Pilomatricoma is a rare and benign tumor affecting children and adolescents. It originates from the matrix cells of hair follicles, the usual sites being head-neck and upper extremities. Due to its rarity, it is often misdiagnosed delaying definitive treatment. We report a case of pilomatricoma over the left gluteal region in a young Nepalese girl that was initially thought to be a calcified granuloma.A six-year-old girl presented with a painful swelling over the left buttock for one year that was gradually increasing in size. On examination, a solitary, well-circumscribed, tender swelling with hard consistency and a bumpy irregular surface measuring 3 × 2 cm was noted over the subcutaneous plane of the left gluteal region. Surgical excision of the mass was done which demonstrated features of pilomatricoma on histopathological examination (HPE). She recovered and remained disease-free at one year follow-up.This case highlights one of the handful presentations of pilomatricoma involving the buttock. Pilomatricoma is rarely considered a differential diagnosis of benign masses, the diagnosis of which is ascertained mostly after an HPE of the excised specimen. Surgical excision with clear margins is not only diagnostic but therapeutic in most situations. •Pilomatricoma is a rare benign tumor that originates from the matrix cells of hair follicles.•It is rarely considered a differential diagnosis of benign masses and imposes a diagnostic challenge preoperatively.•Although rare, pilomatricoma can affect the buttock or the gluteal region.•Surgical excision with clear margins is not only diagnostic but therapeutic in most situations. It accounts for only 0.001–1% of all skin lumps .2A six-year-old girl presented to the pediatric surgical outpatient clinic with complaints of a painful swelling over the left buttock for one year that was gradually increasing in size. On examination, a solitary, well-circumscribed, tender swelling measuring 3 × 2 cm was noted over the subcutaneous plane of the left gluteal region. It had a hard consistency with a bumpy irregular surface and was fixed to the skin but not to the underlying structures. The overlying skin had no visible punctum although it appeared to be slightly thinned out. There were no signs of inflammation in and around the swelling .Fig. 1A Ultrasonography revealed a well-defined, oval, hyperechoic lesion with posterior acoustic shadow in the subcutaneous plane of the left buttock .Fig. 2UlBased on the clinical picture and radiological features, a provisional diagnosis of a calcified granuloma was made. The lesion was excised and sent for histopathological examination.Grossly, the specimen was a skin-covered fibro-fatty tissue with a grey-white and hard outer surface measuring 3 × 1.5 X l cm. . On the Histopathologically, sections revealed deep dermis with a well-circumscribed tumor mass composed of irregularly shaped, islands of “shadow cells”. The shadow cells featured a distinct border and a central unstained area. Clusters of basaloid cells were seen towards the periphery of the islands . A fair She had an uneventful recovery and remained disease-free at one year follow-up.3Pilomatricomas, also known as Pilomatrixomas or calcifying epitheliomas of Malherbe, are benign skin adnexal tumors first described by Malherbe and Chenantais in 1880. Pilomatricoma frequently presents within the first two decades of life, however, no age is exempt from the disease, and females have a slightly higher preponderance . To our Although the etiopathogenesis of pilomatricoma is largely unknown, recent studies have shown that pilomatricomas are frequently associated with beta-catenin mutations . Beta-caPilomatricoma commonly presents as a single, firm, gradually expanding and mobile subcutaneous mass, however, the occurrence of multiple pilomatricomas have also been reported usually associated with disorders such as myotonic dystrophy, MYH-associated polyposis, Turner syndrome, Gardner syndrome, Rubinstein-Taybi syndrome, Sotos syndrome and gliomatosis cerebri .On preoperative evaluation, pilomatricoma presents a diagnostic challenge to the clinicians and is frequently misdiagnosed. Epidermoid cysts, sebaceous cysts, dermoid cysts, non-specific cysts, and foreign bodies were the most common differential diagnoses in an extensive review of 346 cases by Pirouzmanesh et al. [An accurate diagnosis was made only after a histopathological examination of the specimen.Histopathologically, basaloid cells and shadow cells are the two most important cell types that sum up the diagnosis of pilomatricoma. These cells are responsible for the characteristic biphasic appearance, with small, darkly colored basaloid cells in the periphery, evolving into bigger, pink shadow cells in the center .The basaloid cells are the matrix cells that fail to differentiate into a hair follicle. The islands of basaloid cells contain sparse cytoplasm, hyperchromatic nuclei, plentiful mitoses and indistinct cell borders predominantly arranged in the periphery. The shadow cells are formed when the basaloid cells mature and keratinize and lose nuclei by karyolysis. These shadow cells are sited towards the center of cell islands. Although basaloid cells have high mitotic activity, the diagnosis of an even rarer entity, the pilomatrix carcinoma, is established in the presence of cytological atypia, local aggressive behavior, vascular invasion and infiltrative basaloid nodules .Spontaneous regression of pilomatricoma has not been observed and therefore complete surgical excision remains the treatment of choice. Total excision with clear margins and inclusion of the overlying skin in cases showing tumor adherence to the dermis is preferred to avoid the possibilities of recurrence or malignant transformation. The reported incidence of recurrences being 2–6% in the available literature . Future 4Herein, we presented a case of pilomatricoma in a young girl that was clinically misidentified as a calcified granuloma. This case illustrates an uncommon condition that can impose a diagnostic challenge to the clinicians. An early and accurate clinical diagnosis can result in a curative surgical excision avoiding recurrences and risks of potential malignant transformations.This case series is exempt from ethical approval.None.AM drafted the manuscript. ALS conceived the study, was involved in patient care, supervised and provided valuable intellectual insights.1.Name of the registry:2.Unique Identifying number or registration ID:3.Hyperlink to your specific registration (must be publicly accessible and will be checked):ashishlalshrestha75@gmail.com.Dr. Ashish Lal Shrestha, Department of Pediatric and Neonatal Surgery, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, PO Box- 12127, Nepal. Tel: +977-1-552-2981, E-mail: Written informed consent was obtained from the patient's guardians 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 information is required for submission. Please note that failure to respond to these questions/statements will mean your submission will be returned. If you have nothing to declare in any of these categories then this should be stated.None of the authors has any conflict of interest to disclose. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.


ACTINOMYCOSIS is a rare chronic granulomatous disease caused by anaerobic filamentous gram-positive bacteria, the most common of which is Actinomyces israelii. Actinomycetes are commensal inhabitants of the oral cavity and gastrointestinal tract, but they may become pathogenic through invasion of breached or necrotic tissue. Pelviabdominal ACTINOMYCOSIS is uncommon and can mimic a variety of disease processes, including abdominal mass mimicking malignancy, acute abdomen, asthenia, and weight loss. We describe a 38-year-old woman who presented with acute abdominal pain and tenderness, as well as constitutional manifestations and elevated inflammatory markers. On initial computerized tomography (CT) and MRI, a large fluid collection underlining the anterior abdominal wall at the false pelvic cavity, as well as parietal peritoneal enhancement and smudging of the mesenteric fat and a bulky fibroid uterus with an implanted IUD, were identified. The ultrasound guided aspiration and anaerobic culture revealed positive growth for Actinomyces bacteria. An exploratory laparoscopy revealed extensive adhesions between the abdominal wall and the small intestine, as well as hyperemic and thickened peritoneum, and peritoneal biopsy confirmed ACTINOMYCOSIS. After the diagnosis was established, the IUD was removed and the patient was given Ceftriaxone 2 gm once daily for 6 weeks before switching to oral doxycycline 100 mg twice daily for another 3 months. A significant regression of the suprapubic fluid collection, and peritoneal-mesenteric changes were confirmed on follow-up. The case is discussed, and the relevant literature reviewed and analyzed. ACTINOMYCOSIS is a rare inflammatory condition caused by anaerobic bacteria Actinomyces species. These are gram-positive bacteria that are naturally present in the human mouth, digestive and genital tracts. Actinomyces requires the presence of an anaerobic microenvironment to proliferate, which necessitates the presence of many other bacteria ACTINOMYCOSIS, primarily affecting the pelvis ACTINOMYCOSIS is the cervicofacial region, followed by the abdomen; in the latter case the disease is almost always unifocal and limited to the right colon, particularly the cecum ACTINOMYCOSIS can be mistaken for those of other benign, inflammatory and malignant disorders such as tuberculosis, diverticulitis, inflammatory bowel disease, and malignancies. As a result, the diagnosis is typically difficult, and the disease is often detected histologically after surgery ACTINOMYCOSIS, presenting with acute abdominal pain, as a complication of long-term use of IUD and illustrate the important role of radiology in diagnosis and at follow-up. The case is discussed, and the relevant literature is reviewed and analyzed.We describe a young female patient with pelviabdominal 3/L) with left shift of granulocytes, HB of 8.5 (gm/L), platelets of 650,000 (103/L), negative hepatitis B surface antigen (HbsAg), hepatitis-C total antibody and HIV tests; normal liver function tests, serum creatinine, serum electrolytes levels, and urinalysis.A 38-year-old female patient with no significant previous medical history presented with acute abdominal pain that had been present for more than 3 weeks; she has 2 children and had an IUD implanted for over 10 years. Other associated symptoms included anorexia, weight loss, and malaise, and on clinical examination she appeared slightly pale but not jaundiced, afebrile, and vitally stable. There was tenderness in the lower abdomen but no palpable organomegaly. The initial laboratory tests showed a high CRP of 36 mg/dl and elevated ESR first hour 115 (mm/h), white blood cell count (WBC) of 17,000 (10A computerized tomography (CT) with contrast was performed A-C, whicActinomyces israelii” growth. The infectious disease team was consulted and the patient was started on intravenous Ceftriaxone 2 g once daily, with noticeable clinical improvement within a week regarding abdominal pain; the inflammatory markers became nearly normal. The IUD was removed and the patient was discharged home in good general condition with no abdominal pain. After discharge, she was scheduled for regular outpatient follow-up visits and continued on intravenous Ceftriaxone 2 g once daily for a total of 6 weeks before switching to doxycycline 100 mg twice daily orally for another 3 months.A diagnostic laparoscopy was performed, and extensive adhesions between the abdominal wall and the small intestine were identified as well as a hyperemic and thickened peritoneum. The laparoscopic findings suggested tuberculosis, however, acid fast bacilli (AFB) by Ziehl Neelsen staining and quantiferon tests were negative. Furthermore, a peritoneal biopsy was obtained and histopathological examination revealed a colony of Actinomyces surrounded by neutrophils and macrophages . In addiFollowing completion of the previously mentioned antibiotic therapy, CT abdomen, and pelvic scans A-C, as wACTINOMYCOSIS and successful treatment of her condition as shown with clinical-, laboratory-, and radiological evaluations. We do believe that her illness was predisposed by a complication due to chronically infected IUD.We described a young female patient with pelviabdominal ACTINOMYCOSIS is a rare inflammatory condition caused by the ubiquitous anaerobic Gram-positive bacteria “Actinomyces species,” the most common of which is “Actinomyces israelii.” This Actinomyces is a Gram-positive, microaerophilic bacterium that is found in the normal human flora and normally colonizes the human mouth, digestive and genital tracts. Actinomyces necessitates the presence of numerous other bacteria, which destroy the over-vascularized regions and converts the aerobic microenvironment to