Omental liposarcoma is extremely rare, and only a few reports have been published in the literature. Due to the rarity of the disease, establishing a clear diagnosis and formulating a treatment plan may be challenging for clinicians.The patient was a 51-year-old woman who presented with a protruding mass and pain in the lower abdomen.Magnetic resonance imaging revealed a tumor measuring 15 cm in diameter in the pelvis. Ovarian cancer was suspected based on pre-operative imaging findings.An exploratory laparotomy was performed. Intra-operative analysis of the frozen section suggested a benign tumor.Postoperative histopathological analysis confirmed the diagnosis of omental liposarcoma. The patient recovered well after surgery.This case report helps clinical oncologists to develop a comprehensive understanding of this disease and treat it accordingly. If infections, tumors, or other diseases occur in the abdominal cavity, the omentum can limit the spread of the disease through the formation of wraps and adhesions. The omentum is a common site for metastasis of ovarian, stomach, and colon cancer, and is also a common site of granulomatous inflammation, including tuberculous infection and fibrosis. Primary omental tumors are rare.,4 According to the 2020 World Health Organization soft tissue tumor classification, most sarcomas originating in the omentum are soft tissue tumors. Soft tissue sarcoma is a rare malignant tumor derived from mesenchymal tissue cells that can occur in any part of the body. Liposarcoma and leiomyosarcoma are the most common soft tissue sarcomas in the abdominal cavity, and the retroperitoneum is the most common site. Omental sarcoma is very rare and can easily be misdiagnosed before surgery. Here, we present the case of a patient with omental liposarcoma, so that other clinical oncologists can develop a comprehensive understanding of this disease and treat it accordingly.The omentum is a double-layered membrane composed of the peritoneum and fatty tissue that contains blood vessels, nerves, lymphatic vessels, and connective tissue.2A 51-year-old female patient was admitted to the hospital with a protruding lower abdominal mass and sudden-onset lower abdominal pain with frequent urination. The patient started noticing a mass protruding from the lower abdominal wall 2 days before initial presentation; however, she did not pay attention to it at the time. The next day, she started experiencing sudden-onset, severe, and continuous lower abdominal pain after 2 hours of activity, which was accompanied by frequent urination. Abdominal ultrasonography performed at a local hospital revealed a large heterogeneous echo in the pelvic cavity. Ovarian tumor or uterine fibroids were suspected, and the patient was hospitalized for further examination and treatment. However, the patient did not seek further medical assistance after symptom relief. Two months later, the patient experienced abdominal pain again and was admitted to our hospital. Physical examination on admission revealed a body temperature of 36.6°C, blood pressure of 100/65 mm Hg, and respiratory rate of 20 breaths/min. On abdominal examination, a poorly mobile solid mass, approximately 15 cm in diameter, was palpable in the lower abdomen. Gynecological examination revealed a smooth mucous membrane of the vaginal canal, a normal cervix, and a large hard mass in the pelvic cavity with poor mobility and mild tenderness. Contrast-enhanced pelvic magnetic resonance imaging (MRI) showed a large mass in the pelvic cavity, which was considered an ovarian tumor. A small amount of fluid was present in the pelvis  helps in establishing the diagnosis and determining the treatment plan.,17 However, since there is a wide variety of omental tumors that have different properties and at different stages of malignancy, the interpretation of ultrasonographic findings may be affected by the presence of intestinal gas and sound energy attenuation, which complicates the diagnosis of omental tumors. Therefore, when omental tumors are suspected, ultrasonography must be performed carefully. Moreover, an operator's cumulative experience and optimization of scanning technology may also increase the detection rate of omental tumors and reduce the number of missed diagnoses. Ultrasound has a certain value in determining the location and internal structure of omental malignant tumors. To determine the tumor properties, it is necessary to combine ultrasonography with clinical examination and other imaging modalities, such as CT and MRI. Surgical resection is the treatment of choice for omental liposarcomas. Depending on the stage of malignancy, local palliative resection, local extensive resection, or radical resection can be performed. For patients who cannot undergo radical surgical resection, palliative surgery, and postoperative radiotherapy and chemotherapy can often reduce the tumor recurrence rate and prolong survival. In the present case, radical resection of the tumor was performed. Partial resection of the corresponding organs should also be performed if the tumor infiltrates or adheres to the abdominal and pelvic organs.Liposarcoma originates from primitive mesenchymal cells and is a common soft tissue malignancy.The surgical procedure usually combines complete resection of the tumor and partial resection of the involved organs. Tumor size and histological subtype are important prognostic factors. Based on the pathology, the prognosis of omental liposarcoma is better. Since most patients present late, when only incomplete surgical resection can be performed, the recurrence rate is high. Most cases of recurrence occur at the original site, and another operation is generally required to remove the recurrence. The literature reports that there is no significant difference in the prognosis between 2 resections and more than 4 resections; therefore, repeat surgical resections can be performed. The patient underwent contrast-enhanced abdominal MRI before the operation. Ovarian tumor and pelvic effusion were suspected based on the MRI findings. Since the origin and properties of the tumor cannot be accurately determined on imaging, pre-operative diagnosis is difficult. Because omental liposarcoma is rare, there is a lack of clinical experience and related research, which makes establishing the diagnosis based on imaging even more complicated. In addition, the inability of pelvic MRI to identify the blood supply to the tumor and the failure to perform percutaneous biopsy before surgery also led to misdiagnosis. Moreover, intra-operative frozen section analysis suggested a benign tumor, and the diagnosis of omental sarcoma was established only after pathological analysis of the resected tumor. General surgeons and urologists were consulted to formulate the postoperative treatment plan, which further highlights the difficulty in diagnosing and treating omental sarcomas. Because there is no standard treatment plan, the clinician should summarize and discuss the plan with the patient, and continuous follow-up should be performed to observe the clinical features and prognosis of this tumor.4In summary, it is difficult to make a clear pre-operative diagnosis of omental liposarcoma, and most cases are diagnosed postoperatively. Currently, there are no reliable investigation modalities or specific markers to diagnose this tumor in clinical practice. Although B-ultrasound, CT, and MRI are the main imaging modalities, due to subjective and objective reasons, the misdiagnosis rate is high, which should garner our attention. Before the operation, a detailed medical history should be obtained, and a careful physical examination and necessary auxiliary investigations should be performed to improve the diagnosis rate. If a pre-operative diagnosis cannot be established, laparotomy should be performed. Due to the delay in treatment and incomplete surgical resection, the recurrence rate is relatively high, which worsens the prognosis. Therefore, early detection and initiation of treatment and thorough resection of the tumor are instrumental in improving the prognosis of omental liposarcoma.www.editage.cn) for English language editing.We would like to thank Editage (Conceptualization: Ying Gao, Yujie Qin, Xiaoyi Wei.Data curation: Ying Gao, Yujie Qin, Yingchao Wang, Xiaoling Quan, Jiaxi Yao.Funding acquisition: Ying Gao, Jiaxi Yao.Investigation: Yujie Qin.Resources: Yingchao Wang, Xiaoling Quan, Xiaoyi Wei.Writing – original draft: Ying Gao, Yujie Qin.Writing – review & editing: Xiaoyi Wei, Jiaxi Yao.


Angiofibromas classically develop in the lateral wall of the nasopharynx from the sphenopalatine region. Extra-nasopharyngeal angiofibromas are rare entities, with the maxillary sinus being the most common site. Parapharyngeal angiofibroma is an extremely rare entity, being seldom reported in English literature. We present a case of parapharyngeal angiofibroma, which came as a diagnostic surprise in a young adult 25-year-old male. The radiological picture showed a highly vascular lesion, which did draw our attention for not going for a direct or guided FNAC, and upfront excision was planned through the transcervical route. A firm 5 * 7 cm mass was excised and sent for histopathologic examination. The histopathology showed angiofibroma like features as a diagnostic surprise as angiofibroma of parapharyngeal space is a known but rare entity. The knowledge of angiofibroma as a differential in parapharyngeal space will help the clinicians to properly deal with these disorders during preoperative evaluation and definitive surgery and thus prevent the chances of vascular injuries and complications associated with an FNAC. A high level of suspicion regarding this differential as a possible lesion in the parapharynx is required for the entity's diagnosis.  Angiofibromas are classically seen in the nasopharynx. Extra-nasopharyngeal angio- fibromas have rarely been reported. Primary sites of extra-nasopharyngeal angiofibromas are mentioned to be the nasal septum, maxillary sinus, and ethmoids -6. Parapharyngeal angiofibroma is an extremely rare entity and is a diagnostic surprise for the clinician. A high level of suspicion is required for consideration of angiofibroma as a differential diagnosis of parapharyngeal space (PPS) lesions. We report a case of extra-nasopharyngeal angiofibroma in a young male. A 25-year gentleman presented complaining gradual change in voice quality, heaviness of throat, and occasional swallowing difficulty for the last two years without definite dysphagia, dyspnea, or odynophagia. History pertaining to other systems and addiction history was negative. Head and neck examination revealed a left lateral pharyngeal wall bulge with medially pushed tonsil. Visible or palpable neck swelling or retro mandibular fullness were absent. It was not bimanually palpable. Intraoral palpation revealed non-pulsatile soft to firm swelling with restricted mobility. Imaging studies revealed a hyperdense soft tissue mass lesion involving the pre-styloid compartment of left PPS between the root of pterygoids anteriorly, styloid process posteriorly, and body of mandible laterally with posteriorly displaced great vessels ; mediallCT angiographic images revealed a highly vascular mass with multiple vessels with posteriorly pushed carotids . A decision to avoid fine-needle aspiration cytology (FNAC) examination was taken considering the vascular nature of the lesion seen in angiography, and surgical excision was planned. Trans-cervical excision with horizontal lateral incision was planned, and mandibulotomy was reserved as an optional approach to increase exposure at the time of need. A 5 * 7 cm firm swelling involving the para pharyngeal space was found and dissected out. The postoperative gross evaluation revealed a well-circumscribed greyish-white mass with a firm fibrotic cut surface . MicroscAngiofibroma is a fibrovascular tumor of nasopharynx seen in young adolescent males accounting for <0.5% of the head and neck tumors  with spoExtra-nasopharyngeal angiofibromas are common in females with older age of presentation compared to the nasopharyngeal angiofibromas . LiteratCurrently, the most accepted theories of its development include testosterone dependent vascular malformations arising from discontinuous vascular basal laminae having focal lack of pericytes, with the irregularity of smooth muscle layers, developing from the persistent fibrovascular nidus in and around the nasopharynx with molecular evidence favoring these ,16-18. As these nidus are also present in the various junction of skull bones and parapharynx and the lateral side of pterygoids and parapharynx, it is postulated that parapharyngeal angiofibromas and extra nasopharyngeal angiofibromas might also be arising by such ways just as nasopharyngeal angiofibromas, however clear evidence for or against any argument regarding the development of extra nasopharyngeal angiofibromas are lacking in literature , becauseSurgical excision is the preferred treatment for angiofibromas, with radiotherapy being considered only for unresectable cases. Extra-nasopharyngeal angiofibromas have a better postoperative prognosis due to the difficulties in surgical exposure and technical expertise involved in surgical excisions of nasopharyngeal angiofibromas . Highly In the present case, the surgical excision was complete and postoperative diagnosis was a surprise. Parapharyngeal angiofibroma is a rare diagnosis. However, the knowledge of its possibility and suspicion helps the surgeon prevent inadvertent complications.


