Chondroblastoma (CB) is a rare, primary, benign bone tumor that commonly affects men aged 15–20 years. It is usually detected in the epiphysis of the long bones, such as the proximal femur, humerus, and tibia. The patella is an infrequent site. CB with secondary aneurysmal bone cyst (ABC) is extremely rare in the patella, which can be easily confused with other common bone tumors of the patella. Thus, it is necessary to make the right diagnosis to get a good outcome.We have presented here the case of a 30-year-old man who was suffering from anterior knee pain for the past 6 months that had aggravated 2 weeks before the presentation. Osteolytic bone destruction in the patella could be detected in both his X-ray and computed tomography (CT) examinations, while the magnetic resonance imaging (MRI) detected a fluid level. Accordingly, secondary ABC was presumed. We diagnosed the condition as giant cell tumor (GCT) with secondary ABC and, accordingly, performed curettage inside the focus region with autogenous bone grafting following the patient’s medical history, physical manifestations, results of physical and ancillary examinations, and the disease characteristics. However, the intraoperative and postoperative outcomes indicated that the patient’s histopathology was consistent with that of typical CB, suggesting a definitive error in diagnosis. Accordingly, the patient was finally diagnosed with patella CB along with secondary ABC.Past studies have demonstrated that the 3 commonest bone tumors affecting the patella are GCT, CB, and ABC. CB with secondary ABC can be easily misdiagnosed as GCT with secondary ABC or ABC. Performing incision biopsy or excision biopsy and conducting histological examination may be the most effective method for suspected CB with secondary ABC. Chondroblastoma (CB) is a rare, primary, benign bone tumor that originates from the cartilages, accounting for only 1–2% of all primary bone tumor cases reported for the whole body , 4, but A 30-year-old man presented with the complaint of pain in his left knee for the past 6 months that had further aggravated in the past 2 weeks. There was no obvious inducement of the knee pain 6 months ago, and, although it was aggravated after certain activities, the pain remained tolerable. The patient did not seek any treatment because the pain did not affect the patient’s daily life activities. However, when the patient experienced increased pain for the past 2 weeks, he sought medical help from the hospital.His physical examination revealed obvious tenderness in the left anterior patella, no swelling in the left knee, and no effusion in the joint cavity. Normal color and temperature of the local skin, a negative result on the floating patella test, and no knee instability and locking were observed. He showed normal knee movement. No abnormality in the laboratory test results was recorded before surgery.Imaging examination included anteroposterior and lateral left knee radiographs before surgery. The density of the left patella was found to be decreased uniformly, and the joint space was normal. Preoperative CT revealed osteolytic cystic bone destruction at the middle and lower portions of the left patella, in a lobulated or worm-eaten shape with sharp edges. The local bone cortex had thinned. The focus boundary was clear, and there was no sign of sclerosis at its edge. Preoperative MRI revealed abnormal MRI signals of patchy shadow with a clear margin in the left patella. The T1-weighted images (T1WI) showed hypo-intensity, while the T2-weighted images (T2WI) showed hyperintensity. The fluid level could be observed on the sagittal or axial view of the T2WI, suggestive of ABC as a concern. Preoperative lung CT revealed no neoplasm metastasis focus  based on the patient’s age and imaging findings. The surgery was performed after completing the preoperative preparations. However, no needle biopsy was performed before the surgery, for the following reasons:The patient was made to lie in a supine position, and his left lower limb and right ilium were disinfected and draped. After approaching the lesion, a cortical window was created. Fresh blood was observed to be oozing out of the focus after the fenestration, and the focus had a multi-dividing shape containing some myxoid substance. The cartilage surface of the patella was relatively complete. A biopsy was obtained for the frozen section to confirm the provisional diagnosis. Through the cortical window, the intralesional curettage of the lesion was extended. Then, a high-speed burr was used to enlarge the tumor cavity, allowing the removal of an additional 1–5 mm of the cavity lining. After repeated irrigation of the tumor cavity with povidone-iodine solution and isotonic saline, autologous iliac bone was harvested from the right ilium, followed by grafting in the focus Fig.  and sutuThe reports for the intra-operative frozen section and postoperative pathological results revealed that the tumor tissues in the left patella were composed of osteoclast-like multi-nucleated giant cells and chondroid matrix. Accordingly, CB was considered Fig. .Fig. 3PThe left knee joint was reviewed after the surgery, and the grafted bone at the focus was found to have a good effect on the anteroposterior and lateral films and 3D-reconstructed CT. The static quadriceps exercise was started immediately after the surgery. The knee joint was next fixed with braces at the extension position for 4 weeks, after which the functional exercise for the knee was started. Two weeks after the operation, the pain was completely relieved. The range of motion of the knee joint was normal 2 months postoperatively. The imaging showed that the grafted bone had been partly incorporated with the host bone (Fig. CB is a benign bone tumor that commonly affects the epiphysis of the long bones. Kolodny et al. reported CB in 1927 and described it as GCT-containing cartilage . This diAccurate diagnosis is a prerequisite for a good prognosis. According to the literature, three bone tumors commonly affect the patella, namely, GCT, CB, and ABC , 14. AmoAlthough pathological results can assist in making the correct diagnosis, accurate pathological results depend on effective biopsy techniques. Therefore, the selection of the optimized biopsy technique becomes crucial. Currently, the frequently used biopsy technologies mainly include fine-needle aspiration, core needle biopsy, incision biopsy, and excision biopsy. Owing to the involvement of small trauma, fewer complications, lower cost, and ease of operation, the first two technologies are favored by bone oncologists , 28. HowH3F3A and H3F3B by immunohistochemistry or polymerase chain reactions is an effective technique. Past studies have shown that [H3F3A mutations in GCT and H3F3B mutations in CB. This feature has encouraged the development of new diagnostic tools [H3F3A and H3F3B or uses the specific antibodies of G34W and K36M mutation for immunohistochemical analysis to enable distinguishing between CB and GCT. However, the wide application of these tools in clinical practice can only be realized after a long time.In case of insufficient tissues available for biopsy or case of the lack of chondrogenic differentiation area in the limited samples, monitoring the K36M mutation on own that , 26 histic tools , 32 thatAlthough the case was misdiagnosed before the operation, the diagnosis was accurately corrected due to the reasonable choice of biopsy technique for pathological diagnosis. In the end, the patient received reasonable treatment and obtained a good prognosis.In conclusion, CB of the patella can be distinguished from GCT or ABC in terms of disease characteristics and imaging findings. However, when CB is combined with secondary ABC, it is easily misdiagnosed because its typical imaging features will overlap with the imaging findings of other tumors that occur at this site, and then become atypical or even be masked. By reviewing recent studies and referring to the results of this case report, we conclude that an incision biopsy or excision biopsy and histological examination may be the most effective diagnostic method for suspected CB with secondary ABC.


Accessory splenosis (AS) is an uncommon condition that is formed from the lateral dorsal mesogastrium due to the defective union of separate splenic masses. It is often found next to the pancreatic tail and the hilum part of the spleen. The retroperitoneal is described as an unusual and uncommon site. We present a case of a 39-year-old woman with an AS who came to the clinic with a right abdominopelvic mass. Computerized tomography (CT) scan revealed a right retroperitoneal abdominal mass. Diagnostic laparoscopy and resection of the retroperitoneal mass were done, and histological investigation showed that the removed mass was a spleen. Although retroperitoneal abdominal AS is an unusual condition, it should be considered in the list of abdominopelvic mass differential diagnoses. Accessory splenosis (AS) is also known as splenules, supernumerary spleen, or splenunucli. AS embryologically develops during the sixth week after the spleen cells' deposition along the midline path, and more commonly occurs left-sided. The most common location of AS is the pancreas tail and spleen's hilum . AS is cA 39-year-old lady was identified to have diabetes mellitus type 2, dyslipidemia, and gastroesophageal reflux disease. She complained of recurrent epigastric abdominal pain of five months duration. There was no nausea, vomiting, diarrhea, constipation, night sweating, weight loss, or history of trauma to the abdomen. The patient had no past surgical history. The results of the physical and abdominal examinations were unremarkable. Laboratory assessment, including inflammatory markers, were normal. Abdominopelvic computerized tomography (CT) scan showed a well-defined right lower quadrant lobulated soft tissue enhancing mass with internal calcification seen in relation to a short segment of terminal ileum Figure .The small and large bowel loops were unremarkable with no signs of bowel obstruction. The appendix was seen filled with air in retrocecal and subhepatic locations with normal size. The liver showed no features of cirrhosis and no focal lesions. There was no biliary dilatation. The patient was referred to our service and underwent diagnostic laparoscopy and resection of retroperitoneal mass under general anesthesia; intraoperative findings include right lower quadrant mass lobulated with attachment to anterior/lateral abdominal wall. The appendix was normal. Later on, the resected mass was histopathologically confirmed as AS (splenule). It consisted of a grey, tan oval mass with a nodular outer surface and surrounding adipose tissue Figure .On gross examination, the mass measured 8 x 4 x 3 cm. Serial sectioning revealed heterogenous well-circumscribed red-tan and nodular cut surfaces with a focal area of calcification. Microscopic examination confirmed spleen histology. The sections showed the classic components of a spleen. The first is the splenic red pulp venous sinusoids and splenic cords. The second is the white pulp formed by sheaths of lymphoid cells around arteries with surrounding mantle zone and outer marginal zones (Figure Numerous embryological anomalies of the spleen have been discovered, including multiple spleens, complete agenesis, small extra accessory spleens, and polysplenia . The locThe exact size of the AS ranges from few millimeters to centimeters ,5,6. In Despite retroperitoneal abdominal AS being uncommon in clinical practice, it is usually present as an asymptomatic retroperitoneal abdominal mass condition. A high index of suspicion should exist for any patient with AS, as well as it should be considered in the differential diagnosis of right lower quadrant soft tissue mass causing concurrent abdominal pain with enhancement in CT.


