Adenocarcinoma originating from chronic perianal fistulas is a rare disease. Due to the lack of evidence of this malignancy, no definitive diagnostic and treatment guidelines have been established. We present two cases of anal fistula cancer and discuss the diagnostic and treatment strategies.A 79-year-old man was referred for a perianal mass. The patient had a history of chronic perianal fistula and was diagnosed with adenocarcinoma originating from a long-standing perianal fistula. The patient underwent laparoscopic abdominoperineal resection (APR) without any adjuvant therapy. The second case was a 42-year old man, who was referred for a prolapsed anal mass. The patient had recurrent perianal fistula over several years. He was diagnosed with mucinous adenocarcinoma originating from a long-standing perianal fistula, but he initially refused surgical treatment. Two years later, the patient revisited with a huge overgrown tumor and underwent laparoscopic APR with wide perineal skin excision, wound debridement and coverage with a bilateral V-Y advancement flap via a one-step procedure.High degree of clinical suspicion is crucial to diagnose this rare disease that can easily be missed at an early stage. Depending on the disease stage, surgical treatment, chemotherapy, and radiotherapy can be considered.Histopathological evaluation should be performed on recurrent and incurable anal fistulas over a long period of time. Intensive surgical treatment with or without neoadjuvant and adjuvant therapy should be considered in advanced cases. It is e22.1A 79-year-old male presented with symptoms of anal pain, change in bowel habits, and perianal mass with anal discharge for one year, and was referred to our hospital. He had a history of undergoing necrotherapy for recurrent internal hemorrhoids 40 years ago. Physical examination revealed a large irregular perianal ulcerated mass with multiple fistula tracts . During 2.2A 67-year-old man with prolapsed anal mass, who had a medical history of hypertension, diabetes mellitus, and endoscopic submucosal dissection (ESD) for early gastric cancer was referred to our hospital. He had suffered from recurrent fistula in the anus over several years and had been symptomatic with anal pain and discharge. Physical examination revealed external openings at 6–9 o'clock position around the anus. Before coming to our clinic, he underwent incision and drainage with biopsy at a local hospital, and pathology revealed adenocarcinoma with mucinous differentiation. We planned APR with the possibility of a malignancy of chronic fistula, but the patient refused surgery because of fear of excision of anal sphincter and permanent stoma. Two years later, the patient revisited our clinic with a rapidly growing perianal mass and aggravation of symptoms. The mass extended widely in the perineum and buttock areas . MRI sho3Although anal fistula is a common benign disease in coloproctology, perianal adenocarcinoma associated with chronic fistula-in-ano is rare, and has been reported in approximately less than 200 cases in the literature . TherefoPatients are usually diagnosed at an advanced stage because symptoms are similar to benign anal diseases and progress slowly. Endoscopic findings cannot usually provide definite diagnostic evidence as the tumor does not pierce the rectal mucosa. Rosser C. was the first to publish seven cases of anal fistula cancer in 1934; thereafter, several researchers have reported case series and established the following three diagnostic criteria for malignancy of anal fistula: (1) the fistula should usually antedate the carcinoma by at least 10 years, 2) the only tumor present in the rectum or anal canal should be secondary to direct extension from the carcinoma in the fistula, and (3) the internal opening of the fistula should be into the anal canal and not into the tumor itself  the only,13. The Surgical resection has been considered the standard treatment option. APR is the most preferred surgical method, and in advanced cases, wide excision of surrounding tissues including the overlying skin is necessary in order to secure a negative resection margin. In advanced cases, chemotherapy, radiotherapy, and chemoradiotherapy (CRT) can be considered as neoadjuvant or adjuvant therapy, however, its effectiveness remains controversial. According to a study of 14 patients with anal fistula malignancy conducted by Gaertner et al., neoadjuvant chemoradiotherapy or adjuvant chemotherapy was found to be an effective treatment option for these patients, as 7 of 14 patients who received neoadjuvant CRT in this study had a complete response . Hongo eAccording to previously published literature, metastases to lymph nodes may be present in advanced cases, and inguinal lymph nodes are the most frequent sites of metastasis, whereas distant metastasis is uncommon. Gaertner et al. reported that 6 of the 14 patients (43%) had nodal involvement, and suggested that nodal involvement was associated with decreased survival . The proIn our case series, both patients were found to meet the diagnostic criteria, and the diagnosis was confirmed by preoperative histological examination. One patient was identified as a case of mucinous adenocarcinoma, and the other was identified as a case of adenocarcinoma. No lymph node metastasis was observed in either of the patients. One patient received only APR, and the other received adjuvant chemotherapy after curative resection. Several studies have reported that mucinous carcinomas have a poorer prognosis than non-mucinous tumors in colorectal cancer ,18. Howe4To summarize, high degree of clinical suspicion is crucial to diagnose this rare disease that can easily be missed at early stage. Besides, it should be noted that fistulectomy for preventing purpose and the histopathological evaluation should be performed on recurrent and incurable anal fistulas over a long period of time. These tumors have a high potential for local recurrence, therefore, curative surgical treatment should be carried out, and multimodal approaches including neoadjuvant or adjuvant CRT should also be considered in advanced cases. Furthermore, large-scale studies should be performed to establish knowledge related to the treatment and prognosis of this disease.The authors report no declarations of interest.This paper did not receive any specific grant from funding agencies in the public, commercial, or not-for profit sectors.Sang Hyun An: Nothing to declare.Ik Yong Kim: Nothing to declare.This is case report study and ethical approval not required.Written informed consent was obtained from the patients 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.The following statement applies for all listed authors:This case report does not require registration.This is a case report.Ik Yong Kim, M.D., Ph.D.Not commissioned, externally peer-reviewed.Sang Hyun An: Writing - original draft, Writing - review & editing. Ik Yong Kim: Conceptualization, Data curation, Project administration, Supervision.


Subgaleal abscess is a rare condition in which suppuration under the galea aponeurotica  occurs. Diagnosis of subgaleal abscess is guided by high index of suspicion, imaging of the head by computed topography will confirm the diagnosis and detect osteomyelitis or intracranial extension.We describe a case of extensive subgaleal abscess that presented as an infected scalp sebaceous cyst, in male diabetic patient, its management and the role of vaccum assisted closure (V.A.C.) dressing which was never described in such atypical presentation.4 The predominant organism isolated from post-traumatic and post-surgical scalp infections is Staphylococcus aureus . Diagnosis and management will be guided by head CT scan, management of subgaleal abscess should focus on effective intravenous antibiotic therapy, immediate surgical drainage of abscess and debridement of necrotic tissue.The subgaleal space is the between the galea aponeurotica and periosteum of the cranial bones, subgaleal abscess may result from hematogenous infection or contiguous spread, and the diagnosis may not be initially obviousSubgaleal abscess is a rare complication, imaging can prevent serious complications. Negative pressure wound therapy is an invaluable tool for closure of the wounds. AbscessThe work has been reported in line with the SCARE criteria and cite the following paper .2A 53 years old diabetic man presented with progressive scalp swelling and headache for 2 weeks, associated with fever. Upon examination the swelling is located at the vertex of skull, with an opening with purulent discharge with cellulitis around the lesion. On examination the swelling is doughy in consistency with no fluctuation, cystic lesion is felt in the middle of the wound. The patient underwent excision of the infected cyst, debridement and drainage of an abscess. Tissue pathology revealed an infected sebaceous cyst and Staphylococcus Aureus was cultured. During post-operative period he improved slightly and cellulitis subsided.On post-operative day 3, the wound was still discharging copious amount of pus though the patient was not febrile. A computed scan of the head revealed subgaleal collection with air pockets and evidence of outer plate of skull bone osteomyelitis . Patient3The subgaleal space is the between the galea aponeurotica and periosteum of the cranial bones, it is a potential space.The subgaleal space is a very extensive area spanning all of the cranial convexity. It is delimited circumferentially by the attachments of the galea aponeurotica: anteriorly to the bony orbital ridge bilaterally; laterally to the zygomatic arch and the auricular muscles on each side; and posteriorly to the nuchal line .Diagnosis of abscess formation in this space is usually related to postoperative complications and encountered in some operative procedure to the head, ears or sinuses. Infection can extended from other close foci, in this case the subagaleal collection can be missed if not suspected. Serious complications can arise if subgaleal involvement is not diagnosed early, skull bone osteomylitis and intracranial extension are difficult clinical scenarios.However, subgaleal abscess may result from hematogenous infection or contiguous spread, and the diagnosis may not be initially obvious .Staphylococcus aureus [Eikenella corrodens [Staphylococcus epidermidis accounted for 58% of positive cultures in one review [The predominant organism isolated from post traumatic and post surgical scalp infections is s aureus . Howevers aureus  and Eikeorrodens  have beee review . Polymice review .Head CT is often needed in the diagnosis of subgaleal abscess, and if operative debridement is not promptly performed, subgaleal abscess may further progress to life-threatening septicemia, osteomyelitis, and even subdural or brain abscess or meningitis .Immunocompromised patients such as diabetics are prone to recurrent infections especially in the skin; managing such infections early and aggressively reduces the risk of extension of these infections to adjacent potential spaces. They should have an urgent brain imaging and urgent management.Management of subgaleal abscess should focus on effective intravenous antibiotic therapy, immediate surgical drainage of abscess and debridement of necrotic tissue. Culture of the infected tissue to identify the causative organism is extremely important with the emergence of resistant microorganisms.Finally, we experienced an impressive result using the negative pressure wound therapy (V.A.C). This type of dressing uses a vacuum machine to apply a negative pressure of -80 to -120 to the wound. It removes all the liquid or semi-liquid infective or necrotic material from the wound and improves vascularity to the tissue, which in result aid in regeneration of healthy granulation tissue. Our patient had the advantage of reusable pocket size VAC therapy and was discharged on repeated changes in the dressing clinic every three days for five weeks. The wound improved dramatically and the skin defect healed by secondary intention.4Subgaleal abscess is a rare complication of infected scalp skin cysts. Early diagnosis and imaging can prevent serious complications, osteomyelitis, and even subdural or brain abscess. Negative pressure wound therapy is an invaluable tool for closure of infected scalp wounds and will help in tissue regeneration and infection clearance.No potential conflict of interest relevant to this article was reported.There are no sources of funding for this research.Not applicable. The study is exempt from ethical approval in our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is submittedNo identifying details have been used in the article.Dr. Mamoun A. Nabri, Dr. Mohammed Alharbi contributed to the paper by collecting all important data and information pertaining to the case.Dr. Amnah Al-Sayyid, Dr. Hussam Al-Jehani and Dr Khairi Hassan contributed to the paper by reviewing the final manuscript.Kawthar Alabdrabalrasol contributed to the paper by reviewing all the available literature related to the case.Not applicable.Dr. Mamoun A. Nabri.None funded, externally peer-reviewed.