Mucosal Schwann cell hamartoma (MSCH) is a rare benign neurogenic tumor characterized by pure S100p positive spindle cell proliferation. Most cases occur in the distal colon. Involvement of the gall bladder is exceedingly rare. There have been no reports of recurrence or a syndromic association with MSCH. Herein, we describe a case of MSCH of the gallbladder in a 55-year-old female patient with prior history of gastrointestinal neurofibromas who presented with abdominal pain. MR imaging revealed choledocholithiasis, gallbladder thickening, and marked biliary and pancreatic ductal dilation. The patient subsequently underwent cholecystectomy with choledochoduodenostomy. Histologic evaluation of the gallbladder showed diffuse expansion of the mucosa with S100p positive cells with spindly nuclei and indistinct cytoplasmic borders and diagnosis of MSCH of the gallbladder was rendered. Laboratory investigations revealed elevated total bilirubin  and alkaline phosphatase . Abdominal ultrasonography revealed elongated nondistended gallbladder with mild wall thickening, trace pericholecystic fluid, and dependent layering sludge. No gallstones were identified. Mild intrahepatic and moderate extrahepatic biliary ductal dilatation with the common bile duct measuring up to 1.9 cm was noted. No obstructing choledocholith or mass was identified. Subsequently, multisequence and multiplanar MR imaging was obtained through the abdomen before and following the administration of gadolinium contrast material which showed, choledocholithiasis with associated marked biliary and mild pancreatic ductal dilatation, mild peribiliary hyperenhancement, and marked gallbladder wall distention with wall thickening. The hepatobiliary surgery team was consulted for surgical evaluation and management. The patient underwent cholecystectomy with choledochoduodenostomy. The gallbladder was sent for routine histologic evaluation.Grossly the gallbladder was intact with a wall thickness ranging from 0.4-0.6 cm. The serosa was pink-tan smooth, with a roughened hepatic bed. Mucosa was tan-gray and hemorrhagic. No gallstones were identified. Histologic evaluation showed diffuse bulbous expansion of the mucosa with cells with spindly nuclei and indistinct cytoplasmic margins -D.The prWe searched the PubMed and Scopus databases from their inception  to August 2021, using the keywords “Mucosal Schwann cell hamartoma of Gall Bladder”, “Gall bladder MSCH” and “Non-colonic MSCH of gastrointestinal tract”. This was followed by a manual search of the included references. Only two articles with confirmed involvement of gall bladder by MSCH were identified.,1,7,Gibson et al.,5,Among the reported cases of gallbladder MSCH, no syndromic association or recurrence have been documented.3The exact etiology and precise pathogenesis of MSCH remain unclear. It is postulated that mechanical obstruction and chronic inflammation lead to the proliferation of the LGR5-positive intestinal stem cells and their plasticity may contribute to the hypertrophy of the nerve fibers in the gallbladder and gastrointestinal tract, eventually developing into an MSCH.9,The histological differential diagnosis for MSCH includes neurofibroma, schwannoma, perineurioma, and ganglioneuroma. Neurofibromas are usually associated with neurofibromatosis and present as a mural mass.1Both schwannomas and Schwann cell hamartomas are composed of a pure population of Schwann cells, however, schwannomas usually show circumscription, perilesional lymphoid aggregates, collagenous stroma and nuclear heterogeneity whereas MSCH lack these features.10-1,10Perineuriomas are benign nerve sheath tumors composed of perineurial cells. Like MSCH, mucosal perineuriomas are usually identified as polyps on routine or screening colonoscopy and they arise frequently in the left colon, particularly in the rectosigmoid.1Ganglioneuromas are composed of a mixed population of S100p-positive Schwann cells, NFP–positive axons, and ganglion cells. The latter two components are absent in the mucosal Schwann cell hamartomas.10Mucosal Schwann cell hamartomas of the gallbladder are extremely rare. There are no confirmed reports of gallbladder MSCH in patients with neurofibromatosis. Although MSCH does not have a syndromic association, the natural history of this lesion in patients with neurofibromas or clinically suspected neurofibromatosis is not yet elucidated.


Median raphe cysts are uncommon benign cysts thought to occur due to improper fusion of the genital tubercle and can occur anywhere along the median raphe, from the glans to the anus, most commonly occurring along the ventral penile shaft. Limited information is available in the literature about the common imaging features of median raphe cysts with available reports highlighting an avascular cystic lesion. Our case demonstrates a 10-year-old male patient presenting with a ventral penile mass that demonstrated interval growth in the absence of trauma without overlying skin changes. Doppler ultrasound examination demonstrated a solid vascular mass measuring up to 1.6 cm at the ventral aspect of the penis with arterial and venous waveforms. The patient underwent elective resection of the mass which revealed a 2.0 cm inflamed glandular subtype median raphe cyst. This report demonstrates an atypical imaging presentation of an inflamed median raphe cyst, particularly that of a heterogeneous solid mass with arterial and venous blood flow on ultrasound. Median raphe cysts (MRC) are rare benign cysts known to occur along the median raphe, which extends from the urethral meatus and continues caudally along the scrotum down to the perineum. They are thought to arise due to abnormal fusion of the genital tubercle during the fourth week of fetal development; however, various theories exist in the literature with the exact pathogenesis uncertain . ApproxiMRCs are often misdiagnosed as more common entities such as Cowper gland cysts, dermoid cysts, steatocystomas, or neoplasms depending on the location . The diaA 10-year-old male with no significant past medical history presented to the emergency department with isolated penile shaft swelling and minimal pain without infectious symptoms, difficulty urinating or hematuria. The patient denied any trauma to the area of concern and was discharged with instructions to follow up with his primary care physician (PCP). An assessment by the PCP on the next day showed the swelling had decreased, and the patient was sent home with hydroxyzine for pruritus and irritation. At the one week follow up with the PCP, the patient displayed continued decrease in edema with a firm palpable mass at the distal ventral aspect of the penis. The patient also reluctantly confirmed that the mass had been present for as long as he could remember, however, he added that it had recently enlarged.Penile ultrasound was then performed which exhibited a heterogeneous solid mass measuring 1.4 × 1.0 × 1.6 cm arising from the subcutaneous tissue of the distal ventral shaft and appeared separate from the left corpus cavernosum and urethra . Color DFive days later, the patient was taken to the operating room where a 2 cm mass was identified. The mass was very adherent to the spongiosum and right corporal body on the ventral aspect below the coronal sulcus. The mass appeared cystic in nature and when entered, thick brown/yellow material was encountered. Cultures displayed no growth however many polymorphonuclear leukocytes and red blood cells were seen. Frozen sections demonstrated a benign cystic lesion lined with mucin-producing cuboidal epithelium and urothelium consistent with an inflamed glandular type median raphe cyst with focal rupture . SubsequOur case is a novel presentation of a median raphe cyst masquerading as a large vascular penile shaft mass. Typical imaging features of MRCs in the literature are those of an avascular cystic lesion on ultrasound –6. Our cGiven the lack of infectious symptoms or overlying signs of cellulitis, consideration of an infected MRC was not immediately entertained. When an MRC is infected, the patient may present with pain, overlying erythema, or exuding pus typically occurring post coitum . In addiOur case also demonstrates the limitations of lesion characterization on ultrasound. Typical cystic penile lesions are seen on ultrasound as avascular anechoic structures and may have internal echoes if complicated by debris such as protein . AcoustiWe present a novel case of a ruptured glandular subtype median raphe cyst in a 10-year-old with recent enlargement prior to presentation. A ruptured MRC should be considered in the differential when a solid vascular subcutaneous mass is noted along the median raphe without communication with adjacent structures and lack of clinical infectious findings. A ruptured MRC may present as a relatively large lesion (>1.5 cm) with arterial and venous flow as demonstrated in our case.