Invasive procedures for colorectal cancer can cause iatrogenic tumor cell seeding. Implantation of these exfoliated cells in the surrounding tissue can result in locoregional cancer recurrence. This has been described in endoscopic procedures and major surgical resections, however recurrence in iatrogenic lesions of the anal canal during minimal invasive rectal surgery has not been shown in literature yet. This is the first reported case of recurrent rectal cancer that developed into an anal metastasis at the site where hooks of the Lone Star Retractor disrupted the epithelial lining of the anal canal during a local excision of early rectal cancer using TAMIS.A 57 year old male was diagnosed with a high risk early stage rectal adenocarcinoma. He was treated with transanal minimally invasive surgery (TAMIS) with the use of a Lone Star retractor and he received subsequent chemo-radiotherapy. 23 months later the patient developed a bleeding mass bulging out of the anus. A true cut and incision biopsy was performed and the pathology report revealed localization of adenocarcinoma at the anal canal which was similar to the earlier diagnosed rectal carcinoma. The patient underwent an abdominal perineal resection and left-sided lymph node dissection.This shows that local recurrence through implantation of exfoliated tumor cells can occur in iatrogenic lesions of the anal canal not only in major but also in minimal invasive rectal surgery. Careful tissue handling and rectal washout may reduce the chance of this implantation metastasis. •Recurrent rectal cancer can occur at the site of an iatrogenic anal lesion.•Anal metastasis can develop due to implantation of exfoliated rectal tumor cells.•Iatrogenic local recurrence occurs in both major rectal surgery and local excision.•Careful tissue handling and rectal washout may reduce the chance of recurrence. Laboratory results showed a normal carcinoembryonic antigen (CEA) level of <5 μg/L. Colonoscopy with polypectomy of nine polyps and biopsies of a malignant lesion in the rectum was performed. Pathology examination revealed a well differentiated adenocarcinoma of the mid rectum. Abdominal MRI showed a lesion with a diameter of 2 cm located 8 cm proximal to the anorectal junction without infiltration of the muscularis propria and no suspicious lymph nodes or extramural vascular invasion (EMVI) . On the 2 twice a day) for 5 weeks. Strict follow-up evaluation and imaging after 6, 12 and 18 months did not show any signs of recurrence.In this case, the patient participated in a randomized controlled trial that compares completion total mesorectal excision (TME) versus adjuvant chemo radiotherapy after local excision in patients with intermediate risk early rectal cancer  However, after 23 months the patient developed a painless, growing and bleeding mass bulging out of the anus . The difThe patient underwent an abdominal perineal resection and left-sided lymph node dissection. Pathology showed stage rpT2N1b with radical removal and 22 resected lymph nodes of which 2 were positive for metastasis. Postoperative recovery was complicated by wound infection for which vacuum sponge and antibiotic treatment was initiated. Furthermore, the patient had an uneventful recovery. The patient is currently at two years follow up after TAMIS and approximately one year after APR surgery. No new recurrence was seen and no further adjuvant treatment was given.3Spreading of rectal tumor cells to the anal canal or perianal region is rare. Colorectal cancer usually metastasizes via hematogenous route to the liver, lungs, bone, nervous system, via lymphatics to regional and distant nodes, and with intraabdominal exfoliated cancers cells to the peritoneum The Lone Star retractor is a frequently used flexible and adjustable self-retaining retractor system designed to maximize visualization during anorectal surgery. It is placed at the anal verge by using small hooks penetrating the mucosa of the anal canal resulting in small scars. In this case, the recurrence was located in the anal canal below the dentate line close to the normal epithelium of the skin where the Lone Star hooks were located during TAMIS. Based on the anatomy and embryological origin of the rectum it is unlikely that the recurrence in the anal canal was due to vascular invasion and therefore we presume that tumor cell implantation occurred in one of the Lone Star lesions. So far only three cases of Lone Star metastasis have been reported of which all of them in the context of anterior resection To prevent implantation metastases, the rectum and anal canal are washed with cytotoxic solutions such as povidone‑iodine. Despite the available conflicting data and lack of randomized clinical trials, rectal washout is recommended to be routinely performed in rectal surgery until more convincing evidence is obtained In this case the patient participated in a clinical trial and was randomized in the experimental arm. The patient received adjuvant chemoradiation instead of standard completion surgery. The chemoradiation field is limited to the rectum and mesorectum, therefore the implanted tumor cells in the anal canal did not receive any of this treatment. Completion surgery might have prevented this if an abdominoperineal resection would have been performed, but not in case of sphincter preserving surgery.4We present a rare case of anal implantation metastasis at the site of a scar form a Lone Star Retractor hook that developed following local excision using TAMIS approach. This shows that this phenomenon can occur not only in major but also in minimal invasive rectal surgery. Careful tissue handling and rectal washout may reduce the chance of implantation metastasis during invasive procedures for rectal cancer.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Waived by ethical committee of our institute.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.A.S. van Lieshout, A.A.J. Grüter and L.J.H. Smits wrote the paper. J.B. Tuynman and P.J. Tanis revised and edited the paper. J.B. Tuynman and P.J. Tanis performed the surgical procedure.This work is registered in Research Registry with the unique identifying number: researchregistry7527.Ass. prof. dr. J.B. Tuynman.Not commissioned, externally peer-reviewed.All authors have no conflict of interest.


Distinguishing between tumefactive demyelinating lesions (TDLs) and brain tumors in multiple sclerosis (MS) can be challenging. A progressive course is highly common with brain tumors in MS and no single neuroimaging technique is foolproof when distinguishing between the two. We report a case of a 41-year-old female with relapsing–remitting multiple sclerosis, who had a suspicious lesion within the left frontal hemisphere, without a progressive course. The patient experienced paresthesias primarily to her right hand but remained stable without any functional decline and new neurological symptoms over the four years she was followed. The lesion was followed with brain magnetic resonance imaging (MRI) scans, positron emission tomography–computed tomography scans, and magnetic resonance spectroscopy. Together, these scans favored the diagnosis of a TDL, but a low-grade tumor was difficult to rule out. Examination of serial brain MRI scans showed an enlarging lesion in the left middle frontal gyrus involving the deep white matter. Neurosurgery was consulted and an elective left frontal awake craniotomy was performed. Histopathology revealed a grade II astrocytoma. This case emphasizes the importance of thorough and continuous evaluation of atypical MRI lesions in MS and contributes important features to the literature for timely diagnosis and treatment of similar cases. The concurrence of glioma and relapsing–remitting multiple sclerosis (RRMS) is uncommon. Multiple sclerosis (MS) is diagnosed by clinical and radiological criteria meeting the 2017 McDonald’s Criteria [The 41-year-old female (non-smoker with a history of ulcerative colitis and celiac disease) had the onset of her MS 14 years ago, characterized by sensory symptoms of the lateral three fingers of the right hand, which resolved completely over several months. Seven years later, she experienced a second relapse characterized by paresthesias to her right hand, which spread to the medial aspect of her right arm, torso, leg, and toes. Following the second attack, she was diagnosed with RRMS based on MRI and clinical criteria. The initial MRI showed a large lesion  imaging) within the left frontal hemisphere involving the cortex and adjacent white matter  and multiple imaging techniques could not adequately distinguish between TDL and early glioma. In addition to standard brain MRI imaging, the patient underwent FDG-PET-CT, which was hypometabolic (favoring TDL), and MRS, which was equivocal . The coTDLs and brain tumors may share several characteristics, including size >2 cm), gadolinium enhancement pattern, and a predilection for the frontal and parietal lobes. Importantly, several studies have attempted to distinguish between TDLs and brain tumors. For example, a study by Abdoli and Freedman [ cm, gadoConsidering that there has been no recent review on this topic, we conducted an up-to-date topical review of the cases of gliomas in persons living with MS. A literature search was done on MEDLINE/PubMed, Scopus (Elsevier), and Google (internet search) using the following search terms: multiple sclerosis, glioma, tumefactive lesion, brain tumor, astrocytoma, ependymomas, and oligodendroglioma. Case reports or case series reporting gliomas in an individual with MS and published in the English language since 2012 were included. We found 14 articles published in the last eight years (2012–2020) . There wLow-grade glioma in an MS patient may pose a diagnostic dilemma as the MRI findings of low-grade gliomas may be similar to those of TDLs . Table 3This case report highlights that slow-growing suspicious TDLs in MS patients with a non-progressive course should be carefully monitored by MRI over time to exclude low-grade gliomas. An elective craniotomy  followed by radiation and chemotherapy may provide favorable outcomes in patients with concurrent RRMS and low-grade diffuse astrocytoma.


