We describe a rare case of classical Hodgkin lymphoma (HL) in a 20-year-old female patient. She presented to our hospital with chest wall swelling after months of post-chest trauma management. The swelling was initially treated as an infected hematoma, and the patient was referred for surgical evacuation. During the surgery, the swelling was found to be a mass that extended to the mediastinum. A biopsy was taken, which later resulted in the diagnosis of a granular cell tumor (GCT). A decision on surgical resection by video-assisted thoracoscopic surgery (VATS) was taken after discussion with the multidisciplinary team of surgery, cardiothoracic surgery, oncology, pathology, and radiology. During the surgery, a frozen section biopsy was taken, which showed no features of lymphoma or granular cell tumors. The surgery was followed by a midline sternotomy to control the bleeding from an accidentally injured major vessel. The bleeding was controlled, and the mass was dissected and sent for histopathological examination. The histopathology showed nodular classical HL features, and the patient was referred for chemotherapy.In our case, the patient was initially diagnosed with GCT, but with complete resection and an adequate biopsy, the mass was found to be a classical HL. Possible cross-cellularity is questioned, and the possible correlation between the two findings encouraged us to report this case. Hodgkin lymphoma (HL) is a rare B-cell malignant neoplasm that usually presents with supradiaphragmatic lymphadenopathy, and 33% of the cases are associated with constitutional symptoms, including fever, weight loss, and night sweats. Classical HL is the commonly diagnosed entity of the disease compared to the non-nodular lymphocyte-predominant HL, and it is subcategorized into four definite subgroups: nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich Hodgkin lymphoma. Nodular sclerosis HL usually affects adolescents and young adults, and it tends to involve the mediastinum and supraclavicular or cervical lymph nodes as a localized disease .The definitive diagnosis of HL requires providing the pathologist with adequate specimens because small biopsy specimens such as fine-needle aspiration and core-needle biopsy may not include sufficient malignant cells . RecentlGranular cell tumor (GCT) is a rare soft tissue tumor, commonly considered a benign tumor with less than a 2% malignancy incidence. It is derived from nerve sheath cells called Schwann cells . GCTs caHerein, we present a case of a 20-year-old female patient who presented to our hospital complaining of chest swelling following a trauma. She has been treated for months for trauma complications and wound site infections. The swelling failed to shrink, and the patient underwent a CT-guided biopsy which revealed a diagnosis of GCT. The patient then was referred for surgery to remove the mass, which was found to be a nodular classical lymphoma on histological examination.The atypical presentation of the HL and the overlap of the diagnosis with the GCT encouraged us to report this case.A 20-year-old female patient with no past medical and surgical history presented to our hospital with a left-sided chest wall swelling of two months duration after direct trauma to the chest. The patient’s chest swelling was treated at a hospital as a hematoma resulting from the trauma, and it was evacuated. The evacuation failed to reduce the swelling; furthermore, it became tender and erythematous. A chest computed tomographic (CT) scan was done and showed an 8*5*4 cm mass in the anterior chest wall associated with multiple mediastinal lymph nodes with features suggestive of an abscess or a liquefied hematomaFigure . Her whiThe patient underwent mass excision and biopsy, and when the mass was dissected, it yielded dirty fluid filled with cheesy material and necrotic tissue. It was difficult to estimate the size of the mass intraoperatively, but it was about 5*6 cm. After dissection and reaching the pectoralis fascia, the mass was palpated deep into the muscle, for which an incision of the fascia was done, followed by muscle splitting. The mass was located in the retro-muscular area and extended to the anterior mediastinum. Part of the mass wall was biopsied and sent for histopathological examination, which showed connective tissue with dense mixed inflammation and granulation tissue formation with no evidence of malignancy.Following the surgery, the patient developed a wound infection and there was no proper healing. Despite her regular dressings and wound care for about a month, she developed a new collection. A CT scan was done and showed an infected area around the wound extending deeply into the mediastinum, in addition to a suspicious mass located in the retrosternal space Figure . A CT-guDuring the VATS, a frozen section biopsy was taken, and it showed no features suggestive of lymphoma, so the surgery continued. The mass was found to be surrounding the major vessels of the mediastinum; consequently, a small injury to the innominate vein occurred, and the trial of placing a clip to stop the bleeding failed. Thus, the decision to proceed with a midline sternotomy was made to control the bleeding. The bleeding from the innominate vein was controlled successfully. The remaining mass was then shaved from the main vessels and sent for histopathological examination. Debulking of the mass was done, but complete resection was not possible. She had three chest tubes inserted and a vacuum attached to her chest wound. Her vacuum was attached with a pressure of about 80-81 mmHg and was producing a small amount of serous material. It was removed on postoperative day 8. The first chest tube (right chest) was removed on postoperative day 4, the second chest tube (left chest) was removed on postoperative day 6, and the third tube (close to the mass in the anterior mediastinum) was removed on postoperative day 9. The patient was then transferred to the surgical intensive care unit (SICU), where she stayed for three days before being transferred to the surgical ward. The results of histopathological examination of the excised mass were consistent with a Hodgkin lymphoma nodular sclerosis variant Figure . A meetiShe was referred to the oncology team, and positron emission tomography (PET) was done, which showed stage II lymphoma. She was initiated on a doxorubicin, bleomycin, vinblastine, and dacarbazine chemotherapy protocol about two months after her last surgery.Her postoperative course was remarkable for a fever with an infected peripherally inserted central catheter (PICC) about three months after her last surgery, for which she had removed it and taken IV antibiotics. This event occurred after her fourth cycle of chemotherapy.In addition to left upper limb swelling and pain as a result of left upper limb DVT, which was confirmed by a duplex US study of the left upper limb. She was initiated on enoxaparin and then on rivaroxaban. There were no significant complaints except for surgical site pain with the dressing. She was given a course of meropenem and vancomycin for about one week after her last surgery (VATS and sternotomy).The thoracic mediastinum is the compartment that runs the length of the thoracic cavity between the pleural sacs of the lungs, extending from the thoracic inlet to the superior surface of the diaphragm. The thymus gland, lymph nodes, and connective tissue are among the organs found in the anterior mediastinum. The mediastinum is an essential part of the human anatomy because of its complex structure, which guarantees the appropriate operation of the respiratory and cardiovascular systems.The mediastinal mass represents a wide entity, referring to a number of benign and malignant diseases that pose a high challenge to most physicians. The mediastinal cavity is divided into anterior, middle, and posterior regions depending on its landmarks. Thymoma, teratoma, thyroid mass, and lymphoma are the most typical causes of an anterior mediastinal mass. Congenital cysts are frequently found in the middle mediastinum while neurogenic tumors are found in the posterior mediastinum .For all types of anterior mediastinal masses, a proper history should be taken, followed by a thorough physical examination. Forty percent (40%) of patients were diagnosed incidentally, with no symptoms. However, symptomatic patients could exhibit either localized symptoms from local invasion, tumor mass effects, or systemic symptoms from the malignancy process itself .If the clinical picture and radiographic findings aren't sufficient, further evaluations can include chest imaging, blood tests, and diagnostic biopsies .Lymphoma is one of the most common etiologies of anterior mediastinal mass, with an approximate incidence of 25% following thymoma, which accounts for 35% . Among aHodgkin lymphomas (HL) are neoplasms arising from the malignant transformation of B-lymphocytic cells. HL is subcategorized into nodular lymphocytic-predominant HL and classical HL. Classical HL has four histological subtypes: nodular sclerosis, mixed-cellularity, lymphocytе-rich, and lymphocytе-depleted . In 2020Nodular sclerosis classical Hodgkin lymphoma (NS-CHL) represents one of the most common subtypes of Hodgkin lymphoma, comprising approximately 50-70% of mediastinal lymphomas. It is the most common type to involve the anterior mediastinum and has a female predominance. Adenopathy is present in 80% of cases, as seen in this reported case .Patients with mediastinal nodular sclerosis and Hodgkin lymphoma present with various pictures. Patients are usually diagnosed incidentally, as most are asymptomatic. Those with symptoms are mostly present with chest pain, dyspnea, coughing, and fatigue. Superior vena cava syndromе is common as well, and the involvement of adjacent structures may lead to other presentations. Around 30% of patients may exhibit B-symptoms . Overall symptoms depend on the tumor type, size, site, and stage .Invasion of the chest wall by Hodgkin lymphoma mimicking chest wall tumors has to be kept in mind, as tumor resection has many consequences, including uncompleted tumor resection, post-surgical complications, higher recurrence possibilities, and systemic involvement. Thus, making the right diagnosis is critical to avoiding unnecessary resection. It has been reported in the literature that 15% of first and second-stage cases of mediastinal HL could invade the chest. A study by Witte et al. described four cases of lymphomas presented as chest wall masses. Among these, a 32-year-old male patient presented with a cough and dyspnea. A CT scan showed an anterior mediastinal mass invading the manubrium, and biopsies confirmed the diagnosis of nodular sclerosis HL . This paThe diagnosis of HL, like any mediastinal mass, requires clinical evaluation, as mentioned above, followed by laboratory studies. A complete blood count (CBC) could show high white blood cells (WBCs), lymphopenia, normocytic anemia, high lactate dehydrogenase (LDH), and erythrocyte sedimentation rate (ESR) are also seen frequently .Although the most definite diagnosis is achieved by histopathological evaluation, medical imaging plays a crucial role in supporting the CHL diagnosis. A chest X-ray with different views is the first to be done, followed by more sensitive imaging. A computed tomography scan (CT) is the preferred initial modality to determine the exact anatomy, and it plays a role in staging. CT scans in CHL show some specific features, including anterior mediastinal solid masses with irregular borders and surface lobulation but no vascular involvement and associated lymph nodes. In large tumors, invasion of the chest wall or mediastinal structures could be seen. Magnetic resonance imaging (MRI) shows the same findings as the CT scan, with higher inflammatory process detection and fat presence to exclude other differential diagnoses. Positron emission tomography-computed tomography (PET-CT) scans, on the other hand, are used mostly for staging purposes and as recurrence determinants in some challenging cases with post-therapeutic fibrosis .The definitive diagnosis is obtained by an adequate tissue biopsy. Using fine-needle aspiration or even core needle biopsy is usually of low value, as CHL needs good structural evaluation due to the fact that this type of tumor is characterized by a scarcity of malignant cells and abundant inflammatory cells. Nodular sclerosis CHL histopathology shows fibrous tissue surrounding cellular modules and, most times, fibrotic mediastinal lymph nodes .GCTs are rare, commonly benign (two-thirds of cases) neoplasms originating from Schwann cells, affecting adults aged 40-70 years with a female predominance. GCT could occur throughout the body, mostly in the skin or subcutaneous tissue of the head and neck and in the breasts, pulmonary system, and gastrointestinal system .GCT in the mediastinum, on the other hand, is extremely rare, with only a few reported cases in the literature. One Korean study reported a 24-year-old male patient with an incidental mediastinal GCT finding on a chest X-ray . DespiteOn microscopic examination, these tumors show small nuclear polygonal cells that appear in nests or cords separated by connective tissue with numerous granular cytoplasm. The granules are positive for acid Schiff stain and resistant to diastase. GCT rarely recurs, but in some incompletely resected cases, recurrence and local invasion could occur .The association between CHL and GCT has been reported only in one case of a 13-year-old boy who developed multiple subcutaneous GCT while in HL remission. This could be explained by the fact that HL survivors are at increased risk of developing second malignancies throughout their lives, so the correlation in this case hasn't been proven to be real or coincidental . To our In our case, the patient had a GCT result for the first biopsies, and with complete resection, the diagnosis of HL was confirmed. Possible cross-cellularity is questioned, and the possible correlation between the two findings urges the need to report this case and complete the patient follow-up in the future.To summarize our case, a 20-year-old female patient with left-sided chest wall swelling following direct trauma was misdiagnosed as having a hematoma. Later imaging confirmed the presence of a mediastinal mass, further evaluation and biopsy revealed a granular cell tumor, a rare unexpected finding. The patient’s journey continued with surgical intervention and infected wound site debridement until the whole mass was excised and examined. Histopathological examination ultimately confirmed the Hodgkin lymphoma nodular sclerosis variant, and the patient was referred for chemotherapy.Understanding the complex structure of the thoracic mediastinum is essential for diagnosing and managing various conditions, including mediastinal masses. Accurate diagnosis of mediastinal masses is crucial, as it influences treatment plans and patient outcomes. While some patients with mediastinal masses may remain asymptomatic, others may present with localized or systemic symptoms, making the comprehensive clinical evaluation essential. Diagnostic approaches for mediastinal masses involve a combination of clinical evaluation, laboratory studies, and medical imaging techniques. However, in some cases like Hodgkin lymphoma, the diagnosis relies on histopathological evaluation through tissue biopsy of adequate samples. Interestingly, the association between Hodgkin lymphoma and granular cell tumors is a rare occurrence, with limited documented cases. This correlation, as observed in our case, underscores the need for further research and follow-up to better understand any potential links between these two conditions.In summary, the diagnosis and management of mediastinal masses require a multidisciplinary approach, combining clinical expertise, imaging techniques, and histopathological evaluation. Continuous research and reports of unusual cases, such as the one presented here, will contribute to our understanding of these complex conditions and improve patient care in the future.