Lymphangioma of the mesentery is a rare benign condition. Lymphangioma usually occurs in children during first few years of life most likely because of congenital abnormality of the lymphatic system. It may also be caused by trauma, lymphatic obstruction, surgery, inflammatory process, or radiotherapy. Lymphangioma of the mesentery represents less than 1% of all lymphangiomas and about 70% of abdominal lymphangiomas.We report the case of the 42-year-old woman who was diagnosed with the lymphangioma. Laparotomy was performed. A cystic lymph-filled tumor of about 12 cm in diameter was removed from the ileum mesentery. Lymphangioma of the mesentery is a rare condition. Despite its benign nature, it can cause serious complications if not treated. Ultrasound and CT are used for detection of lymphangioma. It is important to surgically remove the lymphangiomas even in the absence of symptoms. Lymphangioma is a benign tumor that usually occurs in children within the first few years of life. It most commonly occurs in the head, neck, and axilla, and these locations carry 95% of all lymphangiomas. The remaining 5% occur in the lungs, mediastinum, adrenal glands, kidney, and bone. Less common places are mesentery, gastrointestinal tract, retroperitoneum, spleen, liver, and pancreas . ChildhoThe 42-year-old woman sought medical attention for periodically occurring moderately severe pain in the lower abdomen. Intermittent lower abdominal pain occurred 2 years ago for the first time. She was investigated in the emergency department a few times. The pain gradually increased over the time. Physical examination revealed that the patient was well and no physical changes were identified. CT scan revealed a clearly defined, hypodensic, cystic, without clear solid components mass, about 11 cm × 10 cm × 6,5 cm, ventrally reaching the right side of abdomen wall, dorsally contacting abdominal aorta, inferior vena cava and right common iliac artery, caudally lying on the ileocolic artery and vein . The masAfter multidisciplinary team discussion the decision was made to proceed with the surgical treatment. Midline laparotomy was performed. A cystic lymph-filled tumor about 12 cm in diameter was detected in the abdominal cavity within the mesentery . The tumOn pathological examination, the macroscopically observed partially opened fatty-fibrous cystic formation 9 cm × 5 cm × 3 cm was identified. Cysts of up to 2 cm filled with whitish, turbid substance were visible. 6 lymph nodes up to 0.5 cm were excised alongside with the lymphangioma. Histologically thin-walled cystic tumor structures were separated by areas of adipose tissue with lymphoid aggregates . The cavLymphangioma is a benign condition that results from the proliferation of lymphatic cavities with thinned walls . About 9The etiology of lymphangioma is not entirely clear. They are thought to result from a congenital lymphatic system abnormality that causes sequestration of the lymphoid tissue during embryonic development. This theory would substantiate why lymphangiomas are most commonly seen in children. Also, there are theories that secondary lymphangioma formation is provoked by abdominal trauma, lymphatic obstruction, surgery, inflammatory process, or radiotherapy  3]..3].There are three types of lymphangiomas: simple (capillary), cavernous, and cystic. Simple lymphangioma occurs in the superficial layer of the skin and is made of small thin-walled lymphatic spaces. Cavernous lymphangioma consists of dilatated lymphatic vessels and lymphoid stroma and connects with spaces of adjacent normal lymphatics. Cystic lymphangioma consists of lymphatic spaces of various sizes that contains smooth muscle fibers and collagen in the stroma but have no connections to adjacent normal lymphatic spaces. The cystic type cannot always be clearly distinguished from the cavernous type because a cystic lymphangioma may have cavernous areas .Bowel mesentery lymphangioma is usually asymptomatic until it increases in size sufficiently. The most common symptoms are abdominal pain and increased abdominal volume. However, clinical manifestation may vary. Although mesenteric lymphangioma is a benign tumor, its size and location may lead to morbidity when other adjacent organs and structures are affected. Complications such as secondary infection, rupture with bleeding, and intestinal obstruction may occur .Ultrasound examination of mesenteric lymphangioma usually reveals a cystic multiseptated mass. CT scan shows uni- or multilocular mass with a contrast-accumulating capsule and septa . These sMesenteric lymphangiomas may grow to a very large size and invade the surrounding cavities and organs. The treatment is to surgically remove lymphangioma, even if no symptoms have occurred yet. This treatment may be complicated due the penetration of lymphangioma to other structures such as the main mesenteric artery or intestine. In that case segmental bowel resection may be performed. Sometimes radical resections may not be possible .The small bowel mesentery lymphangioma is a rare condition that can cause serious complications. Lymphangioma of any kind occurs most often in children because of congenital anomalies of the lymphatic system. Cases in adults represent only 10–20%. They can be detected by the ultrasound or CT scans and confirmed via aspiration of milk-like fluid or histological findings. Although lymphangiomas may be asymptomatic, if detected, they have to be surgically removed.


Endotracheal hamartomas are rarely encountered in children. The symptoms of endotracheal hamartoma may include cough, dyspnea, hemoptysis, chest pain, purulent sputum, and fever. The non-specific symptoms often result in a delayed diagnosis. Among the various treatments of this rare disease, surgical resection seems to be the most widely used, while endoscopic treatment is rarely described. Herein, we describe the case of a 9-year-old boy with an endotracheal hamartoma that was successfully excised by electrosurgical snaring via fiberoptic bronchoscopy (FB). The resection of select benign endotracheal tumors in children can be conducted using electrocautery, which can be regarded as an alternative therapy to bronchotomy. Benign lung tumors represent <1% of all lung tumors; among these, hamartomas have an incidence of 0.025–0.32% in the adult population . AlthougA 9-year-old boy was referred to our hospital with a 20-day history of cough, stridor, and shortness of breath. He had showed a poor clinical response to conventional therapy with antibiotics and systemic steroids at a local hospital. He was previously healthy with no relevant family history. A computed tomography (CT) scan performed at a local hospital revealed a homogeneous mass obstructing the trachea. During preparation for rigid bronchoscopy (RB) at the local hospital, he was found to be intolerant to general anesthesia and his dyspnea exacerbated. He was transferred to our hospital immediately. On admission to our hospital, the patient appeared dyspneic and had a characteristic biphasic stridor, with a percutaneous oxygen saturation of 93% under mask oxygen inhalation, body temperature of 37.7°C, pulse rate of 162 beats/min, respiration rate of 34 breaths/min, and blood pressure of 127/74 mmHg.On day 2, contrast-enhanced multidetector CT revealed a mass lesion with a fat density that was nearly totally obstructing the tracheal lumen . The mulThe mass resection was performed on day 3. Pulmonologists, otolaryngologists, thoracic surgeons, anesthesiologists, and pediatric intensive care unit doctors were all in the operating room to ensure the success of the treatment and the safety of the patient. General anesthesia was induced with 8% sevoflurane. After the intravenous administration of 2 mg of midazolam, a laryngeal mask airway (LMA) was inserted to maintain an open airway. Intraoperatively, anesthesia was maintained with 3% sevoflurane. Based on the depth of anesthesia, 10 mg of pentazocine and 50 mg of propofol were intravenously administered intraoperatively. A swivel adapter was used to connect the proximal end of the laryngeal mask to the T-piece anesthesia system. A flexible fiberoptic bronchoscope  operated by an experienced pulmonologist was inserted via the swivel adapter. A large mass covered by smooth mucosa with a stalk was obstructing approximately 85% of the trachea in the mid-tracheal region . A flexiPrimary endobronchial and endotracheal tumors are uncommon in childhood. Of these, benign tumors consist of inflammatory pseudotumors (plasma cell granulomas), papillomas, hamartomas, mucus gland tumors, leiomyomas, and hemangiomas . MalignaHamartomas in the lung are slow and abnormal growing neoplasms that are defined as a tumor-like malformation in which there is abnormal mixing of the normal components of the organ. In other words, a pulmonary hamartoma is a rare benign tumor that originates from the bronchial primitive mesenchymal tissue and can differentiate into various mature mesenchymal components . The hisPatients with endotracheal hamartomas are generally asymptomatic, at least in the preocclusive early phase. The presenting symptoms including cough, dyspnea, hemoptysis, chest pain, purulent sputum, and fever are induced by tracheobronchial obstruction and can lead to recurrent pneumonia . As the Surgical removal of the tumor in older patients should be done only when the tumor is symptomatic or if malignant transformation is suspected. In contrast to adults, peripheral and central hamartomas in children can cause marked symptoms and require prompt surgical removal. The traditional treatment is pneumonectomy or thoracotomy combined with bronchial resection in children . HoweverRigid bronchoscopy is probably the best conduit for managing endotracheal lesions, although bronchoscopy via a LMA is a viable option. However, it is important to note that the parents of pediatric patients tend to choose FB. Because the patient could not tolerate anesthesia at the local hospital, he was unable to undergo RB there. Therefore, we chose to use bronchoscopy via a LMA rather than RB. The advantage of LMA general anesthesia is that it enables control of the patient's breathing and provides a large space for endoscopic operation. If it had been difficult to remove the tumor under FB, we would have attempted mechanical debulking under RB. If the patient had not been able to tolerate laryngeal mask anesthesia, we would have attempted to resect the mass via FB under tracheal intubation anesthesia. If we had been unable to remove the mass via FB under tracheal intubation anesthesia, the thoracic surgeon would have performed an open thoracotomy. The extracorporeal membrane oxygenation team was ready to provide emergency treatment if the vital signs became unstable. The management of pulmonary hamartoma might be highly center- and patient-dependent. There is no gold standard due to the rare nature of the lesion and lack of uniformity in expertise among centers in managing such cases. Therefore, a large prospective clinical trial is needed.The electrocautery snare has the ability to sever a large mass quickly with concomitant hemostasis. The advantages of electrosurgery via FB are the provision of larger tissue specimens for more accurate histological diagnosis, recanalization of the involved airway, better assessment of the tumor base, more accurate surgical planning, stabilization of the patient to enable them to undergo surgery in a better general condition, and minimization of the bleeding risk by simultaneous cauterization of the base of the tumor during resection. Despite the partial residual tissue , our patAs hamartoma is a benign neoplasm, endoscopic treatment is now widely recommended as the first-line approach. Theoretically, hamartoma can be managed endoscopically in all age groups. However, the diameter of the electrosurgical snare and the working channel of the flexible bronchoscope limit the wide application of this technique. The diameter of the flexible electrocautery snare is 1.9 mm, which can pass through the working channel of the flexible bronchoscope BF-P260 . As the smallest flexible bronchoscope with a 2.0-mm working channel, our experience suggests that the Olympus P260 can be used in children older than 2 years. Thus, electrosurgical snaring might be useful for endoscopically managing tumors in children ≥2 years.The common complications of electrosurgical snaring include bleeding, perforation, and endotracheal burns . The maiAlthough interventional bronchoscopy is a mature technique in adults, it is seldom used in children. However, our case suggests that under careful preparation, interventional bronchoscopy can be safe and beneficial in pediatric patients.Endotracheal hamartoma is a rare benign tumor, and bronchoscopic therapy is recommended as the first-line treatment, although surgical resection may be necessary under certain conditions. Clinicians should consider the presence of tracheal lesions such as hamartomas in pediatric patients who obtain no significant improvement of respiratory symptoms after conventional treatment.