Introduction: In this study, we present a case of primary diffuse leptomeningeal melanomatosis (PDLM), without neurocutaneous melanosis syndrome.A female patient (age: 14 years) presented with headache, nausea, vomiting, vertigo, diplopia, and lower limb weakness. The magnetic resonance imaging (MRI) showed leptomeningeal isointensity on T1- and T2-weighted images and hyperintensity on fluid attenuation inversion recovery (FLAIR) sequences. Definitive histological examination showed a densely cellular tumor, characterized by irregular clusters of large pleomorphic cells and melanin in tumor cells. Adjuvant therapy was refused by the parents, and the patient died within six months.Primary diffuse leptomeningeal melanomatosis is recognized as an uncommon and malignant melanoma affecting the central nervous system. In case comorbidities are not diagnosed in patients with unusual symptoms of meningitis, diagnostic methods such as cerebrospinal fluid analysis and central nervous system biopsy can be helpful in identifying other underlying conditions. Primary and malignant melanoma of the central nervous system (CNS) is an uncommon phenomenon, accounting for nearly 1% of all reported cases of melanoma. According to statistics, primary melanoma of the CNS occurs in 0.005 per 100 000 people ; howeverPrimary diffuse leptomeningeal melanomatosis (PDLM) is recognized as a rare type of primary malignant melanoma of the CNS. This condition in children can be associated with neurocutaneous melanocytosis, which is an uncommon congenital condition, identified by large (or various) melanocytic nevi in the skin and melanocytic leptomeningeal tumors  . In thisA female patient (age: 14 years) with headache, nausea, vomiting, vertigo, diplopia, and lower limb weakness was admitted to the pediatric ward of Ghaem Hospital, Mashhad, Iran in May 2014. Neurologic examination showed flaccid weakness of the lower limbs (4/5). Pathologic reflexes were not detected, and sphincters were intact. On fundoscopy, the optic discs were normal.The magnetic resonance imaging findings showed leptomeningeal isointensity on T1- and T2-weighted images and hyperintensity on fluid attenuation inversion recovery (FLAIR) sequences. These findings showed improvement after the intravenous administration of gadolinium, which was suggestive of carcinomatosis or microbial meningitis . Multiple lumbar punctures were found to be dry. Empirical treatment for bacterial meningitis was initiated. However, after a few days, due to clinical deterioration of the patient, particularly progressive paraparesis, the multidisciplinary team planned a biopsy of the lumbar region.After laminectomy of the third lumbar vertebrae, the dura mater was opened. However, no cerebrospinal fluid (CSF) flow was seen, and all nerve roots were edematous and dark gray in color. Samples were collected for biopsy from the posterior rootlets and arachnoid layer. The pathologic study of the samples revealed leptomeningeal melanoma with a densely cellular tumor, comprised of irregular masses of large pleomorphic cells and melanin in tumor cells. Numerous mitotic figures associated with nerve root invasion were detected . HoweverPrimary diffuse leptomeningeal melanomatosis is a rare malignant melanoma of the CNS . SimilarThe symptoms of PDLM are nonspecific and are caused by intracranial hypertension and focal neurological deficits . PrimaryThe typical MRI findings of PDLM include diffuse leptomeningeal hyperintensity on T1-weighted imaging, low T2 signal intensities arising from the paramagnetic features of melanin , hyperinCerebrospinal fluid analysis usually yields nonspecific results, indicating high levels of proteins, improved red blood cell count, normal (or low) level of glucose, leukocytosis, and occasionally characteristic pleomorphic neoplastic cells consisting of intracytoplasmic melanin pigments . In the Immunohistochemical staining was not performed because of the typical finding on hematoxylin-eosin (H&E) staining for melanoma. To exclude extraneural sources , the implementation of different tests  is necessary after definitive diagnosis , 3.The efficacy of therapeutic interventions, such as corticosteroids, radiotherapy, chemotherapy, intrathecal interleukin-2, and surgery, is poor for hydrocephalus and only palliative , 13, 14.Primary diffuse leptomeningeal melanomatosis is a rare and aggressive tumor with no specific presentations, posing challenges to its diagnosis and treatment. In case comorbidities are not definitively diagnosed in patients with unusual symptoms of meningitis, diagnostic methods such as CSF analysis and CNS biopsy can be helpful in identifying other underlying conditions.


Amyand’s hernia is defined as an inguinal hernia which contains the vermiform appendix. This continues to pose a diagnostic and therapeutic challenge. We herein describe the case of an 11-year-old male with a right-sided Amyand's hernia and concurrent appendicitis. A literature review was also conducted, looking at history, aetiopathogenesis, symptomatology, and management. This case highlights the difficulty clinicians can have in reaching a preoperative diagnosis of Amyand's hernia and selecting the appropriate surgical management. Amyand’s hernia is a rarely occurring form of inguinal hernia (1% of cases) [Due to the prevalence with which patients are seen with right-sided abdominal pain, and the operator-dependent nature of ultrasonography, it is important that clinicians are aware of this potential diagnosis and have a high index of suspicion. This will allow the appendicitis to be managed before the condition progresses to sepsis or a perforated viscus.A normally well, 11-year-old male presented with right-sided abdominal pain and associated vomiting for one day. On initial examination, his abdomen was soft with mild right lower quadrant tenderness, with no other positive findings - including any inguinal swelling or tenderness. The initial accident and emergency (A&E) routine bloods revealed a mildly elevated white cell count (WCC) and a normal C-reactive protein (CRP). Repeat bloods 24 hours later showed the WCC had dropped to within the normal range, and CRP remained normal. It is of note that the patient didn't receive any antibiotic therapy in the interim. The results for these two sets of blood tests can be seen in Table Due to ongoing tenderness, the patient was sent for an urgent ultrasound scan. This revealed evidence of a lesion with mixed echogenicity within the right iliac fossa. Surrounding this was an indeterminate area of either necrosis or collection, with an area of 4.5 cm x 2.5 cm.On examination, on day 2, the child was comfortable and reported improvement in symptoms. His abdomen was grossly soft and non-tender. There was a focal tender point in the right groin with no cough impulse. Due to ongoing clinical concern and no clear diagnosis, a computerised tomography (CT) scan of the abdomen and pelvis was performed, which confirmed Amyand’s hernia. The report stated - “a small right-sided inguinal hernia containing the tip of the appendix with fluid, and increased peritoneal enhancement suggesting localized infection/inflammation” Figures , 2.The patient underwent a laparoscopic appendicectomy where the appendix was visualized extending into the opening of the right-sided inguinal hernia Figure . On inspAmyand’s hernia is a condition where the vermiform appendix is contained within an inguinal hernia sac. The first-ever successful human appendicectomy was performed by Claudius Amyand 1735), wherein the appendix was found to be contained within an inguinal hernia . Subsequ735, wherWhilst this condition can present at any age it is more frequently seen in childhood, as a persistent patent processus vaginalis is considered the most common underlying aetiology of an Amyand’s hernia . DespiteThe mechanism of developing appendicitis with the hernia is not fully understood. There is uncertainty in determining if these are cases of primary appendicitis, coincidentally within the hernia, or if the presence of the appendix within the hernia is responsible for the development of the appendicitis. A number of theories have been presented in the literature. Patoulias et al. conducted a literature review and found four possible theories, these are: incarceration of the appendix inducing inflammation; the development of adhesions between the appendix and the hernial sac causing an irreducible hernia which is susceptible to injury; anterior abdominal muscle contraction  causing compression and therefore a functional obstruction of the appendix; lastly, primary inflammation of the appendix may lead to an irreducible hernia - this leads to venous stasis and an impaired microcirculation within the appendix, thereby leading to bacterial overgrowth , 6.Clinically these patients usually present as asymptomatic, occasionally with signs of inguinal hernia; inguinal bulge usually reducible. In those that present symptomatically, the patients present with features of an obstructed or strangulated hernia - painful irreducible inguinal bulge, with swelling of overlying tissue and overlying erythema. In the rare case of acute appendicitis (or even perforation of the appendix) within the hernia, the clinical picture is that of perforated intestine within the hernia. There are no specific features to suggest appendicitis and McBurney's sign is typically found to be negative , 7. ThesDue to the aforementioned preoperative difficulty in diagnosing Amyand’s hernia, most of these patients are diagnosed intraoperatively during the repair of the hernia. Sharma et al. looked at 18 cases of Amyand’s hernia over a 15-year period, of which none of these patients were diagnosed preoperatively. These patients were operated on as emergency cases for either incarcerated or strangulated inguinal hernias and underwent no preoperative imaging [Right-sided abdominal pain is amongst the most common reasons for acute referrals to general surgeons and ultrasonography is usually the first line imaging of choice. Interoperator variability of ultrasonography makes Amyand's hernia challenging to diagnose preoperatively. It is important that clinicians are aware of this condition and the associated clinical ambiguity. Had the authors relied on legacy practice, where acute appendicitis was to be a clinical diagnosis, it is possible that our patient may have gone on to emulate Claudius Amyand’s patient in 1735. When preoperative imaging is performed, the rate of preoperative diagnosis does increase. Okur et al. looked at 21 cases of Amyand’s hernia at a single center. Ultrasound was done in 12 of the cases (57.1%) and diagnosed 75% 9/12) with Amyand’s hernia preoperatively [/12 with Ultrasound findings usually include a non-compressible structure within the inguinal hernia sac . CT imaging of Amyand’s hernia usually reveals a blind-ending structure within the hernial sac, originating at the caecum. Similarly, to the ultrasound findings, when there is concurrent appendicitis there will be some degree of wall thickening, hyperaemia and fat stranding . It is pSince most cases of Amyand’s hernia are diagnosed intraoperatively, it is hard to standardize an approach to treatment and usually left to the discretion of the operating surgeon. Losanoff and Basson, in 2008, proposed a classification system for the management of Amyand’s hernia . This isThis case highlights the diagnostic challenge posed by Amyand's hernia. With the assistance of imaging, a preoperative diagnosis was reached which enabled an appropriate and planned surgical approach. Clinicians should have a high index of suspicion for Amyand’s hernia and a low threshold for investigating further with imaging. Due to the appendix being isolated from the abdomen, blood tests may return as normal and can therefore be very misleading in a suspected appendicitis. With the use of imaging (ultrasound & low dose CT), a pre-operative diagnosis could be made for a significant number of patients. This would allow for a better visualization of the appendix and inguinal canal, and possibly a better surgical outcome.


