum like cells were evident, highly cellular ectomesenchyme was also present impressing that the tumor showed proliferation of both epithelial and mesenchymal components, whereas ameloblastoma shows proliferation of only epithelial component and even the conventional reversal of polarity of the ameloblast-like cells was also not seen, thus ruling out ameloblastoma .AFD is a rare neoplasm of odontogenic epithelium with immature ectomesenchyme and formation of dysplastic dentin. For a while it had also been named as a dentinoma -4.Controversy surrounds the categorization of AFDs. The continuum concept with AF, AFO, and odontomas proposed by Cahn and Blum remains disproved on the basis of age of occurrence, site of occurrence, histopathology, gender, and evidence studied in recurrent cases -5.The 2005 WHO classification included AFDs under AFs, but the periostin activity studied by Chau, et al. disapproves this categorization, as AFs did not show increase in periostin activity in the stroma with increasing maturity, and concluded that AFs should be regarded as a true neoplasm whereas AFO is more likely a developing odontoma -8. The rAFD is very similar to AF and AFO in biological certainty. Generally, a slow growing asymptomatic tumor, in some instances is associated with unerupted teeth and is commonly seen before the age of 20 years with a male predilection, the ratio being 2:1. Most of them are intraosseous tumors in the posterior mandibular region with about 30% showing cortical perforation. It has been observed that AFD in relation to permanent teeth are commonly seen in the posterior region, whereas those in relation to deciduous teeth are seen in the anterior region . RadioloThe present case though was seen in a 14-year-old female patient, presented in the mandibular right posterior quadrant involving unerupted 46, 47, and 48 as a unilocular, well-defined radiolucency of approximately 3 x 2 cm, but no such opacity nor cortical perforation was seen. The alveolar ridge superiorly was completely destroyed allowing the tumor to present as a soft tissue mass extending on the alveolar ridge in the region of 43 to 48.The histopathology of AFD has great resemblance to AF, showing odontogenic epithelium in strands and small islands resembling dental lamina and enamel organ placed in a very cellular primitive ectomesenchyme resembling the dental papillae . Few odoThe present case also showed odontogenic epithelium similar to dental lamina. Small follicles or islands with central stellate reticulum-like cells with few follicles undergoing cystic degeneration were also evident. The ectomesenchyme in which the odontogenic epithelium was arranged was very cellular with plump fibroblasts. Multiple areas showed a halo-like hyalinization around the odontogenic epithelial follicles impressing upon induction, though not bearing resemblance to odontogenic hard tissue. Few areas showed enamel organ in early bell stage like differentiation with no evidence of enamel formation. Different forms of dentin such as osteodentin, dentinoid, and dentin undergoing globular mineralization were noted to be formed in the ectomesenchyme and also in relation to odontogenic epithelium at few areas. Osteodentin-like areas showed matrix with ectomesenchymal cells entrapped in the matrix. At certain places, osteodentin-like areas were bordered by odontogenic epithelium. Large areas with globular mineralization along with unmineralized predentin-like area were seen. Many areas showed non-tubular dentin entrapping cells in multiple lacunae in close relation to odontogenic epithelium resembling dentinoid as reported by Gardner and Farquhar . Most ofIt may be suggested that that all the tissue bearing resemblance to dentin should be considered as odontogenic/dentin induction in normal or dysplastic form. However, hyalinization needs to be categorized as non-dentinal or non-specific induction in the ecto-mesenchyme, the significance of which is not very clear or specific and might be an abortive effect too. More work needs to be done in detailing and recognition of inductive changes in odontogenic tissues and tumors.The present case clearly shows a clinical presentation that depicts a destructive tumor. The histopathology showcases a varied amount and variety of dysplastic dentin, validating true odontogenic induction in the tumor. Hence, we think that this case might add value to understand it as a true independent entity among odontogenic tumors.