Acute appendicitis is a mimicker of a wide range of gastrointestinal and genitourinary pathologies. The diagnosis becomes more challenging when it is associated with intestinal malrotation. A rare case of left-sided acute appendicitis with asymptomatic undiagnosed intestinal malrotation is reported. A 32-year-old male without known comorbidities presented with left-sided abdominal pain. Abdominal ultrasonography and computerized tomography scans showed intestinal malrotation with acute appendicitis. Exploratory laparotomy and appendectomy with Ladd’s band release via midline incision were performed, and the patient had no issues on follow-up. Given the rarity of acute appendicitis associated with intestinal malrotation, an increase in awareness of this anatomical variant is essential among emergency physicians, radiologists, and surgeons for prompt diagnosis and timely intervention. Abdominal pain is one of the most common complaints for emergency visits. Acute appendicitis is the most common surgical cause of acute abdominal pain with overall lifetime risks for males and females being 8.6% and 6.7%, respectively . The preLeft lower quadrant pain caused by acute appendicitis can be due to a right-sided long appendix projecting into the left lower quadrant area and an abnormal left-sided location of the appendix . The abnWe report a case presenting with progressive and constant pain in his left lower abdomen which we presumed to be due to diverticulitis. However, abdominal ultrasonography (USG) and computerized tomography (CT) scans revealed left-sided acute appendicitis with intestinal malrotation, which significantly changed clinical management. He did well postoperatively and was discharged after a successful recovery.A 32-year-old gentleman without known comorbidities presented to the emergency department of Tribhuvan University Teaching Hospital, Maharajgunj, with complaints of insidious, progressive, constant pain in his left iliac fossa without radiation for two days. It was accompanied by nausea, vomiting, and anorexia. There was no history of hematochezia, melena, or fever, and his bowel and bladder habits were normal. He had no prior history of surgeries, drug abuses, or drug allergies. The patient was initially evaluated in a previous center for the same complaint where he was suspected as having diverticulitis.2 sizes , normal liver and renal function tests, and positive C-reactive protein. Urinalysis was unremarkable. The chest X-ray showed no abnormalities. The intraoperative findings were inflamed appendix with appendicolith with healthy base arising from the cecum which was fixed in the left lower quadrant with minimal free fluid. The duodenojejunal flexure with a whole small bowel was seen on the right side and the large bowel was seen on the left side Figure . Ladd’s A person with acute appendicitis typically presents with vague epigastric or periumbilical pain which later migrates to the right lower quadrant, anorexia, nausea, vomiting, and low-grade fever. While examining patients with such symptoms, we usually come across rigidity, positive psoas sign, fever, and rebound tenderness . These cDifferentials of left lower quadrant pain include colitis, diverticulitis, inflammatory bowel disease, intestinal obstruction (including volvulus) or perforation, nephrolithiasis, pyelonephritis, testicular torsion, epididymitis, atypical right-sided appendicitis, left-sided appendicitis, and left-sided primary epiploic appendagitis ,8.In the present case, we initially suspected diverticulitis. The underlying intestinal malrotation delayed the correct diagnosis of acute appendicitis. It was only after the CT scan was done malrotation was identified. Two previous case reports have similarly misdiagnosed left-sided acute appendicitis with intestinal malrotation as diverticulitis ,10. In tIntestinal malrotation occurs when there is either nonrotation or incomplete rotation of the primitive intestinal loop around the SMA axis during the first 10 weeks of fetal life . It is aAcute appendicitis occurs even rarely in association with intestinal malrotation ,17. ThisIt is imperative to differentiate left-sided acute appendicitis associated with malrotation from that associated with situs inversus totalis, in which every organ, including duodenum, duodenojejunal junction, small and large bowel, cecum, and appendix, is located in a mirror position to situs solitus . In addiUSG is considered to be the best initial imaging modality, especially for children. USG may show an inversion of SMA and SMV relationship with a blind noncompressible aperistaltic tube on the left side. CECT of the abdomen may also show an inversion of SMA and SMV, along with large bowel predominantly on the left side, small bowel predominantly on the right side, and concomitant feature of acute appendicitis . CT is uLadd's procedure is generally performed when intestinal malrotation presents as a volvulus by an open or laparoscopic approach. However, this procedure becomes optional when malrotation is found incidentally or with other intra-abdominal pathology like acute appendicitis. Laparoscopic appendectomy is considered to be the gold standard of treatment of left-sided appendicitis . But unlGiven the rarity of acute appendicitis associated with intestinal malrotation, an increase in awareness of this anatomical variant is essential among emergency physicians, radiologists, and surgeons for prompt diagnosis and timely intervention. Although the clinical picture may be confusing, imaging modalities greatly help in diagnosing this condition. Appropriate and timely intervention could minimize the risk of detrimental consequences like intestinal perforation/abscess formation and prolonged hospital stay.


Retrorectal cysts are rare congenital cystic lesions usually diagnosed in middle-aged women. They are generally asymptomatic; however, local pressure may result in complications. Pain or discomfort in the pelvic, sacral, lower back or perianal area are the most common symptoms. The diagnosis is difficult, and multidisciplinary management is required. An epidermoid cyst is the most common type. Surgical resection is the main treatment, and 3 operative approaches are commonly used: abdominal, trans-sacral, and combined abdominosacral. The selection of the approach depends on the nature and location of the lesion. Here, we present a case that demonstrates the trans-sacral approach to a retrorectal cyst is a feasible option in terms of safety and minimal invasiveness for selected patients with this rare type of retrorectal cystic lesion.A 38-year-old woman with no comorbidities incidentally showed a retrorectal cyst on magnetic resonance imaging performed during pregnancy. The patient underwent surgical resection under general anesthesia, trans-sacral incision was performed, the posterior rectum exposed, and the cyst removed. No complications were seen in the postoperative period.Posterior trans-sacral resection (Kraske) is preferred for patients with posterior retrorectal cyst because it provides adequate exposure.en bloc resection.Posterior trans-sacral resection allows proximal extension for elimination of the infection and in cases of adherence of the cyst to surrounding structures or in malignancy, which require  The maj2A 38-year-old woman not known to have any medical illness presented to our clinic with a retrorectal cyst that was incidentally found during her pregnancy. She complained of difficulty in voiding. She denied any history of change in bowel habit, hematochezia, and obstructive urinary symptoms. Upon examination, the patient was vitally stable and afebrile. Local examination revealed a palpable bulge at 12 o’clock position extending 5 cm into the coccyx. Abdominal examination, including per rectum examination was unremarkable.Routine laboratory tests, including complete blood count, hepatic and renal function tests, and coagulation profile, were all within the normal range. Serum tumor markers, including carcinoembryonic antigen and alpha-fetoprotein, were all within the normal range. Computed tomography (CT) of the abdomen revealed a unilocular non-enhancing low-attenuation mass at the retrorectum measuring 5.4 × 4.6 cm .Fig. 1A:MRI confirmed a well-defined homogeneous high T2 intermediate to high T1 signal intensity structure at the lower pelvis, retrorectal in location, approaching the level of the anal canal. Anterior displacement of the rectum was seen with no signs of rectal wall signal alteration to suggest infiltration. Abutting of the inferior margin of the coccyx was seen with no spinal extension or bone destruction .Fig. 2A:Under general anesthesia, the patient was placed in the jackknife position. A trans-sacral incision was made , and the3Retrorectal cysts, which are rare and usually affect middle-aged women, are typically asymptomatic; however, some patients may present with symptoms caused by the local effects of the cyst, including constipation, rectal fullness, and lower abdominal pain with a retrorectal mass palpable on digital rectal examination . RetroreIn mayo clinic experience done over 120 patients showed sigmoidoscopy was useful for large tumors but in the small lesions was negative . In a caComplete surgical resection is the treatment of choice owing to the risk of complications, such as recurrence, local symptoms, and malignancy . Differe4en bloc resection.Trans-sacral excision is a feasible option and safe to perform. It is a minimally invasive and safe option. It allows proximal extension for elimination of the infection and in cases of adherence of the cyst to surrounding structures or in malignancy, which require The authors declare no conflict of interest.This study did not receive any funding from governmental or private organizations.Ethical approval was obtained from the Institutional Review Board of the King Fahad Specialist Hospital, Dammam, Saudi Arabia.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.Study concept or design – AB, TMS, KS.Data collection – AB, TMS, MYD, FFQ.Data interpretation – AB, TMS, MYD, FFQ.Literature review – AB, TMS, MYD, FFQ.Drafting of the paper – AB, TMS, MYD.Editing of the paper – AB, TMS, MYD, KS.Not required.Tlal Matouq Alsofyani.Not commissioned, externally peer-reviewed.


