Ovarian fibrosarcoma is an extremely rare and malignant sex cord-stromal tumor. Due to its low incidence and poor prognosis, until now, very few cases have been reported, and most of the reported cases have been sporadic. Therefore, the treatments and prognostic factors of ovarian fibrosarcoma are still debatable. Here, we report 5 cases of ovarian fibrosarcoma that presented at Peking Union Medical College Hospital over the past 20 years. The 5 patients were 41, 51, 54, 76, and 76 years of age when initial symptoms of pelvic mass or pain appeared. On ultrasound, this disease usually manifests as unilateral pelvic masses, within which uneven echo enhancement and some blood flow signals are observed. No significant increase was observed in the levels of preoperative tumor markers, such as serum CA125 and sex hormones. The final diagnosis depends on postoperative histopathological results since these tumors are easily misdiagnosed when intraoperative frozen sections are used for examination. Pathologic examinations showed that the tumor cells were spindle-shaped with moderate to severe atypia and high mitotic counts. The immunohistochemistry profile is not specific, but the positive rate of Ki-67 was consistent with the degree of malignancy and the prognosis of patients with this tumor. In addition, the tumor may also be positive for Vimentin, α-inhibin, SMA, estrogen receptor and progesterone receptor. Significant differences were observed in the surgical methods used, and no unified chemotherapy regimen has been established. The overall survival was > 15, >7, > 6, <1, and < 1 year for each patient. After reviewing the literature, evidence-based large-scale case studies were lacking. For treatments, complete cytoreductive surgery plus regimens typically used against malignant sex cord-stromal tumors, as described in the NCCN guidelines, are recommended. Due to its low incidence, both multicenter clinical studies and molecular studies are required to provide gynecologists with a better understanding and guidance for future management of patients with ovarian fibrosarcoma. Ovarian fibrosarcoma is a considerably rare neoplasm, as this tumor type accounts for less than 1% of all ovarian malignancies . OvarianA 57-year-old woman was admitted to our clinic with an abdominal mass that had appeared 2 weeks earlier, but she did not experience symptoms of abdominal pain and distension. She had been postmenopausal for 3 years and had no vaginal bleeding after menopause. Vaginal and abdominal examinations confirmed the presence of a cystic mass, with a diameter of 6–7 cm and no pressing pain. Vaginal ultrasonography revealed a mass 9.6*8.2*5.2 cm in diameter that was thickly encapsulated with a liquid and irregular mass in the left ovarian region. The interior of the cyst contained several septa, with medium to strong echo bulges on the septa, the largest of which was 1.8*1.1 cm in size. A slight blood flow signal could be seen on the septum, and a liquid area with a maximum depth of 4.6 cm could be seen in the pelvic cavity. Before surgery, the levels of serum tumor markers were within normal limits.During the laparotomy, an irregular, multilocular, cystic mass 8*10 cm in diameter was found in the right ovarian region, and no metastatic lesions were observed. Total abdominal hysterectomy (TAH) plus bilateral adnexectomy was performed. The pathologic diagnosis of the mass was a well-differentiated sarcoma of the right ovary . The immunohistochemical results were as follows: Vimentin (+), SMA (+), PR (±), ER (-), CD10 (-), Caldesmon (-), Melan (-), α-inhibin (-).The tumor was categorized as an International Federation of Gynecology and Obstetrics (FIGO) stage I C. The patient was given 2 cycles of systemic chemotherapy consisting of Cisplatin + Vincristine + Bleomycin (PVB) at 3-week intervals. After chemotherapy, the patient was followed-up regularly, and no recurrence was seen 15 years after surgery.A 41-year-old female was admitted with an abdominal mass that had appeared 2 months earlier. This patient reported no additional complaints, such as abdominal pain or distension. She had normal menstruation and no abnormal vaginal bleeding. Upon abdominopelvic examination, a firm, unmovable mass 5*6 cm in diameter without tenderness was detected on the posterior left side of the uterus. Vaginal ultrasonography revealed a heterogeneous, left ovarian mass with a clear boundary and a diameter of 6.1*5.5*4.6 cm. No signs of metastasis, lymph node enlargement or ascites were observed. No obvious blood flow signal was detected. The levels of serum tumor markers were within normal limits with the exception of a slightly elevated carcinoma antigen (CA) 125 level (40.4 U/mL). By magnetic resonance imaging (MRI), a heterogeneous mass 48*62*60 mm in diameter that was closely related to the uterus was detected on the posterior left side of the uterus. The possibility of subserosal fibroids was considered, whereas an adnexal tumor was not considered.During laparoscopic exploration, we found a solid mass 7*6 cm in diameter with a clear boundary; the mass originated in the left ovary, and no peritoneal dissemination was observed. The right ovary appeared full and the uterus appeared normal. Ovarian cystectomy, sampling of the right ovary and intraperitoneal washing were performed during surgery. After the mass was cut open, solid granular materials were present. The intraoperative pathological diagnosis was left ovarian follicular fibroma. However, a week later, the final pathologic diagnosis was fibrosarcoma of the left ovary, and the mitotic counts were evaluated > 10 times in 10 high-power fields (HPFs). The immunohistochemical analysis showed AE1/AE3 (-), Calretinin (-), Ki-67 (index 5%), p53 (-), α-inhibin (-). After surgery, the serum CA125 level was 27.1 U/ml.This case was diagnosed as FIGO stage I A. The patient refused chemotherapy and remained disease-free with normal ovarian function during the 7-year follow-up.A 76-year-old woman was admitted with abdominal pain and fever. She had been postmenopausal for 28 years without any vaginal bleeding after the onset of menopause. Fourteen years ago, the patient underwent an exploratory laparotomy due to acute abdomen at another hospital. During the operation, 2800 ml of intraabdominal blood clots and non-clotted blood was removed, and a cauliflower-like tumor 3*3*2 cm in diameter with active bleeding was seen around the left tubal umbrella. Complete hysterectomy and bilateral salpingo-oophorectomy and omentectomy were performed. The postoperative pathology report described a diffuse granular follicular cell tumor of the left ovary with invasion of the left fallopian tube. The clinical FIGO stage was II C. After surgery, she received 3 cycles of systemic chemotherapy consisting of Cisplatin + Cyclophosphamide (PC), after which the patient did not return for follow-up.Four years later (10 years ago), the patient underwent a second exploratory laparotomy at another hospital due to abdominal pain and the presence of a pelvic mass. During the surgery, a solid-cystic mass 3 cm in diameter was observed between the sigmoid colon and the bottom of the left side of the bladder. In addition, a nodule 1 cm in diameter at the bottom of the left side of the bladder bottom and a nodule 0.5 cm in diameter on the surface of the sigmoid colon were seen. The tumor lesions were completely removed, and the postoperative pathological diagnosis was granulosa cell tumor. After surgery, she received 1 cycle of chemotherapy consisting of Taxol + Carboplatin. However, the patient was then lost to follow-up.The next time, she was admitted for abdominal pain and fever that persisted for 1 day. Ultrasound revealed an irregular, cystic mass 10*8.6 cm in diameter with an unclear boundary in the pelvic cavity; the wall was uneven in its thickness, and the size of the cystic portion was approximately 7.21*6.34 cm. MRI revealed a double capsular structure 88*9*7 cm in diameter on the left side of the pelvic cavity. The mass had a thick wall with a relatively clear edge and was located close to the right bowel. The cephalic size of the lesion was approximately 4*2*3 cm, and the signal of the vesicle was slightly low. Vaginal and abdominal examinations confirmed the presence of a hypertonic, cystic mass with a diameter of 16 cm. The preoperative serum levels of CA125, Estradiol (E2), and Follicle Stimulating Hormone (FSH) were 15.5 U/ml, 25.8 pg/mL and 52.8 mIU/mL, respectively.We performed exploratory laparotomy and secondary cytoreductive surgery. During the operation, a solid-cystic multilocular mass 10 cm in diameter was observed between the sigmoid colon and the left lateral wall of the bladder. After 600 ml of yellow intracapsular fluid was removed, the mass was completely excised. Then, we performed adhesion decompression, repair of the sigmoid colon, and partial ileotomy anastomosis. The tumor was then completely removed, and the pathologic diagnosis was ovarian fibrosarcoma. Immunohistochemical analysis showed the following: Melan-A (+), Vimentin (+), AE1/AE3 (+/-), CD99 (-), Calretinin (-), α-inhibin (-), Ki-67 index 10%.After surgery, the patient experienced incomplete intestinal obstruction and received conservative treatment. The patient’s condition gradually improved and she fully recovered after 2 weeks. Since the patient was elderly, she did not receive adjuvant chemotherapy. No signs of recurrence or an increase in the serum E2 level have been observed more than 6 years after surgery.A 76-year-old woman was admitted with abdominal pain and an abdominal mass. She had been postmenopausal for 28 years and had no abnormal vaginal bleeding. Ultrasound revealed a heterogeneous, irregular, hypoechoic mass without a clear boundary that was located on the upper right-hand side of the uterus, with a diameter of 11.4*13.5*8.6 cm. Punctiform blood flow signals could be seen by Color Doppler Flow Imaging (CDFI). MRI revealed a large solid-cystic mass in the pelvic cavity, and we considered the possibility of malignant lesions in the accessory ovary, except mesenchymal tumors. Vaginal and abdominal examinations confirmed the presence of a hypertonic, cystic mass with a diameter of 12–15 cm and no pressing pain. Before surgery, the serum CA125 level was 593.3 U/ml.Her medical history included sleep apnea syndrome for more than 10 years that necessitated supplementary positive pressure ventilation at night.After a multidepartment consultation, surgical contraindications were eliminated, and we performed an exploratory laparotomy. During the operation, approximately 100–200 ml of bloody ascites was observed. A cystic-solid mass with a diameter of approximately 20 cm was seen below the incision. Cauliflower-like tumors with a rich blood supply could also be seen on the surface of the mass. The mass was widely adherent to the surrounding small intestine and colon, the surface of which contained cauliflower-like tumor lesions. The source of the mass and the metastatic tumors was found to be the left accessory ovary, and thus, the left adnexa was removed. The pathology report revealed a malignant spindle cell fibrosarcoma. The mitotic counts were evaluated > 10 times/10 HPFs. Immunohistochemical analysis showed the following: CA125 (-), CD10 , Desmin (-), Ki-67 (index40%), SMA (+), S-100 (-), Vimentin (+), p53 (-), α-inhibin (-).Due to her medical complications and older age, the patient refused adjuvant chemotherapy. One week later, she was discharged and returned home. The tumor relapsed 2 months later, and she died within 1 year of the first operation.A 54-year-old woman was admitted with fever and dull abdominal pain. She had been postmenopausal for 18 months and had no abnormal vaginal bleeding. A physical examination confirmed the presence of a cystic mass 10 cm in diameter on the right posterior of the uterus with clear boundaries, poor activity and no tenderness. The serum levels of CA125 and CA199 were 88.8 U/ml and 40.4 U/ml, respectively. Vaginal ultrasonography revealed a cystic mass full of fine spots that was located in the right ovarian area; the mass had clear boundaries, irregular low echo protrusions on the wall and no blood flow signals on CDFI. A solid mass with clear boundaries and a diameter of 7.3*5.9*6.5 cm was located below the former site of the cystic mass, and abundant arteriovenous blood flow signals could be seen inside and around the mass.Her medical history included 30 years of dysmenorrhea and 18 years of endometriosis and adenomyosis.We performed an exploratory laparotomy, and during the operation, a small amount of bloody ascites was observed. We observed that the size of the uterus was approximately equal to that at 6 weeks of pregnancy, and no obvious abnormality was seen in the left ovarian area. A cystic-solid mass with an approximate diameter of 10*15 cm was seen in the right ovarian area. After 600 ml of thin, chocolate-like intracapsular fluid was removed, we observed that the mass was densely adherent to the surrounding pelvic peritoneum and that the rectal pouch was completely closed. The source of the mass was found to be the left accessory ovary, and thus, the left adnexa was completely removed. After the mass was cut open, the contents appeared pale white, crisp and vortex-free and contained a chocolate-like liquid. The intraoperative rapid pathology report described a right ovarian spindle cell tumor with a large necrotic area, which was considered a sex cord-stromal tumor, but a malignant tumor was not excluded. After communicating with the patient’s family, they wanted to wait for the final pathology results before proceeding with additional surgery. Total hysterectomy and bilateral adnexectomy were then performed.However, 10 days later, the final pathologic diagnosis was fibrosarcoma of the right ovary, with extensive necrosis. The mitotic counts were evaluated > 40 times/10 HPFs. Immunohistochemical analysis showed the following: CD31 (+), Ki-67 (+ 70%), SMA (+), AE1/AE3 (-), CD34 (-), CD117 (-), ER (-), PR (-), Desmin (-). No tumor cells were found during peritoneal washing.After surgery, the serum levels of CA125 and CA19-9 were 109.0 U/ml and 5.6 U/ml, respectively. The patient was given 1 cycle of systemic chemotherapy consisting of Cisplatin + Epirubicin + Ifosfamide (PEI). After 1 course of chemotherapy, the serum levels of CA125 and CA19-9 were 24.9 U/ml and 4.3 U/ml, respectively.We then performed a second laparoscopic exploration, adhesion decompression and partial removal of the pelvic mass 5 weeks after the first surgery and 3 weeks after chemotherapy. During the surgery, a hard, solid mass approximately 5 cm in diameter was palpable at the top of the stump and was located below the adhesion of the bladder and rectum. The surface of the mass was not visible. No obvious tumor lesions were seen on the pelvic peritoneal surfaces and visceral surfaces. Due to the tight adhesion, separation was extremely difficult, and the mass did not have a capsule or boundaries. Only 2/3 of the tumor was removed, and after the mass was opened, solid, brittle gray material was present.The pathology report still described a fibrosarcoma. One week after the second surgery, the serum level of CA125 was 35.1 U/ml, and she received 1 cycle of chemotherapy consisting of Taxol + Carboplatin (TC). After 1 course of TC, the serum level of CA125 was 32.9 U/ml, after which the chemotherapy ended, and the patient died within 1 year after surgery  at 3-week intervals. No elevated CA125 level or recurrence was seen 8 years after treatment. For case 2, ovarian cystectomy and sampling of the right ovary were performed during surgery without postoperative chemotherapy. A secondary cytoreductive surgery was performed in case 3, and the tumor was completely removed, after which chemotherapy was given again. In case 4, the left adnexa was removed without chemotherapy after surgery because of her medical complications and older age. Specifically, total hysterectomy and bilateral adnexectomy were performed as the first surgery in case 5. Then, we performed a second surgery 5 weeks after the first surgery and 3 weeks after PEI chemotherapy in which the pelvic mass was partially removed. According to previous studies, early and thorough surgery is preferred, but the surgical methods differed significantly in reports of individual cases; surgical methods ranged from unilateral adnexectomy to complete cytoreductive surgery  –13. DuriAfter surgery, adjuvant radiotherapy and chemotherapy should be selected on an individual basis. Due to the high degree of malignancy and the high recurrence rate, the prognosis of ovarian fibrosarcoma is very poor. Few studies have reported which adjuvant chemotherapy improved the survival rate of patients with fibrosarcoma, and thus, it is recommended that even patients with early-stage disease should also be given chemotherapy . HoweverP = 0.014) and a positive rate of Ki-67 < 10%  have a better 2-year survival than other patients. From our experiences, the positive rate of Ki-67 may be consistent with the degree of malignancy and prognosis of the tumor. In our cases, the highest positive rate of Ki-67 was 70% in case 5, and the tumor quickly relapsed 5 weeks after the first surgery and 3 weeks after chemotherapy. We performed a second surgery, which involved partial removal of the pelvic mass. The overall survival was less than 1 year.Currently, the treatments and prognostic factors of ovarian fibrosarcoma are still debatable. Although several cases of long-term survival have been reported , 15, theIn our cases, there were significant differences in the surgical methods, which ranged from ovarian cystectomy to secondary cytoreductive surgery. In addition, no unified chemotherapy existed for the above 5 cases, and of these, two patients even refused chemotherapy due to older age and medical complications. Younger patients in their forties and fifties did not receive the appropriate chemotherapy because of personal choice. The last patient (case 5) abandoned all treatments because her tumor relapsed so quickly, and her tumor was not sensitive to chemotherapy. Unfortunately, we could not offer a better solution. The overall survival rates were > 15, >7, > 6, <1, and < 1 year for each patient. The first patient had the best postoperative outcomes with very rare long-term survival. The reason for this seems to be that the sarcoma was well-differentiated, at an early stage (IC) and was completely removed, although not enough cycles of chemotherapy were administered.P = 0.002) [As for the significance of how the treatment method affects the prognosis, it has been reported that patients who received complete cytoreductive surgery combined with adjuvant chemotherapy had better survival outcomes than others, such as patients who received only unilateral adnexectomy or surgery plus radiotherapy  , 13. In Ovarian fibrosarcoma is a rare ovarian malignant tumor with a poor prognosis, and the diagnosis of this tumor should be based on postoperative pathology rather than on intraoperative frozen sections. Patients with an early disease stage and low positive rate of Ki-67 staining have a better prognosis than other patients. At present, evidence-based large-scale case studies are lacking, but nevertheless, we suggest that complete cytoreductive surgery is essential. For adjuvant therapy, regimens used for malignant cord stromal tumors, as stated in the NCCN guidelines, are recommended. Due to the low incidence of ovarian fibrosarcoma, multicenter clinical and molecular studies are required to elucidate its clinical features and to discover new treatments that lead to a better prognosis.


We report the Case of a 35 years old male patient admitted for pulmonary embolism in a febrile context. Transthoracic echocardiography showed a filamentary mass appended to the pulmonary valve whose thrombotic origin has been suggested on data of late gadolinium enhancement magnetic resonance imaging.The patient had a history of deep vein thrombosis in the context of familial thrombophilia with factor V leiden gene mutation in two of his sisters and an inhaled drug addiction to heroïn. There was a biological inflammatory syndrome with negative blood cultures. Transthoracic echocardiography showed a very mobile homogeneous hyperechoic mass measuring 8 cm in the right ventricle appended between the pulmonary valve and the lateral wall of the RV. In LGE-MRI, an isointense, to the myocardium, marginal hall and a central rim enhancement were objectified, suggesting the diagnosis of thrombus rather than vegetation.Despite the notion of drug addiction, the febrile context and the localization of the mass, a diagnosis of RV thrombus rather than infective endocarditis was favored relying on familial thrompbophilia, personal history of DVT and LGE-MRI aspect. The patient was treated with curative heparin therapy and antibiotic therapy. Due to the persistence of the mass after three weeks of treatment and after heart-team discussion, the patient underwent surgical mass removal. The anatomopathological study confirmed a fibrino-cruoric thrombus. •Differential diagnosis between thrombus and vegetation is sometimes a real challenge for the clinician.•Cardiac MRI with its LGE-MRI sequences can help characterizing thrombus with hypointense edges and brighter central zone called “the etched appearance”.