Giant pheochromocytomas are rare silent entities that do not present with the classical symptoms commonly seen in catecholamine-secreting tumors. In many cases they are accidentally discovered. The algorithm to diagnose a pheochromocytoma consists of biochemical evaluation and imaging of a retroperitoneal mass. The female patient in this case report presented with a palpable abdominal mass and was cured with surgical resection. She suffered no recurrence or complications on follow-up. The left retroperitoneal mass measured 27 × 18 × 12 cm and weighed 3,315 grams. Biochemical, radiological, and pathological examinations confirmed the diagnosis of a pheochromocytoma. In this paper, we report on our experience treating this patient and provide a summary of all giant pheochromocytomas greater than 10 cm reported to date in English language medical journals. Our patient's giant cystic pheochromocytoma was the fourth heaviest and fifth largest maximal diameter identified using our literature search criteria. Additionally, this tumor had the largest maximal diameter of all histologically confirmed benign/low metastatic risk pheochromocytomas. Giant cystic pheochromocytomas are rare entities requiring clinical suspicion coupled with strategic diagnostic evaluation to confirm the diagnosis.  A pheochromocytoma (PCC) is a rare catecholamine-secreting tumor that originates from the chromaffin cells of the adrenal medulla. First described by Frankel  in 1886,A 50-year-old East Indian woman with a 6-month history of lower back pain, fatigue, and unintentional weight loss was referred to our surgical outpatient clinic upon detection of a large abdominal mass during an abdominal ultrasound. The mass was located in the left upper quadrant and thought to be possibly splenic or renal in origin. Physical examination revealed a large nontender lump arising from the left upper quadrant and crossing the midline. Initial laboratory investigations were unremarkable except for microcytic anemia with a mean corpuscular hemoglobin concentration (MCHC) of 7.3 g/dL. The patient had no history of hypertension, headache, palpitations, or excessive sweating and no family history of cancer.Computed tomography (CT) revealed a 23.2 × 17.6 × 13.6 cm mass with predominant areas of necrosis, punctate areas of calcification, and irregular contours superior to the left kidney  15-3, CA 19-9, and CA 125 were normal. The differential diagnosis included adrenocortical carcinoma, pheochromocytoma, myelolipoma, metastasis from an unidentified primary tumor, sarcoma, and lymphoma. Biochemical investigations were performed to exclude a functional adrenal mass, and the diagnosis of pheochromocytoma was made upon observation of elevated plasma free metanephrines, urine catecholamines, and their metabolites .Preoperatively, the patient was transfused with packed red blood cells to stable hemoglobin of 10 g/dL. Adequate catecholamine blockade was achieved, after medical consultation using the alpha adrenergic blocker, terazosin (Hytrin). An open left adrenalectomy was performed through a Chevron incision extending to the left flank. The mass was completely resected en bloc with the spleen, distal pancreas, left kidney, and a 2 cm area of the left hemidiaphragm . IntraopPathological examination of the surgical specimen revealed a circumscribed mass, surrounded by a variably thick fibrous capsule/pseudocapsule with relatively scantly attached fat, measuring 27 × 18 × 12 cm and weighing 3,315 g . ResiduaTo determine the clinicopathological features of giant PCCs, an extensive literature search of the PubMed/MEDLINE and Embase databases was conducted. Search terms included “giant pheochromocytoma”, “cystic giant pheochromocytoma”, “English language” and “case reports”. One case in which the tumor lacked dimensions but was found to be one of the heaviest PCCs recorded was included. Full texts were accessed to confirm eligibility for inclusion. A summary of the 36 final cases are presented in Pheochromocytomas are neoplasms that arise from the chromaffin cells of the sympathoadrenal system . Eighty-The malignant potential of a PCC cannot be determined preoperatively unless there is evidence of local invasion or metastases at the time of diagnosis. Previously, it was believed that size was a predictor of malignant potential; however, with numerous case reports of giant benign PCCs, size can no longer be used as a definitive indicator of aggressive disease . HistoloApproximately 20 to 30% of all PCCs are clinically silent . FactorsSurgical resection is the only curative option for these giant lesions. Laparoscopic adrenalectomy is considered safe and effective for tumors up to 12 cm in its greatest dimensions. However, in the realm of these giant PCCs, open en bloc resection is required. Intraoperative manipulation of these tumors is frequently associated with profound hypertension. However, early isolation of the tumor's venous drainage decreases the risk of intraoperative hypertensive crises . This muIn this report, we presented the case of a 50-year-old female with a giant PCC that was the fourth heaviest and had the fifth largest maximal diameter of all reported PCCs. Additionally, using our search criteria, the tumor had the largest maximal diameter of reported, histologically confirmed PCCs with a low risk of malignancy. Our patient, 3 months after resection of the tumor, remains stable and disease-free. Giant PCCs do not present with the classical symptoms associated with smaller PCCs and are usually associated with a lower risk of malignancy. Previously, larger size was believed to be an indicator of malignancy in these adrenal lesions; however, upon review of the 10 largest known PCCs, this belief was unsubstantiated.