Primary chondrosarcomas of the nasal septum are rare, with a variety of clinical features that evade detection and treatment. While endoscopic surgery has become increasingly accessible, open approaches may be needed to provide adequate visualization for tumour ablation and reconstruction. We report the resection and reconstructive considerations of a septal chondrosarcoma.A 75-year-old woman presented with a 3-year history of a slow growing, firm mass in the nasal tip causing protrusion and septal fullness. Computed Tomography scan of the paranasal sinuses revealed a well-circumscribed, 2.2 cm mass at the anterior nasal septum extending into the right vestibule. Biopsy of the cartilaginous lesion confirmed the diagnosis of a low-grade chondrosarcoma by histopathology. The tumour was removed using a transcolumellar open rhinoplasty approach with a large septal resection. Primary reconstruction of the surgical defect was performed using an L-shaped strut from the nasal keystone area to the columella. Follow-up examinations demonstrated no evidence of recurrent disease with satisfactory functional and cosmetic outcomes at 1-year.This report describes a case of nasal septal chondrosarcoma successfully treated with surgical excision using an open rhinoplasty approach. Only 5–10% of chondrosarcomas are located in the head and neck region and arise rarely in the nasal septum in approximately 2–4%. With this mass, an open rhinoplasty approach was required to allow optimal exposure of the margins and to facilitate reconstruction without disruption of normal sinonasal anatomy and function. Although rare, chondrosarcoma of the nasal septum should be considered in the differential diagnosis of nasal masses. Chondrosarcoma is a heterogeneous group of tumors that arise from cartilage-producing cells, most frequently involving the pelvis, femurs, and ribs . Head anRecently, endoscopic surgery has emerged as a less invasive option for removal of nasoseptal chondrosarcomas. In the case reported here, the extensive involvement of the cartilage and large resection of the septum necessitated an open rhinoplasty approach to provide adequate exposure for ablation and to facilitate primary reconstruction. The aim of this report is to highlight that while endoscopic techniques are expanding, open surgical approaches may still be required to provide adequate oncologic control and cosmetic considerations. We also provide a summary of the nasoseptal chondrosarcoma literature, including diagnosis, management, and prognosis.This manuscript was prepared in accordance with the (CARE) guidelines for the reporting of case reports .A 75-year-old female was referred to the head and neck oncology clinic with a 3-year history of slow-growing mass in the nasal tip that resulted in bulbous tip protrusion and septal fullness. She denied other sinonasal symptoms such as nasal obstruction, pain, discharge, hyposmia, and epistaxis, as well as any associated ocular or neurological changes. Her medical history consisted of hypertension, platelet dysfunction, previous pulmonary embolism, and osteoarthritis; she was a life-time non-smoker. Bimanual examination revealed a firm, nontender mass in the caudal septum palpable on both sides, and no other lesions were identified by flexible endoscopic examination. Her physical examination was otherwise unremarkable, with no palpable cervical lymphadenopathy.Computed tomography (CT) of the paranasal sinuses demonstrated a well-circumscribed lesion arising from the anterior cartilaginous nasal septum. The mass measured 2.2 × 1.5 × 1.0 cm and extended into the right vestibule. There was no evidence of bony erosion . Chondrosarcomas are capable of local invasion and destruction of adjacent structures, and late stages may affect multiple surrounding sites. The majority of tumors grow slowly and are often large at initial detection. The clinical presentation of nasoseptal chondrosarcoma depends on the structures involved, such as the nasal cavity, orbit, paranasal sinuses, Eustachian tubes, or other craniopharyngeal or intracranial compartments :1. Chond. The mosImaging studies are essential to evaluate the osseous and soft-tissue characteristics of the tumor, as well as the extent of its growth to guide appropriate preoperative planning. The most commonly used modalities are CT and MRI. Radiographic findings may demonstrate calcification and cortical bone erosion. Chondrosarcomas typically appears as hypoattenuating matrix with the presence of scattered small, finely speckled, amorphous ring-forming calcifications , as well as foci of associated erosion of the septum and adjacent bony structures , 16. HowSurgical resection remains the primary modality of treatment for these tumors. Goals of treatment follow oncological and reconstructive principles and include disease cure, restoration of function, and maintenance of cosmetic outcomes. With surgery, clearance of tumor margins is essential, as the rate of chondrosarcoma recurrence with surgery alone has been reported to increase with positive margins . While tRadical surgical resection is the mainstay initial treatment of nasoseptal chondrosarcomas. The major cause of mortality is uncontrolled locoregional disease and not metastasis; for low-grade chondrosarcomas, complete excision of the tumor with clear margins may be curative . Wider sWith recent advancements of endoscopic endonasal surgery, tumors restricted to the nasal cavity have been shown to be amenable to endoscopic excision with clear margins , 20–24. However, characteristics of particular lesions may necessitate an open approach to ensure clear margins and allow for appropriate reconstruction. Due to the location and size of some tumors, the endoscopic approach may not allow adequate visualization for ablation and reconstruction. In the case reported here, the substantial septal resection necessitated primary reconstruction to restore function and offer an acceptable cosmetic outcome. Furthermore, due to the extreme anterior nature of the lesion, surgery performed with traditional endoscopic instruments was expected to be limiting. The open rhinoplasty approach allowed for excellent visualization of the tumor and facilitated the placement of the L-strut for reconstruction. Camouflaging techniques were also employed through the open approach. Both sinonasal function and cosmesis were excellent at follow-up, and the patient remains disease free.The overall survival of sinonasal chondrosarcoma ranges from 44 to 87% . Local rAlthough rare, chondrosarcomas of the nasal septum may present with a wide range of sinonasal symptoms and can involve adjacent structures. This case demonstrates a chondrosarcoma with extensive involvement of the septum, requiring an open rhinoplasty approach and primary reconstruction. While endoscopic surgery has recently become a feasible option for lesions limited to the nasal cavity, conventional open surgical approaches should be kept in mind for challenging tumors.


Colorectal cancer liver metastasis (CRCLM) concomitant with infiltration of lymph nodes (LNs) in the hepatic pedicle is difficult to manage, and is regarded as an extrahepatic metastasis; undertaking hepatectomy is controversial in such a scenario.Case 1, A 55-year-old woman was diagnosed with multiple liver metastases arising from rectal cancer along with enlargement of a retropancreatic LN. A characteristic image prior to hepatectomy demonstrated evident tumor progression from a metastatic lesion in segment 5 of the liver (S5) to the hepatic hilum along with Glisson 5. Post-operative histopathological examination revealed viable adenocarcinoma cancer cells originating from rectal cancer in all the liver metastatic lesions and retropancreatic LN.Case 2, A 89-year-old woman was diagnosed with transverse colon cancer with multiple liver metastases. Radiological examination before hepatectomy revealed tumor progression from a metastatic lesion in the segment 6 of the liver (S6) to the hepatic hilum along with Glisson 6 and LN involvement in the hepatoduodenal ligament. Post-operative histopathological examination demonstrated adenocarcinoma cancer cells in the liver metastatic lesions and in a hepatoduodenal LN.When encountering tumor progression from liver metastatic lesions to hepatic hilum along with its Glisson branch, the possibility of hepatic hilar LN involvement should be considered. Indeed, the surgical management of CRCLM with hepatic hilar LN involvement is controversial, but could be acceptable if the positive LNs are limited to the hepatic pedicle and retropancreatic area. Indeed, it is frequently considered as a contraindication for hepatectomy because of the poor prognosis . HoweverWe report two cases of CRCLM with hepatic hilar LN involvement. Both the cases had characteristic radiological findings indicating the possible involvement of the hepatic hilar LNs. We would like to especially focus on the characteristic preoperative images and the mechanism of metastasis from metastatic liver lesions to hepatic hilar LNs.This work has been reported in line with the SCARE criteria (Agha) .22.1A 55-year-old woman was referred to our institution with multiple liver metastases from rectal cancer along with the enlargement of a retropancreatic lymph node. She was treated with laparoscopic high anterior resection of the rectum, followed by chemotherapy in the previous hospital. Pathological examination revealed a stage IVA  rectal cancer based on the 8th edition of the Union for International Cancer Control (UICC). On comparison of the pre- and post-chemotherapeutic computed tomography (CT) images, the size of the liver metastasis in segment 8 of the liver (S8) had reduced from 45 mm to 40 mm, metastasis in S4 reduced from 44 mm to 30 mm, and metastasis in S5 reduced from 35 mm to 28 mm following chemotherapy ; V-a∼e. 2.2A 89-year-old woman diagnosed as having transverse colon cancer with regional LN metastases and multiple liver metastases  was transferred to our institution. Therefore, we decided to perform colorectomy first, followed by hepatectomy as a secondary surgery considering the patient’s poor physical status. Pathological examination after laparoscopic transverse colorectomy with regional LN dissection indicated a stage IVA  transverse colon adenocarcinoma based on the 8th edition of the UICC. Subsequent to colorectomy, radiographic images for re-evaluation revealed additional liver metastatic lesions in S3 in addition to the pre-existing ones in S6 and S7 , along with suspected tumor progression along Glisson 6, which probably came from the adjoining S6 metastasis ; I-a∼c. 3We report two cases of CRCLM with hepatic hilar LN involvement; these have helped us gain useful insight into the treatment of CRCLM patients.Firstly, detection of positive LN involvement around the hepatic hilum itself is not so simple. Initially, hepatic hilar LN involvement by means of CT and MRI scans was not evident, because of the small size. Even if LNs around the hepatic hilum could be detected, it is very difficult to distinguish tumor positive or negative LNs, because it is not unusual to notice enlarged LNs in this area. Only after conducting a PET evaluation, we could detect hepatic hilar LN involvement. Certainly, PET is supposed to be one of the most powerful tools for detection of cancer metastasis, but it is available only in certain specific institutions such as high volume hospitals. Therefore, it is important to find radiological evidence of possible LN metastases around the hepatic hilar area with a CT or MRI in cases of CRCLM.Secondly, the issue of whether the hepatic hilar LN involvement arises from the primary colorectal tumor lesion or the liver metastatic site as second-degree metastases, remains unclear. It is probable that the metastases to the hepatic hilar LNs originated from the liver metastases and not from the primary colorectal tumor lesion in the above two cases ,5. As shThirdly, hepatectomy with lymphadenectomy in patients with CRCLM together with hepatic hilar LN involvement should be considered as one potential therapeutic approach, especially if positive LNs are limited to the area around the hepatoduodenal ligament and the retropancreatic area. Jaeck et al. and Adam et al. emphasized that the location of the positive LNs significantly affected the survival rate ,6. Accor4When tumor progression is encountered from liver metastatic lesions to the hepatic hilum along its Glisson branch, the possibility of hepatic hilar LN involvement has to be considered. Indeed, surgical management of CRCLM with hepatic hilar LN involvement is controversial, but could be acceptable if the positive LNs are limited to the hepatic pedicle and retropancreatic area.None of the authors have any commercial or financial involvement in connection with this study that represents or appears to represent any conflicts of interest.None.Case reports are exempted from ethical approval according to our institution policies.Written informed consent was obtained from both of the patients for publication of this Case report and any accompanying images.Kawasaki Y, Satoshi I and Idichi T: Conception of reporting case, data recording, and drafting.Kawasaki Y, Satoshi I and Idichi T: Management of case.Kawasaki Y, Sakoda M, Tanonue K, Kurahara H, Mataki Y, Maemura K, Ueno S and Natsugoe S: Critical revision of article and final approval of the version to be submitted.Not applicable.Kawasaki Y.Not commissioned, externally peer-reviewed.