Duodenal duplication is a rare congenital anomaly and may manifest as pancreatitis, gastrointestinal bleeding, abdominal pain, perforation, and obstruction. Here, we present a case of intraluminal diverticular duodenal duplication (IDDD) in a child with recurrent abdominal pain caused by a large hole-like structure in the duodenal bulb. This condition has rarely been reported. An 11-year-old boy presented with recurrent attacks of abdominal pain. Upper endoscopy examination and barium swallowing led to an initial diagnosis of IDDD; this diagnosis was confirmed by operative findings and histopathological signs. He underwent a subtotal excision and duodenal anastomosis. No serious complications occurred following treatment. The patient was followed up for 8 months, and his condition improved without symptoms. Enteric duplications are uncommon congenital anomalies and account for 5–7% of all gastrointestinal (GI) duplications. By definition, these duplications can be located at any location within the GI tract . The tervia the nose to the stomach until 4 days after surgery when only a small amount of gastric juice was released. A postoperative abdominal drainage tube showed no obvious fluid exudation, and there was no evidence of celiac disease. The boy drank fluids and progressed to semi-fluids after removal of the tube for 2 weeks. No complications were observed after management and the boy remained asymptomatic at the 8-month follow-up examination except that he ate more and had gained of 2 kg.An 11-year-old boy was referred to our department with a 1-year history of occasional abdominal pain and no vomiting. However, his troublesome pain had recurred and worsened over the last month. The parents told us that their son had experienced poor taste in childhood and denied any history of surgery; there was no family history of this condition. The boy was very thin but his physical examination was unremarkable except for epigastric tenderness. Laboratory examinations also revealed normal levels of carcinoembryonic antigen, bilirubin, and amylase. However, barium swallowing demonstrated that there was a peculiar defect in the duodenum; the barium-filled form was very large and had a blind end . Then, wDuplications of the tract are congenital lesions and may or may not communicate with the alimentary tract. These duplications can be cystic and tubular in shape although the most common type is the cystic form which accounts for 82% of all GI tract duplications . DuodenaThe pathogenesis of IDDD remains unclear. It has been suggested that enteric duplications may be caused by the abnormal separation of the embryonic notochord and gastrocoele, a disruption of the recanalization process in the primitive foregut and environmental factors . EntericThe diagnosis of these structures is difficult. Although duodenal duplications are congenital malformations that are mainly diagnosed in infants, 33% of cases have been described in adults over 20 years old . HoweverThere is a wealth of literature relating to imaging facilities for the diagnosis of IDDD. Contrast-enhanced computed tomography and barium swallowing have been used reliably to detect the site and measure the size of such lesions . Endoscovia endoscopic incision , General Guidance Project of Hunan Provincial Health Commission (20201949), Project of Hunan Provincial Health Commission (B2019111), Hunan Provincial Natural Science Foundation Project (2020JJ5504), Hengyang Science and Technology Bureau Guidance Project (2020161), and Scientific Research Project of Hunan Education Department (19C1620).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


About a quarter of all oral pathologies involving the oral cavity and dental apparatus are traumatic injuries, and a substantial number of these cases are the result of sports injuries affecting adolescents and young adults. Here, we report the case of a 25-year-old healthy female referred to the department of Endodontics for the evaluation and management of teeth 1.2 and 1.1 because of a chronic apical abscess in an area involved in a sport-related dental trauma in the past. A multi-modular diagnostic assessment, comprising conventional periapical radiographs, CBCT imaging, ultrasound, and histopathologic examination, led to a final diagnosis of an apical granulomatous lesion connected to both teeth, and an associated sinus tract. During the follow-up period of three years, the patient was reviewed twice a year and showed progressive healing of the bone and absence of the sinus tract. The present report shows the challenges of diagnosing complications arising from past dental trauma. Furthermore, it is the first documented traumatic case where ultrasound examination was fruitfully used. Emphasis should be put on introducing diagnostic ultrasound for the management of both apical periodontitis and the related sinus tract. Traumatic injuries constitute almost a quarter of all pathologic conditions that involve the oral cavity and teeth, and a substantial number of these cases result from sports injuries affecting adolescents and young adults . Indeed,A 25-year-old Caucasian female patient was referred to the Department of Endodontics of the University of Cagliari, with the chief complaint of periodical drainage of purulent material from the opening of a sinus tract located in the buccal mucosa, apical to teeth 1.2 and 1.1, in the fall of year 2017. The sinus tract had appeared a few months earlier. The patient’s medical history was positive for chronic bronchial asthma, and non-contributory for other diseases or medications. The patient reported a sport-related dental trauma due to a body contact with another player, which occurred six years earlier (2011) when she was an agonist volleyball player. As a consequence of that trauma, she reported the enamel-dentinal fracture of teeth 1.1 and 2.1 and the concussion of tooth 1.2, according to the IADT guidelines . In 2014To assess the content and vascular supply of the lesion, a real-time ultrasound examination with the addition of CPD was performed . The exam displayed an echogenic, solid lesion with a poorly defined hyperechoic bone outline and internal blood vessels, suggestive of an apical granuloma  Figure . This exThe secondary root canal treatment was performed in two visits. At the first appointment, the endodontic filling was gently removed, under microscope magnification, using K-files and a solvent . Chemical disinfection with 5% sodium hypochlorite was used throughout the procedure, an apical gauging of 40 was determined with the apex locator and the canal was filled with calcium hydroxide paste, and the access cavity was sealed with a temporary cement. At the second appointment, the root canal was re-accessed, disinfected with the addition of a final rinse of 17% EDTA, and obturated with gutta-percha and zinc-oxide eugenol cement using the continuous wave technique, but the quality of apical instrumentation was deemed not satisfactory due to difficulties in completely removing the previous fill and reaching the full working length. The access cavity was restored with composite resin , and it The present report documents the sequelae of a sport related dental trauma where a multi-modular assessment involving ultrasound examination was necessary in order to correctly manage the case. Traumas of the oral-dental region have a considerable social impact, as these events represent 5% of all injuries for which patients require treatment. Among the multiple long-term complications of dental trauma are apical periodontitis, internal or external root resorption, and vertical root fractures, which can result in the loss of the tooth . The posIn the management of the present case, the contradictory responses of tooth 1.1 to sensitivity, in conjunction with the lack of healing of AP after both orthograde and surgical endodontic treatment, led the clinical team to consider whether to treat tooth 1.1 or extract tooth 1.2. However, it is well known that edentulism, even when affecting a single tooth  is an extreme type of damage and should require early intervention, either prosthetic, orthodontic or conservative, in adolescents and young adults, to avoid long term complications ,21. GiveThis work showed the importance of a multi-modular assessment in the diagnosis, treatment, and follow-up of the sequelae of a sport-related dental trauma. The traditional periapical radiographs were used, as they still represent the gold standard to disclose the presence of the lesions in the bone and of previous endodontic treatments. They are also useful during treatment and for follow-up. CBCT was of utmost importance to observe the size and spatial relationship of the lesion to the surrounding anatomical structures, to plan the endodontic and surgical interventions, and to accomplish a predictable long-term follow-up. The present report further demonstrated the potential use of ultrasound imaging as a diagnostic aid in the maxillofacial district. Indeed, ultrasound examination was of particular interest in non-invasively visualizing and tracing the sinus tract, and revealing the content of the lesion. In addition, this latter technique was demonstrated to be reliable not only in monitoring the periapical lesion, but also in predicting the long-term outcome of endodontic treatment ,13 based


Orbital lymphomas are primarily non-Hodgkin type and can originate from the eyelids, extraocular muscles, soft tissue orbital adnexa, conjunctiva, or lacrimal glands. Orbital malignancies often represent a diagnostic dilemma for clinicians given their varying and atypical presentations.  To report a case of orbital lymphoma mimicking orbital cellulitis.  A 66-year-old male patient presented with sudden onset of painful proptosis with visual impairment in the left eye for 15 days. On ocular examination, best-corrected visual acuity was 6/12 in the right eye and 2/60 in the left eye, abaxial proptosis with hypertropia, swollen and erythematous eyelids, restricted extraocular movement in all cardinal position of gaze, conjunctival congestion with chemosis and tortuous vessels, sluggish pupillary reaction, and chorioretinal folds in the inferior quadrants. The case was diagnosed as left eye orbital cellulitis, and the patient was treated with broad-spectrum intravenous antibiotics and oral steroids. No clinically discernible response was noted despite 7 days of antibiotics and steroids. Contrast-enhanced computed tomography (CECT) orbit showed features suggestive of orbital lymphoma involving the ipsilateral maxillary and ethmoid sinuses. ENT consultation with diagnostic nasal endoscopy and biopsy was done. Histopathological reports showed features of non-Hodgkin lymphoma.  Orbital malignancies masquerading as orbital cellulitis can pose a diagnostic dilemma. A multidisciplinary approach involving ENT consultation, radiological investigation, and pathological sampling can help achieve a timely diagnosis and appropriate management. Orbital lymphomas are a common type of non-Hodgkin Lymphoma (NHL). It primarily originates from the eyelids, extraocular muscles, soft tissue orbital adnexa, conjunctiva, or lacrimal glands. It is most commonly located on the extraconal site, mostly anterior in the orbit or beneath the conjunctiva with a typical “salmon-patch appearance.” Lymphoma originating from one of these locations is called primary orbital lymphoma. Lymphoma originating from extraorbital sites with metastatic spread to the orbit is secondary orbital lymphoma . Among aThe major factor contributing to the pathophysiology of orbital lymphoma is immune-compromised conditions like ageing, HIV/AIDS, or immunosuppressive drugs. Recently, studies have suggested that pathogens like Chlamydia psittaci, H. pylori, and some viruses are associated with orbital lymphoma . LymphomA 66-year-old Nepalese male patient presented in the Outpatient Department (OPD) of Ophthalmology, B.P. Koirala Institute of Health Sciences, with sudden onset of painful proptosis associated with visual impairment in the left eye for 15 days . Other aUSG B-scan of the left eye showed normal intraocular structures with the presence of homogeneous mass behind the globe inferiorly . The patThere was no improvement despite two days of intravenous antibiotics and oral steroid; contrast-enhanced computed tomography (CECT) orbit showed homogeneously enhancing soft tissue component in the retroorbital space involving the intraconal compartment including the optic nerve with an adjacent extraconal spread. It also involved the soft tissue of the anterior orbit with homogenous opacity in the ethmoidal and maxillary sinuses with few probable sites of bony erosions Figures . OtorhinNon-Hodgkin lymphoma (NHL) is the most common malignant tumor of the orbit . MoslehiOrbital lymphoma commonly presents with proptosis or palpable mass causing eyelid swelling , 11, 12.Lymphoma of orbit can masquerade as orbital cellulitis –17. The Radiological imaging, including a CT scan or MRI, can help diagnose and delineate the extension of the malignant lesion such as orbital lymphoma. Diagnostic confirmation mainly depends on the pathological biopsy for grading and staging of malignancy. Further categorization of orbital lymphoma is aided by immunohistochemistry, making it easy to formulate an appropriate treatment plan. In our case, the orbit seems to be the primary locus of the lesion rather than secondary metastasis, as the normal peripheral blood smear finding makes the chance of hematogenous origin less likely. A more systematic evaluation is still needed, however, to rule out metastasis. Regarding the treatment of localized orbital lymphoma, radiotherapy is appropriate for local control , 19. ForOrbital lymphoma masquerading as orbital cellulitis can pose a diagnostic dilemma. Orbital imaging like CT scan or MRI should always be performed in a patient with painful proptosis, especially in unilateral cases, to exclude malignancy. A tissue biopsy is needed to confirm the diagnosis of lymphoma. Therefore, a multidisciplinary approach with ENT consultation, radiological investigation, and adequate pathological sampling is necessary to obtain a correct and timely diagnosis to decrease overall morbidity and mortality.