In recent years, the number of cancer patients who undergo endoscopic surgery has been increasing, and port-site recurrence is becoming a more common complication. A 66-year-old woman underwent thoracoscopic left lower lobectomy with lymph node dissection for pT1aN0M0 adenocarcinoma of the lung. Six years after surgery, CT revealed a subscapular tumor measuring 3 cm at the site of the surgical port wound. Although port-site cancer recurrence was suspected, needle biopsy revealed that the tumor was an elastofibroma. During 6 months of follow-up, MRI revealed no further change, and it was concluded that development of the tumor at the subscapular port site had been merely coincidental. Elastofibromas are benign, connective tissue tumors that grow slowly. They frequently arise between the inferior corner of the scapula and the posterior chest wall. These tumors are infrequent, representing 1–2% of all primary tumors of the chest wall [In 2011, a 66-year-old housewife patient underwent thoracoscopic left lower lobectomy and lymph node dissection for lung cancer. In general, a 10-mm, 30-degree thoracoscope was placed through the intercostal space in the midaxillary line and a 2-cm incision was then made in the seventh intercostal space just beside the scapular angle. Subsequently, an access thoracotomy was always located in the fourth intercostal space in the anterior axillary line. The histopathological diagnosis was adenocarcinoma stage IA3 (pT1cN0M0) . At 6 years of follow-up as an outpatient, CT revealed a fusiform subscapular soft tissue heterogeneous solid mass with muscle-like density measuring 30 × 35 mm at the site of the subscapular surgical port wound (Fig. Elastofibroma is a rare, benign, slow-growing soft tissue tumor that was first defined by Jarvi and Saxen in 1961 . ElastofElastofibromas have characteristic imaging features on MRI and CT that allow definitive diagnosis. In most cases, CT shows poorer differentiation of tumor edges from the surrounding muscle planes in comparison with ultrasonography and MRI: the tumor appears as a semilunar mass of soft tissue with a density similar to that of the adjacent musculature, containing linear areas of low density secondary to fat. MRI is a non-invasive imaging modality with the greatest sensitivity and specificity for detection of elastofibromas because it demonstrates a circumscribed mass with an alternating pattern of fibrous and fatty tissues. On MRI, a typical feature of elastofibroma is that the interposed areas of decreased signal intensity also appear as low signal intensity on T2-weighted sequences –12. HistWe report a patient in whom CT demonstrated a subscapular pleural tumor at the site of the surgical port wound 6 years after thoracoscopic surgery for lung cancer. Retrospectively, CT showed that the tumor had grown slowly for two years, and therefore, we suspected port-site recurrence or a benign soft tissue tumor such as lipoma, hemangioma, or desmoid tumor. As we were particularly concerned about port-site recurrence, diagnostic needle biopsy was performed. Although MRI, which is considered the main imaging technique for diagnosis, clearly demonstrated a heterogeneous ill-defined lesion with an alternating pattern of fibrous and fatty tissue , in the We hope that the present case will add to the growing body of clinical experience, stimulate further investigation, and ultimately lead to better patient outcomes.The significance of differentiation elastofibroma developing at the subscapular port site after thoracoscopic surgery from port-site recurrence is studied.