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants  provided written informed consent to participate in this study.ZC supervised research work. LW and SL participated in collecting information of this case and searched for literatures. All authors participated in the interpretation of the data, contributed to the article, and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Fibrous uterine polyps are very common in women during or after menopause, and less often seen in women of child-bearing age, with a maximum of frequency between 40, and 49 years. They can have various locations in the uterus depending on the patient's age, mostly the body, and fundus. We report a rare case of cervical localization of a pedicled fibrous polyp issuing from the cervix, in a 44-year-old female patient with 3 living children and a history of miscarriage, who had been presenting breakthrough bleeding, and pelvic pain for 3 months. It is essential to remind young radiologists of the different presentations of fibrous polyps, how to explore them better and when to fear malignancy and thus insist in a histologic study, in order to help clinicians to choose the most adequate treatment option. Fibrous uterine polyps are very common in women during or after menopause, and less often seen in women of child-bearing age, with a maximum of frequency between 40, and 49 years We report the case of a 44-year-old female patient with 3 living children and a history of miscarriage, who had been presenting breakthrough bleeding, and pelvic pain for 3 months.Speculum examination showed a prolapsed mass that bleeded at contact. Cervix was not visible.The patient was then addressed to our structure, first, for a suprapubic pelvic ultrasonography, which showed a voluminous cervical mass of heterogenous echopattern, and Doppler signal .Fig. 1MeShe was later on addressed for a complimentary pelvic MRI, which showed a voluminous endometrial polyp issuing from the uterine cervix  in hyposThe patient was consequently operated on in the Gynecology and Obstetrical structure of our hospital, with a total excision of the mass , and simHistologic findings provided us with the final diagnosis of a fibrous polyp of the uterine cervix .Fig. 4LoEndometrial fibrous polyps are a frequent gynecologic condition that can cause abnormal uterine bleeding, pelvic pain or infertility, or remain silent, and get discovered by chance at the occasion of a pelvic imaging examination in an unrelated context. This condition is due to an abnormal, generally localized, endometrial intrauterine overgrowth that can be single or multiple, and may be sessile or pedunculated The reported prevalence of endometrial polyp varied widely between 7.8% and 34.9%, depending on the study, and the definition of a polyp. It appears to be more frequent as one advances in age, especially in post-menopausal women ; however, there might be a selection bias since postmenopausal vaginal bleeding is more likely to be investigated The most common symptoms associated with this condition are abnormal uterine bleeding, but also menorrhagia, irregular menses, postcoital bleeding , intermenstrual bleeding, or infertility, although they can remain asymptomatic for years Microscopically, endometrial polyps are typically a mixture of dense fibrous tissue (stroma), large and thick-walled vascular channels, and glandular spaces of varying shapes and sized, covered by a surface epithelium ,3.Polyps issuing from the uterine cervix represent 23% of all their locations on the uterus As for diagnostic imaging procedures, many are contributive in exploring uterine fibromas of usual locations.The first one is, without surprise, pelvic ultrasonography. It generally shows a hyperechoic lesion with regular contours, within the uterine lumen, outlining the endometrial walls on which it rests, surrounded by a thin hyperechoic halo. Doppler mode can be very beneficial, especially when it is actually able to show the feeding artery ,4.If the lesion's size is too small to be correctly assessed with suprapubic sonography, one can consider the using the transvaginal way. Some authors even say it can be equivalent to hysteroscopy in this indication Another imaging feature used to assess such lesions is sonohysterography, with the use of saline infusion in order to better outline the endometrial cavity, and thus, the contours, size, and location of endometrial polyps However, in the particular case of polyps issuing from the uterine cervix, such imaging modalities are hardly useful because of the difficult access to the lesion. That is why, in this particular case, pelvic MRI can be very helpful.A standard MRI protocol for exploring the pelvis in women with such pathologies constitutes in sagittal, oblique axial and oblique coronal slices in T1 and T2 weighting as well as Diffusion and contrast sequences. It is important to note that Diffusion sequences play an important role in differential diagnosis between endometrial cancer, polyp, hyperplasia, and physiological thickening Polyps appear essentially as large lesions filling the endometrial cavity or, in our case, issuing from the cervix, with variable T1 and/or T2 signal and contrast enhancement, isointense with the myometrium in Diffusion sequences with high ADC values As for histologic diagnosis, the best option available is hysteroscopy with guided biopsy as it allows us to visualize and remove polyps concurrently. Advances in technology permits specialists to even remove them under direct vision, in some cases This brings us to briefly present the different management options, which go from observation to conservative surgical, and radical surgical procedures. The choice of treatment will be based on the importance of the symptoms and their effects on the patient's daily life but also other factors such as malignancy risk, fertility issues, and operator's skills. Clinical outcomes after treatment of endometrial polyps are generally good, with a significant lessening of symptoms such as intermenstrual bleeding Adenomatous uterine polyps are a quite common condition in women, with cervical pedunculated ones being rarer, and of quite a peculiar presentation. Radiologists should know about their different presentations, how to explore them better and when to fear malignancy and thus insist in a histologic study, in order to help clinicians to choose the most adequate treatment option. Our case's lies in its striking iconography and its positive follow-up.Written and informed consent for publication of the case was obtained from the patient.


In children and adolescents presenting with skull base sarcoma, treatment strategies will face challenging decisions due to the unique chemoresistant pathologies, limitations imposed by the not‐yet fully mature anatomical structures, and the small surgical site. In children and adolescents presenting with skull base sarcoma, treatment strategies will face challenging decisions due to the unique chemoresistant pathologies, limitations imposed by the not‐yet fully mature anatomical structures, and the small surgical site. Long‐term survival is achievable in these rare conditions even after multiple recurrences, but success demands a multidisciplinary team.Head and neck sarcomas comprise less than 1% of all head and neck malignancies.Sarcomas constitute a heterogeneous group of malignancies that vary extensively by anatomic location, histology, and biologic behavior. A multidisciplinary approach including surgery, radiotherapy, and, in chemosensitive histopathologies, chemotherapy is necessary. Chondrosarcoma is a neoplastic process associated with cartilage matrix production lacking osteoid, a characteristic of osteosarcoma. Chondrosarcoma may arise at any age but typically occurs in middle‐aged and older adults between the age of 20 and 60 years old, and it is more common in men. It can also develop from malignant transformation in benign cartilaginous lesions such as enchondroma or osteochondroma.Surgical excision is the main treatment option for chondrosarcoma. For low‐grade tumors, which constitute the majority of these tumors, surgical resection alone is sufficient to achieve a high rate of disease control. For low‐grade central tumors, intralesional excision or curettage can be combined with local adjuvant chemical treatment, cryotherapy, or radiation. These approaches often result in good local control.Skull base tumors in children and adolescents impose greater therapeutic challenges. Although many treatment options for such tumors in adults have been suggested, relatively little is reported with regards to therapies in the pediatric population. The unique pathological composition, the limitations imposed by the maturing skull and brain, and the small size of the patients, make it more challenging to select the best approach.2The patient first presented to the surgery clinic in 2010 at the age of 6 with a chief complaint of an exacerbation of left eye proptosis, which had been previously progressing slowly. Brain magnetic resonance imaging (MRI) revealed a tumoral lesion in the infratemporal skull base and maxillary sinus, with sphenoid spread and optic nerve compression. Endoscopic transnasal resection of tumor, left orbital decompression and infratemporal space evacuation was performed. The tumor grossly appeared chondroid and some degree of clivus bone destruction was identified intraoperatively. A pathologic examination demonstrated low‐grade chondrosarcoma, and the patient was discharged with follow‐up recommendation  four months later, revealed recurrence in the right maxillary sinus and endoscopic surgery was planned. Ophthalmologic consultation before surgery demonstrated left eye visual acuity of 1/10 and right eye visual acuity of 10/10.Resection of the tumoral tissue located in the floor, medial, and posterior walls of the maxillary sinus was accomplished with negative margins via endoscopic approach, but complete resection of the superior aspect was not feasible.One year later, following another recurrence, MRI revealed tumor presence in the sinonasal area and skull base without involvement of the brain parenchyma. Endoscopic resection of maxillary sinus and skull base was done. The right optic nerve was involved by the tumor and a resection of the medial and inferior orbital wall was also required.Over the next 2 years, two more recurrences occurred along with some erosion of the clivus bone, which resulted in the patient's blindness due to optic nerve atrophy. Both recurrences were treated with double surgical resections.After 9 recurrences during a period of 10 years, another follow‐up imaging displayed abnormal heterogeneous foci on the left temporal lobe, left retro orbital area, and left parasellar region, with extension to nasal cavity. Contrast‐enhanced imaging demonstrated a 70 × 60 mm multiloculated mass. The case was discussed in the neurosurgery multidisciplinary tumor board and tumor debulking plus radiotherapy was recommended. The patient did not follow the last recommendation and returned to the clinic after 19 months, with massive tumor progression which was referred to a neurosurgeon and was announced unresectable due to fatal surgical risk. He was then referred for palliative radiotherapy which consisted of 20 fractions of 250 (centigray) cGy per fraction .Due to the patient's excessive suffering from ocular edema, deformity, and eye discharge, right ocular evisceration was performed after completion of radiotherapy. Similar problems in the left eye gradually regressed after treatment.Currently, the patient is under follow‐up and the tumor, despite being bulky and locally advanced, has been in a stable status for 6 months Figure . Both ey3Malignant tumors of the skull base are rare and pathologically diverse neoplasms. These tumors may arise from the bony skull base or from the intracranial compartments, but more frequently, they arise from the subcranial tissues.Although there are a limited number of studies on skull base sarcomas, it is reported that among patients with sarcoma, those with tumors located in the skull base region have the lowest overall survival (OS) rates.In an international collaborative study in 2007, Gil et al. evaluated 146 patients with anterior skull base sarcoma in terms of clinical manifestations and prognostic factors. Orbital involvement, involvement of the orbital wall, and intracranial extension were reported in 53%, 46%, and 28% of the patients, respectively. Tumor grade and adjuvant radiotherapy were not significant predictors of survival. Prior radiotherapy, intraorbital extension, positive margins, and postoperative complications were significant predictors of reduced disease‐specific survival on univariate analysis. The presence of positive or close margins was the only independent predictor of poor overall, recurrence‐free, and disease‐specific survival on multivariate analysis. The authors concluded that wide craniofacial resection with negative margins is an important predictor of better treatment outcome.p = 0.014). They also concluded that resection margin status was an independent predictive factor for local recurrence after radical skull base surgery, and conservative surgery is only acceptable in chemosensitive subtypes.In a recent study with similar end‐points, Kobayashi et al., retrospectively reviewed 22 sarcomas with skull base invasion, and reported that cases whose surgical margins were classified as “wide margin positive” had significantly worse 3‐year local recurrence rate than did patients with “margin negative” or “micro margin positive” status (25% vs. 83%, Mandonnet et al., retrospectively reviewed 42 patients younger than 19 who underwent surgery for a skull base tumor between 1992 and 2002, and analyzed the disease spectrum and outcomes. The median age was 13.75 years, and twenty‐two patients harbored a malignant tumor. Gross total resection was achieved in 78% of cases and local recurrence rate was 47%. With the median follow‐up being 63 months, 14% of patients had a neurological deficit and mortality rate was 30%. Middle cranial base tumors resulted in poorer prognosis.Chondrosarcoma is a neoplastic process associated with cartilage matrix production and may arise in different locations of the body. Patients typically present with localized pain in the affected bone with or without involvement of the adjacent soft tissue in the form of a palpable mass or tissue swelling.Type of treatment center and access to health care facilities have a significant impact on mortality and survival for skull base chondrosarcoma. In a review of the National Cancer Database of the United States published in 2021, data analysis demonstrated that higher education and treatment at a high‐volume academic facility were associated with decreased 90‐day mortality for patients with skull base chordoma and chondrosarcoma. The authors stated that academic approach, radiotherapy, escalated doses, and proton radiation facilities were more likely to be available at high‐volume centers.Despite the advances in imaging and diagnostic techniques, surgical techniques that incorporate oncologic principles, conformal radiation facilities, potential targeted systemic therapies, and early access to coordinated multidisciplinary team, extensive skull base chondrosarcoma, especially in cases of intracranial extension portends worse prognosis and lower survival rates.4In children and adolescents presenting with skull base sarcoma, treatment decisions are challenging due to the unique and frequently chemoresistant pathologies, treatment limitations imposed by the immature anatomical structures, and the small size of the patients. Chondrosarcoma may arise at any age but is rare in children and adolescents. It is important to manage these rare diseases through a multidisciplinary team and with a perspective toward the future recurrences and available options. Long term survival is achievable even after multiple recurrences and surgeries, when approached via a multidisciplinary team.Authors declare that they have no conflicts of interest.M.A., M.B., and S.A.J contributed in conception, design and drafting of the manuscript. P.P, M.D., and S.M.H. contributed in data collection. S.M.H., M.N., and J.S.W contributed in drafting of the manuscript. S.A.J. supervised the study. All authors approved the final version for submission.The study was approved by Sabzevar University of Medical Sciences. The study conforms to recognized standards is of Declaration of Helsinki. An informed written consent form was obtained from patient and his guardian and he signed informed consent regarding publishing his data and photographs anonymous.We would like to thank the patient and his guardian for his consent for publication of his data.