•Even in the multimodality imaging era, anatomopathological stady sometimes is needed to reach the correct diagnosis. EchocarComputed tomography and MRI technical developments leading to high-quality images and thorough information in this matter ,3. MultiHowever, very few studies have reported results on the diagnostic value of cardiac MRI to discriminate thrombus from vegetation and no characteristic MRI signs of vegetation have been reported.The work has been reported in line with the SCARE 2018 criteria [We report a Case of a serpentine mass measuring 10 cm appended to the pulmonary valve with a challenging diagnostic between thrombus and right heart endocarditis. We will discuss the contribution of cardiac MRI and especially late gadolinium enhancement sequences in this diagnostic approach. criteria .2We report the Case of a 35-year-old male patient, chronic smoker, with a history of inhaled heroin addiction and DVT treated with Rivaroxaban for 6 months, and there is a family history of Factor V Leiden in two siblings. The clinical examination showed a preserved venous capital with absence of dermatological signs pointing towards intravenous drug injection.The patient presented to the emergency room with dyspnea at rest, associated with chest pain of the right hemithorax accentuated with deep inspiration. Upon presentation, vitals were as follows: oral temperature of 38.7∘ C, blood pressure of 130/90 mmhg, heart rate of 80 beats/min, and respiratory rate of 22 breaths/min, with an oxygen saturation of 88% on room air. The patient was alert and oriented. ECG showed sinus rhythm with a heart rate at 80 bpm. The thoracic CT angiography showed a pulmonary embolism of the right intermediate trunk with images of alveolar condensations which could be related to pulmonary infarctions or septic localizations. TTE revealed a mobile, serpentine, hyperechoic mass, appended between the lateral wall of the right ventricle and the pulmonary valve measuring 8 cm . In addiIn this context of diagnostic doubt between an IE and thrombus, the patient was treated by a curative anticoagulation based on enoxaparin sodium in association with an intravenous antibiotic therapy: vancomycin and gentamycin for one week then Rifampicin and ciprofloxacin because of fever and inflammatory syndrome persistence. Tests of classic causes of blood culture negative endocarditis were negative.Close monitoring of the patient showed improvement with regression of dyspnea, chest pain, fever and inflammatory syndrome after three weeks of antibiotic and anticoagulant therapy, but persistence of the mass with no decrease.A cardiac MRI was performed, it showed a serpentine mass of 10 cm, appended between the pulmonary valve and the RV apex causing a partial obstruction of right ventricular outflow tract. This mass had a signal intensity on the SSFP cine MRI imaging sequences identical to that of the myocardium , a-d. OnGiven the unchanged dimensions of the mass despite antibiotic and anticoagulant treatment, the potential risk of its migration in the pulmonary trunk and after heart team discussion, it was decided to surgically remove the mass.made by experienced team of heart valve surgery from Paris, allowed to complete resection of the mass which was 10 cm long with preservation of the healthy native pulmonary valve. Anatomopathological analysis confirmed the purely thrombotic nature of the mass.The surgical procedure, 3The diagnosis of infectious endocarditis is based on clinical and biological data, including blood culture, echocardiography and 18F-FDG PET/CT. This diagnosis remains difficult due to the atypical clinical manifestations and the low specificity of the Laboratory tests ,3.Echocardiography, either TTE or TEE, is the first imaging technique used for diagnosis purpose, it shows high sensitivity and specificity, especially when performed in a complementary manner. TTE/EET have limited diagnostic performance in differentiating thrombi from vegetations or cardiac tumors, especially at the early stage and when the vegetation is too small (2–3 mm) to be detected or when the mass is appended to a valve. In our Case, TTE and TEE showed a very mobile serpentine mass measuring 8 cm in length appended to the pulmonary valve. But this echocardiographic aspect did not allow to differentiate thrombus or vegetation. The patient was febrile; but blood cultures and tests for classic causes of blood culture negative endocarditis were negative.The role of MSCT and CMR imaging for the diagnosis of infectious endocarditis has been poorly evaluated ,5,6. VegIn Case of thrombus, signal intensity is changing depending on the thrombus age and it does not generally enhance in LGE-MRI sequences. Cardiac MRI late enhancement sequences can detect thrombus with higher sensitivity than echocardiography by describing a high contrast between the dark thrombus and the adjacent structures.Mural thrombus does not enhance during the first-pass perfusion (FPP) and is often depicted with hypointense border and brighter central zone (“etched appearance”) particularly on PSIR-type sequences, allowing high diagnostic specificity .One pattern was found to be more common in thrombi (94%) than in tumor (2%) in a study: hyperintensity at short inversion time (TI), “edged” appearance with hypointense border and brighter central zone at intermediate TI, and hypointensity pattern at long TI. This pattern had the highest accuracy (95%) to differentiate thrombus and tumor . The samFew cases have been reported describing cardiac masses with T1 and T2 relaxation time measurement. Saba et al. reported that the mean T1 relaxation time of the cardiac thrombus and myxoma were 1044.7 ms and 1681.6 ms, respectively .The cardiac ECG synchronized computed tomography provides high resolution anatomical information in cases of infective endocarditis, especially in Case of valve prosthesis. It can highlight vegetation, aneurysm or perivalvular abscess.18F-FDG PET/CT imaging may also, particularly in patients with prosthetic valves, be useful in the IE diagnosis showing abnormal 18F-FDG uptake around prosthetic valve IE with high sensitivity .Conclusion: Differential diagnosis between thrombus and vegetation is sometimes a real challenge for the clinician. Cardiac MRI with its LGE-MRI sequences can help characterizing thrombus with hypointense edges and brighter central zone called “the etched appearance”.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-inChief of this journal on request.This Case report did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.Dr. IJ, and Dr. CT analysed and performed the literature research, Dr. EJ performed the examination and performed the scientific validation of the manuscript. Dr. El ouazzani Jamal was the major contributors to the writing of the manuscript. All authors read and approved the manuscript.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.1.Name of the registry:2.Unique Identifying number or registration ID:3.Hyperlink to your specific registration (must be publicly accessible and will be checked):Research studies involving patients require ethical approval. Please state whether approval has been given, name the relevant ethics committee and the state the reference number for their judgement.Dr. Jamal El ouazzani.All authors have non-financial conflicts of interest related to this report.


Inverted papillomas of the lacrimal sac and nasolacrimal duct are exceedingly rare. Though histologically benign, these tumors are locally aggressive, have propensity for recurrence and are associated with a chance of malignant transformation. These tumors can present in an innocuous manner, masquerading as more common conditions such as primary acquired nasolacrimal duct obstruction. We present our experience with one such case and a review of the literature to emphasize the importance of pre-operative assessment and intra-operative vigilance, so as to aid in accurate diagnosis and early treatment. Inverted papillomas are benign tumors that arise most commonly from the nasal cavity and paranasal sinuses. They rarely arise primarily from the lacrimal sac or nasolacrimal duct. Although histologically benign, these tumors can be locally aggressive and have a tendency to recur following surgical excision. Malignant transformation has been associated with these tumors in a small proportion of cases. We present our experience with a case of inverted papilloma arising primarily from the nasolacrimal system and a review of the literature. A 45-year-old male presented with a five-year history of unilateral recurrent epiphora and swelling over the right medial canthal region. On examination, he had mild right hyperglobus, an increased tear lake with delayed dye disappearance test and a hard stop on syringing. Nasoendoscopy showed a bulge over the lateral wall of the nasal cavity, anterior to the middle turbinate , with normal overlying mucosa Figure .Computed tomography (CT) scan demonstrated a 2.6 cm lesion centered upon the right nasolacrimal duct with adjacent bony remodeling and expansion, as well as partial dehiscence of the medial orbital wall. Radiological features were deemed to be in keeping with a chronic mucocele  showed a mass extending from the frontoethmoidal recess, along the lateral nasal wall into the nasal cavity Figure .Intra-operatively, the tumor was noted to extend from the lacrimal fossa along the lateral nasal wall to the inferior meatus, and had infiltrated the remnant inferior turbinate inferiorly and frontal recess superiorly. Revision surgery was performed via the previous lateral rhinotomy incision, combined with endoscopic transnasal resection. Remaining superomedial aspects of the maxillary bone weret removed to expose the tumor around the lacrimal fossa completely and resection was performed. Intra-operative frozen section and final histology margins were clear, and the patient remains recurrence-free to date, five years post-operatively Figure .Inverted papillomas arising primarily from the lacrimal system are rare and have only been reported in isolated case reports. To date, there are fewer than 10 cases reported in the literature ,3. TheseThe most common presenting symptom is a medial canthal mass, followed by unilateral epiphora resulting from obstruction of the nasolacrimal drainage system . Bloody Patients with neoplastic lesions of the nasolacrimal system often first present to the ophthalmologist because their presenting symptoms tend to be ophthalmological in nature. If there is a suspicion of nasolacrimal neoplasm, we recommend a referral to an otorhinolaryngologist for nasoendoscopy to identify possible extension of the mass lesion into the nasal cavity.Cross-sectional imaging in the form of CT or MRI should be considered in all cases of suspected neoplasms of the nasolacrimal system, so as to establish the epicenter of the lesion and extent of involvement of surrounding structures . This aiThe principles of management of inverted papilloma of the nasolacrimal system are similar to that of inverted papilloma arising within the nasal cavity or paranasal sinuses. Complete eradication of disease via wide surgical excision with clear margins is the mainstay of treatment ,3. IntraChoosing the appropriate surgical approach is important as it determines visualization and access, which are critical in ensuring that the tumor is completely extirpated. The combined open-endoscopic approach has proven to be effective for resection of extensive benign neoplasms of the nasolacrimal system -11. ThisFollowing definitive surgery, close post-operative surveillance is necessary to identify any tumor recurrence early. Surveillance can be performed either via direct nasoendoscopic visualization or via CT or MRI scans if the primary tumor site cannot be adequately visualized on clinical examination. It has been shown that majority of recurrences for inverted papillomas occur within the first two years of definitive surgery . HoweverThe nasolacrimal system is an unusual and uncommon location for inverted papillomas to originate from. Meticulous history taking, physical examination and vigilant intra-operative observation are essential in diagnosis. CT or MRI is helpful in determining the extent of disease to aid in operative planning. The mainstay of treatment is complete surgical excision with clear margins, which is crucial to prevent tumor recurrence. Choosing the appropriate surgical approach is important and the combined open-endoscopic approach is one method that can maximize tumor visualization while minimizing surgical morbidity. Finally, long-term surveillance of these patients is recommended as tumor recurrence can occur even after five years.