anaerobic. Actinomyces can then easily migrate, infect, and multiply in damaged tissue ACTINOMYCOSIS is uncommon, and has been reported sporadically in the literature Primary bowel ACTINOMYCOSIS is reported infrequently in the literature [ACTINOMYCOSIS causing chronically ruptured appendix ACTINOMYCOSIS mimicking malignant large bowel obstruction ACTINOMYCOSIS was reported due to dropped gallstones after a laparoscopic cholecystectomy ACTINOMYCOSIS, resulting in retroperitoneal fibrosis Abdominal terature , and somACTINOMYCOSIS. In 1967, the link between pelvic ACTINOMYCOSIS and IUD use was first identified by Brenner and Gehring [ACTINOMYCOSIS, an IUD must be changed at least every five years [ACTINOMYCOSIS infection.We believe that the IUD was the predisposing factor in our patient's invasive pelviabdominal  Gehring . IUD colve years . The latACTINOMYCOSIS involving the gastrointestinal (GIT) tract in 18 patients with pathologically proven pelviabdominal ACTINOMYCOSIS. Eight of these patients had previously used IUD. In half of the patients, the sigmoid colon was the most commonly involved region, and all had concentric (n = 15) or eccentric (n = 3) bowel wall thickening. With widespread and severe inflammatory infiltration, the thickened bowel wall was enhanced uniformly in 9 patients and unevenly in the other 9. A peritoneal or pelvic mass  was seen in 17 patients adjacent to the involved bowel and appeared to be heterogeneously enhanced in the majority of cases; infiltration into the abdominal wall was seen in 4 patients. The authors advised that ACTINOMYCOSIS should be considered when CT scans show bowel wall thickening and regional pelvic or peritoneal mass with extensive infiltration, particularly in patients with abdominal pain, fever, leukocytosis, or long-term use of IUD ACTINOMYCOSIS. Colon cystic masses are more common in the rectosigmoid colon, whereas purely solid masses are more common in the transverse or ascending colon [Lee et al. ng colon .ACTINOMYCOSIS is an uncommon infection that may have serious and even fatal consequences. Pelviabdominal ACTINOMYCOSIS should be considered as a potential diagnosis in female patients with long-term use of IUD and symptoms of abdominal pain, in association with fever, leukocytosis, and elevated markers of inflammation, provided that other causes with similar clinical presentations have been ruled out.Invasive pelviabdominal ACTINOMYCOSIS while excluding other conditions with similar radiologic findings such as Crohn's disease, intestinal tuberculosis, and excavated malignant tumors. Actinomyces must be identified using histopathological examination of biopsy specimens and/or anaerobic culture growth for aspirated free abdominal fluid for a definitive diagnosis. Ceftriaxone 1-2 g every 24 hours for 6 weeks is a reasonable first-line treatment for severe and invasive ACTINOMYCOSIS infections, followed by 3 months of oral doxycycline.Radiology plays an important role both in diagnosis and at follow-up. Mural invasion with stricture formation, mass effect with tapered narrowing of the lumen and thickened mucosal folds ACTINOMYCOSIS as a complication of long-term use of intrauterine contraceptive device. The important role of imaging in diagnosis and follow-up.Herewith we confirm that a patient consent form has been obtained. For our article entitled: Pelviabdominal


Laryngeal adult-type hemangiomas are very rare lesions, more frequent in men, whose optimal treatment consists of microlaryngoscopical excision. We herein report a case of larynx cavernous hemangioma in a 64-year-old woman with hoarseness for about six months. Histologically, the tumor was composed of multiple vessels embedded in an edematous stroma. According to the 2017 World Health Organization (WHO) Classification of Head and Neck Tumors, epithelial tumors are the most frequent laryngeal neoplasms . ParticuA 64 years old female presented to the “E.N.T. Section” of University Polyclinic of Catania with a history of hoarseness for about six months. The patient reported no history for neoplastic diseases nor smoking habit. Direct laryngoscopic examination showed a supracordal reddish nodular mass without ulceration of the overlying mucosa, of about 4 cm in its maximum diameter Fig. . The lesSmooth muscle bundles embedded within the edematous matrix were also found. Necrosis, mitoses, and nuclear pleomorphism were absent. Immunohistochemically, the endothelial cells were positive for ERG, CD31 and CD34. Based on both morphological and immunohistochemical features the diagnosis of cavernous hemangioma of vocal cord was rendered.Laryngeal hemangiomas are typically classified into adult and juvenile forms , 5. The More invasive treatments, such as thyrotomy, lateral pharyngotomy and transient tracheotomy, can be performed when dealing with larger lesions, which are more likely to be at risk of bleeding during minimally invasive procedures. Steroids and radiation therapy can also be used in case of large-sized tumors to improve obstructive symptoms, but often the results obtained are transient , 9.The present paper emphasizes the concept that hemangioma, especially in the adult form, is an unusual but existing laryngeal neoplasm; accordingly, due to its benign clinical behaviour and its different management compared to the most common epithelial neoplasms, it should be always included in the clinical and histopathological differential diagnosis of laryngeal masses.


Inguinal hernia repair using prosthetic mesh is used as a standard treatment in most countries and considered superior to primary suture repair. Although prosthetic mesh has greatly reduced the risk of recurrence, the risk of mesh infection remains. A 71-year-old man was diagnosed with symptomatic bilateral inguinal hernias. He underwent successful laparoscopic transabdominal preperitoneal (TAPP) repair and was discharged the same day. After 3 days, he was diagnosed with small bowel perforation, and underwent emergency surgery. We found perforation of the distal ileum caused by the fecal impaction and severe intra-abdominal contamination. We performed subtotal colectomy and ileosigmoid anastomosis, but did not remove the prosthetic mesh because the previous TAPP site was intact. The patient recovered well post-operatively. Therefore, contaminated or dirty surgery immediately after the hernia mesh surgery could be a feasible treatment. Transabdominal preperitoneal hernia (TAPP) repair is an approved and common surgical approach for the treatment of inguinal hernia in adults, particularly for bilateral and recurrent inguinal hernia after open surgery . With thA 71-year-old man was diagnosed to the surgical department with symptomatic bilateral inguinal hernias and he had undergone robot-assisted radical prostatectomy 2 years ago. The bilateral inguinal hernias were evident on clinical. He underwent successful laparoscopic TAPP repair and was discharged on the operation day .3/mm3 and hemoglobin was 13.5 g/dL. The C-reactive protein was 34.33 mg/dL, procalcitonin was 14.50 ng/mL and creatinine was 1.79 mg/dL. He underwent computed tomography (CT), which showed focal bowel wall defect at the distal ileum, and complicated fluid collection and free air in the abdominal cavity (Three days after discharge, he presented to the emergency room with severe abdominal pain. He did not have bowel movement for a week. He had severe constipation and frequently dug up the stool with his fingers. A physical examination revealed abdominal distension and rigidity with rebound tenderness. The patient’s blood pressure was 110/70 mmHg, pulse rate was 116 beats/min and body temperature was 38.0°C. In the complete blood count, a white blood cell count was 2.04 × 10l cavity . The patOn laparoscopic exploration, we found perforation of the distal ileum caused by the fecal impaction and severe intra-abdominal contamination . The entNumerous studies revealed that patients who underwent laparoscopic repair had low recurrence rate, had fewer post-operative complications, were discharged earlier and were able to return to their daily lives more quickly than patients who underwent open hernia . Howeveret al. [Belansky et al.  describeet al. [Contamination of a field during a hernia repair can occur due to many factors such as an inadvertent enterotomy or need for concomitant bowel resection at the time of repair . Xourafaet al.  examinedet al. .On the other side, it has not been demonstrated that there is an increased risk of mesh contamination in the event of simultaneous operations performed on the digestive tract. Some authors report prosthetic repair of the abdominal wall after colonic resection with good results , 7. ManyRegarding TAPP surgery, there were some cases of bowel perforation and the timing of occurrence was different for all reported cases, but mesh site infection was not observed during the follow-up . In our


The natural history of intracholecystic papillary neoplasm (ICPN), especially the speed of growth from small benign to a carcinomatous lesion, is quite unrevealed. Here, we report an extremely rare case of ICPN, in which the papillary lesion was observed transforming from small and benign to malignant using abdominal ultrasound (AUS) over 2 years during routine health checks. A 44-year-old man underwent a routine health check-up. The initial AUS showed a small sessile polyp in the gallbladder, which enlarged slightly at the next AUS, a year later. In the third year, the polypoid lesion enlarged markedly, with a maximum diameter of 10 × 9 × 7 mm. Therefore, a laparoscopic cholecystectomy was performed. Microscopically, the 10 mm tumor had intracytoplasmic mucus, and a clear cytoplasm compatible with gastric-type features. Immunohistochemical analysis showed positive staining of atypical cells for MUC6 and PAS. These findings led to the diagnosis of ICPN with high-grade intraepithelial neoplasia of the gastric type. In conclusion, sessile polyps with rapid growth might be a crucial finding in the early stage of ICPN. The intracholecystic papillary neoplasm (ICPN) of the gallbladder, which was established in 2010 by the WHO, and was revised in 2019 is considered a preinvasive neoplastic lesion [IPMN has a well-defined illness concept, and the risk of malignant transformation has been revealed in several studies –5. AdditA 44-year-old man underwent a routine health check-up. He did not have any abdominal symptoms and his blood cell count and liver function test results were all within normal range.The initial AUS performed in September 2017 showed multiple polyps with a maximum diameter of 3 mm and adenomiomatosis (Fig. The preoperative diagnosis was a cholesterol polyp with rapid enlargement or pyloric-type adenoma of the gallbladder. Gallbladder cancer was a differential diagnosis; however, the probability of advanced disease was estimated to be low, and we planned a laparoscopic procedure without rapid intraoperative histological diagnosis. The patient underwent laparoscopic cholecystectomy. Based on the laparoscopic findings, the gallbladder serosa was normal and there were no findings suggesting advanced cancer. The postoperative course was uneventful. Macroscopically, the resected specimen showed a 10 mm polypoid lesion along the narrow fibrovascular stalks, accompanied by obvious background cholesterolosis Fig. . There wIn the present case, we report a significant growth of ICPN from small sessile polyps to polypoid lesions with high-grade dysplasia, which could be successfully observed using AUS for two and a half years. Because the small sessile polypoid lesion was considered as benign lesion at the initial diagnosis, conservative observation was selected as the treatment option and the history of the sessile lesion was evaluated via US every year. The follow-up AUS captured the rapid enlargement of the tumor. Although clinical characteristics, etiology, and treatment options have often been discussed –14, the In general, sessile polyps in the large intestine, which are found more often in people who smoke, are known as precancerous lesions; thus, they are treated as precancerous. The cells of this type of polyp look serrated, or have a sawtooth appearance under a microscope, which gives them their name. Surveillance colonoscopies or other tests may be necessary on detection of large sessile serrated polyps. Regarding a sessile polyp lesion of the gallbladder, Ishikawa et al. . reporteThe accumulation of genetic mutations with tumor progression was suggested in the previous case series of ICPN, and ICPN exhibited slight p53 and MIB-1 staining, reflecting its mutation in a previous case series . PositivIn conclusion, sessile polyps with rapid growth might be crucial findings in the early stage of ICPN, allowing us to consider the malignant potential of the gallbladder polyp. However, this is the first case study in which we followed its natural history annually, and further case accumulation is needed to clarify the clinical features of ICPN.