Accessory scrotum (AS) is rarely diagnosed antenatally, and its prenatal features remain unknown. Here, we report a case of a prenatally diagnosed accessory scrotum with perineal lipoma. A 33-year-old woman was referred to our hospital at 35 weeks of gestation to evaluate a mass in the fetal perineal region. Prenatal ultrasonography showed a 2.0 × 2.0 cm sized, echogenic, and circular mass located posterior to the left scrotum in a male fetus. Magnetic resonance imaging (MRI) showed a mass containing adipose tissue. A 6.5 cm elastic mass (AS and protruding lipoma) was observed in the perineal region, and surgical excision was performed at 8 months of age. Histological examination confirmed the diagnosis of AS with perineal lipoma, and there was no recurrence at follow-up. The typical prenatal presentation of AS was a circular perineal mass located posterior to the normal scrotum and was associated with perineal lipoma. The prenatal detection of AS was feasible with careful observation via ultrasonography, and prenatal MRI was useful in characterizing perineal tumors and evaluating associated anomalies. Accessory scrotum (AS) is a rare developmental anomaly in the perineal region, with only 53 cases reported to date A 33-year-old woman was referred to our hospital at 35 weeks of gestation to evaluate a mass in the fetal perineal region, which was first detected at 34 weeks of gestation at a primary care clinic. The pregnancy was uncomplicated, apart from mild iron deficiency anemia. Prenatal ultrasonography (US) revealed a 2.0 × 2.0 cm sized, echogenic, concentric circular mass located posterior to the scrotum in a male fetus A–B. The Upon physical examination after birth, his penis showed slight phallocampsis, and his scrotum appeared normal with two independent normal-sized testes. The median scrotal raphe was displaced towards the left side, as shown in Once normal anal function was confirmed, we resected the mass under general anesthesia at 8 months of age. The details of the procedures used are as follows: the patient was placed in the lithotomy position. A skin incision was made around the bottom of the lesion. The mass was resected along with the surrounding subcutaneous tissue without any injuries to the rectum or anus. We attempted to reconstruct the perineal raphe in the midline, which was displaced towards the right side by the mass, without excess tension C. An eleThe patient's postoperative course was uneventful, and there was no recurrence or abnormal bowel habits at 2 years of follow-up D.AS is a rare genital anomaly, defined as an additional scrotal-like epidermis, apart from a normally developed scrotum. AS does not contain testicular tissue and usually arises in the perineal region, typically posterior to the normal scrotum. Histologic evidence of smooth muscle fibers below the skin, compatible with tunica dartos, is diagnostic for AS Although there has been a significant advancement in prenatal screening, prenatal diagnosis of AS remains rare. A literature search using PubMed identified five prenatally diagnosed AS cases, including ours Typical prenatal US features of AS in the reported cases included round, lobulated, and sometimes pedunculated masses located posterior to the normal scrotum. The echogenicity of AS was similar to that of the normal scrotum: homogenous internal echo pattern reflecting adipose tissue, with slightly higher linear echogenicity on the surface layer, which may reflect the existence of the tunica dartos The differential diagnosis of a prenatally detected perineal mass includes lipoma, lipoblastoma, sacrococcygeal teratoma, infantile hemangioma, hamartoma, choristoma, and liposarcoma. Moreover, these lesions may be associated with anorectal and genital malformations, such as chorist Oma, labioscrotal fold, anal atresia, imperforate anus, inguinal hernia, and ambiguous genitalia ,10. AccoHerein, we report a rare case of AS with perineal lipoma. The literature review suggested that the prenatal diagnosis of AS remains challenging, but may be feasible with careful observation. Prenatal MRI was useful in characterizing perineal tumors and evaluating associated anomalies.Informed written consent was obtained from the patient for publication of the Case Report and all imaging studies. Consent form on record.HH, YN, ME and RP participated in the prenatal screening and care. KD, CT, MN, RS and YT participated in the postnatal care and the operation. KD, YT and MW drafted the manuscript. OH participated in its design and coordination and helped to draft the manuscript. All authors read and approved the final manuscript.All authors have made substantial contributions to all of the following: (A) the conception and design of the study, or acquisition of data, or analysis and interpretation of data, (B) drafting the article or revising it critically for important intellectual content, and (C) final approval of the version to be submitted.None.


Intraosseous mucoepidermoid carcinoma of the jaw is a rare lesion that has been suggested to originate from the odontogenic epithelium. We report an unusual case of central mucoepidermoid carcinoma in an 18‐year‐old Japanese man with an odontogenic cyst. This report describes the clinical and histologic features of this extremely rare case of central mucoepidermoid carcinoma admixed with a calcifying odontogenic cyst. Pathological examination revealed that the lesion was admixed with a calcifying odontogenic cyst. The odontogenic epithelium of the calcifying odontogenic cyst was highly likely to be the origin of this case. To the best of our knowledge, this is the first report of CMEC of the maxilla associated with a calcifying odontogenic cyst. We report the case of MEC arising from a rare location, with distinct radiographic findings, and discuss the etiology of the lesion with reference to the relevant literature.2An 18‐year‐old man was referred to our hospital in 2019 with a complaint of a painless mass in the left palate. The patient was aware of the swelling in his right palate for approximately 2 years. However, he noticed a recent and rapid increase in the swelling. Hence, the patient initially consulted a general dental practitioner, where a diagnosis of a tumor of the palate was established. The patient was subsequently referred to our department. His medical history revealed febrile seizures at the age of 1 year, and his family history was unremarkable. On initial assessment, no systemic symptoms were observed. An extra‐oral examination revealed facial symmetry. No swollen cervical lymph nodes were palpable bilaterally. Intraoral examination revealed a dome‐shaped mass measuring 4.0 × 3.0 cm on the left side of the hard palate, extending anteriorly to the distal first premolar area and posteriorly to the soft palate. The mucosal surface of the mass was smooth, but partially ulcerated Figure . ComputeIncisional biopsy of the palate revealed the epithelium comprising primarily squamous epithelial cells and the focal presence of clear cells and ghost cells along with the presence of calcifications, which suggested the possibility of benign odontogenic tumors or odontogenic cysts Figure . Based o3To the best of our knowledge, this is the first report of a central mucoepidermoid carcinoma (CMEC) of the maxilla arising from a calcifying odontogenic cyst.Mucoepidermoid carcinoma is the most common malignant salivary gland tumor, accounting for 20% of all malignant salivary gland tumors mainly observed in the parotid gland of the major salivary gland and around 30% observed in minor salivary glands, including the palate, lip, and retromolar area.In 2012, as a first large‐scale study, Zhou et al. reported that in 39 Chinese patients diagnosed with CMEC from 1985 to 2010, the lesion was more prevalent in women than in men, highly prevalent in the fourth to fifth decades of life, and more prevalent in the mandible than in the maxilla. Moreover, according to this study, the most common symptoms were asymptomatic swelling accompanied by the most common radiographic unilocular or multilocular and osteolytic radiolucent lesions. Furthermore, the most frequent histologic type was low‐grade CMEC with favorable prognosis.Although the origin of CMEC of the maxilla remains controversial, various theories have been considered.Because the clinical finding of CMEC of the maxilla may resemble benign lesions such as odontogenic cysts, tumors, or odontogenic infectious diseases, it may be difficult to establish a correct diagnosis initially. Our case report also showed a radiolucent cystic lesion with an impacted tooth, which is a typical radiographic finding of a benign odontogenic cyst or tumor. Therefore, initially, we did not consider the possibility of MEC. However, in our case report, the maxillary radiolucent lesion was accompanied by proliferative and destructive bone lesion in the hard palate and alveolar process. Considering these findings, CMEC or other central malignant tumors should have been considered as a differential diagnosis even if clinical and radiographic findings lacked the features of malignancy.In conclusion, we report an extremely rare case of CMEC of the maxilla arising from a calcifying odontogenic cyst in a young patient. Because information regarding the clinical characteristics of CMEC in young adults is limited, long‐term follow‐up would be desirable considering the possibility of late local recurrence and metastasis. Clinical findings of CMEC in the maxilla may cause confusion during the