Sternal clefts are infrequent congenital malformations, particularly in their complete presentation. There are less than 100 descriptions ofthese defects published in the literature worldwide. We report a clinical case of lower sternal cleft associated with congenital laparoschisis ina 2-year-old boy. Surgery was performed because of recurrent pneumopathy and the risk of cardiorespiratory decompensation in the midterm. A semi-resorbable prosthesis was used for sternal closure. We have not observed any complications with this sternal closure systemin our patient. This approach is easy, safe, effective and not harmful to a child’s growth. Our patient presentedwith an inferior sternal cleft and upper median laparoschisis. Thesurgical operation consisted of the primary closure of the defect.Herein, we report a 2-year-old boy with bifid sternum beyond theneonatal period who underwent early successful primary closureusing semi-resorbable composite mesh with a multidisciplinarysurgical approach between anaesthesiologist, thoracic and paediatricsurgeon in our centre.Sternal clefts are a rare idiopathic chest wall deformity caused by adefect in the sternum’s congenital fusion process that can be completeor partial.A 2-year-old boy with recurrent respiratory infection and an anteriorchest wall swelling was referred by the paediatrician. The childwas delivered via normal vaginal birth at 36 weeks of amenorrhea,eutrophic with no mention of recurrent urinary tract infection in themother during the pregnancy period. The obstetrical ultrasounds wereuneventful. Interrogation did not reveal any parental consanguinity,no height and weight deficit and vaccination status was up to date.The parents did not report cyanosis, dyspnoea, or repeated respiratoryinfections in the neonatal period. On clinical examination, the childpresented with thoracoabdominal balanced breathing without respiratory difficulty. Observation and palpation diagnosed a sternalcleft. Physical examination revealed an enlargement of the caudalportion of the sternum and a laparoschisis responsible for a xypho-umbilical midline eventration that worsened with crying episodesThe rest of the somatic examination was without abnormalityincluding no associated visible malformation and a normal cardiacauscultation. The cardiac ultrasound-Doppler showed a ‘situs solitus’ without any notable cardiovascularabnormality. The chest computedtomography (CT) scan in axial sectionswith 3D parietal reconstruction showed thepresence of small, rounded, hypodense anddiscontinuous formations correspondingto the ossification points in the upper partof the sternal edges and a sternal diastasisin the lower part Primary closure of the defect was necessarygiven the risks of recurrent pneumopathyand long-ter m c ardio-respirator ydecompensation. The operative procedureconsisted of two operative steps, bone andmuscular rapprochement with a compositemesh. It was performed on the patient in asupine position under general anaesthesiawith ventilation via a classic orotrachealintubation. Prior to the incision, the correctanatomical landmarks (jugulum and xiphoid)had to be identified. We then undertook amedian skin incision and a subcutaneouscauterisation from the fossa jugularis tothe xyphoidis, followed by a layer-by-layerdissection down to the sternum to reach thesternal cleft. The sternal bars were dissectedfree and isolated under the periosteum fromthe insertion of pectoralis major musclesanteriorly, and endothoracic fascia posteriorlyThe prosthesiscompensated and bridged the gap betweenthe two sternal bars [3] It is a rare malformation,particularly in the form of an isolated defect that was first reportedin 1740 by London.[3] The first surgical correction was reportedby Lannelongue in 1888, but the first successful repair was publishedby Burton in 1947.[1] Multiple embryological studies of the sternumhave attributed the sternal cleft to a defect in fusion of the twosternal bands from the lateral plate mesoderm, which normallyoccurs around the ninth week of intrauterine life in the craniocaudaldirection.6] The cleft may involve the manubrium and uppersternebrae, the lower sternum saving the xiphoid process, but also theentire sternum. Therefore, sternal clefts are classified as incompleteand partial forms, which can be superior or inferior.5] According toRavitch,[7] classification of sternal clefts may be in the form of:The sternal fissure is a defect in the fusion of the sternum at anearly stage of development of the fetus.Complete bifid sternum with or without ectopia cordis orherniation of pericardium known as Cantrell pentalogy. Fusion at only upper or lower parts of sternal plates . Incomplete fusion giving rise to a hole in the sternum.9] The most common defect is the partial superior form, which affects theupper sternum and the manubrium with normal fusion of the lowerpart.6] Inferior defects are extremely rare and usually associatedwith complex syndromes like Cantrell pentalogy.5] None of thispentalogy was found in our patient. The aetiopathology of sternalclefts remains unclear. Haque[10] identified familial cases in SaudiArabia suggesting an autosomal recessive pathology. Chest walldevelopment studies have suggested the implication of the Hoxb-4gene and have been inconclusively associated it with defects in mice.[11]Other experiments have demonstrated that perturbations of GSK-3β(glycogen synthase kinase-3β) resulted in alterations of the para-axialmesoderm.[12] Other hypotheses have been suggested, such as abuseof alcohol consumption, nutritional deficiencies and hypovitaminosis during pregnancy but have not beenproven.8] There is no reported justification for the predominantlyfemale prevalence of the condition.5] However, studies by Gorlin etal.[13] and Heron et al.[14] showed a predominance of females when thesternal cleft is associated with a bandlike scar from the umbilicus anddiastasis recti.We observed an association between the partial sternal cleftcombined with a median xypho-umbilical laparoschisis. It shouldbe noted that this association is infrequent. Only two other similarcases, to our knowledge, have been reported in the literature.5] Prenatal diagnosis of isolatedsternal cleft is very difficult.[4] However, Yuksel et al.,[15] diagnosedan intrauterine sternal cleft with the help of ultrasonography. Inthe neonatal period, the diagnosis of sternal cleft is easily done byinspection and palpation.[6] Imaging based on conventional radiology,multidetector CT and magnetic resonance show the verticalisation ofthe clavicles, dehiscence of sternal bars, which is more important whenthe patient is seen at a later stage, and associated with malformations.CT is considered the best technique for studying sternal anatomy,offering the possibility to perform 3D evaluation, which can help thesurgeon in surgical repair of the sternal cleft.6]A bifid or sternum cleft is a rare congenital anomaly generallydiagnosed as asymptomatic at birth.[1] carried out the first surgical repairof a complete sternal cleft in 1988. Burton[8] published two casesof successful surgical repair by implanting a cartilage graft in thedefect in the bone in 1947. Subsequently, Maier and Bortone[16]carried out direct closure of two hemi-sternums in infants who were6 weeks old and this technique has become the reference.[8] Whethersymptomatic or not, the sternal cleft requires surgical correctionin the new born, when the flexibility of the chest wall is maximaland compression of underlying structures is minimal to restorebony protection to the mediastinal structures, establish normalintrathoracic pressure relationships, improve respiratory dynamicsand for aesthetic reasons.6] In the literature, repairs performedafter the age of 3 months have always required more supportivepostoperative care, with a higher incidence of cardiac complication.8] Beyond one year, autologous grafts  or prosthetic materials such as Marlexmesh, Teflon mesh, silicone elastomer and acrylic are required.6] Moreover, studies have suggested that it is always preferable touse autogenous tissue for sternal cleft repair and to avoid the useof prosthetic materials, recognising the risk of infection and thenegative impact of inert material on the patient’s development.8]Historically, Lannelongue[17] In addition, this surgical approach avoids major surgical dissection and the use of autogenous or bone bank rib reconstruction,which presents a potential risk of aseptic or septic necrosis of thebone.[18] What sets Parietex apart from other prosthetic material isthe density it can achieve from relatively little pressure. Most otherprosthetics take thousands of pounds of pressure to squeeze theminto soft shapes. The other benefits of using this type of prosthesisare its flexibility and conformity to the anatomy once implanted.The resorbable hydrophilic film promotes rapid parietal integrationto provide a temporary barrier and minimise visceral attachmentto mesh.[19] The nonabsorbable component provides stability andbridge the gap between the two sternal bars while avoiding directapproximation of both sternal halves and to prevent increasingintrathoracic pressure. It allows fast and complete tissue in-growthon one side for efficient reinforcement. A study by Casha et al.[20]evaluated and quantified the rigidity of sternotomy fixation using amechanical model through which six different fixation techniqueswere tested: figure of 8, straight, Ethibond, repair, Sternaband andmultitwist, and showed that Sternaband with its flat ribbon shape,similar to the cutting of the prosthesis in our technique, is moreresistant to cutting than wires. The force imparted by the lateral partof the closure can help bring direct approximation of both sternalhalves, providing stability. To the best of our knowledge, our caseis the first reparation of sternal cleft using composite mesh andsince this defect is so rare, we don’t have a vast experience on thissubject. However, this surgical approach with mesh prosthesis canbe indicated in many types of sternal clefts  to guarantee favourable long term results withreference to the stature growth, reproducibility, and adaptsbility toall surgical centres that train in general surgery.Despite the risk of infection described in the literature, the use ofprosthesis in sternal cleft repair has an advantage in preventing anincrease in intrathoracic pressure such as explained in our technicalnote.The neonatal period is the best period for surgical correctionof sternal clefts due to the elasticity of the sternum and minimalcompression of the underlying structures. All infants with recurrentpulmonary infection should be referred to surgeons for an earlyinvestigation to detect malformation of either the lungs or the chestwall to avoid delayed surgical repair. A considerable variety of sternalcleft repair procedures have been reported in the literature. However,our surgical procedure is safe and relatively easy, preventing anyimpact on the child’s stature and postural development.