Adenoid cystic carcinoma (ACC) is a rare malignancy of the larynx. Surgical excision seems to be the preferred treatment modality; however, a paucity of high-evidence suggestions due to the small number of cases have been noted. Here we present the case of a 35-year-old woman with subglottic ACC who denied laryngectomy and opted for concurrent chemotherapy and radiotherapy. She remains disease-free four years later. This case illustrates that concurrent chemoradiation, instead of laryngectomy, should be considered in selected cases. Squamous cell carcinoma is the most common neoplasm of the larynx . While aLaryngeal ACC develops slowly and the symptoms tend to appear late . The accIn this paper, we report a case of a laryngeal ACC which was treated without surgery and remains disease-free after four years.A 35-year-old woman presented to our emergency ENT department with the chief complaint of progressive dyspnea and inspiratory stridor over the past three months. She reported hoarseness and mild dysphagia for two years but no otalgia or weight loss. She has consumed alcohol socially and smoked 20 cigarettes per day for 15 years. She is otherwise healthy and employed as a babysitter.On physical examination, a subglottic tumor was detected through rigid endoscopic laryngoscopy. The mass was smooth, not ulcerated or friable, located below the vocal folds, and narrowing the airway Figure . Vocal fThe patient had undergone a cervical MRI and chest CT in another hospital three days before her referral to the emergency department of our hospital. These studies revealed a 1.6x1.8x1.3 cm subglottic mass without any involvement of laryngeal cartilages. Neither adenopathy nor distant metastases were identified.An emergency tracheotomy was performed under local anesthesia to secure the airway because of the obstructive nature of the neoplasm. A panendoscopy for assessment of the upper aerodigestive tract was performed. Panendoscopy included biopsies from every site of the upper aerodigestive tract suspicious for malignancy . Biopsies revealed a subglottic laryngeal adenocystic carcinoma of a cribriform pattern Figure .The patient was informed about the pathology results and a total laryngectomy was suggested as the treatment of choice. Every complication of the operation and every impairment in the quality of life was meticulously explained. The patient denied the operation and opted for concurrent chemotherapy and radiotherapy to avoid a permanent tracheostomy. Thirty-three courses of radiation with a total of 66 Gray and seven weekly cycles of platinum-based chemotherapy were conducted. Lymph nodes of the neck were excluded from the radiation field because of the histologic nature of the tumor. Two months after the end of the treatment the tracheostomy tube was removed uneventfully.The patient was informed that a lifelong follow-up is mandatory and that in case of a local recurrence a salvage laryngectomy will probably be the only available modality. Lifelong follow-up includes at least a visit per year even after 10 years of treatment due to the tendency of neoplasm to give late metastasis. A new MRI was performed 18 months after the treatment, which did not reveal any abnormal imaging findings. Four years later and to date the patient remains disease-free with only the minor complaint of dryness in the oral cavity. A flexible laryngoscopy was performed every three months and yearly MRIs did not reveal any recurrence or metastasis.ACC is a rare malignancy of the larynx, with a mean age of diagnosis between the fifth and sixth decade of life and with no clear prevalence between genders . SubglotThere are no specific guidelines or consensus for the treatment of laryngeal ACCs. The literature lacks high-evidence suggestions grounded on organized cohorts or trials, because of the rarity of the neoplasm. The proposals found in the literature are based typically on case reports or small case series -10. SurgA systematic review reported only four cases treated with chemoradiation for organ preservation considerations . Half ofAdditionally, distant metastases leading to late recurrence are not uncommon ,14. RadiThe role of concurrent chemoradiation for laryngeal ACCs, however controversial, has the huge advantage of larynx preservation. Even though surgical resection is the treatment modality of choice, late distant metastases occur often. The successful course of our case so far suggests that concurrent chemoradiation may be equal or even superior to surgical resection in selected cases, especially when combining considerations of patients’ quality of life and survival rate. As more cases are discovered and reported, meta-analyses may serve to uncover more details regarding this treatment dilemma. Furthermore, there is a necessity for large prospective studies to clarify the advantages and disadvantages of every method in an evidence-based fashion.


A 37-year-old female patient presented with shortness of breath, cough, and chest pain complaints from the 12th week of her first pregnancy. At the 28th week, labor induction had to be performed because of severe dyspnea and hyoxemia. Thereafter, a diffused pulmonary embolism was detected by echocardiography and CT angiography, without histological diagnosis. Pulmonary endarterectomy was performed, and it was found during operation that a huge, lobular mass originated in the posterior wall and extended throughout the vasculature of both lungs, and a mucinous pellicle covered the entire pulmonary endothelium. Pathology revealed a low-grade myxofibrosarcoma with positive vimentin and SMA, partially positive CD-34. Low-grade myxofibrosarcoma (LGMFS) is a low-grade malignant mesenchymal tumor, which usually arises in the extremities of 60–80-year-old patients and rarely originates in the pulmonary artery of young patients. Thereby, it is often misdiagnosed as chronic pulmonary thromboembolism. Furthermore, surgical operation for these patients is still a challenge. Here, we report a rather rare case with incidence of LGMFS during pregnancy.A 37-year-old female patient presented with shortness of breath, cough, and chest pain complaints from the 12th week of her first pregnancy. However, no special examination or treatment was performed. Thereafter, these symptoms were gradually worsened. At the 28th week of pregnancy, labor induction had to be performed because of severe dyspnea and hyoxemia. Two weeks later, she was admitted to our hospital for continually aggravated symptoms. She reported an apparent distress at rest but no syncope, palpitations, lower-extremity edema, weight loss, fever, rashes, or arthritis. There was a history of hysteromyoma defined by transvaginal ultrasound but no history of tobacco, estrogen, appetite suppressant, or illicit drug use, deep-venous thrombosis, or familial pulmonary hypertension or thromboembolic disease. No positive sign was found in physical examination.Chest radiography showed patchy alveolar opacities in both lower lobes, right-ventricular enlargement, and prominent central pulmonary artery. Echocardiography revealed a lobulated mass occupying attached by a pedicle to the posterior wall of the main pulmonary artery, a right-ventricular enlargement with an estimated pulmonary artery systolic pressure of 79 mmHg and normal left ventricular size and function. Subsequently, diffuse pulmonary embolism was detected by CT angiography Figure 1The operation was performed at undiagnosed conditions, due to severe dyspnea and hyoxemia. Femoral cannulation of cardiopulmonary bypass (CPB) was performed for preparation of possible ECMO. When the pulmonary trunk was incised, it was found that a huge, lobular mass originated in the posterior wall and extended throughout the vasculature of both lungs, and a mucinous pellicle covered the entire pulmonary endothelium Figure 2Pulmonary thromboembolism is a common complication after pregnancy . The patBecause of the similar clinical presentations and no specific biomarker, it is very difficult to differentiate pulmonary artery tumor and thromboembolism without histological diagnosis, which is often impossible preoperatively , so thatDue to better prognosis in patients with complete resections than incomplete resections , it is aIt has been reported that vimentin and SMA are used for identification of myxofibrosarcoma. In this patient, besides the tumor cells being vimentin and SMA positive, these cells partially expressed CD-34, perhaps representing a novel myxofibrosarcoma cell line or suggesting a conversion from myxofibrosarcoma cells to endothelial cells. Similar reports are absent.