Biliary adenofibroma is a solid microcystic epithelial neoplasm in the liver, comprising microcystic and tubuloacinar glandular tissues lined by a non-mucin secreting biliary epithelium and supported by a fibrous stroma. It is an extremely rare benign tumor with potential for malignant transformation. Herein, we report the case of a 64-year-old woman diagnosed with intrahepatic cholangiocarcinoma arising from biliary adenofibroma.d-glucose (FDG) uptake on positron emission tomography, like conventional intrahepatic cholangiocarcinoma. The dorsal portion showed a well-defined and low-attenuated mass with heterogeneous early enhancement and partial wash-out on CT, marked hyperintensity on heavily T2-weighted images, and low FDG uptake. The patient subsequently underwent extended left hepatectomy.Imaging studies revealed a tumor of 50 mm diameter, consisting of two components in S1 of the liver. The ventral portion of the tumor showed an ill-defined mass with early peripheral and gradual centripetal enhancement invading to the middle hepatic vein on computed tomography (CT), diffusion restriction on magnetic resonance images, and high fluorine-18-2-deoxy-Pathologically, the former was diagnosed as cholangiocarcinoma and the latter as biliary adenofibroma. We discuss the radiological-pathological correlation of the tumor with a literature review.Preoperative diagnosis of biliary adenofibroma is extremely challenging; however, clinically, it is crucial not to miss the presence of malignant findings. AlthougA tumor in S1 of the liver was found on abdominal ultrasound during health examination in a 64-year-old Japanese woman. She did not have any symptoms or notable family, smoking, or drinking history. She had a medical history of appendectomy for acute appendicitis, hysterectomy for uterine myoma, right ovariectomy for ovarian thecoma, right adrenalectomy for Cushing syndrome, and laparoscopic cholecystectomy for a gallbladder polyp. Contrast-enhanced CT performed 9 years ago at the referring hospital did not show any S1 lesions (image not shown). She also had hypertension, hyperlipidemia, and osteoporosis. She had no abnormal physical findings, including lymphadenopathy and hepatosplenomegaly.Laboratory blood examination revealed that liver enzymes and tumor markers such as alpha-fetoprotein, carbohydrate antigen 19-9, carcinoembryonic antigen, and protein induced by vitamin K absence or antagonist II were within normal limits. She was not infected with hepatitis B and C virus.Abdominal ultrasound revealed that the tumor comprised two distinct components on the ventral and dorsal sides in S1 of liver , and no uptake of gadoxetic acid in the hepatobiliary phase , and no uptake of gadoxetic acid in the hepatobiliary phase.On MR imaging, the ventral portion exhibited slightly low signal intensity on T1-weighted images, slightly high signal intensity on T2-weighted images, markedly low signal intensity on heavily T2-weighted images, diffusion restriction (apparent diffusion coefficient [ADC] = 1.1 × 10max = 8.3), and accumulation was mild in the dorsal portion, except the border region, and comparable to background liver accumulation  multiple lesions, 2) restricted diffusion on MR imaging, (3) obscure margin, (4) enhancement in the arterial phase followed by wash-out in the venous phase [ restrictOn FDG-PET/CT, high accumulation was seen in the malignant component and not in the benign component, thereby, clearly distinguishing between the benign and malignant component. The FDG accumulation of the malignant component was compatible with the typical findings of cholangiocarcinoma . To the Biliary adenofibroma can be included in the differential diagnosis when the liver tumor shows a well-marginated microcystic mass with a solid component like pancreatic serous cyst neoplasm. Invasive nature, restricted diffusion, and high FDG uptake of a part of the tumor, like intrahepatic cholangiocarcinoma, might be clues of malignant transformation.


Branchial anomalies are a result of abnormal development during embryogenesis and are a rare cause of lateral neck masses of congenital origin. The second branchial cleft is the most common site of origin, with abnormalities arising from the first, third, and fourth clefts being rarer. Although cysts originating from branchial clefts are infrequent, it is important to consider this pathology in the differential diagnosis of neck masses, particularly those located laterally. This article discusses a rare case of a 49-year-old female patient who presented with the sudden appearance of a lateral neck mass after sports practice. The patient underwent extensive diagnostic studies, including radiological studies, which were compatible with a fourth branchial cleft cyst. The patient remains asymptomatic, and possible surgical treatment is being evaluated by the head and neck surgery service. This clinical case highlights the importance of prompt diagnosis and appropriate management of rare pathologies such as branchial cleft cysts. The branchial arches or gill apparatus contribute to the proper formation of the head and neck during the fourth week of gestation, these arches are composed of ectodermal clefts and endodermal pouches. Congenital anomalies of the ectodermal clefts of the branchial arches occur due to incomplete obliteration, resulting in a cyst in the majority of cases (75%) or a sinus (25%) .These alterations do not have a predilection for sex and can occur at any age, although they are more common in adolescents and young adults . UsuallyHere I report the case of a young adult patient with a neck mass with unusual characteristics and locations, later identified as a fourth branchial cleft anomaly, an exceptional and infrequent case.This is a 49-year-old female patient with no relevant medical history, originally from Holland and currently residing in the city of Bogotá. She suddenly presented with pain and a sensation of a mass in the right posterior neck region after engaging in physical activity. One month after this episode, the symptoms remained unchanged, and she denied experiencing any other symptoms. It is important to consider that she has not had similar episodes in the past.Upon physical examination, an overweight female patient was found to have a soft, mobile, painless mass on the right side of her neck with no additional masses or lymphadenopathy present. The rest of the physical exam was within normal limits. The patient underwent a contrast-enhanced neck magnetic resonance imaging (MRI) study, which revealed a large cyst possibly derived from the fourth branchial cleft on the right side, behind the jugular vein and lateral to the thyroid, almost submerged in that area, with smooth and thin walls Figure .Following diagnosis, the patient received information about the cause, course, and prognosis of the disease and was referred to the head and neck surgery department to explore possible surgical options.Different specific structures of the neck are derived from the branchial arches. Those derived from the fourth branchial arch correspond to the superior parathyroid glands, laryngeal cartilages, pharyngeal and laryngeal constrictor muscles, the superior laryngeal nerve, the left thoracic aorta, the proximal right subclavian artery, and the last branchial body that forms the parafollicular cells of the thyroid. Failure of obliteration of the pharyngobranchial duct and the fourth branchial cleft results in an alteration of neck anatomy .Branchial cleft anomalies comprise 30% of congenital neck masses, and only 2% are fourth branchial cleft anomalies, which are extremely rare. Fourth branchial cleft anomalies usually manifest as cysts in adults and as sinuses or fistulas in the pediatric population. These laryngotracheal sinus tracts begin at the apex of the piriform sinus and extend inferiorly to exit the pharynx through the lateral cricothyroid membrane. They then continue inferiorly to be located around the left aortic arch or right subclavian artery .The clinical presentation of fourth branchial arch abnormalities varies with age. Episodes of dyspnea are the most common presentation in neonates , cervicaStreptococcus, Staphylococcus, Haemophilus, Escherichia coli, and anaerobic germs such as Eikonella corrodens, Citrobacter, and Proteus [In a review of 526 cases of congenital abnormalities of the fourth branchial arch, it was reported that 19% of cases are accompanied by bacterial superinfections as a result of the retrograde transmission of flora from the hypopharynx, where a variety of aerobic microorganisms have been found, mainly  Proteus .Although cysts or sinuses of the fourth branchial arch are a rare pathology, it is important to consider this anomaly as a differential diagnosis of lateral neck masses, along with others such as tumors of the carotid body, paragangliomas, dermoid cysts, thyroglossal duct cysts, neurofibromas, hemangiomas, lipomas, teratomas, and lymph node metastases .A variety of diagnostic methods have been used to explore branchial cysts, among them, the most useful have been barium esophagogram and direct laryngoscopy, which have the best positive predictive value . However, barium studies may not show a sinuous tract if there is inflammation, unlike laryngoscopy. Others such as MRI, computed tomography, and thyroid ultrasound have also been used to diagnose and evaluate cystic lesions. Many abnormalities are diagnosed as incidental findings .Regarding treatment options, in the case of abscesses of the third and fourth branchial arches, incision and drainage are frequently performed, but have a high recurrence rate, up to 90%. The recommended treatment for cysts, sinuses, and fistulas is surgical excision along with partial thyroidectomy due to the risk of severe infection or primary carcinoma . SurgeryEndoscopy with cauterization is a minimally invasive technique with fewer complications and costs. Other methods of cauterization have been described including monopolar electrocautery and chemocauterization with trichloroacetic acid .In terms of complications arising from surgical and cauterization procedures, surgical site infections, salivary fistulas, and vocal cord paralysis have been reported in 5% to 6% of cases. During electrocautery, paralysis of the recurrent laryngeal nerve and the superior laryngeal nerve may occur due to inflammation and edema that can eventually compress these nerves. Although evidence is limited, most studies conclude that cauterization should be the primary treatment because it is minimally invasive, has a lower rate of complications, and can be performed simultaneously with other procedures such as incision and drainage in the case of an abscess ,12. FurtIn conclusion, fourth branchial cleft anomalies are a rare but important differential diagnosis for lateral neck masses. Clinical presentations vary with age, and infectious episodes can lead to serious complications. Diagnosis is typically made using a combination of imaging and direct laryngoscopy, with surgical excision and partial thyroidectomy being the recommended treatment to prevent recurrence and reduce the risk of severe infection or primary carcinoma. Endoscopy with cauterization is a minimally invasive technique that may be preferred in certain cases due to its lower rate of complications. Overall, a thorough evaluation is necessary for any patient with unexplained neck masses, especially on the left side, to ensure prompt diagnosis and appropriate treatment.