Acute gastric volvulus (GV) is a rare disease with high mortality rate often associated with anatomic abnormalities. Thymoma is the most common neoplasm located in the anterior mediastinum. There is no reported relationship between thymoma and GV. Here we reported a case of thymoma with initial symptom of acute gastric volvulus.A 43-year-old man complained of postprandial abdominal pain, nausea and vomiting. Acute gastric volvulus was diagnosed by chest radiograph, upper digestive tract radiograph and CT scan; later type B3 thymoma was diagnosed by biopsy of mediastinal mass. We inferred that gastric volvulus was secondary to thymoma due to phrenic nerve palsy. The patient was treated with endoscopic de-rotation. Further radiotherapy and chemotherapy were given. During treatments, GV still occurred with less severity and a reduced frequency of approximately every three to four months.We report the first case of thymoma initially presented with acute GV. We suspect a pathological mechanism related to the phrenic nerve palsy. This case indicates that thymoma may present alongside rare acute GV. Gastric volvulus (GV) is a rare condition resulting from rotation of the stomach more than 180 degrees [GV is most often found in the elderly, with a peak incidence around 50 . Based oThough thymoma is the most common tumor of the anterior mediastinum, it is extremely rare . ApproxiHere we report a case of thymoma with initial symptom of acute gastric volvulus.A 43-year-old man was admitted to our hospital for acute postprandial abdominal pain, nausea and vomiting, without dyspnea and myasthenia. There was no history of trauma. Physical examination found that trachea shifted to the right, breathing sound over the left lower chest decreased, cardiac border shifted to the right and tenderness in the upper abdomen. A chest X-ray showed that mediastinum and trachea shifted to the right and the left diaphragm lifted. Upper digestive tract radiograph Fig.  showed lThe ultrasound guided biopsy of mediastinal mass was performed for this patient. The pathological findings revealed type B3 thymoma. According to the imaging examination he was diagnosed as Masaoka stage III thymoma. The thymoma of this patient presented with extensive pericardium, aorta arch and its branch infiltration, so it was considered as unresectable disease.The patient with technically unresectable disease received radiotherapy from December 2013. Gastric volvulus still attacked about once a month, with the incentives of cold, satiety or spicy food. Each time endoscopic de-rotation was needed to relieve the symptoms. In 10 months later, CT scan showed the mass in the anterior mediastinum shrank, but a new small lesion developed on the left pleura. CT-guided biopsy of the pleural mass was performed. The histopathology and immunohistochemistry proved thymoma metastasis. The chemotherapy of carboplatin combined with paclitaxel was given for four cycles. He also received radiotherapy on the pleural mass. Disease was stable for 7 months. Gastric volvulus still occurred with less frequency of about every three to four months, and the symptoms were less severe. The left pleural effusion developed in May 2015. The disease was evaluated as progression. Then he received the chemotherapy of ADOC regimen  for 2 cycles. Till March 2017, he was still alive with slow progression. The gastric volvulus still occurred every 3–4 months.Most patients with acute gastric volvulus present with a trio of symptoms known as Borchardt’s triad; these include pain in the upper abdomen or lower chest, retching and an inability to pass a nasogastric tube. Although the symptoms present in about 70% of patients with acute GV, diagnosis of gastric volvulus cannot be made by history and physical examination alone. Plain abdominal radiograph or computed tomography(CT) are recommended for accurate diagnosis. The classic finding of acute GV on plain radiograph is a single large spherical gas bubble located in the upper abdomen or chest with an air-fluid level . CompareFor this patient, GV was considered secondary to thymoma. There was no history of GV or etiological factors such as previous trauma and GV only presented accompanied by thymoma. An underlying pathophysiologic mechanism related to phrenic nerve palsy may be responsible. The phrenic nerve (PN) has been shown to be involved in up to 33% of patients with advanced stage thymoma (Masaoka Stage III and IV) . The B3 GV is a life-threatening condition, thus prompt treatment is imperative after diagnosis. For patients with acute GV and good surgical risk, surgery is recommended , 10. A lWe report the first case of thymoma initially presented with acute GV. The pathological mechanism may be related to the phrenic nerve palsy. This case indicates that thymoma can cause phrenic nerve palsy, presented with rare acute GV. In addition, for patient with GV, secondary etiology should be considered for early diagnosis and therapy.