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.KX carried out data collection and immunohistochemical analysis. LL and G-WZ participated in clinical treatment. H-JL and G-WZ drafted the manuscript. All authors read and approved the final manuscript.Author H-JL was employed by Shenyang Medical and Film Science and Technology Co. Ltd., Shenyang, China.The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Porokeratosis describes a heterogenic group of keratinization disorders in which lesions are papules and plaques that demonstrate central atrophy surrounded by a hyperkeratotic margin. Clinical variants include not only porokeratosis of Mibelli, but also disseminated superficial, disseminated actinic superficial, linear, punctate, and palmaris et plantaris disseminata. Porokeratosis has a risk of malignant transformation. A woman with disseminated superficial actinic porokeratosis (DSAP) whose lesions presented as pruritic plaques and papules is described. The diagnosis was suspected clinically, supported by dermoscopy findings, and confirmed histologically. The condition-associated pruritus was managed symptomatically; her skin lesions will be monitored clinically. Clinical manifestations, dermatoscopic features, pathology findings, and treatment options for DSAP are summarized. Porokeratosis refers to a group of keratinization disorders that are divided into various types based on the clinical picture and lesion distribution. Common clinical variants of porokeratosis include disseminated superficial actinic porokeratosis (DSAP), disseminated superficial porokeratosis, linear porokeratosis, porokeratosis of Mibelli, porokeratosis palmaris et plantaris disseminata, and punctate porokeratosis; some of the less frequent subtypes of porokeratosis include eruptive bullous, follicular, genitogluteal, lichen planus-like, porokeratotic acanthoma, porokeratotic adnexal ostial nevus, and pruriginous. All forms of porokeratosis present with a centrally atrophic area that is surrounded by a well-demarcated hyperkeratotic ridge; the microscopic examination of this ridge demonstrates a cornoid lamella -3.DSAP is the most common clinical variant of porokeratosis; multiple papules and plaques appear bilaterally on sun-exposed areas with a predilection for distal limbs. The lesions have a slightly reddish or brownish hue and the surrounding ridge typically appears more accentuated than the circumscribed interior. Annular or irregular configurations are characteristics as the lesions centrifugally expand ,5.The diagnosis of DSAP is suspected clinically; dermatoscopy and microscopic evaluation of a tissue biopsy specimen are useful aids to support and confirm the diagnosis -12. An oA 67-year-old woman presented with multiple itchy red lesions on the distal arms and legs. The lesions had been present for five years. The patient complained that each year, during the summer, the itch was more intense, the redness was brighter, and the number of lesions increased. She had a history of chronic sun exposure which occurred during gardening. There was no drug history and her family history was negative for similar skin lesions.A comprehensive physical examination did not reveal any systemic abnormalities. Cutaneous examination showed multiple erythematous to brownish plaques and a few papules ranging between 5 mm and 20 mm in diameter. The annular lesions were well-demarcated and had a thin, raised, keratotic margin Figure .Several lesions were examined using a dermatoscope and the dermoscopy features were noted. The lesions showed either a single or double-track at the periphery; the track was white on the inner side and brown on the outer side. In addition, brown dots were densely arranged in larger or smaller areas within the peripheral white or brown lines Figure .Other lesions had a thin, discrete border-that was either white or pale brown-with densely arranged dotted and linear vessels present centrally on a red homogeneous background. White-yellow clods were also noted in the central area. The clods represented follicular involvement of the lesion Figure .Additional lesions showed white and brown tracks at the periphery. They also had discrete, sparse brown dots and vessels in the center Figure . FinallyA punch biopsy was performed from the margin of a lesion located on her left leg. Microscopic examination of the tissue specimen showed an area of hyperkeratosis that included a peripheral column of parakeratosis, the cornoid lamella, situated above part of the epidermis that contained dyskeratotic keratinocytes. The epidermis had flattening of the rete ridges and there was severe solar elastosis in the dermis Figure .Correlation of the patient’s clinical presentation, dermoscopic findings, and lesion pathology established the diagnosis of DSAP. Initial management focused on the symptomatic relief of her pruritus. She was treated with topical methylprednisolone aceponate 1 mg per g cream applied once daily for three weeks. At a one-month follow-up visit, the pruritus had resolved. However, neither the number nor the morphology of her DSAP lesions was changed.The patient declined any other topical or systemic treatment for her condition, including cryotherapy. Photoprotective measures-such as long sleeves, trousers, and sunscreen-were initiated; it was emphasized that these measures should especially be used during the warmer months of the year when she was outside. She will also return for regularly scheduled follow-up skin examinations to evaluate for any premalignant or cancerous changes in her DSAP lesions.DSAP is a benign intraepidermal condition caused by sun exposure or artificial ultraviolet radiation exposure or both in a patient who is genetically predisposed. However, other factors can also be involved in the pathogenesis of DSAP. For example, immunosuppression and aging can cause somatic homologous recombination with an ultraviolet signature in the epidermis ,13.Familial DSAP usually manifests during the third or fourth decade of life and exhibits an autosomal dominant mode of inheritance. The sporadic form of DSAP is characterized by a late-onset and a negative family history of the condition. In addition, congenital heterozygous mutations in genes of the mevalonate pathway have been identified as a causative factor behind both familial and sporadic cases. The patient in this report had the sporadic form; her lesions initially appeared at the age of 62 years ,13.DSAP lesions characteristically appear on sun-exposed skin. Facial lesions of DSAP, albeit rare, can also develop. Indeed, approximately 15% of DSAP patients have lesions on their faces ,13.Sun exposure is likely to be the primary etiologic factor for the development of porokeratotic lesions in the reported patient. Her lesions appeared exclusively on sun-exposed areas such as the forearms and distal legs and were always aggravated after sun exposure during the summer while gardening. The presence of rosettes, visualized using a polarized dermatoscope, within the porokeratotic lesions also supports this hypothesis; rosettes, although not DSAP lesion-specific, are a common finding in sun-damaged skin ,13.Porokeratotic lesions are persistent. Their evolution is marked by changes in color and shape; the changing morphology may be secondary to the degree of inflammation associated with the lesion. These changes can be observed using a dermatoscope. Indeed, investigators have described the clinical and dermatoscopic evolution of DSAP lesions from a non-atrophic lesion with a circular contour to an atrophic lesion that is polycyclic -7,10,12.The initial lesions of the patient in this report appeared as round, non-atrophic brown papules; dermoscopy showed a white and brown annular double-track at the periphery and brown dots in the surrounded central area. Older, evolving lesions, had an atrophic center, and were round and red; dermoscopy showed central vessels and a peripheral white track. Finally, inactive atrophic lesions were polycyclic and skin-colored; dermoscopy showed a white track not only at the periphery but also inside the lesion.The DSAP lesions of the reported patient also had clinical and dermoscopic features of follicular involvement. This was observed clinically as small, round keratotic areas within the porokeratotic lesions. Dermatoscopically, it presented as white-yellow clods. Follicular involvement in porokeratosis is rarely described in the literature; however, retrospective histologic studies have found this feature to be commonly encountered .The histologic sine qua non of porokeratosis is the cornoid lamella. It appears as an oblique column of parakeratosis that indents the epidermis; the granular layer is either reduced or absent and there are dyskeratotic keratinocytes. The cornoid lamella corresponds clinically to the hyperkeratotic ridge. A lymphocytic inflammatory infiltrate or solar elastosis or both may be present in the dermis; the reported patient had severe solar elastosis ,13.Porokeratosis can be a premalignant disease. Malignant transformation occurs in 6.9% to 11.6% of patients . SquamouDisseminated porokeratosis can also be associated with other disorders. These include not only autoimmune diseases treated with systemic corticosteroids but also immunosuppressive conditions such as chronic liver disease, human immunodeficiency virus infection, and solid organ transplant recipients. In addition, hematologic dyscrasias and solid tumors have rarely been described in patients with disseminated porokeratosis .There are several treatment options that have been described for patients with DSAP. They include cryotherapy, diclofenac, 5-fluorouracil, imiquimod, photodynamic therapy, retinoids, and vitamin D3 analogs. Laser therapy-including carbon dioxide laser and fractional photothermolysis-has also been performed. Even superficial radiotherapy-using Grenz ray-has been used ,16-18.Topical corticosteroids for individuals with pruritic lesions, similar to the patient in this report, have also been used -18. In aDSAP is a keratinizing disorder that typically occurs on sun-exposed areas. The woman described in this report had late-onset sporadic DSAP. Her diagnosis was suspected clinically and confirmed by microscopic examination of a tissue biopsy specimen. Dermatoscopic evaluation supported the diagnosis of the patient’s DSAP. Indeed, dermatoscopy is a useful and non-invasive diagnostic procedure that can be used when the diagnosis of porokeratosis is being considered to exclude other conditions. There is a risk of malignancy developing in DSAP lesions. Photoprotective measures should be encouraged in order to minimize the risk of further skin damage. Also, patients should return on a regular basis for monitoring of their lesions.