initial diagnosis considering that CMEC is similar to benign lesion, as in our case report. Therefore, caution should be exercised regarding CMEC in the maxilla regardless of patient's age to establish an appropriate early diagnosis and treatment even if the clinical findings are not suggestive of malignancy.The authors made no disclosures.KT, HT, and MN contributed to the manuscript preparation. MIM, HT, KS, SI, and KF contributed to the patient management. AN and JI are pathologists who contributed to the pathological diagnosis. All authors read and approved the final manuscript.Because this report involves no experiment, ethics approval is waived.A written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Ectopic parathyroid gland is not uncommon, and is associated with primary hyperparathyroidism. Giant parathyroid adenoma (PA) and concurrent presence of enlarged thyroid nodule increases the probability of ectopic location. The combination of a giant PA that is ectopic (within the strap muscle) in the neck is very rare, especially in cases with no previous surgery. The rare histopathological findings of the current case, water clear cell parathyroid adenoma (WCCPA), could explain the patient's presentation, since it has low endocrine function.99mTc-sestamibi scintigraphy identified a left PA. FNAC of the thyroid nodule showed that it was a colloid nodule. She underwent left hemithyroidectomy and excision of left PA. Intraoperatively, the PA was giant and in the sternohyoid muscle. Intraoperative monitoring of intact PTH (IOiPTH) confirmed successful excision.A 56-year-old Qatari female on routine visit to primary health care physician for hypertension, was incidentally discovered to be hypercalcemic and was referred to the emergency department of our institution. Neck ultrasound showed a thyroid nodule on the left side, but Ectopic giant parathyroid adenoma is rare especially with the intramuscular location in sternohyoid muscle in the neck without previous neck surgeries. The presence of thyroid nodule could be a precipitating factor for migration of the PA. Preoperative assessment with the radiological image is crucial for diagnosis but sometimes fail to localized the PA.Giant asymptomatic PA with long standing low function before hyperfunctioning should raise the suspicion of WCCPA. If diagnosis is confirmed, metastasis from a clear cell renal cell carcinoma should be ruled out. •We report a unique rare case of non-iatrogenic ectopic giant water clear cell parathyroid adenoma (WCCPA)•A parathyroid adenoma >3.5 gm, asymptomatic and with low function before hyperfunctioning, should raise suspicion of WCCPA.•History, examination, ultrasound, sesta-MIBI scintigraphy and immunohistochemical studies are important for diagnosis.•High preoperative parathyroid hormone and calcium need monitoring in post-operative period to avoid hypocalcemia. This can arise from abnormal migration during embryogenesis or is acquired. Enlarged glands can be displaced from their orthotopic locations due to their size, the influence of gravity, by mass effect from an associated enlarged thyroid; or can be iatrogenic from surgical auto-transplantation Parathyroid adenoma (PA) is part of a spectrum of parathyroid proliferative disorders that include parathyroid hyperplasia, adenoma, and carcinoma Most PA are solitary, small in size, and weigh <1 g EPA can occur in anatomical locations that may be missed, including thymus (17%), intrathyroidal (10%), undescended glands (8.6%), carotid sheath (3.6%), and retroesophageal space (3.2%) In 1994, the first case of a rare variant of PA, the water-clear cell parathyroid adenoma (WCCPA) was described We report a 56-year-old female with non-iatrogenic ectopic giant WCCPA in the sternohyoid muscle co-occurring with a thyroid colloid nodule and associated with asymptomatic hypercalcemia due to primary hyperparathyroidism. The patient was referred by her family physician to the accident and emergency department of our institution because of hypercalcemia that was incidentally discovered (she was never symptomatic). We report this case in line with the updated consensus-based surgical case report (SCARE) guidelines 2A 56-year-old Qatari female on a routine visit to her primary health care physician for hypertension was incidentally discovered to have hypercalcemia. The physician referred her to the accident and emergency department at our institution for the management of the asymptomatic hypercalcemia. The patient was seen by the medical team and admitted for investigations and management. Her medical background was significant for hypertension and respiratory compression symptoms, particularly when the patient lied down in lateral position. There was no previous history of neck surgery or irradiation, smoking or alcohol consumption. There was also no family history of thyroid disease or malignancy.99mTc-sestamibi scintigraphy, and the findings were consistent with left superior PA  in the upper neck region, not tender, and with no palpable lymph nodes. The systemic review was unremarkable. Her laboratory tests showed high calcium (3.13 mmol/L) and very high parathyroid hormone (PTH 1036 pg/mL) but normal kidney function, suggesting primary hyperparathyroidism. Liver function tests were normal. Ultrasound scan (US) of the neck showed two lesions  suggestierior PA . UltrasoA senior resident surgeon with assistance and direct supervision of a consultant surgeon performed the procedure. Since the patient had compression symptoms, left hemithyroidectomy with parathyroid adenoma excision were undertaken. Intraoperatively, the left PA was found to be ectopic being intramuscular within the strap muscles, and it was giant 9 gm, . IntraopUpon follow-up, the patient was seen at our Thyroid Surgery Clinic after a week. She was satisfied with the procedure and had no complaints, normal calcium level, and normal thyroid function. On her second visit to our clinic 3 weeks after her discharge, her thyroid function showed mild hypothyroidism , and vitamin D level was low (11.0 nmol/L). Hence, the patient was started on thyroxine 50 μg and ergocalciferol 50,000 IU weekly.Gross appearance of the surgical specimen demonstrated a PA  with solid tan homogeneous cut surface. The left thyroid lobe (4.5 × 3.5 × 2 cm) revealed a 1.5 × 1.5 cm well-circumscribed partially cystic solid lesion with focal calcified areas in the upper and middle pole. Microscopically , there wWCCPA is a rare cause of PHPT. Our literature review showed that only 20 cases have been reported in the literature . Histolo3We present a rare case of non-iatrogenic EGPA within the strap muscle (sternohyoid) co-occurring with left thyroid colloid nodule and associated with asymptomatic hypercalcemia due to PHPT. In most cases of PHPT due to solitary PA, the adenoma is usually small and weighs <1 gm In terms of presentation, PA presents classically with PHPT accompanied by recurrent kidney stones, and psychiatric, bone, and gastrointestinal symptoms GPA is not necessarily palpable or symptomatic. The weight of the gland correlates with its functionality and thus serum calcium levels In terms of calcium homeostasis, our patient had high serum calcium and PTH levels. Patients with high preoperative calcium require close postsurgical observation, as feedback effects cause the non-pathologic parathyroid glands to cease their normal function; and resection of the pathologic gland may result in transient hypocalcemia, due to a sudden decrease in calcium level As for investigations, clinical suspicion and laboratory findings followed by imaging studies can confirm the diagnosis. US of the neck is widely used to locate the pathological gland with 75% and 85% sensitivity and specificity respectively The definitive treatment of primary hyperparathyroidism associated with PA is surgical resection. Preoperative localization of the PA is important, and intraoperative quality control of the surgical excision based on intraoperative serum levels of IOiPTH is a gold standard 4We report a unique rare case of non-iatrogenic ectopic giant WCCPA. When a PA is giant (>3.5 gm), asymptomatic and with long standing low function before being hyperfunctioning, this should raise the suspicion of WCCPA. If the diagnosis is confirmed, metastasis from a clear cell renal cell carcinoma should be ruled out. Hence detailed history, physical examination, and investigations including ultrasound, sesta-MIBI scintigraphy and immunohistochemical studies are of paramount importance. Complete excision of the adenoma is curative with good outcomes. Where preoperative PTH and calcium are high, monitoring of calcium level in the early post-operative period is imperative to avoid hypocalcemia and its deleterious complications.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer-reviewed.Approved by Medical Research Center, Hamad Medical Corporation reference number (MRC-04-21-068).Nothing to declare.welansari9@gmail.com.Prof Dr. Walid El Ansari: Not first in Man, hence UIN not required.Walla Mohamed: data collection, data interpretation, writing the paper. Walid El Ansari: study concept, data interpretation, writing the paper. Mohamed S. Al Hassan: study concept, data interpretation, editing the paper. Rayan M Sibira: laboratory and histopathology, data interpretation, editing the paper. Abdelrahman Abusabeib: study concept, data interpretation, editing the paper. All authors read and approved the final version.All authors declare that they have no conflict of interest.