A 56-year-old female patient with colostomy presented with skin-colored cobblestone and verrucous asymptomatic papules on her peristomal skin for three months; she was referred to dermatology. Histopathology revealed irregular acanthosis, tongue-like extension of rete ridges of mature squamous epithelium without atypical morphology, hyperkeratosis, and inflammation of the skin. The histopathologic appearance was evaluated as compatible with pseudoepitheliomatous hyperplasia. No signs of malignancy, fungus, or koilocytes were found. The lesions were diagnosed as pseudoepitheliomatous hyperplasia by clinical and histopathologic findings. In this case report, we review pseudoepitheliomatous hyperplasia associated with colostomy. Ostomy is an artificial opening surgically created between the bowel and the abdominal wall. It allows the excretion of feces or urine by the abdominal wall through the opening. After the procedure, an ostomy bag is attached to the peristomal skin to prevent stool or urine from flowing out . AlthougA 56-year-old female patient underwent a colostomy procedure six months after mesenteric artery ischemia. The patient reported to the emergency department three days earlier complaining of abdominal pain and inability to have a bowel movement. She was admitted to the surgical department with a diagnosis of ileus, which was resolved after the administration of an enema. On follow-up examination, multiple fungi-like papules were noted on the peristomal skin. Therefore, a dermatology consultation was requested. Her medical history revealed that she was taking anticoagulants, quetiapine, and sertraline due to chronic venous insufficiency, lymphedema, and an anxiety disorder, respectively. The patient had the described peristomal lesions for three months. Subjective symptoms such as itching and pain did not occur. Dermatologic examination revealed multiple skin-colored cobblestone and verrucous papules that did not extend beyond the boundaries of the colostomy bag and filled the entire peristomal skin in the colostomy bag Figure .A punch biopsy was performed with the provisional diagnosis of cutaneous tumor, cutaneous metastases, condyloma, deep fungal infection, or pseudoepitheliomatous hyperplasia. Histopathology revealed irregular acanthosis, tongue-like extension of rete ridges of mature squamous epithelium without atypical morphology, hyperkeratosis, and inflammation of the skin. No signs of malignancy and fungal hyphae or spores were found. No koilocytes were noted, indicating human papillomavirus (HPV) effect. The histopathologic appearance was evaluated as compatible with pseudoepitheliomatous hyperplasia Figures -5.The colostomy bag was replaced with a smaller orifice. However, since the patient did not visit the hospital for follow-up and could not be reached, the current status of her lesions is unknown.Peristomal pseudoverrucous lesions are usually detected around urostomies or ileal conduits that allow urinary excretion. Peristomal pseudoverrucous lesions may occur in approximately 20% of patients with stomas. Among stomas, peristomal pseudoverrucous lesions are reported in 22%-23% with urinary diversions ,7. Wide-Goldberg et al.  reportedOnly three cases of colostomy-associated pseudoverrucous lesions have been reported in the literature -12. Of tThe first case of colostomy-associated pseudoverrucous lesions was reported in 1981 by Hjorth et al. They presented the case of a 51-year-old man with sigmoid colostomy after rectal adenocarcinoma surgery . After rThe other two cases were reported in 2003  and 2005Our patient is unique in that she differs from other reported cases in terms of the clinical and histopathologic features, as well as the location of the lesions.Pseudoepitheliomatous hyperplasia  is a benign skin disease clinically manifested as skin-colored or pale pink verrucous papulonodules, while histopathology shows hypergranulotic and hyperkeratotic epidermis extending into the dermis in the form of finger-like protrusions. This condition may be associated with infections, neoplasms, chronic inflammation, and irritation . EpidermIn the differential diagnosis of pseudoverrucous lesions, diseases such as squamous cell carcinoma, cutaneous metastases, and other neoplastic diseases, condyloma, bacterial infections, and Candida should be considered ,15. TherIt is recommended that the opening of the ostomy bag be of adequate size and that the stoma protrude 1-3 mm to avoid chronic irritation and associated pseudoverrucous lesions . UrinaryPeristomal pseudoverrucous lesions are usually observed around the urostomy; however, it is seen around the colostomy in extremely rare conditions. Our patient is the first reported case in the literature that had peristomal wart-like clobstone lesions filling the entire peristomal skin. Multiple papular lesions may be confused with malignancies, condyloma, and fungal infections because they rarely occur around the colostomy. Therefore, pseudoverrucous lesions should also be kept in mind as a preliminary diagnosis in patients presenting with such lesions.


Although intratumoral hemorrhage is common in patients with malignant brain tumors, reports on its clinical course are scarce. This report presents a rare case of a patient with intratumoral hemorrhage with gliosarcoma invading the venus sinus. This invasion and a small draining vein were observed at diagnosis. Magnetic resonance imaging performed 1 week later showed new-onset venous ectasia, which caused intratumoral hemorrhage. This case provides insight into the mechanisms underlying intratumoral hemorrhage and highlights the emergence of new intratumoral vasculature as a potential warning sign for hemorrhage. Gliosarcomas, accounting for ~2–8% of glioblastomas, are characterized by a more aggressive course compared with other glioblastomas . They arA 66-year-old woman presented with progressive cognitive deterioration and dysarthria. Non-contrast-enhanced brain magnetic resonance imaging (MRI) showed a 6-cm intraaxial mass in the left temporal lobe abutting the dura and an incidentally identified pituitary mass .The patient was diagnosed with cerebral ischemic stroke and atrial fibrillation 2 years ago. Since then, she had been taking edoxaban (60 mg/day). She was scheduled to discontinue edoxaban from the day of hospitalization and to undergo brain tumor surgery 4 days later.Physical examination revealed global dysphagia accompanied by mild cognitive decline. The results of preoperative laboratory and coagulation tests were unremarkable. Gadolinium-enhanced brain MRI revealed lesion in the left temporal lobe exhibiting an irregular ring-enhancing component with central necrosis. Follow-up MRI showed new-onset venous ectasia of the tumor-draining vein into the transverse sinus, which was not observed on initial MRI . The draThe next day, the patient experienced sudden headache and rapid consciousness deterioration. Brain computed tomography (CT) revealed acute hemorrhage inside the tumor with a midline shift . NavigatIntraoperatively, the tumor exhibited soft and friable consistency along with the presence of hematoma and cystic region filled with yellowish necrotic material. The tumor invaded the transverse sinus lumen and was firmly attached to the adjacent dura. The venous ectasia was confirmed to be the tumor-draining vein with internal thrombosis . After sPathological examination revealed an IDH wild-type, World Health Organization Grade 4 gliosarcoma. The tumor cells exhibited immunoreactivity for glial fibrillary acidic protein and vimentin in the glial and sarcomatous components, respectively. The glial component had features of a typical glioblastoma with nuclear pleomorphism and microvascular proliferation. Contrarily, the sarcomatous component contained spindle cells with moderate nuclear atypia. Histological examinations were conducted separately for thrombosed venous ectasia. The immunohistochemical results for actin in the vessel wall were negative, suggesting that the vessel wall had developed within a short period without forming a normal vascular structure .Several studies have demonstrated rapid tumor progression accompanied by abnormal angiogenesis and tumor invading large vessels in patients with highly malignant tumors, and both are associated with an increased risk of intratumoral hemorrhage , 6. Prevet al. reported a case of chondromyxoid fibroma invading the transverse-sigmoid sinus junction exhibiting cerebellar hemorrhage [et al. presented a case of meningioma invading the sigmoid sinus, which resulted in intracranial hypertension due to venous hypertension [In addition to tumor inherent malignancy, tumor location is assumed to be associated with an increased susceptibility to intratumoral hemorrhage. Hersh morrhage . Sumi etrtension . Both caOur patient had a structural change in the intratumoral traversing vessel within a mere week, subsequently leading to rupture of the lesion and necessitating emergent surgical intervention. This phenomenon provides insight into the mechanisms underlying intratumoral hemorrhage in gliosarcoma cases. The initial brain MRI showed a gradual narrowing of the tumor-draining vein that joined the left transverse sinus. Hence, we considered that tumor invasion into the left transverse sinus obstructed venous flow, consequently inducing venous stenosis. Brain MRI performed 1 week after the initial MRI revealed new-onset venous ectasia without thrombus. During this examination, the signal void demonstrated reduced flow despite the lumen being open. Just one day later, we observed an abrupt occurrence of intratumoral hemorrhage accompanied by thrombus formation within the venous ectasia. These imaging results suggest that the structural changes in the venous system were directly associated with the intratumoral hemorrhage. Tumor invasion into the venous sinuses disrupts blood flow, leading to flow stasis and subsequent development of proximal venous ectasia. Ectasia is further complicated by thrombus formation within its lumen. These vascular changes, coupled with an increase in arteriovenous pressure in the tumor vessels, ultimately culminate in the manifestation of intratumoral hemorrhage. Notably, the rapidly proliferating nature of gliosarcomas emphasizes the ability of such vascular structural alterations to occur within a remarkably short timeframe, as demonstrated that our patient.To our knowledge, this is the first report of gliosarcoma with intratumoral hemorrhage and new-onset venous ectasia. Details of the present case emphasize the need to consider structural changes in low-flow vascular malformations, such as venous ectasia, as warning signs of intratumoral hemorrhage. Furthermore, clinicians should be aware of the increased risk of intratumoral hemorrhage in patients with rapidly growing tumors invading the venous sinus due to dramatic vascular flow changes.