Nodular lymphoid hyperplasia (NLH) is characterized by the growth of multiple discrete small submucosal nodules specifically confined to the lamina propria and superficial submucosa layers of the intestinal wall. Gastric and rectal involvement of NLH is exceedingly rare. To date, few case reports have described diffuse NLH presenting with multiple submucosal lymphomatous polyposis occurring in the rectum. Our patient is a 39-year-old morbidly obese Hispanic female who presented to the gastroenterology clinic complaining of intermittent hematochezia for the past six months. Colonoscopy showed diffuse nodularity in the sigmoid colon and rectal mucosa, extending 20 cm from the rectal verge. Rectal biopsies revealed moderate active chronic inflammation predominantly of lymphoplasmacytic cells with areas of lymphoid hyperplasia and focal surface ulceration. Immunohistochemistry stains revealed reactive lymphoid hyperplasia (RLH). The NLH is a risk factor for extraintestinal and intestinal lymphomas. When encountering lymphoid hyperplasias, the possibility of malignancy must always be considered. It is crucial to monitor patients with NLH via capsule endoscopies, small bowel series, and colonoscopy for surveillance of new lesions in light of the potential for malignancy.  The formation of several distinct small submucosal nodules exclusively limited to the lamina propria and superficial submucosa layers of the intestinal wall characterizes nodular lymphoid hyperplasia (NLH). Gastric involvement of NLH is extremely rare . In the When these mucosal lymphoid aggregates in the small and large bowel it is known as diffuse lymphoid hyperplasia, which is common and benign . These lThis case report was presented as a poster presentation at the American College of Gastroenterology 2021 conference on October 24th, 2021. A 39-year-old morbidly obese Hispanic female was referred by her primary care physician (PCP) for evaluation of intermittent bloody stools for the past six months mixed with the stools and also with wiping. The patient reported straining with defecation and incomplete evacuation of bowel movement with small-caliber stools . She denied any prior colonoscopy workup, tobacco, illicit drug abuse, or heavy alcohol use. There were no known family members with gastrointestinal tract (GI) tract, uterine or breast cancers in the family. Physical exam was found to be unremarkable with rectal exam revealing no blood, anal fissures, or external hemorrhoids. A digital rectal exam was performed and revealed brown stool, no gross blood, and no melena. The patient was scheduled for a colonoscopy which showed diffuse nodularity in the sigmoid colon Figure  and rectThe patient was advised to adhere to a high fiber diet, increase oral fluids, and start topical hydrocortisone 2.5% cream for symptomatic relief of her hemorrhoids. It is not known how the patient responded to therapy as she was lost to follow-up.Hematochezia is a common presentation that encompasses a broad differential. Diffuse NLH with rectal involvement, although uncommon, represents an important differential that must be kept in mind. When encountering lymphoid hyperplasias the possibility of malignant lymphomas always comes to question, however, the actual findings are difficult to classify as benign or malignant . It is dHelicobacter pylori infection [Lymphoid hyperplasia in the intestine is a rare disorder and diffuse nodular hyperplasia is even more scarce . While tnfection . While the pathogenesis for lymphoid hyperplasia is unknown, there are different theories to its cause varying on the presence or absence of associated immunodeficiency disorders. When immunodeficiency is not present, it is believed that NLH is related to immune stimulation of gut lymphoid tissue . An antiA study of 781 non-protruding colorectal neoplasias (adenomas and early carcinomas) in Sweden and Japan, suggested an association with colonic lymphoid nodules . The conH. pylori infection were studied by Albuquerque et al. In contrast to patients with chronic H. pylori infection, those with eradicated H. pylori had a substantial clinical response and lesions regression/resolution [The focus on the management of lymphoid hyperplasia does not usually require treatment, and is typically directed towards concomitant disorders. Patients with NLH and solution . Howeversolution . Diffuse NLH with rectal involvement is a rare disease and differentiating it from other gastrointestinal diseases is a clinical challenge. We report a case of NLH with no notable prior medical history or risk factors. Due to the potential of malignant transformation, periodic surveillance is recommended. Appropriate investigations are needed to better understand the etiology, pathogenesis, and proper management to improve long-term survival in patients.


Congenital laryngeal cysts are a rare cause of stridor in infants. These cysts may have serious clinical implications if not promptly recognized. A small laryngeal cyst may remain asymptomatic. However, if large, it can block the laryngeal inlet leading to acute airway obstruction, which is potentially life-threatening. In pediatric patients presenting with respiratory distress, prompt diagnosis and surgical management are crucial to avoid infant morbidity and mortality. We describe two cases of laryngeal vallecular cysts in infants and their management in our clinical practice. Stridor in an infant is related to several pathologies of the airway. When accompanied by respiratory distress in a neonate, it must be addressed urgently. More common pathology includes laryngomalacia, tracheomalacia, vocal cord paralysis, and subglottic stenosis, among others. Although rare, congenital laryngeal cysts are one of the causes of stridor in an infant . CongeniCase 1An eight-day-old baby girl was referred to the otorhinolaryngology team for stridor. She was born prematurely at 34 weeks and three days via spontaneous vaginal delivery. Her Apgar score was 4 out of 10 at birth; hence, she was intubated at four minutes of life. She was subsequently extubated on day two of life; however, she persistently required non-invasive oxygen supplementation. She was noted to have noisy breathing. Physical examination found soft stridor with minimal substernal and subcostal recession. She also had features of soft dysmorphism, microcephaly, flat nasal bridge, and hypertelorism. Bedside flexible laryngoscopy was done on her second week of life, revealing a floppy epiglottis, redundant arytenoid mucosa, and secretions at the subglottic region with the presence of a small laryngeal cyst at the valleculae. The baby underwent direct laryngoscopy and examination under general anesthesia, which revealed laryngomalacia (omega-shaped epiglottis with tight aryepiglottic folds) and subglottic stenosis with synchronous vallecular cyst . Bedside flexible laryngoscopy showed features of laryngomalacia, tubular epiglottis, tight aryepiglottic fold, and floppy arytenoids. There was a pooling of secretions at the pyriform fossae. The child underwent direct laryngoscopy under general anesthesia, which revealed a cystic mass over the valleculae Figure . SimilarCongenital stridor can be caused by several conditions. These conditions can be broadly divided into supraglottic, glottic, and subglottic conditions. Laryngomalacia is the single most common congenital laryngeal abnormality causing stridor. This is followed by subglottic stenosis, vocal cord paralysis, and laryngeal webs . LaryngeThe clinical presentation of laryngeal cysts varies according to site and size . A largeMinimally invasive surgery and cyst removal is the treatment choice for congenital laryngeal cysts. However, there are several surgical modalities for the treatment of congenital cysts. They include endoscopic excision, deroofing, and needle aspiration. This is done after securing the airway with endotracheal intubation, and tracheostomy is performed in some cases where endotracheal intubation is not possible . Needle The surgery was performed in the same setting as confirmation of diagnosis with direct laryngoscopy under general anesthesia. A vallecular cyst may often be approached via the transoral route, and an external approach is required only in rare cases due to large and recurrent lesions . In bothBoth cases described how a benign swelling can potentially lead to potentially life-threatening airway compromise. Although rare, congenital laryngeal cysts should be considered as part of our differential diagnosis when attending to an infant with stridor.Laryngoscopy should be included as part of the assessment when approaching a child with stridor. Prompt diagnosis, if made, can lead to early minimally invasive surgical procedures, which are simple, safe, and effective to avoid potentially life-threatening airway obstruction.


This paper retrospectively analyzed the case data of an adult hamartoma of the left chest wall, and combined with the literature analysis, to discuss the clinical characteristics, imaging diagnosis and differential diagnosis of hamartoma. CT and MRI findings of the patient showed medial occupation of the left serratius anterior muscle, with visible fat and calcification. Different from the previous reports of chest wall hamartoma in terms of age, location and imaging characteristics, this case had certain characteristics, and the diagnosis needed pathological confirmation after surgical resection. The word hamartoma comes from the Greek “hamartanein”, meaning to go wrong, and was introduced by AlBrecht in 1904 . It may A 59-year-old female patient was admitted with the chief complaint of “left shoulder mass for 4 years”. The patient underwent chest CT examination in our hospital 4 years ago and showed soft tissue occupation in the left anterior shoulder blade. The patient did not have chest distress, chest pain, palpitation, shortness of breath, chest wall skin swelling and rupture, fever, chills and other discomfort, and no special treatment was given. After that, the patient underwent regular physical examination every year, and the left scapular mass was found to increase slowly. Half a month ago, the patient underwent physical examination again in our hospital, and chest CT showed soft tissue mass in the left anterior scapula with calcified nodules, suggesting further examination. He was admitted for further treatment on August 26, 2021.Physical examination showed that the chest was symmetrical without deformity, the left posterior back was protruded, the texture was hard, there was no tenderness, the skin was not redness, swelling and ulceration, the bilateral respiratory movement was consistent, the bilateral tactile chatter was symmetrical, the bilateral lung percussion sound was clear, the bilateral lung breathing sound was clear, no dry and wet rale was heard, and no pleural friction. The patient had previously received cervical polypectomy in our hospital. Hyperlipidemia was treated with rosuvastatin orally for 5 years.She suffered from depression for 10 years. No infectious or chronic disease or history of trauma was reported.Chest CT Figure 1The patient completed preoperative examination on August 31, 2021 under general anesthesia, “left thoracic wall soft tissue tumor resection + negative pressure suction implantation”. After the patient was admitted to surgery, intraoperative ultrasound examination was performed to determine the location of the tumor, and the skin was cut about 4cm above the left thoracic wall tumor in the armpit to separate the subcutaneous tissue and muscle. There was a mass on the deep surface of serratus anterior muscle with regular shape, clear boundary and good range of motion. The tumor was removed completely along the blunt + sharp surrounding layer by layer. Postoperative histopathological examination Figure 2The word hamartoma comes from the Greek “hamartanein”, meaning to go wrong, and was introduced by AlBrecht in 1904 . HamartoPathologically, hamartoma can be divided into two types: mesenchymal hamartoma and epithelial or glandular hamartoma . MesenchIn terms of imaging, the imaging manifestations of chest wall hamartoma have certain specificity, mainly calcification and fat. CT is sensitive to calcification, and a large range of fat can also be found, but MR shows more clearly for lesions with less fat. MR is more advantageous than CT for the extent of lesions.In 18F-FDG PET-CT, hamartomas usually have no or only slight increase in uptake, and the uptake on delayed imaging is often unchanged or decreased. FDG PET-CT can better determine the benign and malignant lesions than MSCT . Sun PinIn this case,the patient in this case was an adult female who was found to grow slowly, and its clinical symptoms were similar to those of hamartomas in other parts of the body. CT showed oval mixed density lesions on the medial side of the serratus anterior muscle, calcification and fat density lesions could be seen inside, and no clear damage or morphological changes could be seen near the ribs of the lesions on the window of bone. MRI could more clearly identify the scope and composition of the lesions, The lesions showed high signal on T2WI and low signal in strips, isointensity in T1WI, high signal in strips on T1WI, low signal in reverse phase, high signal on DWI, no significant enhancement in the center of the lesion after enhancement, enhancement at the edge, and high signal on DWI. Internal fat is hypointense in the fat-suppressed phase, which is a specific sign of hamartoma. The main differential diagnosis includes teratoma, elastic fibroma and neurogenic tumor. Teratoma is a germ cell tumor derived from primordial germ cells or pluripotent embryonic stem cells, mostly located in the midline or parastinal area of the body. Teratoma of the chest is more likely to occur in the mediastinum, and its imaging features are cystic and fatty, with calcification visible in 50%. The “fat-liquid plane” has more imaging characteristics, so it can be distinguished from chest wall hamartoma in terms of location and imaging characteristics. The location of hamartoma in this case is similar to that of elastic fibroma, which usually occurs in the deep subscapular horn, but the signal of elastic fibroma is similar to muscle tissue due to the large amount of collagen fibers in the tumor. There are fat components included in the tumor, and the imaging manifestation is “lipid striation sign”, but calcification is rare, and the enhancement is not obvious after enhancement. Neurogenic tumors in the intercostals nerve sheath tumor is relatively common, characterized by round or class round soft tissue density with more uneven density, border and clear, the uneven enhancement scan in mild-to-moderate reinforcement, for the larger part of the tumor, change, the necrotic area can see sac tumors had center, no obvious fat component and calcification is rare, therefore with hamartoma.In terms of treatment, hamartoma is a benign tumor with small lesion in biology.Occurs in chest wall hamartomas and is treated in a similar manner to other sites. When there is no obvious symptom in clinic, observation should be the main method. If the lesion is too large to compress the surrounding tissue and cause clinical symptoms, complete surgical resection of the mass can be cured.In conclusion, we report a rare case of thoracic hamartoma in an adult. Although hamartoma may occur in any organ, hamartoma occurring in chest wall without involving ribs is quite rare, and there are few reports in domestic and foreign literature. This article aims to summarize the clinical manifestations, pathological features, imaging findings and differential diagnosis for the mutual learning of the same way.