Composite paragangliomas consist of two components, paraganglioma and ganglioneuroma, representing a rare subgroup of paragangliomas. The purpose of the study is to describe a case of composite paraganglioma of the celiac trunk and a brief review of the existing literature.A 64-year-old female patient with a history of epigastric abdominal pain and a 51 mm-diameter tumor found in a Computerized Tomography of the abdomen was admitted to our surgical department for further evaluation and treatment. After a brief preoperative surgical assessment, the patient underwent a mini-laparotomy for the excision of this tumor. After having the results of the pathology report, a comprehensive review of the international literature was carried out by applying the appropriate search terms.As it was found intraoperatively, the tumor was located at the cephalad aspect of the common hepatic artery, over the portal vein and the inferior vena cava. A negative-margin resection was achieved and the tumor was sent for pathology analysis. The final pathology report revealed a composite paraganglioma, with α paraganglioma and a ganglioneuroma component. Seventeen cases of extra-adrenal composite paraganglioma have been reported in the international literature so far. This case was the first one found in the area of the celiac trunk.Composite paragangliomas comprise rare and potentially malignant tumors with variable prognosis. Establishing their diagnosis promptly is of vital significance. Due to the first-described location of the composite paraganglioma in our case, the differential diagnosis of tumors in this area should also include composite paragangliomas. Pheochromocytomas are rare chromaffin catecholamine-secreting tumors, usually located within the adrenal glands. However, when these tumors arise outside of the adrenal glands, they are defined as paragangliomas. Paragangliomas may occur anywhere along the path of the autonomic ganglia, from the base of the skull to the urinary bladder. Composite paragangliomas, a subtype of paragangliomas, usually consist of the paraganglioma and the ganglioneuroma component. Composite paragangliomas are estimated to be around 3% of the adrenal paragangliomas, being very rare especially outside the adrenal glands . They arA 64-year-old female patient was referred to our outpatient department for surgical evaluation due to paroxysmal epigastric abdominal pain, with mild deterioration after movement and exercise. The patient's medical history referred that she underwent total thyroidectomy, with central and left lateral compartment dissection due to thyroid papillary cancer with nodal metastasis (pT3b(m)N1Mx based on The American Joint Committee on Cancer staging) one year before her admission to our clinic. Moreover, she had already visited a Gastroenterologist, who suggested her undergoing endoscopy of the upper gastrointestinal (GI) system and a Computerized Tomography (CT) scan. The report of the upper GI endoscopy referred that mild esophagitis and gastritis were present while the CT scan revealed a 51 mm-diameter solid mass at the site of hepatogastric ligament applying pressure on the abdominal aorta. Our findings during the initial physical examination and laboratory test were normal . Next, tPheochromocytomas are tumors which in their majority originate from the chromaffin cells of the adrenal medulla 85–90%) and less frequently from the extra-adrenal sympathetic nerve tissue, mainly paraspinal or paraaortic, called paragangliomas . They ca–90% and Ten percent of all the pheochromocytomas have a chance of malignancy, with extra-adrenal gland localization advocating a particularly high rate of malignancy and metastatic disease . On the Regarding composite paragangliomas, they are considered to be rare tumors. In about 70% of them, paraganglioma coexists with a ganglioneuroma component. They affect patients around 40 to 60 years old, with equal distribution across males and females . The sizFewer than 70 cases have been reported in the medical literature, most of which are located in the adrenal glands, while the extra-adrenal composite tumors have been reported only occasionally . In partComposite paragangliomas are often associated with familial neoplasm syndromes, such as neurofibromatosis type 1 (NF1) or multiple endocrine neoplasia type 2 (MEN2) . In mult123iodine-labeled metaiodobenzylguanidine (MIBG) may be helpful for cases in which CT or MRI are inconclusive, even though the pheochromocytoma has already been proven biochemically. Its specificity is reported to be between 82 and 92% and its sensitivity ranges widely, from 53 up to 94% (Imaging studies for the diagnosis of paragangliomas include CT or Magnetic Resonance Imaging (MRI) scan, while scintigraphy may be an alternative utility when these imaging tests fail to localize the tumor. Abdominal CT scan has an accuracy of about 85–95% for detecting tumors with a threshold size of 1 cm . Most ofp to 94% .In this case, we would like to emphasize the difficulty of establishing the diagnosis of paraganglioma preoperatively. The laboratory tests were indicative and the ultrasound imaging revealed a mass, without any special characteristic indicative of its origin. Moreover, an ultrasound-guided biopsy of this tumor suggested the diagnosis of a ganglioneuroma and not a paraganglioma. In addition, images obtained by CT scan were not characteristic of a paraganglioma, while CT-angiography just helped us identify the relations of the tumor with the celiac artery and the other structure of this area. As a result, the definite diagnosis of the composite paraganglioma was established only after the final pathology analysis of the excised tissue, and besides, it is very common to have the precise diagnosis for this kind of tumors only after the pathology examination have been completed .The prognosis of composite paragangliomas varies and depends on the existence of malignancy. For non-malignant disease, the 5-year survival rate is more than 95%. However, in patients with malignancy, the 5-year survival rate is <50% . Dhir etIn conclusion, composite paragangliomas comprise rare and potentially malignant tumors with variable prognosis. Establishing their diagnosis promptly is of vital significance. Based on our case, due to the first-described location of a composite paraganglioma near the celiac artery, the differential diagnosis of the tumors found in this area should include composite paragangliomas as well.GT and AMe were the chief investigators, wrote the manuscript, and collected the majority of the data. AC and AT conducted the histopathological and immunohistochemical analysis. IP and SA were the surgeons who conducted the excision of the tumor. II collected some additional data for the study. TP wrote and corrected the manuscript for its scientific basis. AMi was the director of the Department of Surgery and provided his permission for this study. All authors have read and approved the final manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer SM declared a shared affiliation with all the authors to the handling editor at the time of the review.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


En bloc resection of malignant tumors involving upper thoracic spine is technically difficult. We surgically treated a patient with grade 2 chondrosarcoma involving T1–5, left upper thoracic cavity, and chest wall.A 37 years old, male patient was referred to our hospital for a huge lump involved left shoulder and chest wall. In order to achieve satisfied surgical margins, anterior approach, posterior approach, and lateral approach were carried out sequentially. After en bloc tumor resection, the upper thoracic spine was reconstructed with a 3D-printed modular vertebral prosthesis, and the huge chest wall defect was repaired by a methyl methacrylate layer between 2 pieces of polypropylene mesh. Postoperatively, the patient suffered from pneumonia and neurological deterioration which fully recovered eventfully. At 24 months after operation, the vertebral prosthesis and internal fixation were intact; there was no tumor local recurrence, and the patient was alive with stable pulmonary metastases.This case report describes resection of a huge chondrosarcoma involving not only multilevel upper thoracic spine, but also entire left upper thoracic cavity and chest wall. Although with complications, en bloc tumor resection with combined surgical approach and effective reconstructions could improve oncologic and functional prognosis in carefully selected spinal tumor patients. En bloc resection has been proven as an effective treatment for aggressive benign, primary malignant, and solitary metastatic tumors involving lumbar and thoracic spine –3. SatisHere, we present a case of chondrosarcoma involving T1–5, left upper thoracic cavity, and chest wall. The details of surgical approaches, technique of en bloc resection, reconstruction of spine column, and repair of thoracic cavity have been described thoroughly.A 37 years old, male patient was referred to our hospital for a huge lump involved left shoulder and chest wall. The patient was suffered from severe radiating pain in the left upper extremity for 1 year. Physical examination showed decreasing sensation on the ulnar side of left hand, normal muscle strength in the upper extremity, and normal muscle strength and sensation in the lower extremity (Frankel D). The preoperative imaging findings (Fig. The patient was placed in the supine position, and a longitudinal roll was situated along the spine between the scapulas. The head was gently rotated toward the right. After preparing and draping the patient in the usual sterile fashion, a “trap door” exposure  was carrThe patient was positioned prone on the operating Table. A posterior midline incision Fig.  was madeThe patient was repositioned in the right lateral decubitus position. The upper left extremity was sterilized and draped on the operating Table. A single transverse incision connecting the ventral and dorsal sites was made on the fourth rib interspace Fig. . The preA nearly 3 kg tumor mass Fig.  was compTES which provides the best chance of local and system control for primary spinal malignant tumors has been developed for many years. With the help of modern techniques, wide surgical margin could be expected in some patients receiving spinal tumor surgery. In a review  of 103 pIn order to achieve satisfied surgical margins for spinal tumor resection, different approaches are recommended according to the scope of tumor invasion. Boriani et al.  have proWith aggressive multilevel TES, a large spinal column defect is created and needs effective anterior spinal column reconstructions. Widely used anterior reconstruction techniques include vascularized autograft strut, nonvascularized autograft strut, titanium cage, stackable carbon cage system, structural allograft strut, and PMMA . All theIn addition to spinal defect, this patient had also faced to the problem of large area of chest wall reconstruction. For patients with multi-level thoracic spinal tumors involving huge rib cage, large area of chest wall defects could cause severe respiratory complications, such as paradoxical chest wall movement and respiratory failure . SeveralWe successfully resected a huge chondrosarcoma involving T1–5, left upper thoracic cavity, and chest wall as one piece, and the patient fully recovered his neurologic compromise eventually. Although encountered complications, en bloc tumor resection with combined surgical approach and effective reconstructions could improve oncologic and functional prognosis in carefully selected spinal tumor patients.