Cerebral cavernous malformations (CCMs) are dilated blood vessels which can develop sporadically or in familial form and are the commonest malformations of blood vessels in the spinal cord and brain. The familial form is an autosomal dominant gene mutation disorder. This condition can be diagnosed with magnetic resonance imaging (MRI) and computed tomography (CT) scan, but the modality of choice is MRI because of its high sensitivity. We report a case of a 73-year-old woman with an asymptomatic multiple familial cerebral cavernous malformation (FCCM) which was previously misdiagnosed as multiple cerebral metastases on CT scan. A brain MRI performed correctly diagnosed her condition as FCCM based on the typical MRI appearances. In order not to misdiagnose brain lesions like CCM on CT scan, for cerebral metastases in resource-poor settings, radiologists must recommend advanced imaging modalities like MRI for further evaluation, thereby avoiding unnecessary invasive surgical biopsies. Cerebral cavernous malformations (CCMs) are the most common malformations of blood vessels and can be seen in various parts of the brain and the spinal cord. CCMs are clusters of abnormal blood vessels that are enlarged and irregular in structure –3. Many CCMs are classified into two forms, namely, sporadic and familial forms. The sporadic form is often associated with a single isolated lesion whilst the familial form is associated with multiple lesions and mutations in three genes, namely, K-Rev Interaction Trapped 1 (KRIT1)/CCM1, CCM2, and Programmed Cell Death 10 (PCD10) also known as CCM3. Approximately 40-60% of familial cerebral cavernous malformations (FCCMs) are inherited in an autosomal dominant pattern due to a heterozygous mutation in one of the three genes , 9.Although CCM is often asymptomatic, people with this condition may present with clinical complaints such as seizures, headaches, deficits in neurological function, and cerebral hemorrhage . The incA 73-year-old woman was referred to the MRI unit of the department of radiology, Korle Bu Teaching Hospital, for brain MRI on account of suspected cerebral metastases. Prior to this, in 2019, she had an invasive moderately differentiated adenocarcinoma of the sigmoid colon and had sigmoid colon resection and end-to-end colorectal anastomosis. She underwent chemotherapy and was doing well, but yet to receive radiotherapy. She was not a known hypertensive and diabetic and had no history of amyloidosis. She is the only child of her deceased parents; the cause of death of her father was cerebrovascular accident. She has one child.As part of her metastatic workup, a brain CT scan was done at an outside hospital. The findings  were misThe typical MRI appearance and the high number of the lesions (approximately more than 70) were in keeping with multiple FCCMs. She was counseled on the associated chances of having spinal cord cavernomas, genetic inheritance of the condition, and the risk of cerebral hemorrhage among others. She therefore consented for MRI of the spine which did not reveal any associated cavernomas of the spinal cord. Her only son also underwent MRI of the brain and spinal cord, which were unremarkable. The genetic testing could not be done to determine mutation in any of the genes associated with FCCMs due to unavailability of the testing facility in the country.CCMs are the most common type of vascular lesions in the central nervous system, comprising 5%–15% of all vascular malformations. Prior to the use of MRI in diagnostic imaging, symptomatic lesions were more dominant among the 163 CCM cases reported in literature as at 1976, and with the availability of MRI, at least 40% of CCMs are identified incidentally , 12. OutFCCM syndrome is defined as the presence of multiple CCM  or when at least two family members have CCM or a mutation in one of the three genes causing FCCM . Our 73-In our patient, the larger lesions on T1W, T2W, and FLAIR showed hyperintense core and peripheral rim of hypointensity due to hemosiderin, and this suggests that there was a recent minimal bleeding but she remained asymptomatic. The lesions became more prominent on GRE/T2∗ and SWI due to signal loss and blooming artefacts, and these two sequences are crucial in detecting the cavernomas. MRI can also characterize cavernomas into four types, namely, type 1, 2, 3, and 4 lesions, as originally reported by Zabramski et al. . Type 1 From the descriptions of the types of lesions, it is obvious that our patient has all the 4 types. Some authors also described type 4 lesions as small malformations that can only be seen on GRE/T2∗ as hypointense foci and are thought to be capillary telangiectasias . It is aIt is also important to differentiate multiple cerebral cavernomas from amyloid angiopathy. On imaging, these have typical subcortical distribution and our patient's lesions do not show this type of distribution. Hypertensive microbleeds are another differential diagnosis, but the patient was not a known hypertensive and hypertensive bleeds have a predilection for basal ganglia region . HemorrhGenerally, 50% of patients with FCCMs remain asymptomatic throughout their entire lives as seen in our patient partly due to the slow course of FCCMs. Conservative management and symptomatic treatment are highly recommended for this condition. A surgical intervention is only recommended in patients with multiple lesions, hemorrhage, progressive neurological deficits, and seizures . Our patIn order not to misdiagnose brain lesions like cerebral cavernous malformations on CT scan for cerebral metastases, radiologists must recommend advanced imaging modalities like MRI for further evaluation.MRI is more sensitive in the detection of CCMs than brain CT scan. Hence, radiologists can use the typical MRI features to diagnose CCMs, thereby avoiding unnecessary invasive surgical biopsies and be aware of the mimics of multiple CCMs.


Internal hernias represent the penetration of intestinal loops through congenital or acquired openings within the mesentery or peritoneum. One such hernia is the paraduodenal hernia, which is the most common type of internal hernia (53%)  In this paper, the case of a 39-year-old patient with left paraduodenal hernia will be presented. He arrived in the emergency department with sudden onset of abdominal pain. The diagnosis was established using computed tomography and an open surgical procedure was successfully performed.Clinical presentation of paraduodenal hernia ranges from asymptomatic to manifest. The greatest difficulty regarding management of paraduodenal hernias lies in their diagnosis. Many studies have shown that the best option for diagnosis is computed tomography (CT). Open and laparoscopic techniques are used in treatment of paraduodenal hernias with similar results.The case of a 39-year-old male patient with LPDH with non-specific symptomatology was presented. CT scan is the best diagnostic option for this condition. Open surgical approach was used with great success. •Internal hernias represent the penetration of intestinal loops within the mesentery.•Most common internal hernias are paraduodenal hernias.•Best diagnostic tool for internal hernias is computed tomography.•Surgical options for internal hernias include open and laparoscopic approach. These dInternal hernias are rare and pose a significant diagnostic and therapeutic challenge for both radiologists and clinicians There are two types of paraduodenal hernias: left-sided  and right-sided  Treatment consists of surgery, either in the form of open surgery or laparoscopy. Laparoscopic surgery is preferred by experienced surgeons in high volume centers. Recovery after the laparoscopic procedure is faster, but long-term outcomes are similar for both methods 2There are numerous classifications of internal hernias, and one of the more acceptable ones is suggested by Welch, who divides internal hernias into 8 types: 1a: left paraduodenal hernia, 1b: right paraduodenal hernia, 2: foramen of Winslow hernia, 3: pericecal hernia; 4: sigmoid mesocolon-related hernia; 5: transmesenteric hernia; 6: transomental hernia; 7: supravesical and pelvic hernia. Doishita et al. classify internal hernias into 3 main groups according to the type of hernia opening  Left paraduodenal hernias (LPDHs) occur when the proximal jejunum or parts of the duodenum prolapse through the paraduodenal fossa or Landzert's fossa 3Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal upon request.A 39-year-old patient came to the emergency surgical department in the district general hospital in 2020, with the clinical picture of spasmodic pain in the epigastrium, with suprapubic propagation in the left back. The patient vomited 5 to 6 times. He took a tablet of ibuprofen, after which the abdominal pain intensified and he vomited a few more times. He was drenched in cold sweat and felt faint. He had a normal stool and did not complain about any urinary problems. He did not suffer from any serious illnesses. He consumed gastroprotective drugs, had appendix surgery, and multiple lipomas of the anterior abdominal wall. The medical history showed that he had experienced similar abdominal pain in the past, since childhood, with a weakened appetite and occasional nausea and vomiting after eating. In the past, he was treated conservatively, without an accurate and definitive diagnosis. He last underwent a gastroscopy 3 years ago, when he was diagnosed with chronic gastritis and gastroesophageal reflux disease (GERD). On that occasion, a biopsy and pathohistological diagnosis were performed – active chronic gastritis, Helicobacter pylori – positive. Eradication therapy was performed. The patient did not smoke, drank alcohol occasionally, and denied any drug allergies. In regard to drugs, he took pantoprazole 40 mg once a day and ibuprofen as needed.Status praesens: conscious, in contact, oriented, eupneic. Skin and visible mucous membranes normal. Cor: rhythmic action, clear tones, no murmurs. Arterial blood pressure on both arms 140/80 mmHg. Lung: right basal, impaired respiratory noise. Abdomen: meteoric, soft, tender to deeper palpation in the epigastrium and suprapubic, without organomegaly, peristalsis weakened. Lumbar succussion negative, bilateral. Limbs: no edema, normal peripheral arterial pulsations. Digitorectally: ampoule filled with feces, no neoplasm is felt at the fingertips, normal colored stool on the glove. Laboratory findings are unobtrusive, except for leukocytosis of 11.4 × 109/L.Radiograph of the abdomen, standing: air and residual intestinal contents without pathological aeroliquid levels or intestinal distension. There is no accumulation of free air under the domes of the shield , which showed the following result: in the left hemiabdomen, several convolutions of the jejunum of the distempered lumen 41 mm, filled with feces with abrupt conical narrowing, without clear cause, correspond to small bowel obstruction. A shorter segment of weaker wall opacification may indicate initial ischemic changes, with no signs of perforation. Intestinal convolutions aboral from the site of obstruction of empty, normal wall thickness. No pneumoperitoneum. Several reactive lymph nodes regionally, shorter in diameter up to 7 mm. Several tiny cysts of the liver and spleen without other focal or perfusion abnormalities. Bile ducts, gallbladder, pancreas, adrenal glands, kidneys, and small pelvic organs of normal size and density, without focal or perfusion abnormalities. There was no free fluid. Pulmonary bases without nodular lesions, consolidation and pleural effusion. Bone structures without signs of pathological bone remodeling .Fig. 2AxAfter preoperative preparation, the patient was placed under general anesthesia and surgery was performed. The procedure was performed by a general surgeon with 15 years of clinical experience. A laparotomy was performed through an upper midline incision. There was a minor serous effusion in the abdominal cavity. An inspection of the abdominal cavity followed, and a peritoneal sac was found with the proximal third of the jejunum twisted inside, located to the left of the fourth segment of the duodenum and the duodenojejunal junction. Collapsed loops of the small intestine were observed, aboral of the obstruction. With a meticulous preparation technique, the hernia sac was opened, the trapped jejunum loops were decompressed, the hernia sac was resected and the hernial opening was closed by sutures. Clamped jejunum loops showed signs of maintained vitality . This was followed by inspection and palpation of other structures of the abdominal cavity, and no other pathology was found. Rinsing of the abdominal cavity with warm saline and irrigation solution was performed, followed by drainage and closing of the wound in layers  and 4. T4LPDH represents herniation of the mesentery of the small intestine in the left paraduodenal or Landzert's fossa in the posteroinferior direction Clinical presentation of paraduodenal hernia is not unambiguous but ranges from asymptomatic to manifest . Among the rarer symptoms that can be mentioned are secondary pancreatitis, biliary colic and palpable tumefaction in the upper left part of the abdomen. In many cases, the patient has non-specific symptoms which last for years. This case report concerns such a patient, a 39-year-old man. The greatest difficulty in management of paraduodenal hernias lies in their diagnosis, especially in the case of asymptomatic patients or non-specific symptoms of a chronic nature. In such cases, with a well-taken medical history, clinical examination of the patient, abdominal X-ray, abdominal ultrasound and laboratory tests and possible angiography of the upper mesenteric artery , contrast CT scan of the abdomen plays a crucial role , and in most cases confirms the diagnosis 5In this paper, a case of a 39-year-old male patient with LPDH with non-specific symptomatology was presented. Due to the intensification of abdominal pain, nausea and vomiting after meals as a result of intestinal obstruction, we approached a detailed diagnostic treatment of the patient and arrived at the above diagnosis. We performed open surgery, which confirmed the formation of LPDH, and we resolved it in the usual way, by releasing the trapped loops of the small intestine and closing the hernia orifice with sutures. Unrecognized LPDH leads to delayed surgery, which can cause intestinal perforation, peritonitis, and even death. Computed tomography of the abdomen helps us make an accurate diagnosis and perform timely surgery, which was the case with our patient.We did not have any source of funding.We did not use any personal date of patient included in this case report.Written informed consent was obtained from the patient for publication of this case report.N/A.Dragan Manojlović.Dragan Manojlović: case report concept, writing the paper.Nenad Čekić: Writing the paper.Mario Palinkaš: data collection, writing the paper.There was not any conflict of interest.