The paper at hand presents a unique case of leiomyosarcoma (LMS) involving the left leg in a 56-year-old patient. This individual experienced pain and the presence of a mass for approximately eight months before seeking medical attention. A diagnostic biopsy revealed the presence of multinucleated pleomorphic cells arranged in intersecting fascicles upon immunohistochemistry (IHC) staining for vimentin, caldesmon, and smooth muscle actin. The rarity of LMS in the extremities highlights the need for further understanding and research to determine the most suitable treatment approaches for such patients. In this specific case, the patient underwent tumor excision followed by reconstruction using a megaprosthesis. This report emphasizes the importance of considering unique treatment strategies when dealing with rare neoplasms like LMS in the extremities. As medical knowledge continues to evolve, gaining insights into the optimal management of such cases will be crucial for improving patient outcomes and overall prognosis. Leiomyosarcoma (LMS) is a prevalent type of soft tissue sarcoma (STS), constituting approximately 7% to 10% of all STS cases . The retIts characteristic appearance on plain radiographs is that of a lytic lesion without a surrounding sclerotic rim. Immunohistochemistry (IHC) plays a crucial role in diagnosis, with positive staining for smooth muscle actin (SMA), desmin, and h-caldesmon often observed .Given the complexity of LMS cases, successful management requires a multidisciplinary approach involving various medical specialties. However, the cornerstone of treatment remains complete surgical resection of the tumour. Following surgery, additional therapeutic measures such as chemotherapy and radiotherapy are utilized to provide complementary support.This report highlights a distinctive case of LMS originating from the proximal tibia. The tumour was successfully treated through a comprehensive approach involving a complete, wide resection of the affected area. Additionally, joint reconstruction using a megaprosthesis was employed to restore the function of the affected bone.A 56-year-old male presented to our outpatient department (OPD) with the chief complaint of pain and swelling in his left knee for eight months Figure . The paiAn MRI of the left leg revealed a heterogenous expansile space-occupying lesion (SOL) in the upper 1/3rd shaft of the tibia, destroying the bone and breaking the continuity of the cortex. It was hypointense in T1-weighted images while hyperintense in T2-weighted and fat-suppressed inversion recovery images. Extraosseous extension of SOL was seen outside the cortex, involving muscles and extending even up to the subcutaneous fatty tissue. The lesion measured 49x52x80 mm (anteroposterior (ap) x transverse (tr) x superior to inferior (si)). There was altered bone marrow signal intensity suggestive of bone marrow edema infiltration in the tibia around SOL, while normal bone marrow signal intensity was seen in the fibula. The major neurovascular bundle appeared normal , lungs , inguinal region, right femur, and right iliac bone.The patient underwent a core needle biopsy from the left proximal tibia and was reported to have fibrocollagenous tissue with an invasive tumour arranged in fascicles and whorls with nuclear pleomorphism and eosinophilic cytoplasm. It was positive for vimentin, CK, S100, and SMA on IHC Figure . These fAfter a complete evaluation, the patient underwent a wide resection and reconstruction with a megaprosthesis and medial gastrocnemius flap Figure .The patient withstood the surgery well. There were no postoperative complications. He was later admitted to the medical oncology department for postsurgical excision of the LMS proximal tibia in view of adjuvant chemotherapy with an ifosfamide-adriamycin regimen and is under regular follow-up in our department. Currently, after five months of surgery, the patient is well, with no evidence of any recurrence or metastasis with ongoing follow-up.Leiomyosarcoma is a spindle-cell sarcoma variation and a kind of STS. They account for 7% to 10% of soft tissue sarcomas overall ,3. Less There has been an equal incidence of LMS reported in men and women. Age predilection has been the subject of inconsistent findings. One set of authors claimed that this tumour was more prevalent in people in their seventh and eighth decades of life, while other researchers claimed that there was an equal distribution of ages ,7.There are actually two competing hypotheses for the origin of primary bone LMS. The first postulate demonstrates the involvement of vascular smooth muscle cells in the bones. The second postulate maintains that intermediate cells, in particular fibroblasts, can differentiate into smooth muscle cells that are capable of producing a matrix of connective tissue and myofilaments ,8,9.The most common signs are discomfort and localized swelling. Large tumours can be palpated as they spread beyond the bony structures. It has been documented that 15% of patients exhibit pathological fractures as a result of the osteolytic nature of the lesion causing cortical damage .Leiomyosarcoma of the bone presents as a lytic lesion with fuzzy borders and a permeative pattern on plain radiographs. Bone lesions exhibit osteolysis as well as varying degrees of assertiveness, such as the absence of a sclerotic rim and cortical breach ,11,12. BThe LMS in the bone has similar histopathological characteristics as other sites, showing elongated cells with abundant cytoplasm, sporadic vacuoles, and pleomorphic nuclei. It is eosinophilic, and the fascicles of these cells permeate the stroma. In high-grade tumours, there is evidence of mitotic activity, including aberrant forms . The mosPrior to surgery, imaging examinations must be used to determine the degree and extent of the surgical resection. A thoracic-abdomen-pelvic CT scan and MRI are advisable to determine the ideal spread of the tumour and any occurrence of skip-metastasis. A PET-CT may help with staging, grading, and assessing the effectiveness of neoadjuvant therapy .The most effective form of treatment and the only curative one is surgical resection. The R0 resection provides better outcomes microscopically than the R1 resection; therefore, the resection must be large with free margins .Another therapeutic option is radiotherapy; however, its effectiveness is debatable. Antonescu et al. found similar survival among patients who underwent surgery alone and those who underwent radiation therapy following surgery, possibly because this form of tumour is resistant to radiation .The primary strategy for metastatic LMS is chemotherapy. The most effective medications for treating bone sarcoma include cisplatin, doxorubicin or doxorubicin-based chemotherapy, and dacarbazine, which are typically used in first-line metastatic situations .The evolution and survival rate appear to be unaffected by anatomical location and histological grade. A retrospective trial by Antonescu et al. showed hardly any difference in life span between patients who got chemotherapy and those who received surgery . Age ≥40Our patient with metastatic bony LMS was treated with wide resection and reconstruction with a megaprosthesis right lower limb and medial gastrocnemius flap following multidisciplinary evaluation. He is currently leading a healthy life, with no evidence of local recurrence reported on follow-ups. He continues to be followed up at our institute's medical oncology department for adjuvant chemotherapy with the ifosfamide-adriamycin regimen.In this study, we presented a case of LMS occurring in the proximal tibia of a 56-year-old male, along with metastasis. To gain a better understanding of this case, we conducted a comprehensive evaluation, comparing it with other similar cases documented in the literature. The patient’s response to treatment was highly positive, as he showed significant improvement after undergoing wide resection and megaprosthesis. Notably, he experienced relief from pain and showed no signs of local recurrence during the follow-up period. When a patient presents with swelling in or around the knee region, it is essential to undertake a multidisciplinary evaluation. This process should involve various diagnostic methods, such as imaging, histological examination, and IHC studies. Considering the potential spectrum of differential diagnoses, it is crucial to include LMS as one of the possible considerations. This case highlights the importance of thorough evaluation and diagnosis when dealing with knee swelling, as prompt and accurate identification of LMS can lead to timely and appropriate treatment strategies. The success of wide resection and megaprosthesis in this case underscores the significance of tailored approaches in managing LMS, offering potential positive outcomes for patients with similar conditions. Continuous research and analysis of such cases in the medical literature contribute to advancing our understanding and refining treatment protocols for this rare but impactful neoplasm.