Malrotation of gut is a congenital anomaly of foetal intestinal rotation and it is mostly discovered in early childhood as acute intestinal obstruction. This condition is very rare and often silent in adults.We present a case of 44-year-old woman admitted with acute abdominal pain. The abdominal CT scan showed ileal conglomerate loops with thickened walls filled of liquid in the right iliac side. Laparotomic Ladd's procedure was performed successfully with division of Ladd's band, adhesiolysis, reconstruction of Treitz's ligament, small bowel resection and Merckel's diverticulum removal. The patient made an unremarkable recovery.We discuss the rarity of intestinal malrotation in adulthood and made a literature review comparison of the therapeutic options available.Intestinal malrotation is a rare entity and adult presentation is even rarer. Some cases are asymptomatic, but when symptomatic a volvulus should be promptly suspected to avoid complications such as bowel ischemia. Intesti2A 44 year old woman attended the emergency department, complaining acute abdominal pain in periumbilical, bilateral iliac and hypogastric regions. The routine blood tests showed: WBC 13120, Hemoglobin 11 g/dl, HCT 33,5, CRP within normal limit. She was admitted and managed with nasogastric tube decompression and intravenous fluid hydration for a suspected bowel obstruction. X ray abdomen showed signs of bowel obstruction with air-fluid levels of midgut; chest x-ray was unremarkable. The abdominal CT scan revealed an conglomerate ileal loops with thickened walls filled of fluid in the right iliac recess .A diagnosis of bowel obstruction was made and the patient sent for urgent laparoscopy. At laparoscopy , the patWe first untwisted the volvulus, then returned the bowels in the abdomen in a non-rotated position. We performed the closure of the internal mesenteric passageway , Fig. 6 3The normal development of mid-gut occurs in three stages of rotation process between the 4th and 12th week of embryonic period. During this phase duodenum is fixed retro-peritoneally at the ligament of Treitz in the left and caecum on right lower abdomen. Malrotation of the gut is due to complete or partial failure of 270 degree of clockwise rotation of the midgut around the superior mesenteric pedicle; it results in anomalous position of small bowel loops in the right side with absence of ligament of Treitz and appendix, caecum and ascending colon on the left side. A peritoneal fibrous band – also known as Ladd’s band – can compress duodenum causing duodenal obstruction. Intestinal malrotation is a disease of newborn as it frequently manifests in the first month of life; adult manifestation is very rare. Clinical presentation in adults is more variable  and can It is still controversial if asyntomatic patients with documented malrotation require surgery. Many authors recommend elective Ladd’s procedure in all patients with intestinal malrotation . Ladd’s 4Intestinal malrotation is a rare entity and adult presentation is even rarer: it has been reported that the incidence in adults is approximately 0.2%. Evidence from post mortem studies suggest that gut malrotation may affect up to 1 in 6000 ,20. SomeIt is argued that elective Ladd’s procedure should be performed in all patients with intestinal malrotation. The surgical treatment remains as it was first described by Ladd in 1932: mobilization of the right colon and duodenum, division of Ladd’s bands, division of adhesions around the superior mesenteric artery, appendectomy . GeneralNone.None.This clinical case report is exempt from ethical approval.The authors obtained from the patient written informed consent for the publication of this case report and images.CG and FB were responsible for the surgical intervention. CG conceived the case report. LC and GP researched and drafted the manuscript. MDD, GMC and PC revised the manuscript. All authors read and approved the final manuscript.NA.Carmine Grassi.Not commissioned, externally peer-reviewed.


A 44-year-old male initially presented with a right thalamic brain tumor that was confirmed with stereotactic biopsy to be glioblastoma (GBM). The patient was treated with radiotherapy and temozolomide for 6 weeks. At 1 month after completing chemoradiation therapy, the patient underwent follow-up imaging that revealed the primary lesion had mildly responded to chemoradiation, but a secondary lesion had developed along the biopsy needle tract. This secondary lesion was outside of the field of radiation therapy for the primary tumor and concluded to be intracranial spread of GBM along the biopsy tract. The patient’s final imaging 4 months after initial diagnosis revealed the primary and secondary lesions had enlarged. Subsequently, the patient clinically deteriorated and died 7 months after initial diagnosis. A 44-year-old male presented to the emergency department with a 6-week history of headaches that had been initially worked up for a sinus infection. His headaches were worse with standing, bending, and lying down. He also reported having nausea, but denied vision problems, weakness, and numbness. In addition, the patient denied seizures and had not experienced any loss of consciousness. Past medical history was notable for hypertension and amyotrophic lateral sclerosis (ALS). His ALS had been diagnosed in 2010 and he had responded well to two stem cell injections in 2010 and 2011 as part of a clinical trial. Neurological examination was notable for new onset of motor weakness in the left upper and lower extremities.MRI of the brain revealed a mass measuring 3.5 × 2.4×3.3 cm in the medial right thalamus-pineal region . The lesDue the location of the lesion, the patient was not considered a candidate for surgical resection. Therefore, stereotactic biopsy was performed to establish a definitive diagnosis. The biopsy was performed via a right frontal trajectory to minimize functional morbidity and utilized an outer sheath. The inner needle was removed separately for each biopsy while the outer sheath remained in place. In addition, the patient had a ventriculoperitoneal (VP) shunt placed to address their acute hydrocephalus. Histopathology analysis revealed the mass to be glioblastoma (GBM). The molecular profile of the tumor was determined to be isocitrate dehydrogenase-negative and O-6-methylguanine-DNA methyltransferase indeterminate. Post-operative axial CT imaging  demonstrOne month after the completion of chemoradiation , follow-up imaging revealed that the tumor in the right thalamic-pineal region had modestly increased in size (3.7 × 3.2×3.2 cm) with central necrosis . However1–3 however, his platelet count continued to remain too low for adjuvant temozolomide. The patient remained clinically stable for the next 2 months, but deteriorated likely due to tumor progression. The patient died 7 months after initial diagnosis.Two months after completing chemoradiation therapy and 1 month after the secondary lesion received stereotactic radiosurgery , the patient reported having increased frequency of falls and significant gait instability. MRI at this time showed a considerable increase in size of both the primary and secondary lesions with a significant increase in edema , compare4 However, when brain lesions are located in areas of the brain that cannot be surgically resected, such as the thalamus, stereotactic needle biopsy is an effective way for safely determining the diagnosis while minimizing complications.5,6 Complications for stereotactic biopsy are rare, with a reported rate of between 0.4 and 4%.7,8 The most common complications are hemorrhage, infection, and the risk of inconclusive results.5,6 Metastases resulting from seeding of the stereotactic biopsy needle tract is considered a rare complication. However, there have been case reports in the literature that discuss seeding of the biopsy tract in patients with brain metastases,9 pineoblastoma,10,11 anaplastic astrocytoma,12 and GBM.13,14 In addition, malignant seeding has been well documented in needle biopsy procedures for tumors in the liver,15–17 lung,18 breast,19,20 prostate,21,22 kidney,23 and GI tract.24The ideal treatment protocol for GBM consists of surgical resection, followed by radiation therapy and concomitant temozolomide chemotherapy.13,25 Pierallini et al reported that the volume of the primary tumor was unchanged between 3 and 6 months after radiotherapy treatment, but the metastatic lesion outside of the radiation field demonstrated further growth during the same time period.The secondary lesion that developed in this patient occurred along the biopsy tract and was outside of the radiation therapy field for the primary lesion . Any mal26,27 A clinical trial of 40 patients with malignant pleural mesothelioma that underwent needle biopsy via thoracoscopy showed that 100% (20/20) of the patients who received radiotherapy to the biopsy tract did not develop secondary lesions, while 40% (8/20) of the patients who did not receive radiotherapy developed metastases.28There are several important conclusions from this case and similar reports of intracranial metastasis outside of the radiation field. First, radiologists interpreting tumor imaging and oncologists following brain tumor patients should be aware of the risk of developing secondary sites of disease along the biopsy tract. Second, generous clinical target volume expansions are typically added in treatment target delineation so that biopsy tracts are subsequently included, but there are rare cases such as this in which volume expansions still do not entirely cover the entire biopsy tract. Consequently, although spread along the tract is rare, extending the irradiated volume to include the entire biopsy tract in patients receiving radiotherapy after undergoing stereotactic biopsy of deep cerebral lesions could potentially be beneficial. This concept has been demonstrated in patients with malignant pleural mesothelioma, which has a high rate of malignant seeding along the biopsy needle tract.This case adds to the existing body of literature that that malignant seeding of the needle tract during stereotactic biopsy for brain tumors is a possible complication, especially if the biopsy tract extends outside of the radiation field. Due to this tumor’s deep location in the thalamus, the biopsy tract was longer than what would be needed for superficial lesions. As a result, the radiation field focused on the primary lesion and left the superficial portion of the biopsy tract susceptible to malignant seeding. Therefore, clinicians should consider the location of the tumor and the length of the biopsy tract when planning radiation treatment. In clinical situations where lengthy biopsy tract is unavoidable, clinicians may consider expanding the irradiated volume to target the entire biopsy needle tract to decrease the risk of this complication.Intracranial metastases associated with needle biopsy are a rare complication in brain tumor patients, but have the potential for impacting the patient’s clinical condition and outcome.The region of the needle tract that is outside of the radiation field is at increased susceptibility to malignant seeding during stereotactic biopsy.Prophylactic treatment of the biopsy tract with radiation therapy in addition to the tumor may decrease the risk of malignant seeding in patients with long biopsy tracts not likely to be covered by the primary field.