Sinonasal Undifferentiated carcinoma (SNUC) comprises 3% of the head and neck tumors, including metastatic neoplasms. Herein we report the case of a 60-year-old male who was brought dead to our institute with previous records of a contrast-enhanced CT scan of the brain and MRI with evidence of tumor in the maxillary sinus with intracranial extensions. The histopathological examination of the mass in the maxillary sinus proved to be SNUC with metastases to the brain, lungs, and around the aorta. These tumors are undifferentiated and are distinct from other poorly differentiated tumors in deriving their origin from the Schneiderian epithelium. The aggressive nature of the tumor renders the prognosis quite dismal. SNUCs need to be early recognized and distinguished from other poorly differentiated carcinomas with the help of immunohistochemistry. The patient was seeking treatment for his illness, as documented by accompanying relatives. Further detailed clinical history was not available, as the case was brought for medicolegal autopsy by police personnel due to sudden death.The patient’s available records included a contrast-enhanced CT scan of the head taken 3 months back, showing two hyperdense, and non-enhancing brain parenchymal lesions in the left frontal and occipital lobes, measuring 11×10 mm and 10×10 mm, suggestive of neoplastic origin. The brain MRI showed multiple heterogeneous signal lesions with peripheral enhancement and perilesional edema involving the right capsular region, left frontal lobe, left occipital lobe, and the right cerebellum. The lesions were considered as possible metastasis. The paranasal sinus showed a large heterogeneous signal mass lesion involving the right maxillary sinus with an invasion of the right zygomatic bone, arch, and right sphenoidal vein with intracranial extension, causing compression over the right anterior temporal lobeAn autopsy was performed.The external examination revealed a body of a well-nourished man with a swelling over the zygomatic and maxillary area. The examination of the skull revealed a pinkish-white soft mass over the anterior and middle cranial fossa. The mass involved the right sphenoidal bone and extended into the right maxillary sinus and zygomatic bone. The left lung weighed 672 g, and the right lung weighed 780 g . On cut section, reddish-brown fluid oozed out. There was no evidence of thrombus or consolidation. The thoracic cavity was unremarkable.In the mediastinum, a hard mass was found surrounding the aortic arch. The Pathology Department received a soft mass from the maxillary sinus, the whole brain, the whole dissected heart, a fragment of the abdominal aorta, part of the aorta with the hard mediastinal mass, both lungs, two pieces of the kidneys, pieces of the pancreas, spleen, and liver.The soft mass from the maxillary sinus measured 5.7×3.5×2.5 cm and weighed 27.4 gm. The cut section was grayish-white .The brain weighed 1308 g after autopsy and 1364.5 g post-fixation (reference range:1179-1621 g). The external surface showed normal gyri and sulci. No herniation was seen. On the coronal slicing, a lesion, measuring 1×0.8 cm, was seen in the insular cortex at the junction of the right cerebral hemisphere’s grey and white matters. On the left hemisphere, a lesion measuring 1×0.8×0.8 cm was seen at the grey and white matter junction A. The leThe representative fragment of the aorta with an attached hard mass measured 10.1×6.7×41 cm. The aortic arch measured 4.5 cm in length .The luminal diameter at one end was 2 cm, and at the other end was 1.5 cm. The whole hard mediastinal mass weighed 178.1 g. The cut section of the mass showed a whitish growth with central blackish discoloration abutting the wall of the arch of the aorta, which was thickened and showed atheromatous plaque.Both lungs’ gross examination showed irregular and whitish nodules, which at the cut surface exhibited a central blackish discoloration measuring 2×2.5×4.2 cm and 4×3 cm, respectively.The representative samples of the kidneys, pancreas, spleen, and liver did not show any gross evidence of tumor involvement.Sections from the mass in the maxillary sinus showed a tumor arranged in the acinar and pseudo-alveolar pattern. The individual cells showed conspicuous to inconspicuous nucleoli with mild to moderate nuclear pleomorphism and abundant eosinophilic cytoplasm along with areas of necrosis. Increased mitotic count was observed. Occasional nuclear grooves and bizarre forms were identified. Numerous entrapped nerves were seen 4B. ThereSections from the cerebrum and cerebellum showed metastatic deposits A.Sections from the heart showed atheromatous changes in the main coronary artery and left anterior descending artery with 50% occlusion at a distance of 3 cm from the origin. However, no myocardial infarction was noted, ruling out an ischemic myocardial disease as a possibility of the immediate cause of death. The wall of the abdominal aorta showed atheromatous plaque.Sections from both lungs’ lesions B and theThe sections from the soft mass subjected to immunohistochemistry (IHC) reacted positively for CK 8/18 A and higThe post mortem diagnosis was of Sinonasal Undifferentiated Carcinoma arising from maxillary sinus with metastasis to brain, soft tissue around the aorta, and bilateral lungs.Sinonasal Undifferentiated carcinoma (SNUC) is a rare and highly aggressive tumor with an uncertain histogenesis and etiology.SNUCs are more commonly seen in males and are more prevalent in the elderly with the mean age ranging between55-60 years of age.The most common symptoms are nasal obstruction, periorbital pain and swelling, proptosis, diplopia, and cranial nerve palsies.On light microscopy, the tumor is generally arranged in sheets, nests, and trabecular patterns with small to medium polygonal cells with moderate amphophilic cytoplasm, vesicular to hyperchromatic nucleus and inconspicuous to prominent nucleoli. Brisk mitotic activity is noted along with prominent areas of necrosis and angiolymphatic invasion, which were observed in our case.1SNUCs are immunoreactive for CK7, CK8/18, CK19, and epithelial membrane antigen (EMA) and negative for synaptophysin, chromogranin, vimentin, and CD45, these reactions are necessary to rule out all the other poorly differentiated malignancies of the head and neck region.,The differential diagnosis of SNUCs includes (i) nasopharyngeal carcinoma; (ii) esthesioneuroblastoma; (iii) neuroendocrine carcinoma; (iv) rhabdomyosarcoma; (v) lymphoepithelioma; (vi) lymphoma; (vii) melanoma; and (viii) poorly differentiated adenoid cystic carcinoma. Therefore, immunohistochemistry is fundamental for the precise diagnosis.Our case was immunoreactive for CK 8/18, high molecular weight cytokeratin and focally for p16and negative for vimentin, desmin, p40, synaptophysin, Human Papilloma Virus (HPV), Leucocyte Common Antigen, and HMB45, thus ruling out the possibility of a sarcoma, squamous cell carcinoma, and melanocytic tumour.Moreover, to rule out SNUC-like neoplasms, immunohistochemistry for INI-1 and IDH-2 was also performed. INI-1 was found retained ruling out the possibility of SMARCB1 deficient tumors, and IDH-2 mutant was positive reinforcing the diagnosis of SNUC. Hence, a diagnosis of SNUC was rendered. The differential diagnosis of SNUC-like tumors has been enumerated in the ,The treatment protocol advised in the available literature is radical curative resection followed by adjuvant chemo-radiation.Unfortunately, in our case, the patient succumbed to the disease with widespread metastases due to a probable diagnosis delay. As we could not elicit a detailed clinical history, we cannot comment upon the disease’s course.The purpose of this case report is to emphasize the highly infiltrating nature of this tumor and the various implications it may have due to widespread metastasis if left untreated. Ignored cases might lead to the infiltration and failure of multiple organ systems to such an extent that the tumor itself may not remain the only cause of the patient’s suffering or death.


Jejunal adenocarcinoma, a small bowel adenocarcinoma (SBA), is a rare cause of small bowel obstruction. Jejunal adenocarcinoma classically presents with vague clinical symptoms, i.e., abdominal pain, discomfort, and weight loss, making timely diagnosis challenging. Owing to its diagnosis at a late stage, the prognosis of jejunal adenocarcinoma is poor. Curative resection of the tumor at the early stages remains a treatment of choice. Here, we report a case of a 55-year-old man presenting with symptoms of nausea, vomiting, abdominal pain, abdominal distension, and relative constipation. Computed tomography (CT) scan showed dilated small bowel loops. Exploratory laparotomy was performed, which revealed a jejunal stricture and dilated small bowel loops proximal to it. Suspicious stricture, along with the diseased portion of the intestine, was removed through en-bloc resection. Histopathology and metastatic workup revealed moderately differentiated adenocarcinoma with stage IIB (T4aN0M0). We conclude that, although rare, jejunal adenocarcinoma should be kept in mind when dealing with a patient presenting with symptoms indicating small bowel obstruction. Our purpose is to emphasize laparotomy as both a diagnostic and surgical modality for SBAs in early stages, especially in setups of low economic countries where advanced imaging techniques are relatively inaccessible. Small bowel obstruction is a surgical emergency that we encounter very frequently in our day-to-day practice. Postoperative adhesions (60%) are the most common cause of small bowel obstruction, followed by hernias, Crohn's disease, malignancy, and volvulus. Other less frequent causes of bowel obstruction differ between young adults and elderly patients; gallstone ileus is more frequent in elderly patients while Crohn’s disease in young adults . Rarely SBA usually presents in people aged between 55 and 65 years. It presents with vague symptoms such as abdominal discomfort, pain, nausea, vomiting, gastrointestinal bleeding, and intestinal obstruction. SBAs are often diagnosed in an emergency setting, either as an obstruction 40%) or bleeding (24%) 0% or ble. There iHere, we present a case of a 55-year-old man with a small bowel obstruction caused by a primary jejunal adenocarcinoma.A 55-year-old man, with no known co-morbidities or any previous abdominal surgery, presented to the emergency department of Dr. Ruth K. M. Pfau, Civil Hospital, Karachi, with a two-week history of abdominal pain and distention, and a one-week history of constipation, nausea, and vomiting. He also reported a 10-kg weight loss in the past six months. Furthermore, he was kept on anti-tuberculosis therapy for six months when diagnosed with pulmonary tuberculosis two years ago. The patient's family history was insignificant.On examination, vitals were normal; the patient was mildly dehydrated and resuscitated with ringer lactate. The abdomen was distended, with exaggerated bowel sounds. No mass was palpable on examination. The digital rectal examination (DRE) and the rest of the systemic examinations were unremarkable. Hematological investigations are demonstrated in Table Intraoperative inspection showed stricture at the proximal jejunum. Adhesions in the whole abdomen and 200 ml of frank pus surrounding the stricture were observed. The small bowel was distended proximal to the stricture and collapsed distal to it Figure . IntraopHistopathology confirmed a moderately differentiated adenocarcinoma of the jejunum, invading the serosa with tumor-free resection margins. On sectioning of the specimen, the mucosa of the bowel wall exhibited a lesion measuring 2 cm x 2 cm. We also recovered 27 lymph nodes; none of them was involved by the tumor. However, seven lymph nodes showed complete hyalinization. Lymphatic and vascular invasion was not identified. The cancer was, thus, staged as IIB (T4aN0M0). Postoperative CT scan of the chest, abdomen, and pelvis (CAP) showed no metastasis. Carcinoembryonic antigen (CEA) levels were 3.7 ng/ml . Follow-up with a surveillance CT scan of CAP at six-month showed no recurrence of the disease. The patient remains well to date.Although small bowel comprises 90% of the mucosal surface area and 75% of the length of the entire gastrointestinal tract, malignancies of the small bowel are relatively rare, accounting for about 3-5% of all gastrointestinal malignancies . The mosSBAs are slightly more common in men than women and are commonly diagnosed at 60 years of age . Risk faSBA may present asymptomatically with microcytic anemia as the only sign of development . HoweverVarious investigational modalities are available to detect small bowel tumors. Older imaging techniques have limited importance in the diagnosis of SBA. Barium meal follow-through has a sensitivity of 50% in the detection of the tumor whereas push enteroscopy can only assess up to 40% of the small bowel length . AccordiStudies suggest that CT scan has an accuracy of 47% for the diagnosis of SBA and is a valuable mode of investigation for detecting local, regional, and distant metastasis; however, it may have value in diagnosing small primary lesions ,13. It iThe treatment of choice for SBA is complete en-bloc resection of the tumor as it offers the best overall survival. It involves the removal of the primary tumor with clear margins together with loco-regional lymph node resection. Thus, curative surgery offers a 50% chance of cure. Recurrence at distant sites is the predominant pattern of failure following a curative resection . To dateIn addition, patients of SBA have a poor prognosis with a five-year overall survival (OS) rate ranging from 14% to 33%. The five-year OS is related to the tumor stage, with stage IV tumor having the worst prognosis (3-5%). Moreover, the male gender, age greater than 55 years, lymph node invasion, and distant metastasis also contribute to a poor outcome. Several studies suggest that a primary duodenal tumor has a worse prognosis than a primary jejunal or ileal tumor .We aim to report a case of adenocarcinoma of the jejunum as a rare cause of small bowel obstruction and emphasize the importance of laparotomy in the management of patients with small bowel obstruction, where CT findings are inconclusive. If SBA is suspected based on operative findings, cancer-directed surgery proves to be the most appropriate approach to its best survival outcome. A surgeon should be wise enough to make timely decisions, thereby preventing a delay in diagnosis and subsequent poorer outcomes of aggressive disease.Jejunal adenocarcinoma, a small bowel adenocarcinoma, is a rare cause of small bowel obstruction. Patients usually present with vague clinical features such as abdominal discomfort, pain, and weight loss, which makes the timely diagnosis challenging. Although rare, jejunal adenocarcinoma should be kept in mind when dealing with a patient presenting with symptoms indicating small bowel obstruction. Laparotomy may serve as both diagnostic and surgical modality for SBAs in early stages, especially in setups of low economic countries where the facilities of advanced imaging techniques are not readily available. Further, profound research is required to assess the potential applications of newer investigative strategies and treatment modalities to achieve better disease outcomes.