Follicular dendritic cells help advance B-Cells in becoming memory B-Cells or antibody-producing plasma cells in the light zone, or undergo additional affinity maturation in the dark zone. Follicular dendritic cell sarcoma (FDCS) is an extremely rare soft tissue malignancy derived from follicular dendritic cells. Autoimmune disease increases the risks for the development of hematological malignancies. To the best of our knowledge, there are few cases of FDCS development in the setting of underlying Sjogren’s syndrome (SS). Therefore, in this report, we present a novel case of FDCS associated with new-onset SS. In SS, the follicular dendritic cells are organized within germinal centers within the glands it infiltrates and is involved in B-Cell development. Because FDCS is derived from follicular dendritic cells, our report postulates that the unregulated follicular dendritic cell proliferation that may occur in SS could increase the risk for FDCS. Due to this possible connection observed in our patient, we highlight FDCS as a differential diagnosis when considering soft tissue cancers. We urge additional research to outline and explore the possible pathologic link between SS and FDCS. Follicular dendritic cells are immune cells derived from mesenchyme that can be found predominantly in lymphoid tissue but can be found throughout the human body . These cIt is also known that follicular dendritic cells may contribute to autoimmunity via the display of autoantigens . ResearcA 72-year-old Caucasian male presented to his primary care office with a dry mouth, dry eyes, and a swollen lump below his right jaw for two weeks. The patient stated that he has a dry mouth and noticed a progressive decline in saliva production. Physical examination revealed right salivary gland enlargement without masses and tenderness to palpation. The patient was advised to alleviate his symptoms with a warm compress and salivary gland massages. A CT of the salivary gland was ordered and showed no abnormal mass, fluid collection, or enhancement under the right submandibular region. The salivary gland revealed normal morphology and signal intensity of the parotid and submandibular glands. At this moment, the patient was diagnosed with SS. On imaging, there were enlarged superior mediastinal lymph nodes incidentally noted with the largest measuring 5 cm x 3.90 cm. The etiology was determined as unknown but further evaluation was warranted. The chest CT without contrast revealed three large abnormal mediastinal lymph nodes with the largest measuring 5.2 cm in greatest dimension. Neoplasm was considered until proven otherwise. Post the chest CT findings, a positron emission tomography (PET)/CT skull-thigh was ordered to analyze the patient’s abnormal mediastinal lymphadenopathy due to clinical suspicion of B-cell lymphoma. The PET/CT skull-thigh revealed hypermetabolic lymphadenopathy within the right paratracheal and prevascular spaces of the mediastinum. There was no hypermetabolic adenopathy beyond the mediastinum. Additionally, a fluoroscopic-guided bone marrow biopsy of the patient’s left iliac bone revealed normal iliac bone anatomy. Seven months after the incidental mediastinal lymphadenopathy finding, the patient was admitted for surgical resection, and a complete blood count was collected Table . A complA right video-assisted thoracoscopic surgery (VATS) resection of the mediastinal mass was completed along with multiple biopsy attempts. At this time, marginal zone lymphoma was determined. After the VATS procedure, the patient was discharged home without complications and began a chemotherapy regimen of bendamustine and rituximab for six cycles for eight months. In the following year after completing chemotherapy, an outpatient postoperative follow-up revealed progressive enlargement of the right paratracheal lymphadenopathy. The PET/CT scan showed an increase in size and metabolic activity of the right paratracheal lymphadenopathy measuring up to 3.4 cm x 3.1 cm (previously 2.2 cm x 2.1 cm), with maximum standardized uptake values (SUV) of 22.2 (previously 10.3). Additionally, the imaging revealed an increase in the size of a 17 mm x 12 mm prevascular lymph node with a maximum SUV of 2.8. Due to the enlarging right paratracheal adenopathy, the patient underwent right robotic-assisted partial resection of the paratracheal mass. The necrotic abnormal tissue was excised and submitted for frozen analysis, which confirmed lymphoma involving the right paratracheal mass. Additional tissue was submitted for fresh pathology, permanent pathology, and microbiology. A month later, the right paratracheal mass biopsy and immunohistochemistry highlighting podoplanin D2-40 Table  revealedOne month following the diagnosis of FDCS, the patient underwent genetic testing for cancer-type relevant biomarkers, genomic signatures, and pathogenic alterations. The patient’s tumor was positive for programmed death-1 ligand 1 (PD-L1) expression, a FANCM pathogenic variant, and a Janus kinase 1 (JAK1) pathogenic variant. Genomic signatures were analyzed and revealed no additional abnormalities. After genetic testing, the patient was advised to follow up with oncology. During this visit, the PET scan results and the treatment plan was reviewed with the patient. The patient’s case was discussed with the tumor board, and local therapy surgery +/- radiation therapy was recommended. At this stage, the literature supported surgical resection of the sarcoma as the main treatment approach and suggested that the role of adjuvant therapy was questionable. The patient was advised to follow up with radiation oncology to discuss the role of radiation therapy. Physical examination revealed no palpable adenopathy, B-symptoms, or respiratory symptoms. During the consultation, it was discussed that FDCS may be on a continuum of lymphoid lesions that are at increased risk in patients with SS. Because mantle zone lymphoma commonly appears in the salivary glands, it was suggested that the mediastinal lymphoma may have been related to FDCS initially. Combined surgery and radiation therapy were scheduled for a month from the radiation oncology consultation. An additional CT chest with IV contrast was ordered for sarcoma staging. Imaging revealed a slight interval increase in the size of anterior and middle mediastinal lymphadenopathy without any new sites of metastatic disease in the chest. Following sarcoma staging, cardiology was consulted to perform a preoperative cardiovascular exam. Based on the American College of Cardiology and American Heart Association (ACC/AHA) guidelines the patient was determined to be at moderate risk for a perioperative cardiovascular event during the planned right thoracotomy. And so, the patient accepted that he would be at higher risk for postoperative atrial fibrillation. At this stage, the patient was scheduled for a right thoracotomy for FDCS analysis and underwent a successful resection.Monocytes and related-immune cells are divided into two types: 1) phagocytes, and (2) dendritic cells. The dendritic cells include follicular dendritic cells, interdigitating dendritic cells, intestinal dendritic cells, Langerhans cells, and indeterminate cells . Follicu phagocytDue to its rarity, FDCS is frequently misdiagnosed and is often not considered in a differential diagnosis . HistoloAlthough FDCS usually has an indolent course, local recurrence is common. Nodal involvement occurs in 15% of cases, with 25 cases involving the mediastinum . FollicuCastleman disease (CD) is a benign tumor that develops in the lymph nodes and is typically associated with abnormalities of follicular dendritic cells, especially in the hyaline-vascular histological subtype and in the multicentric variant . The conAlthough SS elevates the risk of developing non-Hodgkin’s lymphoma , the assIn this report, we discussed the unique case of a 72-year-old male who was diagnosed with FDCS, an exceptionally rare malignant soft tissue neoplasm that may be linked to his diagnosis of SS two years prior. Patients with autoimmune disease, specifically SS, are placed at greater risk for hematological malignancies. Due to this possible association, further research to definitively outline the possible pathologic link between SS and FDCS is needed. Currently, there exist only 809 cases of diagnosed FDCS in English literature. Follicular dendritic cell sarcoma ordinarily follows an indolent course, however, local recurrence is frequently seen. The vast majority of FDCS malignancies involve extranodal sites such as the liver, spleen, and gastrointestinal tract. The FDCS literature lacks definitive and optimal treatment regimens, however, surgical resection with adjuvant chemotherapy and radiation likely provide the best outcomes. In light of this unique case presentation, we hope to bring more light to FDCS as a differential diagnosis when considering soft tissue malignancies and hope to stimulate further research that explores the possible pathological link between SS and FDCS.


Inguinal hernias containing the stomach are extremely rare, and have never been described in females. We are reporting the case of a 79 year old female who presented in septic shock with a left inguinal hernia containing the stomach, resulting in gastric perforation and loss of abdominal domain. She underwent emergency exploratory laparotomy with manual reduction of the hernia, wedge resection of the perforated gastric segment, abdominal washout, and closure of the abdominal fascia using biological mesh. She had a complicated but successful postoperative course, and was discharged to a rehabilitation center three weeks after hospital admission. Inguinal hernias are commonly encountered in general surgery, accounting for 75% of all abdominal wall hernias, and undergo operative repair 1 million times a year in the United States. Inguinal hernias occur more commonly in males than females, with a lifetime risk of 27% and 3%, respectively . Here, wA 79 year old female patient presented to the emergency department with a two-day history of intractable nausea, vomiting, and diffuse abdominal pain. She reported having had a left groin bulge for 70 years and did not regularly see a doctor. Vital signs were notable for tachycardia and tachypnea. Physical exam was notable for a firm and distended diffusely-tender abdomen; and an associated giant incarcerated left inguinal hernia, extending down to the thigh. Laboratory evaluation revealed leukocytosis (WBC 20 K/uL), lactic acidosis (lactate 5 mmol/L), and acute kidney injury (Cr 1.5 mg/d). Computed tomography imaging demonstrated a giant left inguinal hernia containing the stomach, proximal duodenum, right hemicolon, and omentum, and associated free intraabdominal fluid and pneumoperitoneum . AggressThis is the first reported case of a stomach-containing inguinal hernia in a female. Inguinal hernias containing the stomach are rare and medically challenging. They are typically large in size and may be complicated by perforation or loss of domain. Their surgical management involves the following steps: care of associated critical-illness, repair of gastric perforation, closure of the abdomen when there is loss of domain, and, finally, inguinal hernia repair.Patients with stomach-containing inguinal hernias may present acutely and critically ill. Perforation of the stomach can lead to septic shock. Gastric outlet obstruction can lead to volume depletion and concomitant hypovolemic shock. Fluid resuscitation, early antibiotic administration, and source control are key upfront therapies . IntensiPatients with stomach-containing inguinal hernias may go on to develop acute gastric perforation, likely due to a combination of factors: pressure-related injury from gastric distension , 7, tearPatients with stomach-containing inguinal hernias may develop loss of domain, thereby making closure of the abdomen more complicated . Giant hPatients with stomach-containing inguinal hernias may benefit from a staged repair of their inguinal hernia depending on the degree of contamination or hemodynamic instability . Inguina