Periosteal fasciitis (PF), a subtype of nodular fasciitis, is an uncommon benign soft-tissue mass that originates from the periosteum or tissues adjacent to bones. PF has rarely seen in children, especially involving in the mandible. This case report presents a rare case of PF originating from the periosteum of the mandible in an 11-year-old girl. She was referred to our hospital with fast-growing painless swelling in her left mandible. Computed tomography revealed an exophytic juxtacortical mass eroding the lower part of the left mandible and lower mandibular cortex with a periosteal reaction. The mass showed low signal intensity on T1-weighted magnetic resonance imaging (MRI) and high signal intensity on T2-weighted MRI. The apparent diffusion coefficient (ADC) of the lesion found to be moderate. Dynamic contrast-enhanced MRI revealed a gradual increment pattern in the central region of the mass. On 18F-fluorodeoxyglucose (FDG) positron-emission tomography/computed tomography (PET/CT), relatively high 18F-FDG uptake was observed on the early scan and the 18F-FDG uptake was declined on the delayed scan. The clinical and conventional radiological findings of the mass were suggestive of malignancy. However, the findings of ADC and dynamic MRI and dual-time-point FDG-PET/CT favored benign etiology over malignant etiology. Histological and immunohistochemical findings along with reactive ossification of the periosteum confirmed the diagnosis of PF. Currently, comprehensive examinations, such as clinical, imaging, and histopathological examinations, are recommended for the definitive diagnosis of PF, while MRI and dual-time-point FDG-PET/CT could have a potential usefulness to differentiate from malignancy. Nodular fasciitis (NF) is a rare and benign soft-tissue mass often misdiagnosed as a malignant neoplasm because of its fast and infiltrative growth pattern. It is considered a reactive process that involves the proliferation of mesenchymal origin cells, such as fibroblasts and myofibroblasts; however, the pathogenesis of NF remains unknown . NF was NF demonstrating a direct association with bone is termed periosteal fasciitis (PF). PF is a rare, benign, self-limiting condition that originates from the periosteum or tissues adjacent to bones and is considered a subtype of NF. It exhibits histological features similar to subcutaneous NF, constituting approximately 4% of all NF cases . AdditioThe imaging features of NF are non-specific and variable. Although NF is generally seen as a relatively well-defined, soft-tissue mass of superficial location on computed tomography (CT) and magnetic resonance imaging (MRI), there was no definitive image pattern for the diagnosis of NF in previous studies –6. Also,We report a case of PF in the mandible in an 11-year-old girl. To the best of our knowledge, pediatric cases of PF located in the mandible are rare, and this report may be the third report. In addition, we present and discuss the potential usefulness of MRI and dual-time-point FDG-PET/CT features in this case, to distinguish PF from malignant tumors, and eventually, in the diagnosis of PF.An 11-year-old girl was referred to our hospital for further examination and treatment of swelling in the left mandible. She noticed a rapidly growing painless swelling in the left mandible over 2 weeks. Physical examination revealed a hard mass in the left mandible with no pain or signs of acute inflammation. She had no history of trauma or other diseases. Panoramic radiography revealed a rounded radiolucent lesion with irregular border   of 3.7 Fig. a. IntereThe rapidly growing hard mass with radiological findings of bone destruction and high 18F-FDG uptake was suggestive of a malignant etiology rather than a non-neoplastic or benign lesion. However, the possibility of a benign lesion could not be discarded because the radiological findings of ADC and dynamic MRI and dual-time-point FDG-PET/CT favored benign etiology, such as non-ossifying fibroma, giant cell tumor, or Langerhans cell histiocytosis.A biopsy was performed one week before surgery and it was diagnosed as fibroma. The lesion was surgically excised. The extirpated spherical mass (diameter: 1.5 cm) was encompassed with the connective tissue. Bone-like hardness was partly palpable on the surface of the extrusive hemisphere toward the periosteum  was partially circumscribed with a thin shell-like bone at the periphery. Thick fibrous tissue attached to the bone surface, regarded as the periosteum, was observed Fig. b. The inThe swelling of the left cheek improved after surgical excision. At 1.5 years after the operation, a CT scan was performed to re-evaluate the status of the mass. CT scan revealed that the mass had disappeared and that the bone had occupied the area Fig. a, b. TheIn this report, we presented a case of PF in the mandible in a child. PF is considered a subtype of NF and is far less commonly reported. Few studies have been published on NF and/or PF in children, and notably, the occurrence of mandibular NF and/or PF in children is extremely rare. Bemrich-Stolz et al. reported 18 cases of NF in pediatric patients over a 12-year period . Among tTo the best of our knowledge, in pediatric population, only three mandibular PF patients, including our case, have appeared in the literature , 10. AllThe radiological (CT and MRI) findings of NF and/or PF are non-specific and variable. MRI of NF shows various signal intensities, probably because of the combination of variability in cellularity, amount of collagen, amount of cytoplasm and water content in the extracellular space, and vascularity in the individual lesion , 11. Theb-values of 500 s/mm2 and 1000 s/mm2 was higher than the standard value for head and neck malignancies, such as squamous cell carcinoma and malignant lymphoma [Dynamic contrast-enhanced MRI using gadolinium revealed increased heterogeneous enhancement. The time-intensity curves showed a gradual increment pattern in the central region of the mass and a pattern of rapid uptake followed by a gradual decrease in the peripheral region of the mass. The time-intensity curve pattern observed in the peripheral region of the mass is similar to that of malignant tumors; however, the gradual increment pattern in the central region of the mass was atypical for malignant tumors and favored benign lesions. Also, in the present case, the ADC of the lesion was suggestive of a benign etiology rather than a malignant etiology. ADC calculated from diffusion-weighted imaging using two lymphoma . HoweverFDG-PET visualizes glucose metabolism and is widely used to differentiate between benign and malignant lesions, as well as to stage/restage various malignancies. In a number of cases, the degree of 18F-FDG accumulation is used to differentiate between malignant and benign lesions. However, numerous other benign conditions, such as abscess, pulmonary granuloma, tuberculosis, and sarcoidosis, may also present with increased 18F-FDG uptake , 8; thusThe diagnosis of PF is challenging; however, the radiological characteristics of the present case, such as the time-intensity curves and ADC of MRI and dual-time-point FDG-PET/CT findings, could have potential usefulness in differentiating PF from malignancy and, thus, in the diagnosis of PF.Because the diagnosis of PF is difficult with clinical examination and imaging, histological examination is necessary to confirm the diagnosis. Histologically, PF shows features similar to NF. Allen identified four features that are common in nearly all cases of fasciitis: 1) presence of spindle-shaped fibroblasts that tend to be arranged in long fascicles, which are slightly curved, whorled, or S-shaped; 2) presence of small vascular clefts or slit-like spaces that often separate the fibroblasts; (3) extravasation of erythrocytes; and (4) presence of mucoid interstitial ground substance  presence, 17. In  presenceOnce the diagnosis of NF and/or PF has been made histologically, local excision is usually the appropriate treatment. After surgical excision, the prognosis of NF and/or PF is good and the recurrence is exceedingly rare , 11. In In conclusion, this article describes a rare case of PF originating from the periosteum of the mandible in an 11-year-old girl who exhibited clinical and radiographic features of a neoplastic disorder. The mass was surgically excised, and the diagnosis of PF was verified histologically. PF shares common clinical and radiographic features with neoplastic mandible tumors and is often misdiagnosed as a malignant tumor of the soft tissues. Incorrect diagnosis may lead to overtreatment, potentially causing disturbed orofacial development in children. In this article, we imply the potential usefulness of MRI findings  and dual-time-point FDG-PET/CT findings to differentiate PF from malignancy. Nevertheless, in contrast to neoplastic tumors, PF has a good prognosis after surgical excision. Therefore, PF should be included in the differential diagnosis when neoplastic lesions of the mandible are encountered.