Gallbladder volvulus is a rarely reported and diagnosed condition. We present a case of an elderly female with right lower quadrant pain mimicking acute appendicitis without conclusive imaging; however, due to worsening serological laboratory findings and sepsis picture, an exploratory laparotomy was performed. A necrotic gallbladder was removed, diagnosing gallbladder volvulus. A systemic literature review showed the difficulty in making a diagnosis and the uniqueness of our patient presentation. A high level of clinical suspicion for gallbladder volvulus must be maintained and should be included in the differential diagnosis in elderly women with an acute abdomen, as complications can be severe. Gallbladder volvulus (GV) occurs when the organ twists along its long axis, resulting in vascular compromise. This condition affects women more often and has a higher incidence in the geriatric population. There have been only 400 cases recorded since it was first described in 1898; however, its incidence appears to have increased in the twenty-first century as the average life expectancy increases [We present a case of an elderly female with GV who experienced acute lower abdominal pain in the setting of a palpable abdominal mass.This article was previously presented as a meeting abstract at the ACG 2020 Annual Scientific Meeting & Postgraduate Course on October 23, 2020.An 86-year-old woman with a history of hypertension, hypothyroidism, hyperlipidemia, colon cancer with sigmoid resection, and lumbar disc disease presented with one day of severe, sharp epigastric pain radiating to the right lower quadrant associated with nausea and vomiting. Physical exam revealed distention, tenderness, guarding, and a palpable mass in the right lower quadrant of the abdomen. There was no rebound tenderness and bowel sounds were hyperactive. Laboratory tests revealed leukocytosis, elevated erythrocyte sedimentation rate, and c-reactive protein. The complete metabolic panel, amylase, lipase, and lactic acid were within normal limits. A CT scan of the abdomen Figure  was compOriginally described by Wendel in 1898 -6. In 19Due to advances in imaging modalities, the incidence of preoperative diagnosis of gallbladder volvulus has increased from 10% to 26% . BecauseA pre-operative radiologic diagnosis is challenging, as the actual volvulus may not be visualized. The preferred diagnostic modality is an ultrasound that will commonly demonstrate findings similar to those of acute cholecystitis: a thickened gallbladder wall with pericholecystic fluid ,13. BaseDespite advances in imaging, volvulus is not usually discovered until surgical intervention, as in our patient. Early diagnosis of gallbladder torsion can help prevent life-threatening complications such as gallbladder gangrene, perforations leading to bilious peritonitis, and other infections. GV should be included in the differential diagnosis of an elderly patient with an acute abdomen. A high index of clinical suspicion is needed in elderly patients, as early diagnosis and definitive surgical intervention reduce morbidity and mortality.


Vulvar leiomyosarcoma is a rare smooth muscle malignant neoplasm but it is the commonest type of vulvar sarcomas. It may mimic benign tumors and misdiagnosis could delay proper management. We report a case of a 38-year-old pregnant woman with leiomyosarcoma of the vulva. The patient presented to her primary general practitioner with a small vulvar mass that she had first noticed one year prior. The tumor was suspected to be benign Bartholin’s cyst and treated with antibiotics. The patient declined improvement and had many consultations to different clinics where she had been diagnosed and treated the same. The tumor size started to grow rapidly after she got pregnant, and the patient was referred to our hospital where she underwent tumor resection. Histopathology revealed leiomyosarcoma. The patient had further assessment and close follow-up and has had no recurrence for 12 months. There is little literature available on vulvar leiomyosarcoma, most of which are case reports, and most gynecologic oncologists will go through their whole careers without seeing a single case. Vulvar sarcomas are rare representing 1-3% of all malignancies arising in the vulva. They are of a mesenchymal origin and the most common types of vulvar sarcomas are leiomyosarcoma, rhabdomyosarcoma, liposarcoma, angiosarcoma, malignant peripheral nerve sheath tumor, malignant fibrous histiocytoma, and epithelioid sarcoma .Leiomyosarcoma is the most common sarcoma of the vulva. It is a rare malignant neoplasm of the smooth muscle. Furthermore, its association with pregnancy is very rare. Limited data is available about its biological behavior, prognosis, and treatment strategies in pregnancy.Especially given the increasing incidence over the past several decades, however, this increase may be attributed to genetic predisposition, lifestyle, and sexual habits , 3In an attempt to add to the scarce literature data on this rare tumor, we are presenting this case report. A review of the course, incidence, diagnosis, and therapeutic approach of leiomyosarcoma of the vulva in pregnancy will be provided in this case report.A 38-year-old woman, gravida 5 para 4 presented at 32 weeks pregnancy. She first presented to her primary general practitioner with a small vulvar lesion that she had first noticed one year prior. The lesion was suspected to be benign Bartholin’s cyst and treated with broad-spectrum antibiotics. The patient noticed no improvement and had many other consultations with different clinics when she was advised to the same diagnosis and treatment. Initially, the swelling was constant in size, however, it started to enlarge rapidly. A significant increase in size was noticed during pregnancy and causing a restless feeling. The patient was thus referred to our hospital. She had no significant gynecological past history, prior gynecological malignancies, or condyloma acuminatum. Her pregnancy course was uneventful. The medical, surgical, and family histories were noncontributory. No medications had been taken before or during the pregnancy.Physical examination revealed a healthy woman with normal vital signs. Abdominal examination reveals a gravid abdomen with a fetus of appropriate size for the gestational age in cephalic presentation. Pelvic examination revealed a vulvar mass at the left side involving left labia majora and minora. The mass was painless, rounded, firm in consistency, freely mobile with an intact overlying skin measuring 15.0 x 10.0 cm diameter  with heterogeneous signal intensity on T2-weighted images showed a solid mass with no cystic signal intensity measuring 4.8 x 7.2 x 10 cm. The radiological impression was labial leiomyoma. At 34 weeks of pregnancy, the patient was admitted for tumor resection. A baseline fetal cardiotocography and obstetrics ultrasound were normal. Under spinal analgesia, the tumor was completely resected with minimal blood loss Figures , 3. No fThe patient was referred after delivery to the Gynaeoncology department for follow-up and further assessment and management. She had pelvic MRI, abdominopelvic CT with contrast, PET-CT head, neck, and lower extremity and show no tumor deposit, neither local nor distal. On post-surgical follow-up, the patient was asymptomatic and had no radiotherapy or adjuvant chemotherapy. She had no evidence of recurrence or distant metastasis on 12 months follow-up after surgery.HistopathologyGross evaluation revealed a well-defined mass measuring 10.0 x 8.0 x 5.0 cm and showing tan whorly cut surfaces with no obvious areas of hemorrhage or necrosis. Histological examination showed smooth muscle neoplasm arranged in fascicles. The tumor cells showed mild to moderate nuclear atypia and increased cellularity. The mitotic index was nine mitotic figures per 10 high power fields (HPF) when the high method is used. Small foci of tumor necrosis were identified Figure . The tumThis case is the first case of vulvar leiomyosarcoma showing a rapid increase in size during pregnancy reported in the Gulf region. The literature review revealed few documented cases of leiomyosarcoma located in the female genital tract -7.The deleterious effect of pregnancy on the course of leiomyosarcoma remains a matter of dispute among the authors. Several reports contain evidence that pregnancy may have a deleterious effect on the course of the tumor, however, this is not well supported through adequate data.The presentation can vary according to the stage of disease, but generally, patients will present predominantly with an asymptomatic, slowly growing vulvar mass, pain, discomfort, bleeding, and voiding difficulty with a size range between 1.5-16 cm ,5,7,8. WMany vulvar cancers are initially misdiagnosed as inflammatory conditions, Bartholin’s gland cyst, lipoma, fibroma, or leiomyoma, which could delay the diagnosis and worsening prognosis ,4,5,7. DThe final diagnosis of leiomyosarcoma is usually done on histopathological examination of the tumor. Pregnancy may induce histologic changes that may confuse the examining pathologist, therefore notifying the pathologist with pregnancy status is essential as it may require careful assessment.There are two classification systems that are used for smooth muscle tumor (SMT) of the vulva (site-specific criteria). In 1979, Tavassoli and Norris issued the first criteria for vulvar SMTs , and in Surgery is the cornerstone of therapy for vulvar cancer, but because of its rarity, evidence-based treatment algorithms are not available -3. The tThe decision for preterm delivery was based on several factors including the balance between the risk of cancer progression and the risk of delivery of a premature fetus. Leiomyosarcomas are aggressive tumors and patients whose disease appears to be initially confined to the vulva may experience a clinically aggressive disease course . The undIn conclusion, we report a rare case of vulvar leiomyosarcoma diagnosed in young pregnant women with a good outcome for both the mother and the baby. Leiomyosarcoma should be included in the differential diagnosis of vulvar masses. Progressively enlarging vulvar lesions should be biopsied even during pregnancy. Complete excision with pathologic confirmation of negative margins should be the goal. Dissection of unsuspicious lymph nodes is not recommended. Despite its rarity, both gynecologists and pathologists should be aware of vulvar leiomyosarcoma.