Posterior choroidal leiomyoma is an extremely rare tumor, to our knowledge, less than 10 cases reported in the literature. The definite diagnosis can be confirmed by immunohistochemistry, and local resection is preferable to enucleation for the posterior choroidal leiomyoma.Two adult Asian women presented with progressive vision loss in their right eyes. Ophthalmic examination revealed an amelanotic dome-shaped choroidal mass located in the fundus with yellowish exudative retinal detachment. Clinical differential diagnosis of a nonpigmented choroidal neoplasm mainly includes amelanotic melanoma, atypical hemangioma, metastatic carcinoma, as well as the rare posterior choroidal leiomyoma. Considering the choroidal lesion was more likely to be a benign tumor, then we performed the treatment of local resection by pars plana vitrectomy and the histopathological examination confirmed the diagnosis of choroidal leiomyoma. The best corrected visual acuity of the patients was more than 20/100 on 6-month follow-up.From these two posterior choroidal neoplasm cases, we were able to demonstrate local resection by the 23 to 25-gauge mircoinvasive vitrectomy for excision of intraocular tumors is a feasible treatment for choroidal leiomyoma. Posterior choroidal leiomyoma (CL) is a rare tumor, to our knowledge, less than 10 cases reported in the literature . ClinicaA 34-year-old Asian woman presented with a 3-month history suffering of burred vison of the right eye. The woman had a history of small uterine fibroids and was not treated surgically. Dilated fundus examination showed a large amelanotic mushroom-shaped choroidal mass located the superotemporal quadrant with peripheral exudative retinal detachment involving the macula, and the tumor surrounded by yellowish intraretinal exudation Fig. A. Color A 33-year-old Asian woman presented complaining of blurry vision for 2 weeks. The woman had no family history of systemic (uterine) fibroids or any subtle clinical signs associated with the disease. Fundus examination demonstrated a yellowish-white choroidal neoplasm located the inferior nasal quadrant of the right eye , A- and B-scan ultrasonography  and fluoOf these cases of CL described above, 6 underwent enucleation due to the malignant tumors were considered to be diagnosed clinically , 3, 6, 7Microscopically, the intraocular tumor is composed of interlacing bundles of spindle cells and blunt-ended oval nuclei, with moderate amount of fibrous cytoplasm. On trichrome staining, there was no significant difference between intercellular myoglial fibrils and neurofibrils of Schwann cell tumor. Mitosis is rarely present. Many CL lesions showed a muscular appearance under light microscopy.In addition, immunohistochemistry showed that the expression of smooth muscle actin and muscle specific actin were consistent in the two tumors mentioned in this report. The immunohistochemical results of our cases are consistent with those of smooth muscle tumors.Local excision was performed in two patients by the 23 to 25-gauge mircoinvasive vitrectomy and reconstruction of the eyeball. No serious complications such as hemorrhage occurred during the operation. The retinas were well attached with silicone oil 3 months after operation. Follow-up for 2 years, there was no sign of tumor recurrences or retinal detachment. The best corrected visual acuity of the two patients were 20/66 and 20/100 respectively.Neither of our patients developed metastasis.In summary, we described 2 cases of CL, a rare, benign neoplasm which arises exclusively in the posterior choroid. Clinically, CL mimics malignant  and benign lesions. Under light microscopy, it is similar to the neural tumors, so muscular immunohistochemical markers and morphology should be carefully evaluated before the confirmed diagnosis.And, if a tumor is suspected benign or malignant is uncertain, especially if the tumor is mainly located in the choroid, local resection is a reasonable treatment to its management, in that it preserves eye structure and remains visual function. However, optimal treatment methods of choroidal leiomyoma remain to be determined. More cases are needed to analyze the characteristics for more precise differentiation from other tumors, prognosis and appropriate therapeutic options.


This report highlights an extremely rare genetic condition constitutional mismatch repair deficiency (CMMRD) in an Indian pediatric patient with dual malignancies, who suffered from transient encephalopathy, a rare side effect of the drug Nivolumab and the associated challenge during CSF protein electrophoresis interpretation. This report highlights an extremely rare genetic condition constitutional mismatch repair deficiency (CMMRD) in an Indian pediatric patient with dual malignancies, who suffered from transient encephalopathy, a rare side effect of the drug Nivolumab and the associated challenge during CSF protein electrophoresis interpretation.  The patients suffer from a broad spectrum of aggressive and pediatric cancers, like hematological malignancies, brain tumors, and colorectal cancers. In contrast to CMMRD, Lynch Syndrome is an autosomal dominant heterozygous monoallelic germline loss‐of‐function mutations in one of the four MMR genes with an increased risk of colorectal cancer, endometrial carcinoma, and other malignancies in the fourth and fifth decades of life.Confirmation of the diagnosis involves the analysis of microsatellite instability panel (MSI) and/or immunohistochemistry (IHC) followed by mutation analysis.We herein report a case with CMMRD with childhood colorectal cancer and brain tumor, GBM (glioblastoma multiforme). He received adjuvant treatment for GBM with Nivolumab, a monoclonal antibody but showed signs of suspected immune encephalopathy post‐Nivolumab, an extremely rare complication of Nivolumab.We would like to present this case because of its rarity and severity, rare side effect like encephalopathy with new immune modulators like Nivolumab, and its unusual electrophoretic interference.The study received approval from the local ethics committee.2The patient is a 11‐year‐old boy from Jharkhand, India, who presented with pain abdomen and intermittent constipation two years back. Colonoscopy revealed polypoid stricturing lesion suggestive of sigmoid colon tumor which was further confirmed by radiological findings on CT (Computed Tomography) scan abdomen. Among nonmalignant features, dermatological feature in the form of hypopigmented macule on left shoulder was present. On reviewing the family history, there was a history of neural tube defect and early death in sibling, and a history of sudden death in paternal cousin at 14 years of age. Few incidences of consanguineous marriage were reported in their extended family. The patient underwent exploratory laparotomy and resection of polypoid segment of sigmoid colon. Histopathological examination (HPE) revealed moderately differentiated adenocarcinoma of the sigmoid colon (pT3N0). The MMR IHC done on colonic tumor block showed intact nuclear staining with MSH2, MSH6, and MLH1 while immunostain for PMS2 was attempted thrice but cannot be reported as the results were noncontributory. In view of the absence of PMS2 in both the tumor tissue and the native normal tissue, a possibility of constitutional MMR deficiency (CMMRD) was considered. The patient was planned for chemotherapy with Capecitabine. After 2 months of chemotherapy, he again presented with episodes of headache, vomiting with left‐sided facial weakness of supranuclear type and difficulty walking. CECT (Contrast‐Enhanced Computed Tomography) scan of the brain revealed right frontoparietal space‐occupying lesion (SOL) with perilesional edema and midline shift suggestive of brain tumor (glioma) for which he underwent craniotomy and surgical removal of the tumor. CNS panel on biopsy diagnosed glioblastoma multiforme (GBM) WHO grade IV. Microscopic examination revealed a proliferating glial tumor with marked nuclear pleomorphism, mitosis, necrosis, and microvascular proliferation. IHC showed tumor cells to be positive for GFAP , IDH1(R‐132H) was negative signifying a poorer outcome, ATRX was retained, high Ki67 index of around 70% signifying higher tumor recurrence and progression. IHC done for mismatch repair (MMR) proteins on the block of GBM showed intact nuclear staining for MLH1 and loss of nuclear expression for PMS2 in both the tumor cells and the normal brain tissue. Because of the absence of PMS2 in the tumor tissue, native normal tissue of both GBM block and prior colonic block, the diagnosis of constitutional MMR deficiency (cMMRD) was established. Mutation analysis subsequently confirmed the diagnosis. The patient had a disease‐free survival for next 3 months after which he again showed signs of progressive disease He was then further managed by salvage immunotherapy with Nivolumab, a monoclonal antibody. After 2 courses of Nivolumab given 1 month apart, the patient had episodes of vomiting, but not associated with headache or other clinical signs of raised intracranial pressure. As a part of management, Ommaya shunt was done with drainage of approximately 7.5 mL clear CSF . CSF routine examination showed cell counts within normal limits and absence of any malignant cell while on biochemical evaluation CSF glucose was within normal limit but protein concentration was highly elevated to 563 mg/dL, the reference range being 12‐60 mg/dL. No metabolic abnormalities or acute infections or disease progression was evident from pathological, or microbiological work up. Contrast‐enhanced MRI (Magnetic resonance imaging) brain revealed right frontal lobe enhancing lesion and significant cerebral edema. CSF electrophoresis was done to rule out any inflammatory or noninflammatory neurological disease. In CSF electrophoresis (CSF‐PEP), an unusual faint M band near the beta region was seen, while in SPEP and CSF‐IFE, no heavy or light chain restriction pattern was noted corresponding to the M band as shown in Figure S1, S2, and S3, respectively. The patient was therefore diagnosed with immune encephalopathy post‐Nivolumab therapy. He was further treated with intravenous dexamethasone. The patient became stable and was discharged after 2 days on a dexamethasone taper. Considering the benefits of significant tumor reduction in contrast to the rare side effect malignant cerebral edema as reported by 3The patient was diagnosed and treated for CMMRD at Tata Medical Center, Kolkata, India. The characteristics are evaluated and presented in supporting information Table S1. Age at diagnosis is consistent with the available literature on CMMRD.In a study done byIn conclusion, this case report justifies the need for CMMRD reports in a developing country like India and a better understanding of the associated challenges in electrophoresis with emerging therapeutics like Nivolumab.None declared.1 and SC2: planned the study. CP1: collected the data and wrote the report. SC3 and KG1: analyzed, edited, and revised the manuscript.All authors: involved in the conception, design, write‐up, and revision of this article. CP4This study was approved by the institutional ethical committee of the TATA MEDICAL CENTER, KOLKATA, INDIA. All personal identifiers  were removed from the data set, and analyses were carried out at the institution level.Figures S1‐S3Click here for additional data file.Tables S1‐S2Click here for additional data file.