We present the case of a 68-year-old woman, referred to our department for critical upper limb ischemia, which had occurred a few days after homolateral surgical ligamentotomy for carpal tunnel syndrome, diagnosed and confirmed by electromyography, and performed with a brachial tourniquet. The patient was later admitted for subsequent progressive necrosis of the first three fingers of the left hand, accompanied by signs of upper limb ischemia. An accessory cervical rib was identified, completely obliterating the subclavian artery distally at the origin of the suprascapular artery. A complete humeral artery occlusion was also found at the middle third of the humerus. The accessory rib was resected and the subclavian artery recanalized. A few days later, necrosis of the distal third of the first two fingers appeared and surgical resection was performed. Despite this chronic condition, the acute occlusion of collateral circles was probably induced by the brachial tourniquet. This represents a rare event, never previously reported in the literature: a case of critical upper limb ischemia due to a brachial tourniquet in a patient with misdiagnosed thoracic outlet syndrome. Until specific electrophysiological criteria for this syndrome can be found, attention should focus on history and clinical examination in patients with suspected carpal tunnel syndrome. Several other disorders, such as narrowed cervical foramina and decreased cervical range of motion,1 have also been associated with CTS. However, its statistical association with Thoracic Outlet Syndrome (TOS) is still unclear in the literature. In these cases, diagnosis, defined by the association between clinical symptoms, physical examinations and electrophysiological studies, may turn out to be difficult and even insidious, and further examinations should be considered prior to surgery. We report here a case of a woman in whom an inaccurate diagnosis and subsequent surgical therapy resulted in severe consequences for her hand. This very unusual case induced us to reflect on the possible reasons of the unexplained failure of surgical treatment for CTS.Carpal tunnel syndrome (CTS) is the most common peripheral neuropathy,We present the case of a 68-year-old woman, referred to our department for critical upper limb ischemia, which had occurred a few days after homolateral surgical ligamentotomy, after diagnosis of Carpal Tunnel Syndrome (CTS). A few months earlier, the patient had complained of pain, numbness and paresthesia in the left hand, affecting in particular the first three fingers at night. She had no history of cardiovascular diseases, arteriopathy, diabetes, smoking, or other vascular risk factors. She was clinically diagnosed with CTS, which was confirmed by electromyography of the left upper limb. A few weeks later, according to clinical and electrophysiological findings, surgical ligamentotomy was performed with a brachial tourniquet at a small local hospital. No complications occurred during the operation or after it, and the patient was sent home. The next day she returned to the hospital, complaining of extreme pain affecting the whole arm, but this was judged as wound pain and was medicated with painkillers. The following day she again returned to the emergency department, with the same incoercible pain, together with initial necrosis of the first three fingers of the left hand. She was immediately referred to the department of vascular surgery at the reference hospital. Clinical examination revealed delayed acute limb ischemia, in the absence of brachial pulsatility. A thoracic and upper limb angio-TC was immediately performed, and showed complete thrombotic occlusion of the left axillary artery and the first tract of the brachial artery with its distal rehabitlitation. An accessory cervical rib was found, completely obliterating the left subclavian artery distal to the origin of the costocervical trunk  and a poDue to the persistent absence of a brachial pulse, caused by dissection of the brachial artery, a venous graft was created, consisting of a left subclavian-proximal brachial artery bypass. At the end of the surgical procedure, a radial pulse was perceptible. Prescribed medical treatment included antiplatelet and anticoagulant therapy. After the first surgical treatment, complete necrosis of the distal third of the first two fingers became marked and a surgical resection was performed in the department of Plastic and Reconstructive Surgery . In the post-operative period, an eco-color Doppler demonstrated the patency of the venous graft. Currently, the patient does not complain of any pain, her pulse is clinically perceptible, the temperature of her hand is normal, and the wounds have healed . The pat1,3 It may be caused by compression between the first rib and the clavicle ,4 between the costoclavicular space and the thoraco-coraco-pectoral space during hyperabduction of the arms (hyperabduction syndrome), and at the narrowing of the interscalene triangle .1,5Thoracic Outlet Syndrome (TOS) is now defined as a group of clinical disorders involving suffering of the brachial plexus and subclavian vessels at their thoracic outlet.3 A case of undiagnosed TOS, complicated by acute ischemia of the upper arm after surgical treatment for CTS in a 