Case Presentation. This is the report of a 23-year-old man who had experienced lower abdominal pain for three months before presenting to the emergency department with severe right lower abdominal pain, nausea, vomiting, and anorexia since the day prior to admission. The patient was diagnosed with acute appendicitis, and the pathology report confirmed cytotoxic PTCL of the appendix.  Lymphoma of the appendix is a rare cause of acute appendicitis; however, acute appendicitis is a common first manifestation of appendiceal lymphomas. Cytotoxic peripheral T cell lymphoma (PTCL) is a type of aggressive non-Hodgkin lymphoma that portends a generally poor outcome. Cytotoxic PTCL of the appendix is extremely rare with few cases reported in the literature.  Patients with appendiceal PTCL commonly present with signs and symptoms of acute appendicitis due to luminal obstruction by the tumor. Therefore, appendiceal tumors such as PTCL should be considered in the differential diagnosis of patients presenting as acute appendicitis. In addition, since there is no standard chemotherapy regimen for cytotoxic PTCL, this and other case reports hopefully help in providing the clinical evidence needed for establishing appropriate treatment guidelines. Acute appendicitis is one of the most common causes of acute abdomen worldwide . One rarA 23-year-old man came to the emergency department because of right lower quadrant (RLQ) abdominal pain. He had a history of 3 months of vague lower abdominal pain, which became progressive and was more severe since the day prior to admission. It was associated with anorexia, nausea, and vomiting. He had no urinary or bowel changes and he had no significant past medical history. He reported that his brother had died at 46 years of age, but the reason was not determined.On physical examination, he was ill with generalized malaise. He had a pulse rate of 105, blood pressure of 110/55 mmHg, respiratory rate of 18, and oral temperature 38.1°C. RLQ abdominal tenderness and guarding were present. The physical examination was otherwise insignificant.The patient was admitted to the surgery ward with the impression of acute appendicitis. Appendectomy was performed and acute appendicitis was confirmed. However, the patient's condition started to deteriorate and further workup was performed. Ultrasound examination of the abdomen and pelvis showed multiple pathologic lymph nodes with varying sizes at the right lower quadrant with the largest one measuring 20 mm. In addition, mild free fluid between bowel loops was observed.Abdominopelvic CT scan revealed thickened small and large intestinal wall as well as lymphadenopathy. The pathology sample was reviewed and sent for further molecular analysis which revealed the final diagnosis, i.e., PTCL of the appendix. Unfortunately, the patient died four weeks after admission during the first round of chemotherapy.βF1 positive. Some of the cells expressed CD30. Markers such as CD79α, CD20, ALK1, CD56, and FOXP3 as well as Epstein Barr Virus (EBV) “in situ” hybridization (EBER) and HHV8 were negative. The proliferative index evaluated with ki67 was high, more than 80% of the atypical cells. According to these findings, the diagnosis of cytotoxic PTCL was made.Histopathology of the appendix showed the presence of atypical lymphoid cells around mucosal lymphoid follicles in appendiceal as well as the adjacent bowel wall and also neutrophilic infiltration in the muscular layer of the wall consistent with appendicitis and periappendicitis Figures –3. DiffuAppendiceal lymphoma is a rare malignancy that can present as acute appendicitis . The gasth and 7th decades of life [With regard to the presentation, patients with appendiceal PTCL, like other neoplasms of the appendix, commonly present with signs and symptoms of acute appendicitis due to luminal obstruction by the tumor –4, 10. H of life .The diagnosis of appendiceal PTCL is made with imaging modalities—the most useful of which is computed tomography (CT) imaging, histopathology, immunophenotyping, and genetic analysis. CT scan in appendiceal lymphoma patients demonstrates appendiceal enlargement with the maintenance of its vermiform appearance. Diffuse mural thickening and aneurysmal dilatation of the appendiceal lumen can also be present. It is suggested that the appendix diameter exceeding 15 mm on CT scan should raise suspicion about appendiceal tumor since the diameter is less when the appendicitis is not associated with a tumor . NHLs teDue to the very small number of appendiceal PTCL cases, it is difficult to establish standard management guidelines for them. PTCLs, unlike B cell lymphomas, do not respond well to conventional chemotherapy and have a generally poorer outcome. The reason for this might be the fact that PTCLs disseminate early in the course of disease and that there are virtually no specific chemotherapy drugs for them and the current regimens are according to guidelines for diffuse large B cell lymphoma treatment. Treatment modalities include surgical exploration and resection, chemotherapy, and radiotherapy. The first-line chemotherapy regimen for PTCL has conventionally been Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone (CHOP) or CHOP-like regimens. This regimen leads to a 5-year overall survival of 32%. Novel drugs that target specific cellular pathways have been suggested that when used in combination with the conventional chemotherapy have shown promise . PTCL isIn addition, in cases where imaging such as ultrasound is performed, attention must be paid to atypical findings such as lymphadenopathy in the pelvic cavity.Considering the fact that most appendiceal lymphomas initially present as acute appendicitis, physicians should always consider this disease in the differential diagnosis of acute abdomen. This is especially important since early diagnosis can change the management and surgical approach and since a late diagnosis is associated with a poorer prognosis. In addition, all appendectomy specimens in cases of acute appendicitis should be evaluated by histopathology even if they appear normal by gross examination.


Secondary osteoma cutis is a phenomenon that may occur in several conditions. When it occurs in a melanocytic nevus it is named osteonevus of Nanta, an event considered uncommon and characterized by the presence of bone formation adjacent or interposed with melanocytic cells. There are reports of its occurrence in various melanocytic lesions, being more frequently associated with intradermal nevus. We report a case of osteonevus of Nanta in combined nevus, possibly the first description of this association. Secondary skin ossification is a phenomenon that can occur in several conditions, such as pilomatricoma, basal cell carcinoma, acne, scars, mixed skin tumors, sites of inflammation and trauma.The osteonevus of Nanta is characterized by the presence of adjacent bone tissue or interposed with melanocytic cells,We report the case of a woman with exuberant lesion on the scalp, whose histopathological analysis was conclusive of osteonevus of Nanta, being possibly the first report of this association.A 38 year-old woman with an occipital lesion since she was born, which increased in the last 14 years, without associated symptomatology. At the examination, there was an exophytic, pedunculated, normocromic tumor with irregular surface and depressed areas with dark brown to blue pigmentation, fibroelastic consistency, measuring about 3 × 2 cm in diameter . The patThe histopathological analysis showed diffuse proliferation of melanocytes arranged in nests of regular size and shape in the papillary dermis and in strings in the reticular dermis surrounding adjacent structures with focal area of melanocytes of dendritic pattern with abundant melanic pigment in the cytoplasm. In addition to collagen fibroplasia, there was a foreign body type granulomatous reaction around free hair shaft and homogeneous bone formations compatible with cutaneous ossification. The histological diagnosis was osteonevus of Nanta in a combined nevus – intradermal and blue . The immThe first report of ossification in a melanocytic nevus was made in 1908 by Heidingsfeld, although the phenomenon was detailed published just in 1911 by Nanta, when it became known as osteonevus of Nanta.From its initial description, the phenomenon was reported in several melanocytic lesions such as blue nevus, Spitz's nevus,According to the literature, the lesions are mostly located in the head and neck, especially on the face, being less than 5% in other locations, as forearms.About its pathophysiology, disembrioplasia (hamartomatous lesion) and metaplasia are the main mechanisms postulated so far to explain the occurrence of the ossification of these lesions. According to the disembrioplasia, there would be the presence of primitive mesenchymal cells of osteocytes at erroneous sites with simultaneous expression of structures of ectodermal and mesodermal origin.4Regarding the higher incidence of osteonevus in female reported in a large part of the studies, it is believed that estrogen has a potential role in bone formation.In the histological analysis, ossification is usually observed as small islands of lamellar or amorphous compact bone at the base of the melanocytic lesion,In the case reported there was the characterization of combined nevus – intradermal melanocytic nevus and blue nevus – associated with the presence of homogeneous bone tissue in the reticular dermis near hair follicles and a foreign body type granulomatous reaction. No reports have been identified in the literature about combined nevus ossification, which is probably the first description of this association.The findings of ossification near hair follicle and granulomatous reaction of the foreign body type, associated with the localization of the lesion in the scalp, site rich in hair follicles and target of repetitive trauma, guide the reasoning regarding the possibility of metaplasia resulting from a chronic follicular inflammatory process, leading to secondary ossification of the combined melanocytic nevus.Culver and Burgdorf in 1993 reported the only case in the literature of malignant melanoma associated with osteonevus of Nanta. In the report, the female patient had extensive superficial melanoma and intradermal nevus with two ossification foci at its base.Although the phenomenon does not appear to be related to a poor prognosis, the possibility of metaplasia as a pathophysiological mechanism and an association with melanomaNone declared.Elaine Dias Melo: Conception and planning of the study; elaboration and writing of the manuscript; obtaining, analysis, and interpretation of the data; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature.Patrícia Amaral Couto: Elaboration and writing of the manuscript; obtaining, analysis, and interpretation of the data; critical review of the literature.Antônio Pedro Mendes Schettini: Approval of the final version of the manuscript; elaboration and writing of the manuscript; effective participation in research orientation; critical review of the literature; critical review of the manuscript.Carlos Alberto Chirano Rodrigues: Conception and planning of the study; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases.None declared.