Pneumatosis is always an alarming sign that may result from life-threatening bowel ischaemia and infarction; however, benign intramural gas can also result from a variety of secondary conditions including chronic obstructive pulmonary disease (COPD). Pneumoperitoneum and pneumoretroperitoneum can be seen with both entities. Therefore, thorough discussions with the referring clinicians regarding the patient’s medical history, clinical examination and laboratory results are mandatory. Benign causes can be managed conservatively, however, emergency exploratory laparotomy is often required in suspected life-threatening conditions. Misinterpretation of this finding can lead to incorrect diagnosis and unnecessary surgery. A 79-year-old male presents with 1-day history of lower abdominal pain, nausea and vomiting. Biochemical markers of inflammation were raised, and examination findings revealed generalised tenderness with guarding in the left iliac fossa. His past medical history included chronic obstructive pulmonary disease (COPD), large bowel diverticulosis, conservatively managed diverticular perforation and recent acute cholecystitis.A CT of the abdomen and pelvis enhanced with contrast in the portal venous phase was performed to investigate for acute diverticulitis. Previous CT imaging was available for comparison.Images demonstrated a small volume pneumoperitoneum within the abdomen and pelvis and associated widespread areas of pneumatosis intestinalis. There were prominent fluid-filled small bowel loops with no signs of bowel obstruction in keeping with an ileus. No portal venous gas was demonstrated. Additional evidence of uncomplicated large bowel diverticulosis and a thick-walled collapsed gallbladder consistent with recent cholecystitis. No free fluid or drainable collection. The visualised lungs demonstrated pulmonary emphysema with multiple small cystic changes .Based on the CT findings of diffuse intramural gas and intraperitoneal free gas, a clinical diagnosis of pneumatosis cystoides intestinalis (PCI) with a small volume pneumoperitoneum was made. Differential diagnoses for these radiological appearances include a hollow viscus perforation with associated pneumatosis intestinalis, however, there was no definite site of perforation appreciated on the CT. Correlation with the overall clinical picture and biochemical markers including lactate were advised.Following initial investigations, the patient was observed by the surgical team. Despite 4 days of intravenous antibiotics, the patient continued to have abdominal pain with mild generalised abdominal tenderness on examination and persistently raised biochemical inflammatory markers.A repeat CT of the abdomen and pelvis enhanced with contrast in the portal venous phase revealed less extensive pneumoperitoneum and resolving pneumatosis compared to the CT images performed four days prior. There were no other significant interval changes with no free or localised intraperitoneal fluid collection . After cIn this case, sequel CT examinations excluded a life-threatening cause of intramural gas and supported the diagnosis of this relatively uncommon finding of benign pneumatosis intestinalis. It is often idiopathic and asymptomatic, however when extensive, as with this case, an underlying cause is often present. PCI was felt to be secondary to the patient’s COPD. CT findings vary from focal to diffuse gas collections along the gastrointestinal tract forming round cystic collections in the subserosa layer which are often benign features. They can also be found tracking along the submucosal bowel wall creating linear or cresenteric gas patterns; the latter is often, however, not always, indicative of bowel ischaemia.The mechanical theory of intramural gas occurs when air migrates from the gastrointestinal lumen into the submucosal or subserosal layer of the bowel wall as a result of increased intraluminal pressure during coughing or intestinal obstruction. PCI can be divided into a primary and idiopathic aetiology (15%) or a secondary type (85%) which is caused by various predisposing factors including COPD.PCI is a rare disease affecting approximately 0.03% of the adult population and is often a self-limited process.The pulmonary theory of PCI involves alveolar rupture and air leakage because of coughing and high airway pressure in COPD. Gas then migrates through vessels within the mediastinum and bowel mesentery before breaching the bowel wall and becoming trapped forming air-filled cyst within the wall.To our knowledge, we can assume that the latter has occurred in this case. Furthermore, dissection of intramural gas through the serosal layer of the bowel or rupture of a cyst in PCI are hypothetical causes of pneumoperitoneum which result can result in acute abdominal distension and pain. PCI has a vague and broad spectrum of symptoms including diarrhoea, constipation, abdominal distention, nausea, vomiting, weight loss. It can easily be misdiagnosed clinically; however, the radiographic finding of pneumatosis intestinalis should raise the clinician’s concern about the development of life-threatening conditions.PCI can occur anywhere within the gastrointestinal tract mainly reported in the large intestine, then small intestine and less frequently in the stomach.Surgery is only recommended when complications are suspected. The combination of bowel wall soft tissue thickening, free fluid and periintestinal fat stranding and portal venous gas are worrisome features and associated with a life-threatening cause and higher mortality rates. Clinical awareness along with close surveillance is vital in the accurate diagnosis and management this condition. Surgical consultation is mandatory if there is any uncertainty or clinical deterioration.In benign cases, in the absence of such additional concerning features, the referring clinicians must rely on the patient’s medical history, clinical examination and laboratory results to make life-saving clinical decisions about management. Conservative management includes antibiotics, nasogastric decompression, intestinal rest and oxygen treatment for pulmonary emphysema if appropriate.Intramural bowel gas  is the presence of air within the bowel wall secondary to dysbacteriosis of gastrointestinal flora, poor mucosal integrity, high intraluminal pressure or migration from pulmonary gas.CT imaging is sensitive for detecting subtle intramural gas, for which the lung window is beneficial. Multiplanar reformatted images enables different patterns of gas collections within the submucosal or subserosal bowel wall to be identified which can help differentiate the underlying pathological process.Obstructive airways diseases (asthma and COPD), systemic diseases , intestinal inflammation and iatrogenic drugs are some of the causes of benign intramural gas. Yet, this radiological sign also occurs in life-threatening cases of bowel ischaemia and is associated with emergency surgical intervention and a high mortality rate.Awareness of the causes of intramural gas alongside discussions with the referring clinicians about the patient’s history, examination and laboratory results are critical to avoid misinterpretation, incorrect diagnosis and unnecessary surgery.


Duodenal gangliocytic paragangliomas are rare neuroendocrine tumors primarily localized in the periampullary area. Though mostly asymptomatic, they can present with various symptoms, most often jaundice, anemia and abdominal pain. The present paper is a case series report, describing our personal experience with patients presenting to the Emergency Unit with different symptoms due to duodenal gangliocytic paraganglioma. Endoscopic resection is safe and indicated in most of the cases, being also associated with lower medical costs. EUS plays a central role in the pre-resection management and in surveillance, and immunostaining is decisive to ascertain the tumor histologic origin. In addition to reporting our experience, we researched the literature regarding these rare tumors and performed a comprehensive review. Duodenal gangliocytic paragangliomas (GP) are rare neuroendocrine tumors, with less than 300 cases being reported worldwide, most of them as single case reports. Duodenal GPs arise mainly in the second part of the duodenum and in the periampullary region. Unlike other neuroendocrine neoplasia, GPs have a benign clinical course, being rather asymptomatic and sometimes incidentally found during imaging studies or autopsy ,2. When Due to their low incidence and vague presentation, their diagnosis could prove difficult without a skilled eye. Awareness about their clinical and endoscopical presentation, as well as their histological features should be raised. In this paper, the authors of the study report their experience with three patients that presented with different clinical manifestations of duodenal GPs, which were eventually managed endoscopically or surgically during the period of one year. The main aim is to highlight inapparent clinical, endoscopic diagnostic features of the pathology as well as its therapeutic management. Moreover, we searched multiple databases  for studies reporting the presence of GP and realized a literature review on this topic. Following the international guidelines, our study could add to a relatively scantily represented segment of medical literature, mainly in the field of patient management and diagnosis .We present the case of a 29-year-old male, without any significant medical history except for first grade obesity, who was sent to our hospital for recently debuted sclero-tegumentar jaundice, pruritus and epigastric pain, with a preliminary tomographic diagnosis of both vesicular lithiasis and choledocholithiasis established in another medical unit. Biochemically, no anemia or leukocytosis were noted, and outside of slight cholestasis and hepatocytolysis, no other changes were identified. Given the young age, we initially directed him toward endoscopic ultrasonography (EUS) for confirmation of the diagnosis. Later on, we sent the patient to an endoscopic retrograde cholangiopancreatography (ERCP) procedure for papillosphincterotomy and stone extraction. After carefully examining the ampullary region, we noticed a slightly elevated, inapparent, tumor-shaped protrusion adjacent to the major papilla, with a normal mucosal lining. A differential diagnosis was made between a benign tumor, an eerie biliary stone impaction or a bulge of mucosa resembling a tumor. Therefore, we set for cannulation and biliary tract contrast injection, which showed, along with two lacunar round images suggestive of calculi, an enlarged common bile duct (CBD) of up to 10 mm and a distal biliary stenosis in the last 15 mm. Stone extraction was performed using a basket, and a 10 Fr/7 cm stent was placed .TM, Boston Scientific Corporation, Marlborough, MA, USA), three passes being conducted through the tumor ; afterwards, micrometer-thick sections were sliced at a microtome. Morphological studies using Giemsa and Hematoxylin–Eosin (HE) colorations were carried out, and then immunostaining was performed. Microscopically, the tumor cells were ovoid, monomorphic, presenting mild nuclear atypia, low mitotic activity and no necrosis. As for the immunohistochemistry staining, the fragments were positive for synaptophysin, chromogranin A and CD56 and focally positive