68-year-old woman, is described here. However, in our patient, the original diagnosis of CTS may have been correct. Proximal anatomic abnormalities may in fact increase the susceptibility of peripheral nerves, resulting in symptoms such as peripheral compression of the ulnar, radial and median nerves.1Post-traumatic compression of neurovascular structures between the clavicle and the first rib have been described in the literature.6 indicating two points of compression of the nerves of the upper limbs. Proximal neural damage probably disturbs normal axoplasmic flow and thus the metabolism of peripheral nerves.5 This syndrome may be mistakenly identified as CTS more often than distal compressive disease.6,7 Clinically revealed CTS has been found in varying percentages of patients affected by TOS, from 44%6,8 to nearly 0%,8 demonstrating the controversy and difficulty of diagnosing clinical TOS correctly.6This situation is called “double crush syndrome”, a term coined in 1973 by Upton and McComas,6,9 no appreciable statistic link was found between CTS and true TOS: the frequency of proximal dysfunction in women affected by CTS was found to be less than 1%.6 True TOS is generally unilateral, with a history of paresthesia and pain starting in the supraclavicular fossa and irradiating through the medial arm, forearm and hand, especially in areas innervated by medial antebrachial cutaneous and ulnar nerves. Weakness, followed by atrophy, affects the thenar eminence and occasionally the medial forearm.3In addition, in two studies,4 and was also found in our case. Symptoms may be caused by specific maneuvers, such as postural exacerbation.9 Four provocative tests 5 have been proposed to diagnose TOS, but they are of low specificity.9Numbness and tingling are found in about 50% of patients, sometimes accompanied by sensory loss. Neurological and vascular symptoms are usually related to a post-stenotic aneurysm of the subclavian artery. This has been reported in 85% and 96% of patients with vascular abnormalities, respectively by Criado and Durham,9 Our patient was referred to us when a clearly defined vascular impairment appeared and diagnosis was apparently easy. No data regarding vascular or nervous symptoms or signs were reported when she went to her general practitioner, nor whether a complete examination was performed, but the patient stated that the only symptom was peripheral paresthesia in median territory.Vascular examination is also very helpful, but it must be performed at an angle of abduction and external rotation not exceeding 90°, this point being essential to maintain good test specificity and reliability. Several studies have found modifications of the radial pulse during abduction and external rotation of the arm, especially in women, with higher clinical values than with Doppler ultrasound.9 Our patient had also had electromyography when the first symptoms appeared, and they suggested CTS, as did the clinical examination. X-ray examination is essential for proper views of anomalous osseous structures, whereas MRI is more reliable in identifying fibrous bands at the thoracic outlet. Surgical treatment should not be based only on radiological findings, but also associated with clinical and EMG results.3Neurophysiological tests in patients with suspected TOS generally involve examination of the sensory nerve amplitude potential (SNAP) of the medial antebrachial cutaneous nerve (MABCN), which has been used to identify true neurogenic TOS since 1993.3TOS due to an accessory rib is treated with surgical resection of the rib and, if necessary, revascularization of the occluded artery, as in our case. Several surgical approaches have been proposed in the literature; resection of anatomical abnormalities is always suggested when possible, but first rib resection is also often performed in these patients and is considered to be safe, although discomfort and morbidity may be substantial. The results of surgical treatments are difficult to evaluate and considerable heterogeneity is reported in the literature. Surgical indications are progressive neurological deficit and failure of conservative treatments.In our patient, resection of the accessory rib and revascularization of the left brachial artery by subclavian-brachial artery bypass interrupted further necrosis and wound healing. Despite the patient’s chronic condition, as we first stated, acute occlusion of the collateral circles was probably caused by the brachial tourniquet during surgery. Although spontaneous necrosis of the distal part of the upper limb in TOS patients has occasionally been reported, we could not find a similar situation in the literature. We believe our case represents “the tip of an iceberg” of unclear diagnoses of CTS, which subsequent surgical treatment cannot necessarily resolve. Since diagnosis of CTS is clinical, the surgeon must identify suspicious cases and perform specific clinical tests. In addition, if suspicion is confirmed or remains unclear, electromyography should be carried out in the specific hypothesis of TOS; otherwise, only CTS may be identified if a non-specific question has been made. In order to complete the diagnosis, radiological examinations should subsequently be carried out.The authors declare no conflict of interest.