Amyloidosis is a disorder of protein folding in which various proteins automatically aggregate into a highly abnormal fibrillar conformation. Amyloidosis is classified into systemic and localized forms depending on whether the abnormal proteins deposited in several different organs or only a single organ. In localized amyloidosis of the head and neck regions, laryngeal amyloidosis is common; however, localized amyloidosis of the nose is extremely rare. We herein report a case of localized amyloidosis of the nose and review the relevant literature on localized sinonasal amyloidosis. A 41-year-old man presented with a history of severe nasal obstruction, which had persisted for two decades. Nasal endoscopy and imaging studies showed extensive thickening of the bilateral nasal mucosa and diffuse submucosal deposition of calcification. After histopathological and systemic examinations, he was diagnosed with localized amyloidosis of the nasal mucosa. Septoplasty and bilateral inferior turbinoplasty, which consisted of mucosal resection using an ultrasonic bone curette, was performed and his symptoms markedly improved. Localized sinonasal amyloidosis has a good prognosis and surgical resection should be selected as a first-line treatment; however, clinicians should recognize the high probability of recurrence. Amyloidosis is a disorder of protein folding in which various proteins automatically aggregate into a highly abnormal fibrillar conformation . AmyloidAmyloid proteins can be deposited anywhere in the body . The depLocalized amyloidosis in the head and neck region is a rare entity , and locWe herein report a case of isolated localized AL amyloidosis of the bilateral nasal mucosa that was treated with debulking surgery using an ultrasound bone curette (UBC) and review the relevant literature on localized sinonasal amyloidosis.A 41-year- old man with a history of atopic dermatitis and allergic rhinitis visited our hospital with a complaint of severe nasal obstruction that had gradually worsened since childhood. He also suffered from hyposmia and watery nasal discharge. On nasal endoscopy, the nasal septum was deviated to the right, the nasal mucosa was smooth and pink with nodular thickening, and the common nasal meatus was so narrow that the bilateral middle turbinates or posterior part of the nasal cavity were not visible . Plain cIn order to improve his main symptom of nasal obstruction, septoplasty and bilateral inferior turbinectomy were performed at 4 months after the patient's first visit to our department. In the inferior turbinectomy, we applied the submucosal resection with powered instrumentation to preserve the ciliary function of the mucosa and to prevent postoperative crusting and bleeding. A mucosal incision was made in the anterior head of the inferior turbinate, the mucosa on the sides of the common and middle nasal meatus was detached from the bone, and submucosal resection was performed to remove as much thickened submucosal tissue as possible using a microdebrider. The bone of the inferior turbinate was not removed in consideration of the possibility of reoperation for recurrence of the lesion in the future. In septoplasty, the Killian approach with cartilage preservation was used and the deviated perpendicular plate of ethmoid bone and vomer were resected. The thickened submucosal tissues of the bilateral septal mucosa were reduced in the same manner as in the inferior turbinectomy. Since the submucosal tissue with severe calcification was very hard and difficult to resect with the turbinate blade of a microdebrider , an ultrWe only identified 21 case reports of localized amyloidosis originating from the nasal cavity and paranasal sinuses that included detailed patient information in 20 English-language articles published from 1990 to date , 14–32. In the 22 case reports included in our literature review , the meaRegarding the past medical history of the patients, 4 cases (18.2%) had no history and 3 cases (13.6%) had a history of nasal surgery. In addition, diabetes, hypertension, obesity, radiotherapy for a nasal cavity lymphoma, nasal trauma, rheumatoid arthritis, monoclonal gammopathy, and ear discharge were reported in one case each (4.5%). The past medical history was not described in 9 cases (40.9%) reported in 8 studies. Three cases (13.6%), including our case, had no relevant family history, and the remaining case reports did not describe the family history.n = 18; 81.8%), bleeding , rhinorrhea or nasal discharge . Facial swelling and olfactory impairment (including anosmia or hyposmia) were found in 4 cases each (18.2%). Mouth breathing, epiphora and hearing impairment [including aural fullness, described as “auricular plenitude” in the case report by Saas et al.  , 2 years in 4 cases (18.2%), and since early childhood in 2 cases (9.1%). One patient each (4.5%) reported that the duration of symptoms was several months, 3 years, 4 years, 7 years, 8 years, and several years. The duration of symptoms was not described in 4 reports.n = 20; 90.9%), maxillary sinus or antrum , ethmoid sinus , frontal sinus  and sphenoid sinus . Extra-nasal or extra-sinus extension to the sella, parasellar region, clivus, hard palate, pterygoid process and nasal bone were found in one case each (4.5%) in 4 case reports.The locations of lesions included nasal mucosa  , and right- and left-side regions in 5 cases each (22.7%).n = 4), pale pink or pale (n = 3), friable (n = 2), smooth (n = 2), soft to firm (n = 2), and non-tender (n = 2). Local examinations revealed adhesion (or synaechie) and nasal polyp in 3 cases each. Thickening of the mucosa of the nasal cavity, especially affecting the nasal septum and nasal conchae was reported in 5 cases (22.7%) and localized lesions in the nasal mucosa were reported in 2 cases, which were described as a partially yellow granular nodule (n = 1) and an erythematous irregular area (n = 1).Regarding the findings of local examinations, a mass in the nose was found in 14 cases (63.6%). The mass was characterized as erythematous , 21 (95.5%), and 10 (45.5%) cases, respectively. Of the 21 patients who underwent CT, all showed increased lesions of soft tissue density located in the nose or sinuses; findings included opacification, mass or mucosal thickening of the nasal meatus or sinus, and masses. Among them, calcification in the lesion was observed in 5 cases (23.8%), osteitic, osteotic, or reactive bone change were observed in 3 cases (14.3%), and destructive changes of the bone or osteolysis were observed in 4 cases (19.0%). MRI signal intensity of the amyloid legion was documented on T1-weighted images in five cases and T2-weighted images in six cases. For T1-weighted images, three of the five cases (60.0%) were hypointense and two were hypointense to isointense (40.0%); for T2-weighted images, three of the six cases were hypointense (50.0%), one was hypointense to isointense (16.7%), one was dominantly hyperintense (16.7%), and one was hyperintense in the periphery and hypointense in the center (16.7%).n = 1 each).Regarding the type of amyloid protein, 11 cases in 10 studies were subjected to an immunohistochemical examination. Among them, serum amyloid light protein (AL) was positive in 8 cases, including kappa immunoglobin light chain in 2 cases, lambda immunoglobin light chain in one case and both kappa and lambda in one case. The remaining 3 case reports described negative studies for AL, serum amyloid A protein (AA) and both (Seventeen cases (81.8%) were treated with surgery. Among them, 4 cases (18.2%) were treated bv diagnostic surgery or excisional biopsy alone. The remaining 13 patients underwent resection or debulking of the lesion, with sinus surgery performed as needed. In one case, chemotherapy  was administered prior to surgery, but no reduction was seen . ExternaThe mean follow-up period was 20.4 months .In this latest review of the relevant literature, we aimed to clarify the clinical features of localized sinonasal amyloidosis. From an epidemiological point of view, our review of the literature revealed that localized sinonasal amyloidosis occurs in a wide age range (8–81 years), has a female predominance , and tends to not be associated with family history. Regarding past medical history, three of the cases had undergone nasal surgery prior to the diagnosis of amyloidosis, which suggests that patients with undiagnosed amyloidosis are mixed in among those undergoing nasal surgery to improve nasal symptoms. The most common clinical symptoms were nasal obstruction (81.8%), epistaxis (40.9%), and nasal discharge 31.8%), in that order, and there no symptoms were specific for localized sinonasal amyloidosis. However, there were some cases with symptoms suggestive of severe inflammation or malignancy, such as headache, facial pain, visual impairment or cerebral fluid leak from the nostril , 29–31. .8%, in tOn local examination, sinonasal amyloidosis tended to present as erythematous, pale pink or pink, friable, smooth-surfaced masses. Localized mucosal lesions showed a yellowish, granular, irregular mucosa. A certain number of non-operative or early postoperative cases showed mucosal adhesions, which were thought to be a feature of amyloidosis.The imaging features of localized amyloidosis in the head and neck are not specific . On CT, In order to make a diagnosis of amyloidosis, the first step is to obtain histological evidence of amyloid, followed by looking for evidence of systemic amyloid deposition in the patient. The next steps are the detection and (in the case of AA and AL) quantification of the precursor proteins in the blood to confidently determine the type of amyloid . The preAfter the detection of amyloid, the type of amyloid should be identified by the immunohistochemical analysis of biopsy samples using specific antibodies . AL is tFor localized amyloidosis of the head and neck region, symptom-based management is recommended. If systemic amyloidosis is ruled out by appropriate testing, treatment may include observation in the absence of symptoms or conservative resection for symptom relief followed by annual follow-up for up to 10 years . In our Inferior turbinoplasty is an effective adjunctive procedure to septoplasty for patients with inferior turbinate hypertrophy, and surgical procedures involve resection, ablation or crushing of part, or all, of the turbinate to increase the size of the nasal airway . Among t2)  translocation is present  . Inducti2) . In inte present . Cycloph present . The com present . In high present . In cont present . In our  present , suggestRadiotherapy targeting the presumed clonal proliferation seems to be a useful treatment method for localized amyloidosis and is effective for stopping the progression of disease and improving symptoms caused by localized amyloidosis . In locaWe presented an extremely rare case of localized, idiopathic, primary nasal AL amyloidosis. Based on our review of the relevant literature, localized sinonasal amyloidosis shows a slow progression of nonspecific symptoms, such as nasal obstruction, epistaxis, and rhinorrhea, over a period of several years, and non-specific findings, such as mass formation in the nose or thickening of the mucosa; thus, clinicians should always suspect the presence of amyloidosis. The presence of adhesion of the nasal mucosa in the absence of previous surgery is a clue to the suspicion of amyloidosis. The only way to diagnose amyloidosis is to demonstrate amyloid deposits in the tissue by biopsy. CT often shows a fluffy appearance, which is an osteogenic reaction adjacent to sinonasal bone, or a large degree of punctuate calcification in soft tissue. MRI shows low to moderate signal intensity on T1-weighted images and variable signal intensity, ranging from low to high, on T2-weighted images. Chemotherapy is not effective for localized sinonasal amyloidosis, and the first-line treatment is resection or debulking of the lesion without impairing the sinonasal function; however, long-term follow-up is necessary because recurrence is frequently observed after treatment.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.The studies involving human participants were reviewed and approved by Ethics Committee, Toyama University Hospital. The patients/participants provided their written informed consent to participate in this study.HTak, HTac, KT, and HS: conception and design. HTak, HTac, KT, and JI: literature search and obtaining of images. HTac, JI, and HS: writing the article. All authors critical revision and final approval of the article.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.