for pan-cytokeratin AE1/AE3. They stained negative for S100, CD34, MSA. The Ki67 score was 1%. Finally, the pathologist classified the lesion as a neuroendocrine well-differentiated tumor (NET grade 1).Afterwards, the patient underwent further imaging studies, computed tomography (CT) and magnetic resonance imaging (MRI), for preoperative stadialization, which rendered the tumor resectable . TherefoMorphological studies of the whole resected tumor showed a nodular, well-defined structure, covered with duodenal lining with clear resection margins. Microscopically, it featured three-phasic tumoral proliferation consisting of Schwannian-type spindle-shaped cells, ganglia-like cells and neuroendocrine-type cells with nests, acini and trabeculae; rare glandular structures were noted. These findings were consistent with the diagnosis of gangliocytic paraganglioma. The recovery after the surgical intervention was excellent and, after one year of regular follow-up, the patient has no tumoral recurrence and no symptoms .The second patient was a 43-year-old male, with a medical history of primary arterial hypertension and obesity class 1, referred to our Emergency Unit for black, tarry stools, known as melena, suggestive of upper gastrointestinal bleeding, epigastric pain, hypotension and asthenia. These symptoms started 7 days before presentation. Biologically, a hemoglobin value as low as 5 g per deciliter was detected. The patient was hemodynamically stabilized with blood transfusions and plasma expanders and also received high-dose proton pump inhibitor therapy. An upper gastrointestinal endoscopy was performed, and in the second part of the duodenum, in the nearby vicinity of the major and minor duodenal papilla, an extramucosal, ulcerated, polipoid lesion of about 20/15 mm was noted, with a hematic clot covering the ulcer base and a diffuse slow hemorrhage noted at the clot’s extremities. At first, hemostasis was conducted by perilesionally injecting 20 mL of adrenaline 1:10,000. After this, we performed a CT exam that excluded the possible local or distant spread of the tumor. The next day, the upper endoscopy by means of a side-viewing duodenoscope was repeated with the intention to remove the tumor .Therefore, under Propofol sedation, the patient underwent endoscopic resection with the use of a 25 mm polypectomy snare. Therefore, we injected diluted adrenaline  into the nearby duodenal wall, and the tumor was resected into two pieces with dimensions of 20/15/5 mm and 15/10/5 mm, respectively .Hemostasis by means of an adrenalin 1:10,000 injection and electrocoagulation with a 10 Fr bipolar probe was used to minimize the risk of bleeding .Microscopically, we observed tumoral proliferation in three components: spindle-shaped cells, which vastly outnumbered the others, focal ganglia-type cells and areas with medium-sized epithelioid cells with a neuroendocrine phenotype, disposed in a tubulo-papillar fashion. There was no obvious mitotic activity, and no necrosis was detected. The overlying duodenal mucosa, as well as the peripheral tissue contained no tumor elements .The immunochemistry analysis showed S100 and synaptophisine diffusely staining, as well as chromogranin staining in the epithelioid tumor component, which was also partially stained for EMA, AE1/AE3 and PGR. The Ki67 score was 2–4% .The histopathological results and immunochemical assays were consistent with the diagnosis of gangliocytic paraganglioma.There were no periprocedural complications, and the patient was discharged after 5 days of surveillance. Six months later, the patients underwent endoscopic and endosonographic evaluation, which prompted no further therapeutic steps, the resection spot being barely visible, with no recurrence signs by EUS.The third patient was a 41-year-old male, without any relevant medical history outside of obesity class 1 and essential hypertension, presenting to endoscopic evaluation for symptomatic reflux disease and abdominal pain. The blood tests only showed a slightly elevated creatininemia and minimal anemia, with no other pathological changes, including tumoral markers detection. The Helicobacter pylori stool antigen test rendered a positive result. Abdominal ultrasound scanning yielded normal results. At first, the patient underwent endoscopy by means of a standard frontal-viewing endoscope and, a 4 mm sessile polyp Paris Is was identified in the bulbar area, close to the pyloric orifice, hidden by the posterior sphincterian wall . BiopsieThe next logical step was preparing for polypectomy, and 3 days later we examined the lesion again with a frontal-viewing endoscope which visualized the aforementioned, inapparent lesion, but we were unable to attain an optimal resection position of the endoscope. Therefore, we withdrew the standard endoscope and entered the duodenum with a lateral-viewing duodenoscope, which offered a much better strategic position A. ElectrA morphological analysis showed cells with hyperchromic nuclei, elongated, spindle-like or round-oval in shape, with a low cytoplasmic volume, alternating with cells with vesicular nuclei and a high-volume, clear, granular cytoplasm. Lymphoid tissue was present in an intertwined fashion with the tumoral tissue . NecrotiThe patient was monitored shortly after the procedures and on the long run, endoscopy being performed after 3, 6 and 12 months, revealing complete local healing in a normal clinical context, outside of a mild persistent abdominal pain managed by proton pump inhibitors, non-steroidal anti-inflammatory drugs and pain medication. A rapid urease test was performed, with negative results due to eradication therapy that the patient was offered meanwhile. Furthermore, endoscopic ultrasound was conducted shortly after the resection and 12 months after, in order to confirm complete resection.Outside of the persistent abdominal pain, which seemed to be functional, another persistent feature, important in the context, was the continuation of arterial hypertension at a certain degree; so our patient was sent for Holter monitoring of blood pressure for 24 h, which showed a range of systolic blood pressure of 113–144 mmHg and a range of diastolic blood pressure of 65–101 mmHg. The patient was prescribed a calcium channel blocker and sent for continuation of investigations. Serum chromogranin A, gastrin or serotonin (5-hydroxytryptamine) and urinary catecholamines, vanillylmandelic acid or 5-hydroxyindoleacetic acid were tested and yielded normal results. Later on, even positron emission tomography (PET) with radiolabeled somatostatin analogs and a neuroendocrine tumor protocol were carried out, with no gastro–duodenal, bilio–hepato–pancreatic, retroperitoneal or adenopathic anomalies detected .Pheochromocytomas (PCCs) and gangliocytic paragangliomas (GPs) are rare abnormal growths of chromaffin cells in the adrenal glands and elsewhere in the body, respectively. They can occur in almost any location, except for the bones and brain, all tumors having metastatic potential, with 20% of all tumors presenting in at least two primary locations (multicentric gangliocitic paraganglioma) . Their hThe first-ever description of a duodenal GP by Dahl in 1957 as a duodenal ganglioneuroma was soon followed by the account of Taylor and Helwig in 1962 of a benign nonchromaffin paraganglioma ,9,10. GaThere is limited research on the use of anti-angiogenic drugs in the treatment of GPs, but some studies have shown promising results of this treatment in combination with conventional therapies. Combining anti-angiogenic therapy and immunotherapy is an emerging strategy in the treatment of gangliocytic paragangliomas. However, more research is needed to determine the optimal combination and dosing of these therapies for gangliocytic paraganglioma .Our tripartite study scoped patients with three particular clinical modes of presentation of GPs: jaundice and abdominal pain, anemia and upper gastro-intestinal bleeding, and symptoms resembling a possible carcinoid syndrome, all tumors being located into the first and second part of the duodenum, i.e., right after the posterior pyloric sphincterian wall, in the nearby vicinity of the minor duodenal papilla, and adjacent to the major papilla.Interestingly, the demographic and history features were somewhat similar—all patients were young men  and were from at least overweight to obese grade I . Two of the patients also presented primary arterial hypertension, which in the circumstances of a possible hormone-secreting tumor, may raise questions about the etiology of the raised blood pressure, but the context of our three case reports was not linked to the tumoral manifestations spectrum.Regarding the diagnostic algorithm, endoscopic ultrasound plays a central role in the early management of every polypoid duodenal tumor, especially for those covered with normal mucosal lining, which can trick the endoscopist into identifying them as normal mucosal bulges, duodenal hyperplastic polyps, diffuse duodenal lymphoid hyperplasia or other common or rare lesions. EUS has the ability of visualizing the origin layer or allowing the tissue acquisition through EUS-FNA or EUS-FNB, very important to obtain a fast diagnosis or in post-endoscopic resection follow-up. Both surgical and endoscopic resection play separate important roles in the treatment of GPs, as our study showed, the latter being advantageous for tumors with superficial location and when local or distant extension is not detected in sectional studies, also lowering the total costs of these patients’ management. Extensive endoscopic and imaging evaluation, as well as biological special tests, are to be done before endoscopic or surgical resection is planned. After this, extensive morphological and staining analyses are needed to ascertain the tumoral origin and differentiate the tumor from neuroendocrine tumors or other benign or malignant nodular lesions. On the long run though, endoscopic, EUS, possibly bioptic, and imaging follow-up are necessary for excluding tumoral relapse, which will raise the costs, despite the tumor’s mainly benign course.Our three cases highlight another warning sign for complete and exhaustive endoscopic duodenal exploration with both frontal-viewing and lateral-viewing endoscopes when such pathology is suspected.GPs can manifest with diverse clinical and endoscopic presentation, but they also have some common features, such as demographic or history characteristics—male gender and obesity—and post-resection prognosis, which is much more favorable compared to that of other duodenal tumors, with predominantly excellent survivability and curability. Even though recurrence and metastasis cannot be completely ruled out, a careful preoperative assessment should be conducted ,16.Limitations of the study include the lack of genetic testing as well as of the application of a more personalized approach. The lack of long-term monitoring can represent a further limitation.Gangliocytic paragangliomas have a polymorphic clinical presentation. These tumors should be suspected in the presence of a polypoid duodenal lesion associated with obstructive jaundice which seems benign by sectional studies, upper digestive hemorrhage and anemia, or symptoms of carcinoid syndrome. EUS plays an important role in the pre-resection management and in surveillance. At the same time, immunostaining is decisive to ascertain a tumor’s histologic origin. Endoscopic resection is safe and indicated in most cases, being associated with few complications, a quick recovery and low medical costs. Surgical resection is recommended when endoscopic resection is unsafe, the tumor is invading the profound layers or metastasis in the lymph nodes are present.