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.This study involving a human participant was reviewed and approved by the ethics Committee of the Aerospace Central Hospital. The patient provided her written informed consent to participate in this study. Written informed consent was obtained from the individual for the publication of any potentially identifiable images or data included in this article.All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Anterior mediastinal masses are rare conditions that can become symptomatic through compression of the airways and vascular structures. Fatal or severe complications can occur during anesthesia and surgery. With this review we aim to describe the state of the art in peri-anesthetic management of mediastinal tumors, which we illustrate with a clinical case.We report a case of a young female patient suffering from a large anterior mediastinal mass that underwent an open biopsy after intercostal nerve blocks (INB) in six consecutive right intercostal spaces (2nd to 7th). A right anterior mediastinotomy was performed and an excellent analgesic effect was achieved. The patient was awake and did not experience significant pain or cough, having received paracetamol 1 g and returned home later in the day. The diagnosis of non-Hodgkin's lymphoma was later confirmed.Our review showed that anesthesia for mediastinal masses' resection or open biopsy is rare and prone to severe complications. Such complications are more important in children, patients in supine position, under general anesthesia and already symptomatic prior to the procedure. INB presents some advantages against paravertebral block (PVB) and thoracic epidural anesthesia (TEA), is easier to reproduce and has a shorter learning curve. Airway stenting with a rigid bronchoscope can be an alternative.Multilevel medial axillary line INBs are safer and easier to reproduce than PVB, have less hemodynamic repercussion than TEA and can, therefore, be preferable for open anterior mediastinal biopsies or small masses resection. •Solo INB for anesthesia in anterior mediastinal masses open procedures is rare.•Symptomatic patients seem to be more at risk for anesthetic complications.•INB presents some advantages against PVB or TEA and seems easier to reproduce. Thoracic epidural anesthesia (TEA), multilevel intercostal nerve blocks (INB), and paravertebral blocks (PVB) are the most frequent locoregional techniques, used alone or in combination with other techniques through different levels of consciousness We report a case of a large anterior mediastinal mass that underwent an open biopsy under multilevel INB, while providing a comprehensive literature review and an algorithm proposal for the management of such lesions. We ensure that this report follows the SCARE 2020 criteria 2A 24-year-old female patient, presented with SVCS. An X-Ray showed an enlargement of the mediastinum . Subsequ2 at 6 L/min were administered and the patient was positioned in a slight anti-Trendelenburg (15°). A short hypnosis was performed. An ultrasound (US)-guided INB was repeated in six consecutive right intercostal spaces (2nd to 7th) at the medial axillary line. The US linear probe  was moved till optimal sagittal views of the ribs, intercostal muscles and parietal pleura were obtained. The needle was inserted in a caudal-to-cranial direction. When the tip of the needle was located under the internal intercostal membrane, at the base of the superior rib, the depression of the parietal pleura by the injection of the local anesthetic confirmed the correct position, after negative aspiration of air or blood. We injected 5 milliliters (ml) of the anesthetic solution (lidocaine 1 % + ropivacaine 0.5 %) per space, with a total of 30 ml. The onset of sensory loss occurred approximately 10 min after the injections. Before starting the incision, 15 cm3 of lidocaine 1 % was subcutaneously injected. Electrocautery was used to divide intercostal muscles at the superior board of the inferior rib, the parietal pleura was opened, a retractor was positioned, and multiple biopsy specimens were taken. An excellent analgesic effect was achieved during the operation, and she returned home later that day, comfortable from an analgesic point. The diagnosis of a non-Hodgkin lymphoma was further confirmed.She was scheduled for a right anterior mediastinotomy aiming for a biopsy of the mass. The first and third authors were assigned to the procedure. Midazolam 1 mg I.V. and O3Current knowledge arises from a couple of randomized control trials (RCT) and retrospective case series of anterior mediastinal masses Moreover, the aggressivity and duration of the procedures described in the literature is diverse with probably longer resections being performed under GA and open biopsies or debulking under locoregional techniques and thus influencing the estimation of complication rates. To this matter, careful revision of the CT scan angiogram for venous structures compression and review with the surgical team of the operative plan are of utmost importance to anticipate difficulties and complication probability. Other preoperative factor that can help predict severe intraoperative complications is pericardial effusion (even more if an AG or positive-pressure ventilation is added). If it is the case, a pericardial drainage, cardiopulmonary bypass, or both can be performed prior Hnatiuk et al. Some strategies are proposed in the setting of an extensive and aggressive surgery in patients at risk of having complications under GA: Awake fiber-optic intubation, intubation distal to the airway compression, inhalation induction with spontaneous ventilation, avoidance of muscle relaxants, avoidance of the supine position, positioning changes, and immediate availability of rigid bronchoscopy if needed. For symptomatic patients requiring a diagnostic open biopsy, a locoregional technique shall be used. Another important aspect to consider is eventual phrenic or recurrent laryngeal nerve compression or lesion which may increase the need for post-operative mechanical ventilation.There are a few locoregional options to obtain proper analgesia. Numerous authors reported on the successful use of TEA to perform several thoracoscopic and open procedures. Also PVB and TEA provide both a good intra- and postoperative pain relief  When we compare INB with TEA, Mogahed et al. showed that INB can achieve an excellent analgesia while blocking stimulation from the parietal pleura. INB are also better in patients with inadequate cardiovascular reserve US-guidance in both PVB and INB allows a better accuracy. The paravertebral space (PVS) is a wedge-shaped space lateral to the vertebral column where the spinal nerves emerge from the intervertebral foramina. The lateral paravertebral space tapers and continues laterally into the proximal intercostal space (PICS), a transition that occurs near the costotransverse joint. Both the PVS and PICS contain the intercostal nerves, and the PVS also communicates inferiorly and superiorly with adjacent segmental PVSs, allowing a passage- way for local anesthetic (LA) to spread to several adjacent levels. As the trajectory of the biopsy in this specific case was anterior, a block proximal to the medial axillary line showed to be sufficient without the need to take the risks of coming closer to the spinal canal. The visualization of the intercostal nerves and pleura is also easier in such locations as the thickness of the musculature is less significant. In anterior mediastinal locations like our case, it is our belief that medial INB present fewer risks than PVB for both spinal canal and pleura. It is also an easier technique, for junior consultants or senior staff with less locoregional experience, to reproduce in a safer and confident manner.4Patients with an anterior mediastinal mass and high risk of perioperative complications can be identified by the presence of cardiorespiratory signs and symptoms at presentation, tracheal compression higher than 50 % or pericardial effusion in the CT scan and combined obstructive and restrictive patterns on PFTs. These findings should prevent the use of GA. Tumor biopsy using a locoregional technique is preferable .Fig. 2AlMaintenance of spontaneous respiration during anesthesia is considered mandatory, particularly for biopsy procedures. Airway stenting, with a rigid bronchoscope in the first instance, is advocated by some. Multilevel INB are safer and easier to reproduce than PVB, have less hemodynamic repercussion than TEA and can, therefore, be preferable for open anterior mediastinal biopsies or small masses' resection.None.


Acute phlegmonous esophagitis is a very rare, life-threatening form of esophagitis, characterized by diffuse bacterial infection and pus formation within the submucosal and muscularis layers of the esophagus. We describe a case in which contrast-enhanced chest CT was useful for evaluating the severity of phlegmonous esophagitis, which was overlooked and underestimated by endoscopy. A phlegmon is a localized area of acute inflammation of soft tissues, and the term ‘phlegmon’ is used to describe inflammation related to a bacterial infection. Phlegmon can be associated with the formation of suppurative exudate or pus ,2, and pA 76-year-old woman with no specific medical history visited an otolaryngology clinic because of neck pain and a foreign body sensation in her throat after eating Korean pancakes containing mussels. A laryngoscopic examination revealed no visible abnormalities and the patient returned home. However, the patient subsequently visited the emergency room due to worsening neck pain and fever development.Streptococcus Viridans groups. Endoscopy was performed to detect possible esophageal perforation, but no mucosal lesions were observed. Antibiotic (meropenem plus levofloxacin) treatment was started immediately to treat pneumonia and esophagitis. After 5 days of antibiotic treatment, the pneumonia improved, as determined by chest radiography. However, posterior mediastinal lesions obscuring both paravertebral stripes were observed  with no evidence of leukocytosis . The initial anteroposterior chest radiography revealed mediastinal widening and patchy consolidation in both lower lobes (not shown). The patient then underwent contrast-enhanced chest CT for further evaluation. The CT images showed diffuse circumferential esophageal wall thickening with low-density submucosa along the whole esophagus, increased mediastinal fat attenuation, and peri-esophageal fluid collection . Esophagobserved , and, thobserved A–C reveaobserved D. In addobserved . Based oFor the diagnosis and management of acute phlegmonous esophagitis, the patient was transferred to our hospital, and follow-up endoscopy and subsequent endoscopic ultrasonography were performed. Esophageal mucosa had a normal appearance when examined by endoscopy, but a small opening was seen at the upper esophagus . EUS revThis is the first report that we are aware of to be issued on the usefulness of contrast-enhanced chest CT for the evaluation of the severity of acute phlegmonous esophagitis caused by mucosal injury by a foreign body. The term ‘phlegmonous’ refers to pus formation by a diffuse inflammatory process, and phlegmonous infections can spread throughout the gastrointestinal tract. The stomach is the most commonly involved site. More than 100 such cases have been described in the literature, with a reported mortality rate of 42% . Acute pThe pathogenesis of acute phlegmonous esophagitis is unclear. The reported predisposing factors include immunosuppression, alcoholism, diabetes mellitus, old age, and mucosal injury . Its patA CT examination and EUS can provide an accurate, prompt diagnosis . CT imagThe treatment options for phlegmonous esophagitis include managing the infection with antibiotics, internal pus drainage, or surgical intervention . There iPhlegmonous esophagitis is easily overlooked because it is a rare disease with no specific signs or symptoms. Therefore, radiologists should be well aware of the CT findings and risk factors of this disease, and advise clinicians on its appropriate management.We report a rare case of acute phlegmonous esophagitis in which contrast-enhanced CT was useful for diagnosis and evaluating extent, which was underestimated by initial endoscopy. This section is mandatory and should contain the main conclusions regarding the research.