Desmoplastic Small Round Cell Tumour (DSRCT) is a rare malignancy that has only a few cases documented in the literature. We report a case of DSRCT in the abdomen and pelvis that was identified following ultrasound-guided biopsy of one of the numerous liver lesions seen on imaging in a 13-year-old Afro-Caribbean female with increased abdominal girth. The tumour was characterized by all routine imaging modalities available at the time. To our knowledge, this is the first reported and published case in the English speaking Caribbean. In the review of the literature, we correlate the imaging findings with previously reported cases. The diagnosis of DSRCT cannot be made solely using standard imaging techniques, but radiologists should be suspicious of DSRCT as a differential diagnosis in a young patient with increased abdominal girth, multiple liver and peritoneal deposits seen on imaging. Written informed consent for the case to be published  was obtained from the parents of this patient for publication of this case report, including accompanying images. This is the case of a 13-year-old female known to have asthma since early childhood with her last attack being approximately 1 year ago. She was on maintenance medication of beclomethasone 50 mcg, and Salbutamol inhaler, two puffs as needed. She was non-compliant with her medication citing lack of symptoms as her reason for non-compliance. She presented with complaints of generalized weakness and fatigue for the past 6 days, along with weightloss of an uncertain number over the previous months. The patient noted that her clothing began fitting more loosely, particularly over the past 7 days. She reported no history of fever, joint or muscle pain, rashes, but stated that she was experiencing constipation, loss of appetite, but no nausea, vomiting, or any abdominal pain. Additionally, she reported early satiety; only being able to tolerate approximately two spoonfuls of food before she felt full. This was also associated with increased abdominal girth which was noted during the previous months. There was no reported yellowing of the eyes, dark urine or pale stool. Menarche was reported to be at age 12, with regular periods that lasted approximately for 5 days, requiring 3–4 changes of partially soiled pads per day. There was no history of sexual contact, intravenous drug use, or blood transfusions. She also reported having significant exercise intolerance; only being able to walk about 20.0 m before she became tired. There were no palpitations or chest pain experienced but has shortness of breath on exertion. No urinary symptoms or history of seizure activities were reported. There was no other significant finding in her medical history. She presented to the hospital for further management. Pertinent examination findings were limited to the abdominal examination where our patient was noted to have mild epigastric prominence with a hypopigmented patch noted to the central abdomen. No caput-medusae, angiomas, hernias or pulsatile masses were appreciated. The abdomen was soft but tender in the right upper quadrant on deep palpation. A negative Murphy’s sign was appreciated. The liver span measured 24.0 cm, with no splenomegaly appreciated. The kidneys were not ballotable and there was no renal angle tenderness. There was no shifting dullness or transmitted fluid thrill appreciated. Notably, the patient was underweight with a calculated body mass index of 17.6. A clinical assessment of tender hepatomegaly of unknown cause was made, to rule out a primary versus a secondary hepatic mass.A complete blood count, urea and electrolytes, coagulation profile , hepatitis B, hepatitis C, and liver function tests were ordered along with an abdominal ultrasound, urinalysis, and urine beta-human chorionic gonadotropin (HCG). The urine β HCG was negative, the liver function tests were normal except for elevated alkaline phosphatase and gamma-glutamyl transferase. The complete blood count, the urea and electrolytes, as well as the coagulation profile, were within normal limits. The urinalysis was also normal.The abdominal ultrasound revealed an enlarged coarse appearing liver (24.2 cm in span), with multiple ill-defined isoechoic masses of varying sizes along with anechoic perihepatic free fluid .Multiple heterogeneous masses were also seen within the pelvis which appeared hypovascular on colour doppler interrogation. Moderate volume pelvic free fluid (ascites) was also visualized. These findings were highly suspicious for an underlying malignancy and the recommendation was made to have a CT scan of the abdomen and pelvis done. The CT scan of the abdomen and pelvis within the same admission period revealed a large ill-defined, heterogeneous solid mass with low attenuation central areas measuring 9.2 (L) x 9.4 (W) x 6.3 (AP) cm in the pouch of Douglas within the central pelvis .Centrally, this lesion displayed a few subcentimetre foci of calcification. No intralesional fat density was seen within this mass. The mass demonstrated mass effect on the uterus anteriorly and a clear plane of separation was not seen. Posteriorly, the adjacent sigmoid rectum was collapsed and draped over the superior extent of the mass. It was also inseparable. The involvement of the latter structures could not be excluded. Normal appearing ovaries were not identified. Multiple similarly appearing solid masses consistent with extensive peritoneal deposits were seen in the right and left adnexa, posterior to the bladder, along the pelvis side-walls with the largest focus seen in the bowel mesentery measuring 6.8 × 5.1 cm. The liver was markedly enlarged (25.0 cm measured in the midclavicular line) and the left lobe extended into the left hypochondrium. The liver demonstrated innumerable, predominantly rounded low attenuation (35.0 Hounsfield unit) masses measuring from a few mm to a maximum of 6.5 × 5.6 cm; the largest lesion was seen in segment 7. There were patchy areas of heterogeneous hepatic enhancement. The intrahepatic portion of the inferior vena cava, hepatic veins, and the peripheral portal veins were compressed; however, blood flow was maintained. There was also left renal vein stenosis due to mass effect from an exophytic hepatic lesion.Innumerable enlarged abnormal lymph nodes were also noted circumferentially encasing the celiac trunk, portal vein, aorta, common and internal iliac vessels. Mesenteric adenopathy was also seen. The constellation of findings on imaging was highly suspicious for malignancy and therefore the patient was referred for ultrasound-guided biopsy of the liver lesions, which was then sent for histopathology and immunohistochemistry (IHC).The biopsy consisted of three cores of tan to dark-brown tissue ranging in size from 1.5 × 0.1 × 0.1 cm to 1.0 x 0.1 × 0.1 cm .in situ hybridization detected EWSR1 gene rearrangement. The final diagnosis was, therefore, a desmoplastic small round cell tumour.The biopsy was sent to The Hospital for Sick Children, Toronto, Canada, through the SickKids Caribbean Initiative, for ancillary studies. IHC studies reported tumour cells positive for pankeratin, EMA, WT1 (C19) and vimentin with focal, dot-like positivity for desmin, partial mild staining for CD99 and focal staining for NSE. The desmoplastic stroma stained positive for SMA. There was intact nuclear staining with BAF47. Tumour cells were negative for WT1 (NH2), PAX 8, inhibin, glypican-3, CDX2, CK5/6, CK20, calretinin, CD30, myogenin, Heppar-1, and CD45. Cytogenetic studies using fluorescence 1 One of the largest intra-abdominal DSRCT case series of 19 cases, Gerald et al, reported that the male to female ratio is greater than 5:1 and the age of presentation is approximately 18.6 years.2 The mean survival time was found to be approximately 29 months, with the longest recorded survivor up until the published date of the study is 101 months (approximately 8.5 years).3 The index case is female; no data or case was found suggesting differences in the course of the disease between the sexes. Most of the literature characterizing this tumour, however, was written in the late 20th century, with more recent studies emphasizing modern treatment modalities and their efficacy. There is a paucity of data outlining the variable clinical presentation of DSRCT. Less than 200 cases have been documented in the literature worldwide.4 Based on our review of the literature, to our knowledge, this is the first clinically as well as radiologically documented and published case of DSRCT in the Afro-Caribbean population. Abdominal pain, increased abdominal girth, and a palpable abdominal mass are identified as the first and most common symptoms experienced by affected patients.3 Additionally, obstipation, nausea, vomiting, anorexia, and weight loss are common presenting complaints.5 The index case presented with altered mentation, reduced bulk, increasing abdominal girth and firm non-tender hepatomegaly, not dissimilar to the clinical findings cited by other studies and case reports. It is possible that our patient’s altered mentation was due to hepatic encephalopathy; the liver function tests were normal except for elevated alkaline phosphatase and gamma-glutamyl transferase. The CT scan of the brain was normal. Fundamentally, the presentation of this tumour is highly non-specific, with histopathology and IHC the diagnostic methods of choice after identification with imaging. The observed literature reveals a modest benefit for the use of radiological investigations in the diagnosis of DSRCT. Published ultrasound images representative of this disease process were found to be almost non-existent during our literature search. In a study conducted by Gerald et al, the classic sonographic features were lobulated peritoneal masses, ascites, serosal hepatic metastasis and a thickened peritoneum.1,6,7 In another study by Bellah et al, CT was used to further characterize the radiological features of DSRCT, which included bulky intra-abdominal soft tissue masses involving omental and serosal surfaces, without any distinct organ of origin. Solid dominant heterogeneous pelvic masses in the retrovesical or rectouterine spaces, as well as concurrent metastases, which were common at the time of diagnosis were also reported, principally those involving lymph nodes and liver parenchyma.6 The CT scan of the abdomen and pelvis done for the indexed case demonstrated a large pelvic mass with extensive peritoneal deposits, nodal disease, hepatic metastases and ascites, largely similar to the findings described in the aforesaid study. The study investigation by W. Zhang et al found that T1 weighted magnetic resonance images demonstrated mild heterogeneous enhancement of the lesions in the aforementioned sites, with T2 weighted images showing mixed iso- and hyperintense areas. Positron emission tomography demonstrated multiple nodular foci of increased metabolic activity within the abdominopelvic masses.7 These studies highlight the relatively nonspecific features of DSRCT on imaging, but strongly suggest that the diagnosis should be suspected in young patients with multiple bulky heterogeneous peritoneal soft tissue masses, as well as suggest the use of imaging for staging and to guide biopsy. The differential diagnosis for these imaging findings included other small round blue cell tumours, namely rhabdomyosarcoma, malignant lymphoma, neuroblastoma along with atypical malignant mesothelioma. Gerald et al in 1991 described DSCRT as a characteristic lesion, when it was discovered that DSCRT has specific immunohistochemical and karyotypic features from other round blue cell tumours. Before 1991, DSCRT was considered an atypical presentation of small round blue cell tumours.8 ESWR1 gene rearrangement was detected via IHC of the histopathology sample taken from an ultrasound-guided liver biopsy of the indexed case. Interestingly, this particular phenomenon was found to be the most common finding elucidated in a large institutional comparative series by Mohamed et al of IHC techniques to diagnose DSRCT, with 91.3% of the tested samples in the study showing ESWR1 rearrangement using the fluorescent in situ hybridization technique, and 92.8% with the reverse transcription-polymerase chain reaction technique.9 These figures