A retained foreign body (RFB) is a rare but possible complication of surgery. Among the most common retained foreign bodies are sponges, which may include lap pads and gauze pieces. Surgical never events are errors in medical care that are identifiable and preventable but have serious consequences for the patient, making it an important problem in terms of the safety and credibility of a healthcare facility. They also pose a major medicolegal threat to healthcare organizations and a diagnostic challenge for surgeons. Herein, we present the case of a 35-year-old woman who presented with signs and symptoms of acute intestinal obstruction. She revealed a history of Caesarean section 11 months prior. She had a stormy postoperative course then and had to undergo a diagnostic laparoscopy for pus aspiration three months after surgery, where no finding other than pus was reported. Upon presentation at our tertiary care center, she was examined and found to have an RFB for 11 months. She was managed surgically with successful laparoscopic removal of the gossypiboma and consequent resolution of all her symptoms. Though rare, the possibility of an RFB, especially after open surgery, should be kept in mind when diagnosing patients who present with pain, mass in the abdomen, or symptoms of an infection. Laparotomy is the mainstay of treatment for gossypiboma, but successful laparoscopic removal of the RFB provides a definite treatment with the super-added benefits of laparoscopy. Laparoscopic removal of gossypiboma has been reported in the literature and demonstrated in our tertiary care center. Gossypiboma, textiloma, or cottonoid are the common terms used to describe a mass in the body caused by a retained surgical sponge surrounded by a foreign body reaction. A surgical sponge is the most common type of retained foreign body (RFB) . The exaWe present the case of a 35-year-old female patient who presented with signs and symptoms of acute intestinal obstruction. She revealed a history of Caesarean section 11 months prior and had been more or less asymptomatic since then.A 35-year-old woman presented to the gastroenterology outpatient department with complaints of pain in her abdomen and inability to pass stools for five to six days. The pain was dominantly in the infraumbilical region, acute in onset, dull in nature, nonradiating, not associated with food intake or movement, and nonresponsive to oral pain medications. Occasionally, she also felt a slight discomfort in the periumbilical region. She denied any episodes of vomiting or fever and mentioned that she was able to pass flatus.In early 2021, the patient underwent a laparoscopic myomectomy for a large uterine fibroid. Following that, she received a trial of in vitro fertilization, which resulted in the implantation of three fetuses, of which two were in the uterus and one was tubal ectopic, for which she underwent a second laparoscopy. She carried the uterine twin pregnancy to term and delivered via Caesarean section in January 2022. Her postoperative course was complicated by abdominal pain and nonpassage of flatus, but she was managed conservatively for subacute intestinal obstruction.In March 2022, she experienced vague abdominal pain and underwent ultrasonography. Free fluid was observed in the pelvis; therefore, the patient underwent a diagnostic laparoscopy, during which pus was aspirated from the pelvis, with no other significant findings.The patient presented to our tertiary care center with the aforementioned complaints. On examination, she was tachycardic with a heart rate of 105 beats per minute, and the rest of her general examination results were unremarkable. Her abdomen was nondistended and soft but tender in the left iliac fossa and infraumbilical region. A nasogastric tube was inserted, and the patient kept nil per oral and was managed in the ward with fluids and adequate analgesia.InvestigationsNone of the hematological investigations revealed any abnormalities. Erect radiography of the abdomen revealed air-fluid levels. An ultrasonography study was reported to show multiple dilated jejunal loops and an echogenic foreign body of approximately 8 cm, with posterior acoustic shadowing in the lower midline above the bladder. The findings from a contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis resonated with the ultrasonography findings, showing 40-mm wide jejunal loops and jejunoileal junction narrowing. Radiologists also reported a 10 cm × 5 cm oblong low-density structure with thick walls closely adherent to the bowel loops and adjacent uterus  and the Swahili word boma . An RFB discovered by a fresh set of surgeons poses a professional and personal dilemma. For this reason, gossypiboma cases remain grossly underreported. However, the incidence of gossypiboma was reported to be 1 in 100-5,000 surgical procedures and one in 1,000-1,500 intra-abdominal operations . GawandeAn RFB can lie dormant in the body for long periods, and the body deals with it in two ways: first, through an exudative, infectious response that leads to abscesses and fistulas, and second, an aseptic fibrinous response that leads to encapsulation and subsequent mass effect ,6. A patA gossypiboma can be difficult to diagnose. Theoretically, all radiological investigations such as ultrasonography, radiography, CT, and MRI can identify RFBs but may be challenging. In the case of radiography, the sponge may be hidden, like in our case, where it was in a pelvic pouch, or the skiagram may be complicated by gas shadows or incidental findings such as renal stones. Our patient's radiograph only showed features of small bowel obstruction. The RFB may not be detected on MRI because the radiopaque marker is not magnetic. For all these cases, a CT examination is the investigation of choice. The characteristic appearance is that of a spongiform pattern with gas bubbles .When a gossypiboma is found, surgical removal of the foreign body is mandatory. The two available options for dealing with a gossypiboma are exploratory laparotomy and diagnostic laparoscopy, both followed by the removal of the RFB and needful surgery. While an open method can be quicker and easier and provide better exposure, laparoscopy has advantages, including a less painful postoperative course, smaller scars, early ambulation, early oral intake, lesser risk of hernia, and overall quicker recovery and return to daily life activities ,7. Howevnever events are errors in medical care that are identifiable, preventable, and serious in their consequences for patients, making it an important problem in terms of the safety and credibility of a healthcare facility [A gossypiboma is classified as a surgical never event. According to the definition of the US National Quality Forum, facility . To prevThough rare, an RFB should not be completely ruled out when making a differential diagnosis. To avoid RFBs, multiple checks at multiple levels should be ensured and the nursing staff should be given proper education about safety checklists and the importance of instrument and gauze counts. Although laparotomy is the go-to procedure for RFB removal, successful laparoscopic removal allows for diagnostic exploration, with the added advantages of early ambulation, decreased postoperative pain, early diet tolerance, and overall early return to normal life.


Congenital peribronchial myofibroblastic tumor (CPMT) is an extremely rare lung disease in infants. It shows benign behavior and has a favorable survival after surgical treatment. CPMT was reported only in cases. Here, we report the longest follow-up known case of CPMT and review the clinical, radiographic and histopathological features of the published literature.ETV6-NTRK3 fusion or ALK rearrangement was detected. Gene mutations in JAK2 and SMO were detected by NGS. She is currently alive for 8 years with no evidence of disease recurrence.Ultrasound examination at 30 weeks of gestational age of a healthy 29-year-old female revealed a solid mass in the left lung. Computed tomography (CT) revealed a mass in the left lower lobe. The tumor was removed by lobectomy and pathologically diagnosed with CPMT. The tumor was composed of cartilage, spindle cells and oval cells. Vimentin was strongly positive. Smooth muscle actin (SMA) was positive in the spindle cells. The histopathologic and immunohistochemical features were similar to those in the literature. No JAK2 and SMO were detected, which may be associated with the formation of CPMT.CPMT is a rare lung tumor in infants. Surgical treatment is recommended for CPMT. The prognosis after successful surgery is favorable. The final diagnosis was histopathologic findings. Due to its cellularity, mitotic activity and rapid growth, long-term follow-up should be strengthened. The present patient is alive and well for 8 years after the surgery without recurrence. Gene mutations in  Lung lesions rarely occur in utero or infancy. With the development of ultrasonography, lung lesions in infants can be detected mostly by prenatal ultrasound examination. Congenital lung lesions included abnormalities in lung development, such as pulmonary sequestration (PS) and congenital pulmonary airway malformation (CPAM), and primary pulmonary tumors, such as pleuropulmonary blastoma (PB), congenital-infantile fibrosarcoma, and fetal lung interstitial tumor (FLIT).Congenital peribronchial myofibroblastic tumor (CPMT) is an extremely rare lung disease in infants. The tumor was first reported in 1949 as “hamartoma” . Accordith case and the second case in China and give a review of the literature with clinical, radiographic, and histopathologic characteristics.CPMT was reported only in case reports, and there were just 24 cases reported in the published English-language literature –22. Here. A healthy 29-year-old female, gravida two, para two (G2P2), her first child had died a few days after birth of respiratory distress syndrome (RDS). The present case was her second pregnancy. Prenatal ultrasonography was regularly scheduled, and she had no complicated pregnancy. She did not reveal polyhydramnios or hydrops fetalis. Ultrasound examination at 30 weeks of gestational age revealed a solid mass in the left lung, indicating CPAM. The neonate was delivered by cesarean section at the 34th week of gestation. The birth weight was 2.4 kg, and the newborn was 48 cm long. The Apgar scores were 10, 10, and 10 at 1, 5, and 10 min, respectively. She had no respiratory difficulty at birth. The infant was discharged home with stable vitals. The infant was suddenly cyanotic at 20 days after birth, and she was admitted to West China Hospital, Sichuan University. Chest computed tomography (CT) showed a large mass lesion in the left lower lobe, as shown in FigOn gross examination, the lung showed a solid mass measuring 3.3 cm × 2.5 cm × 1.7 cm in size. Lung specimens were stained with hematoxylin and eosin for microscopic examination. Microscopically, the tumor surrounded the airway with peribronchial growth , leading to a frameshift mutation in exon 7. SMO found a point mutation at the acceptor splicing site of exon 5 (c.1140 + 1G > A).No The final histological diagnosis was CPMT. No complications occurred in the postoperative period. The patient is now 8 years old, and she is doing well without further treatment after surgery. There was no clinical or radiologic evidence of recurrence or metastasis.Congenital peribronchial myofibroblastic tumor (CPMT) is classified as a neoplastic lesion, which was first described by Jones, C. J. in 1949 , 17, 18.JAK2 mutation and SMO mutation were detected by 56-NGS in the present case, which may be associated with the formation of CPMT. Janus-activated kinase 2 (JAK2) is a member of the JAK protein family, and the activation of JAK2/STAT3 signaling is frequently detected in many tumors and plays an important role in oncogenesis, angiogenesis, and metastasis of many cancer diseases [The etiology of CPMT is unknown. The tumor is thought to originate from the pluripotent mesenchymal cells around proximal bronchial branches, which are destined to differentiate into cartilage and myofibroblasts to form the bronchial wall . Cytogendiseases , 24. Actdiseases . Our resThe histopathological features in the present case were similar to those in the published literature. The tumor surrounded the airway with peribronchial growth, composed of cartilage, spindle cells interlacing fascicles and oval cells. Strong positivity for vimentin was observed in the spindle cells and oval cell components, indicating that CPMT may originate from mesenchymal cells. Smooth muscle actin (SMA) positivity in the spindle cell component indicated that CMPT differentiated into smooth muscle tissue.ETV6-NTRK3 fusion gene. Most pleuropulmonary blastomas include DICER mutations. Some inflammatory myofibroblastic tumors (IMTs) have ALK rearrangement fusion genes and positive immunoactivity for ALK-1. Some synovial sarcomas have the fusion gene SS18. In the present case, ETV6-NTRK3 fusion and ALK rearrangement were not detected. Immunohistochemical staining of ALK-1 CD34, desmin and OCT3/4 was negative.Lung lesions can be detected by ultrasonography, chest-ray or computed tomography, sometimes with mediastinal shift, but the imaging features of CPMT are nonspecific. It is characterized by rapid growth and is easily misdiagnosed as a malignant tumor. The final diagnosis of CPMT is histopathologic findings. The differential diagnosis included other congenital abnormalities and pulmonary tumors, such as pulmonary sequestration (PS), congenital pulmonary airway malformation (CPAM), chondromatous hamartoma, teratoma, pleuropulmonary blastoma (PB), fetal lung interstitial tumor (FLIT), inflammatory myofibroblastic tumor (IMT), congenital-infantile fibrosarcoma, monophasic fibrous type synovial sarcoma and so on. Most infantile fibrosarcomas have an There were no symptoms in the present preterm case, with a lung mass approximately 3.3 in diameter. The present case received no further treatment after surgery. The patient is now 8 years old, and she is doing well, with no recurrence or metastasis. There were just 24 cases reported in the published English-language literature –22. A suJAK2 and SMO were detected, which may be associated with the formation of CPMT.CPMT is a rare lung disease, typically presenting as a solid lung mass in the fetal and neonatal period. CPMT is composed of immature cartilage and spindle cells in fascicles and oval cells. CPMT has a favorable survival after successful surgical resection. The present case of CPMT was detected at 30 weeks by ultrasound. The patient is doing well for 8 years after the surgery, with no recurrence. Gene mutations in