CYP17A1 gene and that her parents were all heterozygous carriers of this pathogenic variant. Due to the variable clinical manifestations of 17-OHD, meticulous assessment including genetic analysis is necessary. Further study is warranted to unravel the mechanism of gonadoblastoma in a patient with normal karyotypes.17α−hydroxylase/17,20−lyase deficiency (17-OHD), caused by mutations in the gene of the cytochrome P450 family 17 subfamily A member 1 (CYP17A1), is a rare type of congenital adrenal hyperplasia (CAH), usually characterized by cortisol and sex steroid deficiency combined with excessive mineralocorticoid. Gonadoblastoma is a relatively rare ovarian tumor that is frequently seen among patients with 46,XY gonadal dysgenesis. Rarely have they been reported in female patients with normal 46,XX karyotype. Here, we report an interesting case of an 11-year-old Chinese girl who presented acute abdominal pain that was later attributed to tumor rupture of right ovarian gonadoblastoma with dysgerminoma. Further evaluations revealed hypertension and hypokalemia. Hormonal findings showed increased progesterone, hypergonadotropic hypogonadism, and low cortisol levels. Her chromosome karyotype was 46,XX without Y chromosome material detected. Genetic analysis revealed that the patient had a homozygous pathogenic variant c.985_987delTACinsAA (p.Y329Kfs*90) in exon 6 of the  CYP17A1 gene, located on chromosome 10q24.3. Mutations in the CYP17A1 gene lead to a deficiency in 17α-hydroxylase/17,20-lyase. Due to blockage of sex hormone synthesis, 17-OHD causes differences/disorder of sex development (DSD) in 46,XY, while sexual infantilism and primary amenorrhea in 46,XX. Moreover, insufficient glucocorticoids in turn increase adrenocorticotropic hormone (ACTH) secretion, leading to adrenal hyperplasia and excessive mineralocorticoid, causing hypertension and hypokalemia  refers to a group of syndromes caused by inherited deficiencies in one of five enzymes involved in the biosynthesis of cortisol from cholesterol . The majGonadoblastomas are relatively rare ovarian tumors consisting of sex cord and primitive germ cell components . AlthougAn 11-year-old girl was admitted to the local hospital for abdominal pain in April 2022. CT revealed a large pelvic mass measuring 10 × 8 cm in diameter, with suspicion of adnexal torsion or tumor rupture. Serum tumor markers, including carbohydrate antigen (CA) 125, CA199, carcinogenic embryonic antigen (CEA), alpha-fetoprotein (AFP) and β-human chorionic gonadotropin (β-HCG) were in the normal range. An emergent laparoscopy showed a ruptured ovarian tumor measuring 10 × 8 × 6 cm on the right side, along with approximately 50 ml of intra-abdominal bleeding. The root of the right adnexa was twisted 720 degrees. The uterus and the left ovary were small in size, and no enlarged retroperitoneal lymph nodes were noted. Right salpingo-oophorectomy was performed. The sample was placed in an endo bag and exteriorized through the incision. Microscopically, a mixture of germ cells, sex cord elements, and hyaline bodies were arranged in large nests and lobules. The sex cord cells were arranged at the periphery of these nests  and testosterone (T), along with increased levels of progesterone (P), follicle stimulating hormone (FSH) and luteinizing hormone (LH). Furthermore, decreased cortisol and elevated ACTH levels were observed Table 1.in situ hybridization (FISH) analysis using a probe specific for the sex-determining region of Y-chromosome (SRY) gene and zinc-finger Y (ZFY) gene locus at the Y chromosome was performed and no signal was detected for the SRY and ZFY locus. To exclude the possibility of somatic mosaicism, additional molecular analyzes were conducted in both the gonadal stroma and tumor tissue. Quantitative fluorescence-polymerase chain reaction (QF-PCR) results showed no amplification of the SRY in both the gonadal stroma and tumor tissue. Further whole-exome sequencing of the patient indicated that she was homozygous for c.985_987delTACinsAA (p.Y329Kfs*90) in exon 6 of the CYP17A1 gene  staging classification for cancer of the ovary, the pathological staging was stage I gonadoblastoma with dysgerminoma, and no adjuvant chemotherapy was needed based on the National Comprehensive Cancer Network (NCCN) guidelines. Glucocorticoid supplementary treatment was initiated. The patient was prescribed prednisone starting at 5 mg/day. Three months later, her blood pressure varied between 117-130/72-85 mmHg, and plasma levels of potassium, cortisol, and ACTH returned to the normal range. After communicating with the patient and her parents about her condition, supplementary treatment with sex hormones will be used later to promote the development of secondary sexual characteristics and induce an artificial menstrual cycle. During the follow-up period, physical examination, blood parameters, including sex hormone, cortisol, ACTH, aldosterone, blood electrolyte levels, and pelvic ultrasound will be regularly monitored. Currently, the follow-up is still ongoing.17-OHD is a rare kind of congenital adrenal hyperplasia (CAH) characterized by the failure to synthesize cortisol, adrenal androgens, and gonadal steroids . DefectsCYP17A1 gene, and most are associated with a classic phenotype of combined 17α-hydroxylase/17,20-lyase deficiency. A smaller number of CYP17A1 missense variants have been reported to exhibit partial impairment of 17α-hydroxylase/17,20-lyase activity. Based on the degree of enzyme deficiency, 17-OHD is classified chiefly into complete and partial type deficiency. Specific CYP17A1 gene variants have been reported to cause a partial loss of 17a-hydroxylase/17,20-lyase activities or dissociation of the 17a-hydroxylase and 17,20-lyase functions  was changed by AA. This frameshift pathogenic variant finally leads to the premature stop codon of 418TGA and generates a truncated protein containing only 417 amino acids without the heme-binding region of codons 435 and 455, which plays a key role in the catalytic function of the enzyme. Therefore, the loss of the pivotal functional domain results in the complete loss of 17-hydroxylase/17,20-lyase activity.Until now, numerous pathogenic variants in the 7 enzyme . TyrosinCYP17A1 gene pathogenic variants. In total, genetic variants were reported in 181 Chinese cases; of them, 70 (38.6%) had a pathogenic variant p.Y329Kfs*90.In 2003, a Korean compound heterozygote patient with primary amenorrhea, hypokalemia, and hypertension, was first diagnosed with this pathogenic variant . SubsequTSPY gene was established as the putative gene for GBY  patients had gonadal tumors, one with a Leydig cell tumor and the other with a Sertoli cell tumor . The gonIn the management of 17-OHD, glucocorticoid administration is the key therapy to suppress adrenal hyperplasia and normalize blood pressure as well as plasma potassium level. Treatment should be individualized and the dose should be adjusted according to the patient’s blood pressure, plasma potassium, and hormone levels . In caseIn summary, 17-OHD is uncommon and challenging in clinical practice. We describe the extremely rare case of a 17-OHD female patient with normal 46,XX karyotype accompanied by ovarian gonadoblastoma with dysgerminoma. Due to the variable clinical characteristics of 17-OHD patients, a meticulous assessment is necessary, including genetic analysis. Further study is warranted to unravel the mechanism of gonadoblastoma in patient with normal karyotypes.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.The studies involving human participants were reviewed and approved by Peking Union Medical College Hospital. Written informed consent to participate in this study was provided by the participants’ legal guardian/next of kin.MY: Writing and literature search. JY and QT: Concept, design, and medical practice. XZ: Analysis and interpretation. All authors contributed to the article and approved the submitted version.


External jugular vein aneurysms presenting as neck masses is very rare in the literature. This case report presents an 80-year-old female, who was referred to the office due to an incidental finding of a left submandibular neck mass. The patient presented to her primary care physician initially complaining of bilateral intermittent ear pain that was present for several years. After extensive workup, the patient was diagnosed with a left external jugular vein aneurysm. When asymptomatic, this rare condition can be followed safely on an outpatient basis. Vascular surgery consultation should also be considered. Venous aneurysms located in the head and neck are reported to have an indolent clinical presentation frequently associated with local pain or tenderness without significant risk for severe complications such as embolism and rupture , 2. The An 80-year-old female was referred to the otolaryngology clinic by her primary care provider with the chief complaint of bilateral intermittent otalgia. During the encounter, the patient recalled mild, left submandibular neck edema associated with tenderness that would resolve in 2–3 days. These symptoms were long-standing and were deteriorating several months prior to her presentation to our office. The patient presented with a computed tomography (CT) ordered by her primary care provider, which revealed a hyperdense mass within the left submandibular gland measuring 1.8 cm anterior–posterior, 1.4 cm transverse and 1.8 cm in height . This fiThe patient has a history of long-standing hypertension managed with enalapril and amlodipine. She also has hyperlipidemia which is managed with diet modification. On the day of the encounter, her vital signs were within normal range besides a mildly elevated blood pressure reading of 161/88 mmHg. Her physical examination and laryngoscopy were unremarkable, except for fullness on the left submandibular neck, without palpation of distinct masses or nodules. The discrepancy between physical examination and imaging prompted additional workup with an ultrasound (US) of the neck and request for fine-needle aspiration of the reported left submandibular neck mass. During the pre-intervention US, the vascular nature of the lesion was confirmed . After rThe most common presentation for aneurysm in the neck region is a palpable soft mass that enhances with Valsalva maneuver , 5. DoppThere are rare reports of external jugular vein aneurysms in the literature , 10, 11,The management of external jugular vein aneurysms can be observational, provided that these lesions are asymptomatic. Surgical intervention can be indicated either for cosmetic reasons when there is significant deformity or when complications arise due to phlebitis or thrombosis. Aneurysmal ruptures and pulmonary embolism are considered to be rare and surgical excision is thought to eliminate the risk of these severe complications . AdditioThis case report illustrates the need to consider external jugular vein aneurysm in the differential diagnosis of upper neck masses. A meticulous head and neck physical examination, including Valsalva maneuver, should be performed and ultrasonography can confirm the diagnosis when suspicion is raised. When uncomplicated and asymptomatic, the lesion can be managed with watchful waiting. If intervention is required, appropriate preoperative planning will decrease the risk of intraoperative vascular complications.