are consistent with the other existing literature exploring the cytogenetic diagnosis of DSRCT. Ultimately, despite aggressive multimodal therapy in combination with surgery, the prognosis of DSRCT remains very poor. Radiological investigations remain paramount in contextualizing the extent of organ involvement as well as facilitating biopsy to confirm this pathological entity. Multiplanar imaging is also critical in the assessment of the disease response to treatment. This patient was subsequently referred to the haematology and oncology service for chemotherapy where she was started on etoposide 155 mg, vincristine 2 mg, ifosfamide 2790 mg, and doxorubicin 116 mg intravenously. A CT scan of the chest, abdomen, and pelvis was done at 4 months after the first imaging was done which coincided with the completion of four cycles of chemotherapy to assess treatment response. The images of the chest were unremarkable. A significant response to treatment was observed in the CT scan of the abdomen and pelvis. Treatment strategy for DSRCT may also include radiation therapy, surgical resection of the tumour with peritonectomy and heated intraperitoneal chemotherapy. These treatment options were differed in this case. The images below depict a significant response to treatment 1 year after diagnosis post 10 cycles of chemotherapy  is a rare undifferentiated tumour associated primarily with serosal surfaces, particularly the peritoneum. Historically, this tumour demonstrates a predilection for adolescent males, with a typically aggressive course.otherapy .This response though early in treatment appears promising for this patient. More data are needed to properly qualify the incidence of this condition in different population subtypes, to ascertain any genetic predisposition and/or other potential risk factors; environmental or otherwise that may lead to the development of DSRCT.DSRCT can mimic many other abdominopelvic malignancies and cannot be diagnosed on radiological imaging only. Histopathology and immunohistochemistry are essential in making the diagnosis.Whilst a useful initial investigation, abdominopelvic ultrasound is insufficient as it requires thorough characterization of the extent of the intra-abdominal and pelvic involvement of the tumour.The presentation of DSRCT is fairly consistent, but non-specific, with increasing abdominal girth in a young patient being a constant feature.DSRCT shows a strong predilection for the liver and peritoneum, relative to the other intra-abdominal and pelvic serosal organs.


Laparoscopic duodenojejunostomy is a common surgical treatment for SMA syndrome. Although there are successful cases of laparoscopic duodenojejunostomies on malignant conditions, cancer patients with opioid-induced bowel dysfunction could struggle in maintaining an oral diet despite surgical treatment of the mechanical obstruction.A 66 year-old woman with a chemotherapy history of 18 months for Stage 4 jejunal cancer near the ligament of Treiz presented with vomiting and dehydration. She had a gastrojejunostomy constructed prior to the induction of chemotherapy. CT scan and endoscopic studies confirmed the stricture of this anastomosis due to tumor invasion. Laparoscopic duodenojejunostomy was performed, but tolerable food intake was not achieved, likely due to limited bowel movements caused by opioid use and tumor invasion of the celiac plexus. A side-to-side jejunojejunostomy was constructed, since accumulation of food in her jejunal loop was thought to be a significant cause of her limited food intake and vomiting. She was able to tolerate oral intake after the second intervention and was discharged home.Successful cases of laparoscopic duodenojejunostomy in malignant strictures of the duodenum have been reported. In this case, the outcome was not so well due to limited bowel movements caused by opioid use. Literature review of laparoscopic duodenojejunostomy on SMA syndrome revealed some cases to be unsuccessful in enabling oral feeding or resolving nausea, and methods to treat such cases could be discussed further.Laparoscopic duodenojejunostomy is an option for malignant strictures of the duodenum, but a favorable outcome could not be achieved in our case. A side to side anastomosis of the jejunal loop and the efferent jejunum may help in improving the outcome. The initial evaluation revealed metastasis in her para-aortic as well as suprapancreatic lymph node, thus diagnosed as stage 4, and 1st line therapy was chemotherapy. Due to tumor obstruction, a gastrojejunostomy was constructed prior to chemotherapy. FOLFOX + Bevacizumab was started 18 months ago. The regimen was changed 4 months ago to FOLFIRI + Ramucirumab due to disease progression. There was also a complaint of left epigastric pain 7 months ago suspected to be cancer-related, for which treatment with opioids was started. In the course of her treatment, she presented with vomiting and dehydration. Contrast-enhanced computed tomography (CT) revealed a dilated gastroduodenum and a collapse of the efferent jejunum loop, indicating obstruction at the gastrojejunostomy. This was thought to be caused by a disseminated tumor invading into the anastomosis a, b. SheLaparoscopic duodenojejunostomy was performed a week after admission. Decompression with a nasogastric tube was done for 3 days prior to surgery. A 5-port procedure a was takAnastomotic passage could not be confirmed in an assessment with gastrografin on post-op day 3, but since no significant reflux was observed for 2 days, oral feeding was started. Repeated vomiting occurred 5 days later and a nasogastric tube was placed . With thSurgical intervention was thought to be essential for symptom improvement, and thus laparoscopic side-to-side jejunostomy of the jejunal loop and the jejunum anal to the duodenojejunal anastomosis was performed 30 days after the previous surgery. The EZ Access™ system was inserted into the umbilicus via the open method, and 3 trocars were used a. The di3Anastomotic strictures pose a threat to surgeons in a variety of surgical methods, including gastrectomy, colorectal resection, etc. In laparoscopic Roux-en-Y gastric bypass for obesity, rates of anastomotic strictures occur in 5–15% . When faLaparoscopic duodenojejunostomy is a surgical method well reported for SMA syndrome. Single-center case series show no mortality, no anastomotic leaks, and a mean post-operative length of stay ranging from 4.5 to 7.5 days  Table 1Table 1. Although laparoscopic duodenojejunostomy was not successful in achieving the initial goal of our patient, there are reported cases in which symptom improvement or resolution and oral nutrition was gained in patients with SMA syndrome and malignant strictures, suggesting that it is the choice of operation. However, methods to treat cases in which symptoms persist should be discussed further.4Laparoscopic duodenojejunostomy is a common surgical intervention of SMA syndrome, but there are also cases which failed to enable oral feeding or resolve nausea. Appreciable food intake and bowel flow may not be achieved in patients with limited bowel movements, and methods to treat unsuccessful cases should be discussed further. In our case, a side-to-side jejunojejunal anastomosis involving the loop was a solution.None.Non required.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.Yugo Matsui: Responsible for literature review, writing and manuscript preparation. Operator of both surgeries.Teppei Murakami: Responsible for manuscript preparation. Assisted in the laparosope-assisted jejunojejunostomy.Satoshi Ishida, Ryuichi Mikami: Assisted in the laparoscopic duodenojejunostomy.Shotaro Matsuda, Aoi Tayama, Ryutaro Sakata, Takehisa Harada, Masahiko Takeo: Responsible for manuscript review.Not applicable.Yugo Matsui.Not commissioned, externally peer-reviewed.The authors report no declarations of interest.


Clear cell tumors of the lung (CCTLs) are rare and mostly benign pulmonary neoplasms arising from perivascular epithelioid cells. Only approximately 100 cases have been reported, and half of them were in China. Limited details about CCTLs often cause diagnostic or therapeutic problems.We describe a case of a 28-year-old woman with multiple gradually replicating and enlarging nodules in the left lower lobe. The patient underwent fine-needle aspiration biopsy and was diagnosed with CCTL. A left lower lobectomy and mediastinal lymph node dissection were performed. The gradual changes in size (1.4 cm to 2.8 cm) and quantity (10 to 49) of the CCTLs in this case were the biggest differences from previously reported cases.CCTLs are very uncommon and mostly benign PEComatous tumors with no specific morphologic features. We present a case of CCTL with multiplicity and rapid growth, which may indicate its aggressive nature. The accumulation of similar cases will help clarify the exact nature and improve our understanding of the disease. Clear cell tumors of the lung (CCTLs) are extremely rare and benign lesions first described in 1963 by Liebow and Castlemen . CCTLs bA 28-year-old woman consulted the hospital for left chest paroxysmal pricking in 2014. Chest plain computed tomography (CT) scans revealed approximately 10 demarcated, round, homogeneous, unequally sized nodules without evidence of calcification, necrosis, or cavitation in the left lower lobe. Regular follow-up was suggested, and the same CT findings were obtained in 2016. In March 2019, she was admitted to our hospital for further evaluation because a plain chest CT scan showed larger, more numerous nodules. There were approximately 49 nodules, the largest of which was 2.8 cm in diameter Fig. . The larHE staining showed that the tumor consisted of round, clear cells with distinct cell borders and a granular eosinophilic cytoplasm Fig. a. HistolThe present clinicopathologic study of CCTL, otherwise defined as “sugar tumor”, is based on a small number of sporadic reports. Limited details about CCTL may cause diagnostic or therapeutic problems. Therefore, every single case is essential for pathologists or surgeons to better understand the nature of CCTL.CCTLs belongs to a family of “PEComatous tumors, benign” of the lung , and surRadiographically, malignancy cannot be excluded based on CT findings alone, and CCTLs are often considered primary or metastatic lung cancer due to the rich vascular stroma revealed by intense postcontrast enhancement on CT findings . HoweverHistologically, the lesions consist of round or oval clear cells with distinct cell borders and a granular eosinophilic cytoplasm. The characteristic feature of the lesions is a scanty intervening stroma with thin-walled sinusoidal vessels . Strong Biologically, CCTLs have traditionally been considered benign, but we report this interesting case to question the benign nature of CCTLs because of the multiplicity and rapid increase in size of the lesions. The clinicopathological features were consistent with those of the described cases. We analyzed 5 nodules and did not find vascular invasion or permeation of the tumor cells, and there were no histological differences among these 5 nodules. It is unclear whether the lesions were the result of intrapulmonary metastases or consisted of multiple primary lesions. Approximately 100 CCTL cases have been reported to date, and most of the nodules did not show malignant behavior. What would be the destiny of these patients if they did not undergo surgery? We speculate that patients with solitary CCTLs may experience long-term survival. However, surgical resection with VATS is a safe and curative approach, and we should not accept the low risk of local growth and distant metastasis. Whether or not surgery should be performed remains unclear because we seldom diagnose such tumors preoperatively and have not performed the RCTs necessary to answer this question. In 1988, we reported on a CCTL patient who died from hepatic metastases 10 years after resection , while tCCTLs are very uncommon and mostly benign PEComatous tumors with no specific morphologic features. We present a case of CCTL with multiplicity and rapid growth, which may indicate its aggressive nature. The accumulation of similar cases will help clarify the exact nature and improve our understanding of this disease.