This case report looks at a 47 year-old patient, presenting with a rapid onset mass of the left face. This report examines the case, the investigations undertaken, its management and undertakes a literature review of this uncommon condition and rare presentation.This patient had experienced functional deterioration, restriction and an evident swelling of the left face. Having identified the mass to arise from the mandibular condyle, an incisional biopsy was undertaken. The histology was suggestive of an arteriovenous malformation. Surgery was undertaken to excise the entire left mandibular condyle along with the lesion and then replace the temporomandibular joint with a custom made prosthesis.Arteriovenous malformations, or Haemiangiomas, can arise in any part of the body and can often grow over time. Although benign, lesions can be locally invasive and distort anatomy, resulting in functional issues. Their risk for hemorrhage and potentially significant blood losses is also a risk factor in their presence. Rarely arteriovenous malformations can occur in hard tissue, as in this case distorting and obliterating the temporomandibular joint. One’s only recourse is to remove and eliminate the lesion, however in cases such as this significant morbidity could ensue due to functional deficit. Hence consideration must be given to reconstruction.Having excised the mass and reconstructed the temporomandibular joint with a titanium prosthesis, the patient experienced a good recovery with a return to normality. They arAVMs lack the dampening effect that capillaries would usually have on blood flow, as a result they can become progressively larger over time, with the surrounding tissues becoming deprived of blood flow and suffering ischaemic effects. The resulting tangle of blood vessels is often termed a “nidus” and can be extremely fragile and prone to bleeding due to the abnormal direct connections between high-pressure arteries and low-pressure veins .AVMs can be asymptomatic and are generally detected due to causing resultant effects on surrounding structures. Within the jaws AVMs are very rare, with a prevalence of less than 1/100,000 person years .2This case pertains to a 47 year-old causacian female who presented to the Oral and Maxillofacial Surgery Department at Salisbury NHS Hospital. The patient was otherwise medically fit with no significant medical, drug or familial history.The presenting complaint involved non-painful swelling of the left cheek/preauricular region that arose over a couple of weeks. There was no history of injury to the area, nor any discharge or associated rubor/calor/dolor. The lesion was firm and appeared to distend the overlying tissues with the appearance of arising from deeper structures. Intra-oral examination did not reveal any dental pathology and salivary expression was normal.In a differential diagnosis, a lesion of the parotid salivary gland was amongst the more likely potential explanations. Physical examination alone was insufficient to determine the lesion’s origins, hence CT scanning was undertaken.Imaging demonstrated a highly unusual lesion associated with the left temporomandibular joint. The lesion was well circumscribed, confined to the mandibular condyle, partially lytic in appearance and did not appear to be causing any destructive effects on the surrounding tissues. The overall size was large, measuring at 32 × 23 × 29 mm. Generally the appearances were benign, however a firm diagnosis could not be achieved. As such the decision was taken to perform an incisional biopsy .Fig. 1CTSamples of abnormal bone were obtained along with surrounding soft tissues. Macroscopic analysis revealed an irregular bony surface with distortion and expansive signs. The sample was decalcified for microscopic analysis. This showed areas of proliferative blood vessels between bony trabeculae, certain areas also showed leashes of small to medium-sized blood vessels which lacked elastin in their walls and were dilated. Much of the bone was replaced with these vascular structures. The remaining, expanded bony surface was covered with fibrous tissue. Two histopathology consultants agreed that these findings were consistent with an AVM .Fig. 2HiFollowing the International Society for Study of Vascular Anomalies (ISSVA) classification system, this AVM would be categorised as an Intraosseous Vascular Malformation (VMOS), under Simple Vascular Malformations Type III Category . There wDue to the expanding nature of the lesion and increasingly deleterious effects on the patient, the decision was taken to operate. The surgical plan being to resect the left mandibular condyle head, neck and a portion of the ramus, essentially obliterating the temporomandibular joint. Although curative intent would be achieved through this approach, the resulting effects on the patient’s quality of life and function would be significant. As such, consideration was given to reconstructive options available for such a resection. With the glenoid fossa and skull base unaffected, a custom joint replacement was thought to be the best option, restoring both normal anatomy and function.Given the anatomical variations often seen with temporomandibular joints and their need to work in tandem with the opposite side, a custom prosthetic option was selected. This would allow for precise planning and replication of the anatomy, in what was likely to be a challenging and non-standard resection. The CT scans obtained allowed for digital planning, design and manufacture of the titanium prosthesis .Fig. 33DSurgery was performed by a Maxillofacial team, including a consultant surgeon and specialty doctor surgeons, all team members were certified and held tri-collegiate fellowship/membership with The Royal College of Surgeons. An approach was selected to allow sufficient access for resection as well as placement of the prosthesis. A preauricular incision with temporal extension was done for initial access. This allowed for direct dissection down to the field. A second incision was made using a retromandibular approach to aid access.Using a surgical guide, bony cuts were made so as to resect the affected field, leaving a portion of the ramus. As the AVM was completely intraosseous there was no uncontrollable haemorrhage. The prosthesis was then placed on the remaining bony surface of the mandibular ramus and fixated with bicortical screws. Throughout the procedure the patient was placed into rigid intermaxillary fixation to keep the jaws locked together, maintaining the occlusion of the teeth and the position of the temporomandibular joint of the opposite side. Layered closure was done with a drain placed to prevent accumulations. Following an uneventful recovery the patient was discharged the following day.Recovery in the postoperative phase was routine with no complications or facial nerve weakness. Allowing for sufficient time, healing and oedema to subside at home, the patient returned to relative normality with their function and facial profile over 3 weeks, eating a normal diet with no discomfort. Postoperative imaging taken at 3 months indicated a good position of the prosthesis with integration to the mandible. The final histology supported the diagnosis of a benign AVM.3Although AVMs of the jaw are rare, one should appreciate their part of a differential diagnosis when considering options for unexplained symptoms and pathology. Studies have shown that although AVMs have a risk of haemorrhage , intraosGiven the slow-growing nature of AVMs they are often quiescent for a significant period and do not tend to show increased incidence for a particular age group . With thAVMs of the jaws are very rare, accounting for (0.5–1%) of all lesions . LiteratRadiographic features of AVMs can be similar to other lesions so definitive diagnosis based solely on imaging is difficult . Key radDefinitive diagnostics can only reliably be achieved through biopsy and histopathological examination. Features are fairly consistent in AVM histology, proliferative epithelial cells and fibrous tissue are often seen surrounding the lesion and the remaining bony trabeculae are interspersed with vascular structures and endothelial cells .There are various treatment options available for AVMs, the key aims being; elimination, haemorrhage control and preventing recurrence. Some AVM lesions may be inaccessible and in these cases radiotherapy is the only treatment modality available. Cases have been reported of radiotherapy successfully controlling AVM lesions , howeverDirect surgery with or without embolisation of major afferent feeding vessels is the accepted treatment of choice . RadicalIn this case, wherein the entire joint was excised, the use of a custom prosthesis was selected. Given the essential functions of mastication, speech, airway support and deglutition are directly supported by the temporomandibular joint, restoration of function was of principal concern. Indeed it has been shown that improvement of temporomandibular joint function with the use of a prosthesis results in an improved quality of life of up to 85% over the long term , and thi4Due to the complex presentation and multifactorial nature of AVMs a step-wise diagnostic approach should be undertaken. CT scanning can guide and aid in surgical planning, but would be inappropriate to solely rely upon for definitive diagnosis. Given the spectrum of imaging presentations seen and the crossover with other lesions, microscopic histological examination is the gold standard for diagnosis. Of the various treatment modalities available the primary goals remain; elimination, haemorrhage control and preventing recurrence. With reconstruction bearing importance depending on the functional deficits likely to be encountered through surgery.This case has been reported in line with the SCARE 2018 criteria [The authors report no declarations of interest.We have no funding sources to report.Institutional approval was obtained for the study. Ethical approval is not applicable to this submission type.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.Blackhall, Kristian K: Lead author & corresponding author. Design of report and literature review. Literature search. Data analysis. Writing of the paper.Ling, Eugenie: Assisting in writing of the paper. Literature search and analysis.Kunjur, Jayanth: Supervising consultant surgeon. Clinical lead for this surgical case. Writing of the paper.1.Name of the registry: N/A2.Unique identifying number or registration ID: N/A3.Hyperlink to your specific registration (must be publicly accessible and will be checked): N/ABlackhall, Kristian K.Not commissioned, externally peer-reviewed.