Tumor-related osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by severe hypophosphatemia and osteomalacia. The diagnosis of TIO can be very difficult because of its nonspecific nature of clinical manifestations. Here we reported a case of young TIO patient with “painful knee joint with difficulty in moving” to improve the clinical diagnosis and treatment levels.68Ga-DOTATATE PET/CT, and then was surgically resected. Upon pathologic assessment, the tumor was diagnosed as phosphaturia stromal tumor (PMT) with positive Vim staining. After the surgery, serum phosphate level rapidly recovered and symptoms significantly improved.The patient’s clinical features were consistent with TIO. A tumor was successfully located in left tibial by TIO should be considered in patients with chronically hypophosphorus osteomalacia in the setting of no family history. Early removal of the responsible tumors is clinically essential for the treatment, and imaging examination is of great significance for tumor localization. TIO is a rare paraneoplastic syndrome caused by tumors that ectopically overproduce fibroblast growth factor-23 (FGF-23), leading to decreased renal tubular reabsorption of phosphate. It is characterized by severe hypophosphatemia and osteomalacia . SymptomThe 22-year-old female patient was admitted to the hospital on August 25, 2021 with the chief complaint of “double knee joints pain for 10+ years, aggravation for 2+ months, accompanied by groin pain for 2+ weeks”. 10+ years ago, she had recurrent pain in both knees without obvious inducement, occasionally with knee valgus and knee dislocation. 2+ years ago, she developed low back pain which was relieved after rest, but the spine moved freely. 1+ years ago, she experienced severe pain in her low back and loss of mobility. She needed help to get up and down stairs, usually could only walk slowly. 2+ months ago, the pain became progressively worse and she developed pain in the groin area and the right 12th rib after a tumble. MRI showed bone marrow edema in the right intertrochanteric, femoral neck, lower left femur, and tibial intercondylar crest. 2+ weeks ago, she had occasional wandering pain of joints all over the body, accompanied by difficulty in walking and increased pain after weight bearing. Biochemical tests revealed hypophosphatemia (0.47 mmol/L), high serum alkaline phosphatase (257 IU/L), low 25-OH Vit D (41.1 nmol/L), and dual-energy X-ray absorptiometry (DXA) demonstrated reduced bone density. Then she was admitted into the Department of Endocrinology of our hospital for further treatment. She had lost 3 cm in height over 10 years. Her medical history included polycystic ovary syndrome (PCOS) for nearly 3 years, with irregular menstruation. Deny bad eating habits, long-term absence from sunlight, chronic diarrhea and the history of taking adefovir dipivoxil or other drugs. No history of recurrent skin hemorrhages or tooth loss or fracture. No family history of skeletal, metabolic or hormonal disorders.She is 142 cm tall and weighs 46 kg, with no obvious positive signs in heart, lung and abdominal examination. Lower limbs are unequal in length and circumference  (Table (1) Alkaline phosphatase: 270.4 U/L (n: 35-100 U/L); (2) Phosphorus: 0.52 mmol/L (n: 0.85–1.51 mmol/L); (3) Calcium: 2.29 mmol/L (n: 2.11–2.52 mmol/L); (4) Magnesium: 0.91 mmol/L (n: 0.75–1.02 mmol/L); (5) Creatinine: 42.9umol/L (n: 41-73umol/L); (6) Calcitonin: 15.3 pg/ml (n: 0.0–18.0 pg/ml); (7) Parathyroid hormone: 61.23 pg/ml (n: 8.70–79.60 pg/ml); (8) 25-OH Vit D: 16.00 ng/ml (deficiency: < 20 ng/ml); (9) Total procollagen I N terminal peptide: 90.84 ng/ml ; (10) β-Crosslaps: 623.40 pg/ml ; (11) N-MID osteocalcin: 15.30 ng/ml  , and a small nodular, vertical strip abnormal signal outside the bone (1.5 cm) Fig. .Fig. 2MRL1-L2 –1.5; L1-L3 –1.5; L1-4 –1.7; L2-L3 –1.7; L2-L4 –1. 8; L3-L4 –1.8; Neck −2.9; Wards triangle −2.7; Great trochanter −2.4; All −2.3.Osteomalacia, with pseudo fractures of the anterior segment of the right 2nd rib, the axillary segment of the left 7th and 8th ribs, right side of the sacrum, left acetabulum, and right femoral neck Fig. .Fig. 399Abnormal density of left middle and upper tibia with increased somatostatin receptors (SSTR) expression. Pseudo fracture of multiple ribs  and right femoral neck with increased SSTR expression. TIO was considered in the above lesions Fig. .Fig. 468Combined with the above clinical history, physical examination, laboratory evaluation, the patient was initially confirmed to have TIO. The responsible tumor was located in the left proximal tibia. The patient then underwent resection and biopsy of the left tibial tumor on September 6, 2021. During the operation, there was a bony protrusion and bone destruction in the left proximal lateral tibia, and white soft tissue beside the bone. The damaged bone tissue and soft tissue beside the bone were taken and sent for pathological examination. Then we used Kirschner wire to drill holes in the lesion area, and used electric knife to kill the lesion tissue at high temperature. Postoperative histopathological examination demonstrated a spindle cell tumor with prominent vascularity, smoky matrix, “grungy” calcification and a small amount of multinucleated, osteoclast-like giant cells Fig. . Immunoh2D, serum calcium and parathyroid hormone [2D synthesis, and osteomalacia [TIO is an acquired metabolic bone disease caused by tumor, and is one of causes of osteomalacia hypophosphorus in adults. Laboratory tests in TIO show refractory hypophosphatemia, elevated alkaline phosphatase, reduced tubular reabsorption of phosphate, low or normal 1,25(OH) hormone . Among tomalacia . Klotho,omalacia . The ectomalacia . Solubleomalacia . Howeveromalacia . Histoloomalacia , 13. The18F-FDG PET/CT) is also poor because PMT is mostly benign with low metabolic activity [Surgery is the only established, definitive treatment of TIO , 15. Howactivity .111In-pentetreotide or octreotide is the first approved radioactive drug for neuroendocrine tumor (NETs) imaging. Its high affinity for SSTR2 and SSTR5 makes 111In-pentetreotide single-photon emission computed tomography (Octreoscan-SPECT/CT) useful for the localized diagnosis of TIO [68Ga-DOTATATE, like octreotide, is an antagonist of the SSTR, which upon receptor binding is internalized resulting in accumulation of radioactivity in tumor cells [68Ga-DOTATATE PET/CT has been proved to be superior to Octreoscan-SPECT/CT for possible reasons: (1) 68Ga-DOTATATE has higher affinity for SSTR2 and SSTR5 than octreotide [68Ga-DOTATATE imaging has advantages in terms of shorter acquisition time and lower radiation exposure because of its short half-life of 68 minutes, compared with 2.8 days for 111In, the isotope used in Octreoscan-SPECT/CT imaging. 68GA-DOTATATE PET/CT maximum standardized uptake value (SUV max) also correlates with the clinical and pathologic features of NETs and is an accurate prognostic index [68Ga-DOTATATE PET/CT demonstrated abnormal density of left middle and upper tibia with increased SSTR expression, thus successfully locating the tumor.Since most responsible tumors are mesenchymal tissue derived and express the SSTR, SSTR-based functional PET/CT has been used successfully to detect TIO tumors. s of TIO , 23. 1,4or cells . 68Ga-DOtreotide , 26; (2)treotide . In addiic index . In thisAt present, TIO is mostly reported to start in middle age. However, the onset age of this case was prepubescent (12 years old), which is obviously younger than majority of reported cases. Thus it needs to be differentiated from hereditary hypophosphatemia, mainly including: (1) X-linked hypophosphacemic rickets (XLHR); (2) autosomal dominant hypophosphatemic rickets (ADHR); (3) autosomal-recessive hypophosphatemic rickets (ARHR) . Althoug2D stimulates aromatase activity [2D and calcium may be necessary for normal ovarian follicular formation. Loss of apoptotic mechanisms or overexpression of anti-apoptotic factors may lead to the appearance of polycystic ovary [This patient was diagnosed with PCOS 3 years ago, while sex hormones returned to normal 5 days after surgery. It indicates the possible association between PCOS and TIO. However, as far as we know, its specific mechanism remains to be elucidated. Previous studies have found that 1,25(OH)activity , 32, dowactivity , and up-activity . Thus, vic ovary , 36. In ic ovary .2D replacement therapy can be given when TIO responsible tumors cannot be localized or completely resected. Serum phosphorus and symptoms can be improved to some extent, but cannot be cured [Neutral phosphorus solution and 1,25(OH)be cured . The usebe cured , 39. FGFbe cured , 41. How68Ga-DOTATATE PET/CT is recommended for the location of responsible tumors. Surgical removal and close follow-up are essential for the treatment of TIO. Patients unable to undergo surgery can be treated with neutral phosphorus solution and 1,25(OH)2D.TIO, as a rare paraneoplastic syndrome, is easy to be misdiagnosed. When patients develop unexplained progressive multiple bone pain, muscle weakness, hypophosphatemia and elevated alkaline phosphatase, the possibility of TIO should be considered.