BRAF K601E mutation in ovarian tumor tissues. Preoperative serum Tg concentration was >300 ng/mL, which decreased to 38.2 ng/mL after gynecological surgery with undetectable anti-Tg antibodies. The patient underwent total thyroidectomy with no cancer detected on histopathological examination. The patient was treated with I-131 and showed no recurrence 4 years after the diagnosis. Conclusions: Malignant struma ovarii is diagnosed by surgery. Because papillary carcinoma in struma ovarii is rare and there are no guidelines regarding the management of this type of cancer, therapeutic decisions should be made individually based on clinical and pathological data.Introduction: Struma ovarii accounts for 2% of mature teratomas. Struma ovarii is diagnosed when thyroid tissue accounts for >50% of the teratoma. Malignant transformation is rare, occurring in <5% of struma ovarii cases. Case presentation: A 17-year-old patient was diagnosed with papillary thyroid cancer in struma ovarii. The patient exhibited menstrual disorders. Abdominal and pelvic CT revealed a 17 cm mass in the left adnexa. Laparoscopic removal of the left adnexa with enucleation of right ovarian cysts was performed. Histopathological diagnosis was a follicular variant papillary carcinoma measuring 23 mm in diameter. Immunohistochemical positive expression of CK19, TTF-1, and thyroglobulin (Tg) confirmed the diagnosis. Molecular analysis detected the  Germ cell tumors account for 15–20% of ovarian tumors, and most are mature teratomas ,4,5. StrA 17-year-old girl, nullipara, virgin, came to the Outpatient Gynecological Clinic with complaints of menstrual disorders with polymenorrhea. The gynecological interview revealed that the first menstrual period at 12 years old was spontaneous, with regular menstrual periods every 28 days. For the past 2 years, menses had been scanty, occurring every 2 weeks. Physical examination showed the presence of a large mass in the lower abdomen reaching the navel. Ultrasound (US) examination through the abdominal wall revealed a cystic-solid tumor measuring approximately 15 cm adjacent to the left ovary. To clarify the diagnosis, a multi-phase CT scan of the abdominal cavity and pelvis with contrast was performed, and a blood sample was taken for analysis of basic tumor markers. CT scan of the abdominal cavity and pelvis confirmed a vast cystic-solid lesion measuring 8 × 13 × 17 cm, including a solid fragment measuring 4 × 5 × 5 cm, originating from the left adnexa, suggesting the presence of a tumor in the left ovary . We confBasal tumor markers were as follows: CA-125 17.3 U/mL (range 0.0–35); beta HCG <1.2 mlU/mL (range 0.0–5.0); CA19-9 3.54 U/mL (range 0.0–37); CEA <0.5 ng/mL (range 0.0–5.0). The patient underwent laparotomy to remove the left adnexa because of the large tumor mass, and a section was taken from the omentum. During the procedure, approximately 5 cm of the right ovarian cyst was detected and enucleated. The collected material was examined intraoperatively, which showed no presence of cancer. The results of histopathological examination revealed struma ovarii malignum “post intram”. A follicular variant of papillary carcinoma (23 mm in size) was detected within the left struma ovarii. No infiltration of the capsule was found .No tumor cells were detected in the enucleated right ovarian cyst or in a slice of the sample from the omentum. The diagnosis of cancer was confirmed by immunohistochemistry. Immunohistochemically, the tumor cells were strongly positive for thyroglobulin (Tg), transcription thyroid factor type 1 (TTF-1), and cytokeratin (CK19), and were negative for estrogen receptor .ABL1, EZH2, JAK3, PTEN, ACT1, FBXW7, IDH2, PTPN11, ALK, FGFR1, KDR, RB1, APC, FGFR2, RET, ATM, FGFR3, KRAS, SMAD4, BRAF, FLT3, MET, SMARCB1, CDH1, GNA11, MLH1, KIT, SMO, CDKN2A, GNAS, MPL, SRC, CSF1R, GNAQ, NOTCH1, STK11, CTNNB1, HNF1A, NPM1, TP53, HRAS, NRAS, VHL, ERBB2, IDH1, PDGFR, ERBB4, JAK2, PIK3, and EGFR. NGS identified a mutation in the BRAF p.K601E gene (c.1801A > G p. Lys 601Glu), whereas no mutations were detected in other genes . The hot spots in 50 genes in which mutations are present in various types of cancer were sequenced  using the Cancer Panel v2* kit (Thermo Fisher Scientific). The panel includes the following genes: er genes .The patient was referred to the Endocrinology Clinic to determine further treatment. The patient’s physical examination showed no abnormalities. The serum Tg, TSH, aTg, and TPO levels were determined from the serum, which was frozen before gynecological surgery. Tg concentration before surgery was >300 ng/mL (range 0.0–55 ng/mL), and it decreased to 38.30 ng/mL after surgery; serum TSH was 1.92 µIU/mL (range 0.35–4.94); no serum Tg or thyroid peroxidase antibodies were detected . Thyroid US examination showed a hypoechogenic focus in the lower pole of the left lobe. Fine needle aspiration biopsy of the focus performed under US detected a benign lesion  . Whole-b2, and blood pressure was 100/60 mmHg. The results of hormonal tests showed no deviations (androstenedione 3.43 ng/mL (range 0.30–3.70); total testosterone 0.39 ng/mL (range 0.14–0.49); free testosterone 1.780 pg/mL (range 0.3–3.05); DHEAS 454.5 µg/dl (range 61.2–493.6); FSH 5.14 mIU/mL (range 2.8–11.3); LH 4.9 mIU/mL (range 1.1–11.6); estradiol 41 pg/mL (range 21–251); total prolactin 179 mIU/L (range 40–530); 17-OH-Progesterone 0.766 ng/mL (range 0.27–2.31); beta HCG <1.2 mIU/mL (range 0.0–5.00); Ca-125 8.2 U/mL (range 0.00–35); AMH 3.26 ng/mL (range 1.52–9.95); SHBG 37.42 nmol/L (range 25–135); HOMA 1.4 (range <2.5); features of PCO on US). An oral contraceptive tablet was included in the treatment (ethinylestradiolum 0.02 mg + desogestrelum 0.15 mg). Currently, the patient has discontinued oral contraception because of maternity plans. She remains in endocrine and gynecological control.The patient also remains in gynecological control. After gynecological surgery, menstruation occurred every 30–35 days. Two years after gynecological surgery, the patient showed increasing features of hyperandrogenization in the form of hirsutism of the skin, including the face, chin, abdomen, and along the linea alba, and she underwent hormonal and imaging tests of the remaining ovary. BMI was 24 kg/mBRAF mutations are associated with the occurrence of some histological types of papillary thyroid cancer. These authors also reported that the BRAF K601E mutation is unique to the follicular papillary thyroid cancer variant [BRAF mutations , suggesting the presence of a common pathogenetic pathway for all papillary thyroid cancers regardless of location [BRAF gene were reported by other authors [NRAS [HRAS [RET/PTC rearrangements [BRAF K601E mutation in our patient’s DNA.Most of the struma ovarii in which thyroid-type carcinomas arise are unilateral and more often affect the left ovary , as was  variant . Schmidtlocation . Similarlocation . Mutatio authors ,32. Poinrs [NRAS ,34 and HAS [HRAS  genes, angements , were alThere is an ongoing discussion regarding the therapeutic management of malignant struma ovarii. Treatment guidelines remain to be established, and the optimal surgical treatment and postoperative management are controversial. Recommendations are based on individual case reports or review work. Surgery might consist of total hysterectomy with excision of the adnexa and ovaries or sparing surgery including unilateral oophorectomy . RadicalBecause our patient was young and nulliparous, gynecological surgery was limited to unilateral oophorectomy to preserve fertility and hormonal function in the second ovary. The opposite ovary should be examined during surgery to exclude pathological changes. In the present patient, a tumor was detected during gynecological surgery in the opposite ovary that was ultimately identified as a simple cyst postoperatively. In cases of malignant struma ovarii with distant metastases, the consensus is that a more aggressive treatment approach  is warranted. The goal of I-131 treatment is ablation of thyroid remnants and destruction of metastatic foci of thyroid cancer. This enables monitoring for disease using whole-body I-131 scanning and analysis of serum Tg levels . One of Yassa et al. emphasized the role of patient stratification according to the risk of recurrence in the management of malignant struma ovarii. In patients with a low risk of recurrence , they propose unilateral salpingo-oophorectomy and levothyroxine treatment to maintain serum TSH levels at 0.1–0.5 mIU/L [However, high-risk patients with tumors larger than 2 cm or aggressive histopathological features require additional treatment  to detect and potentially treat recurrences .Regardless of the type of treatment, Makani et al. recommends long-term monitoring for at least 10 years including clinical monitoring and assessment of Tg levels . The bioWell differentiated thyroid cancer in struma ovarii is rare and has a high percentage of metastases and recurrences ,9,15. ToMalignant struma ovarii is usually diagnosed during surgery. Immunohistochemistry and molecular tests can help determine risk groups. Because papillary carcinoma in struma ovarii is rare and there are currently no guidelines for the management of this type of cancer, therapeutic decisions should be made individually based on clinical and pathological data.