Cervical chondrocutaneous branchial remnants are rare, benign, congenital anomalies, frequently seen bilaterally.Here, we report the case of a 4-month-old female infant who presented with bilateral lower neck skin tag since birth.The patient underwent mass excision. The final pathological diagnosis was bilateral cervical chondrocutaneous branchial remnants with hyaline cartilage.No complications were observed after excision. One-year follow-up revealed no recurrence.Bilateral chondrocutaneous branchial remnants are rare anomalies. They are often associated with cardiac or genitourinary abnormalities. Therefore, additional preoperative imaging of the abdomen and heart are recommended. To date, 117 cases have been reported  in medical literature. Bilateral CCBR are rare, and multiple differential diagnoses should be considered while diagnosing it according to the location. Herein, we present a case of bilateral CCBR presenting as a neonatal neck mass at the sternocleidomastoid (SCM) muscle level and investigated previous medical literatures about bilateral CCBR case reports predominance in male (11 of 17);(2)high incidence of associated anomalies (76%) involving the auditory , respiratory , oro-gastrointestinal , genitourinary (hydronephrosis), cardiovascular , musculoskeletal and visual systems;(3)presence of a cartilage core;(4)a scarcity of bilateral lesions (1 of 17);(5)located in the middle or lower third of the neck; and(6)increased prevalence anterior to the SCM muscle. Ultrasonography can be useful for describing the lesions, which have the characteristic presence of a tubular cartilage that extends to the SCM muscle. CCBR is often associated with cardiac or genitourinary abnormalities, which have been reported in 11% to 76% of cases. Therefore, preoperative additional imaging studies of the abdomen and heart are recommended.CCBR can have either rod-shaped elastic or hyaline cartilage core surrounded by normal skin and subcutaneous tissues. They are located in the middle or lower third of the neck, anterior to or over the SCM muscle. The lesion presents at birth and has no or very slow growth. The lesion has no connection with deep structures but adherence to the fascia of the SCM muscle is often reported. There is no report of underlying sinuses and cysts.In our study, we examined the previous medical literatures and inferred that there was no correlation between anomalies on unilateral CCBR and bilateral CCBR Table .As treatment for CCBR and to obtain an accurate histologic diagnosis, complete surgical excision is recommended. If the patient has problems with tolerating anesthesia, the excision can be postponed.,15Tarmir et al suggested surgical treatment of CCBR just before starting school, which allows minimization of surgical complications and spares the child of the psychological complications; however, it can be postponed to a suitable and safe age.5Bilateral CCBR is a rare condition. CCBR should be included in the differential diagnosis for congenital neck lesions in pediatric patients. The treatment of choice of CCBR is complete surgical excision. Further, careful preoperative assessments are needed for investigating associated lethal anomalies by abdominal ultrasound and cardiac examination.Conceptualization: Taejin Park, Han Shin Lee.Data curation: Taejin Park, Hyo Jung An, Han Shin Lee.Formal analysis: Han Shin Lee.Investigation: Tae Han Kim.Methodology: Jae Yool Jang, Jung Woo Woo.Project administration: JinKwon Lee.Resources: Sang Ho Jeoung.Supervision: Eun Jung Jung.Validation: Han Shin Lee.Visualization: Han Shin Lee.Writing – original draft: Han Shin Lee.Writing – review & editing: Taejin Park.


Gastric inflammatory fibroid polyp (IFP) is a rare polypoid lesion of the stomach that is characterized pathologically by the presence of spindle cells, a prominent network of blood vessels, and inflammatory infiltration of eosinophils. IFP is mainly located in the gastric antrum and is usually semi-pedunculated and covered with normal mucosa. There have been several reports of large IFPs with ulceration on the surface, at the apex, but no report of the IFP with ulceration at the fornix of the stomach. We report a case of IFP with ulceration that was suggested to be gastric cancer and was resected for diagnostic treatment.A 79-year-old woman presented to our hospital. During mass screening for cancer, stomach fluoroscopy revealed an abnormal shadow. Endoscopy showed an ulcerated tumor at the fornix of stomach; hence, gastric cancer was suggested because of the polypoid lesion with irregular ridges and ulceration. Pathological diagnosis of gastric biopsy specimens revealed an inflammation of the gastric mucosa, and specific findings for gastric cancer were not obtained. Because we could not exclude gastric malignancies such as cancer or gastrointestinal stromal tumor, we performed a partial resection of the stomach with a 2-cm margin using the laparoscopic-assisted method. Pathological examination of the resected specimen revealed that the tumor was present in the submucosal layer and consisted of collagen fiber containing inflammatory cell infiltration of mainly eosinophils. A prominent network of blood vessels was also found in the specimens. Immunohistochemical staining revealed mild positivity for CD34, and α-SMA and was negative for c-kit, DOG-1, s-100, desmin, ALK, and IgG4. The lesion was thus diagnosed as an IFP. The postoperative course was uneventful. The patient is currently asymptomatic and has shown no recurrence.IFPs have variable locational, morphological, histological, pathological, and immunohistochemical features. We reported that the gastric IFP was located at the fornix of the stomach and was similar in morphology to gastric cancer. This case is clinically significant to avoid over-surgery. Inflammatory fibroid polyps (IFPs) are benign polypoid lesions of the stomach. They are typically noninvasive, non-ulcerated, and eosinophilic submucosal tumors (SMT) with spindle cell proliferation. They occur in a variety of sites in the gastrointestinal tract, but the most common site of IFP is the gastric antrum –5. In 14A 79-year-old woman presented to our hospital. An abnormal shadow using stomach fluoroscopy was detected during mass screening for gastric cancer. The examination showed an irregular-shaped defect with a central ulceration at the fornix of the stomach Fig. . CEA, CAIFP is a rare benign polypoid lesion of the gastrointestinal tract. In the very first report in 1937, Kaijser described the gastric lesion with eosinophilic cell infiltration . The lesThe most common localization of IFP is the antrum of the stomach –5, but aPathologically, IFP is characterized as a submucosal lesion containing spindle cells with a prominent vasculature and inflammatory cell infiltration, mainly eosinophilic cells. A specific feature is a perivascular “onion skin” appearance, but approximately half of IFPs do not have this appearance . Our preImmunohistochemically, IFP was positive for CD34 and negative for c-kit, DOG-1, s-100, and desmin, and ALK. In the present case, the α-SMA was mildly positive only in fibrobrast cells and negative in spindle tumor cells. Recently IgG4 related lesions of the stomach have attracted attention for the ulcerative lesions of the stomach, which are difficult to diagnose; IgG4 contained cells were not found histochemically Fig. .Fig. 6ImAlthough several treatment options except the operation including re-biopsy, endoscopic resection, and follow-up were present, we selected the surgical operation to prevent bleeding from the tumor and to rule out the malignancies. Endoscopic resection was judged to an be-off label lesion from the shape and size of tumor. In general, the treatment of IFP depends on the shape, size, and location of the lesion. The current standard treatment is complete resection by endoscopy or gastric surgery, because it is considered as benign and noninvasive lesions –12. HoweHelicobacter pylori (H. pylori) was detected on the surface of gastric epithelium by Giemsa staining using the surgical materials, the association with IFP and H. pylori infection was not excluded [Etiology and pathogenesis of IFP remain unclear. The local inflammatory response underlying an underlying granuloma such as trauma, tuberculosis, Crohn’s disease, and sarcoidosis were reported. In our case, the history of trauma, tuberculosis, Crohn’s disease and sarcoidosis was not recognized. Because excluded . Familiaexcluded . In 2008excluded . The preIn conclusion, we presented a rare case of IFP with ulceration mimicking gastric malignancies at the fornix of the stomach, which was treated using laparoscopic-assisted partial resection.


The anterior digastric muscle belly (ADMB) may present significant variations of substantial surgical importance. We present an unusual complex bilateral asymmetry of an accessory ADMB found when dissecting the submental area in a 72-year-old Greek male cadaver. A rare variant was recognized in the submental area constituted by a combination of bilateral asymmetry of the ADMB with unilateral absence of the intermediate tendon. The complex variant caused an obvious morphological asymmetry in the submental area. Such muscular variations may alter the surgical approach to the submental region. Clinicians involved in the treatment of this area should be aware of any possible variant, particularly when dealing with neck mass patients.  The digastric muscle (DM), located in the suprahyoid region, consists of an anterior and a posterior digastric belly (ADMB and PDMB), each of them arising from different embryological precursors and thus supplied by different nerves. The ADMB is innervated by the mylohyoid nerve , while the PDMB is supplied by the facial nerve. Different innervation is explained by the separate derivation of the two bellies from the first and second brachial arch mesenchyme. The ADMB and PDMB are converged to the intermediate tendon (IMT) connecting with the hyoid bone (HB) through a fascial sling closely associated with the stylohyoid muscle (SHM) insertion. The ADMB originates from the digastric fossa. An aponeurotic layer gives off the tendon to the HB body and great horn. The DM may lack the IMT, thus be attached directly to the HB. The muscle is vascularized by the submental artery  .The ADMB presents a great variability in size, shape, and form. The ADMB variations may present double or extra slips spreading to the jaw or the mylohyoid muscle (MHM) or crossing over the midline to the opposite MHM or the opposite digastric fossa. These unilateral or bilateral muscle bundles or slips may occur as a result of a phylogenetic DM reduction or an unusual development of the ontogenetic material . The ADMThe current case report highlights the occurrence of a bilaterally asymmetrical accessory ADMBs and the unilateral absence of the IMT. The complex muscular variant caused an obvious asymmetry over the submental area. Knowledge of this kind of variability is of paramount importance in neck mass imaging interpretation and differential diagnosis. During the submental region dissection in a 72-year-old Greek male cadaver, a bilateral accessory ADMB was observed Figure . On the Contralateral, the left SHM typically attached to the HB, via a single bundle, coursing along the inner aspect of the IMT and ending up into an extended strong fibrous band (representing an IMT extension in terms of function) without any attachment to the HB. Towards the superior and inferior aspect of the SHM, thin muscle bundles attached in a pennate fashion, the superior one ultimately crossed the midline over the MHM, while the inferior counterpart spread out freely over the HB. The main left-sided ADMB possessed two origins, one larger extended over the inferior mandibular rim, and a separate smaller one originating from the digastric fossa. Both parts merged further, forming a common belly and typically attached partially to the anterior end of the IMT and partially to the commencement of the fibrous band Figure .Embryological background of DM aberrationsZlabek, studying the ADMB variants, found that the muscle expansion towards the midline was characterized by its rectangular oblongation . In aberThe ADMB variability among various studiesSeveral anatomical studies described the ADMB and DM fibrous sling variations ,10. The Clinical significanceAsymmetrical or symmetrical ADMBs variations are of clinical importance since they indicate asymmetry cases over the anterior region of the neck or even in the movement of the floor of the mouth or the temporomandibular joint and perhaps imbalance in laryngeal movement . The asyThe DM variability is of paramount importance during platysma myocutaneous flap mobilization in reconstructive procedures ,20. ThorBy using computed tomography (CT) and magnetic resonance imaging (MRI), typical and variable anatomic structures and pathologic entities of the floor of the mouth can be evaluated .The described aberration represents a variant in the MHM-DM group in the floor of the mouth. This kind of ADMBs aberrations has to be taken into consideration when evaluating the floor of the mouth, as they can be confused with pathological conditions in imaging. Therefore, normal anatomical variants have to be considered on CT and MRI when an asymmetry over the floor of the mouth is encountered .Submental and submandibular areas are very well vascularized, and knowledge of their muscular abnormalities is essential when a myocutaneous flap is mobilized in reconstructive plastic surgery. Vascular variations may coexist, and in order to maintain and increase flap’s viability, vessels’ branching pattern must be well preserved, intraoperatively. CT and MRI are valuable imaging tools in the differential diagnosis of the muscular abnormalities from pathological lesions, as due to the muscular tissue density, accessory, and hypertrophied DM can be easily misinterpreted as a tumor or a lymph node. Cases of asymmetrical aberrancies in submental region should be very carefully identified in order to avoid misinterpretation.


Neuroendocrine carcinomas (NECs) are rare malignancies that originate from the hormone-producing cells of the neuroendocrine system. They can grow in most organs of the body but are commonly found in the gastrointestinal and respiratory tracts. The nasal cavity is a rare site for NECs.We report a case of NECs in a 45-year-old woman who presented with epiphora in the right eye for a year owing to an unknown reason.The diagnosis was initially confirmed via histological and immunohistochemical assays. Postoperatively, computed tomography of the neck revealed C4 vertebral bone metastasis.The tumor was endoscopically removed from the right eye. The patient received a full course of adjuvant polychemotherapy.Six months after diagnosis, the patient died due to bone metastasis.Diagnosing nasal neuroendocrine carcinoma is clinically challenging. It must be distinguished from other orbital masses, such as chronic dacryocystitis or nasal polyps. The treatment should be further investigated for this rare malignancy in near future. Owing to the complexity of its histopathological diagnosis, the pathological mechanisms of NECs remain elusive. NECs in the nasal cavity display aggressive local invasiveness and have a high recurrence rate. NECs usually have adjacent tissue invasion or distant metastasis with poor prognosis because of adjacent tissue invasion or distant metastasis. Clinical symptoms of this disease include rhinological syndrome and ophthalmic signs, but these are nonspecific. Surgical excision, multiple-agent chemotherapy, and radiation therapy are often utilized for its treatment. We report a rare case of nasal neuroendocrine carcinoma that primarily manifested as epiphora, including its imaging features and pathological evidence.Currently, less than 100 cases have been reported.2A 45-year-old woman was admitted to our institution because of epiphora in the right eye. This was accompanied by pain when she woke up in the morning. The symptoms lasted for 1 year. Her symptoms started 2 months ago when she experienced progressive right nasal obstruction. Symptoms such as bleeding, blurred vision, diplopia, nausea, and dizziness were not observed. The patient did not experience any pathological neurologic changes. No cervical lymphadenopathy was noted. Normal visual acuity and ocular mobility in both the left and right eyes were observed on ophthalmologic examination. The patient provided informed consent for the publication of this case.Physical examination and biochemical laboratory test results, such as a complete blood count and serum chemistry panel, were within the normal range. Definitive lesions were not identified on plain chest radiography. A flaky soft tissue shadow in the right nasal cavity and lachrymal sac was noted after injection of contrast through sinonasal computed tomography (CT). Through direct nasopharyngoscopy, an exophytic fleshy mass (approximately 3 × 4 cm) was observed in the right nasal cavity. Combining both clinical and imaging features, the presumed diagnosis was chronic dacryocystitis or nasal polyp on the right side. The tumor was surgically removed through a minimally invasive endoscopic strategy with a lachrymal sac nasal anastomosis in the right eye.Pathologically, the nuclei of dysplasia cells were large and stained dark. Cytoplasmic staining was scanty Fig. A. Tumor 3 The NECs were divided into 3 stages, namely well-, moderate-, and poorly differentiated NECs. The poorly differentiated neuroendocrine carcinomas were further divided into small-cell NEC and large-cell NEC (LCNEC). Clinical presentations of primary NECs of the nasal cavity can lead to nonspecific symptoms such as nasal obstruction, epistaxis, or chronic sinusitis. On nasal endoscopy, the tumor mass may be seen with the naked eye as gray-white or reddish, polypoid, brittle, and it may easily bleed upon contact. Mass shadows can be found via CT examination of the upper nasal cavity. This is involved in the ethmoidal sinus and maxillary sinus with bone destruction. Uniform hyperintense signal on T1WI and T2WI was indicated in the enhanced MRI.The World Health Organization (WHO) 2017 Classification of Head and Neck Tumors (“Blue Book”) included a new classification of neuroendocrine carcinomas (NEC) of head and neck, neck, according to the degree of malignancy. reported a “pigeon” pattern in the bilateral ethmoidal sinus as specific MRI features in SCNEC tumors. CT or MRI imaging of the nasal cavity has more advantages than conventional radiography when evaluating the extent of local invasion of a neoplasm. These examinations will benefit the planning of further treatment. CT has several advantages in evaluating skeletal changes. MRI complements CT because it effectively characterizes soft tissue components and evaluates the extent of tumor invasion beyond the wall of the bone sinus. However, the images of NECs lacked typical symptoms and special detection methods, which resulted in misdiagnosis of sinusitis or nasal polyps. Therefore, it is important to use CT/MRI or enhanced CT/MRI in all cases of tumors in the nasal cavity prior to surgery. Frozen pathological sections during surgery are necessary to distinguish benign and malignant tumors.It is important to determine the extent of local tumor invasion and distant metastasis through radiological examination. CT and MRI scanning were particularly indispensable for identifying primary NECs. Zhu et al,13 Generally, it is recommended to use at least 2 or more staining results combined with hematoxylin and eosin staining by synthetic analysis to determine the diagnosis of NECS.Hematoxylin and eosin staining was used to observe histopathological changes that appeared as nests, cords, and trabeculae in neoplastic cells with small cytoplasm, nucleolar deficiency, and active mitosis. For immunohistochemical staining, the neuroendocrine markers were positive for NSE, Syn, and CgA while the epithelial markers were significantly positive for CK.,15 The American Joint Committee on Cancer TNM  staging was reported to be a predictive factor for the survival of NECs patients. Our patient had a C4 vertebral bone metastasis with T3N1M1 stage IV. The prognosis of the current case was advanced. Although no definite treatment for NECs has been established, combined surgery, chemotherapy, and radiotherapy was the primary treatment option that provided a high cure rate, despite the low survival.Due to the rarity of NECs of the nasal cavity, most patients with the disease were diagnosed at later stages and developed metastasis to the liver, lung, bone, and brain. 70% of patients presented with American Joint Committee on Cancer stage IV disease, and 37 patients (46.3%) were alive after a mean follow-up of 30.8 months . The most common treatment modality used in 26.3% patients was combination chemotherapy and radiation therapy.The side effects of chemotherapy included gastrointestinal damage, bone marrow suppression, and liver damage. In our patient, we excised the tumor with a minimally invasive nasal endoscope and a 2-course cisplatin-based postoperative polychemotherapy. This induced stage IV bone marrow suppression, and the patient eventually died of bone metastasis. In a systematic review of 80 SCNEC patients studied by Rivero, It was documented that the 1-year survival rate was 57%, and the 5-year survival rate was 10%. For many patients, early diagnosis and intervention improved the outcome.However, the second and third most common treatment modalities were combination surgery and chemoradiotherapy (21.3%), and surgery alone (18.8%), respectively. At present, the most common treatment for NECs is multimodality therapy. Previous reports showed that the local recurrence rate of SCNEC was 33%, and that of distant metastasis rate was 31%.4Neuroendocrine carcinoma of the nasal cavity is a rare but aggressive disease. Current treatment with a standard of multimodality approach should be considered for the management of NECs. However, considering the limited methods for diagnosing the disease, a multicenter study is necessary in the future.www.editage.com) for English-language editing.The authors thank Editage (Conceptualization: hong fei liao.Data curation: hong fei liao.Formal analysis: Weiqi Wu.Methodology: Yaohua Wang.Project administration: Puying Gan.Resources: Weiqi Wu, Puying Gan.Validation: Qihua Xu.Visualization: Qihua Xu.Writing – original draft: Weiqi Wu.Writing – review & editing: Weiqi Wu.