Arteriovenous malformation (AVM) results from errors in vascular development during embryogenesis; absent capillary beds lead to shunting directly from the arterial to venous circulation. Although it is common in the head and neck region, AVMs located in the parotid gland are quite rare. Here, we report two cases of arteriovenous malformation of the parotid gland that presented to our out-patient setup with swelling in the parotid region and were diagnosed as arteriovenous malformation on histopathological study after surgical resection. The parotid gland is the most common site for salivary gland tumors. But, it is rare to find a vascular anomaly in the parotid gland in adults. The general terms, “Angioma” is still used for both vascular tumors and vascular malformations. Vascular tumors can be either benign or malignant, and on the other hand, vascular malformations can be capillary, venous, arteriovenous, etc.Earlier, all vascular lesions were referred to as haemangiomas. Nowadays, they are classified into haemangiomas and vascular malformations on the basis of the endothelial characteristics. The vascular malformations are either low flow such as capillary, lymphatic, and venous malformations or high flow such as arteriovenous malformations (AVMs) .We hereby present two interesting and rare cases of arteriovenous malformation of the parotid gland.A 32-year-old female presented to our out-patient department with complaints of swelling below the right ear for 8 years, which was insidious in onset and gradually progressive in size. It did not alter in size on chewing of food, opening and closing of mouth, neck movements, or weather changes. It was associated with a mild-to-moderate dull ache radiating to the ipsilateral side of the face.On physical examination, the patient had a soft, compressible, nontender, nonfluctuant, nonreducible, nonpulsatile, well-defined mass in the right parotid region measuring 4 × 3 cm extending from the right lobule to 1.5 cm below the angle of the mandible in vertical dimension and 3 cm in front of the lobule in horizontal dimension . On auscOn ultrasonography, the mass showed multiple pinhead circular calcified foci in it . The colThe patient was taken up for right superficial parotidectomy. Intraoperatively, there was diffuse, dilated vascular channels replacing parotid tissue, with retromandibular vein as the draining vessel to the mass . The facGross examination of the specimen showed hemorrhagic areas on the cut section. Histopathological examination conducted microscopically showed variable size vascular channels. The vessels with thick muscular walls showed hyalinisation and focal calcification. The venous calibre vessels showed intraluminal fresh thrombus, which at places showed recanalization and endothelial proliferation, consistent with A-V malformation .A 43-year-old male presented in ENT OPD with complaints of swelling below and in front of the left ear for 1.5 years, which was gradually progressive in nature, painless, and had alteration in size due to external factors and did not relieve with anti-inflammatory drugs. On physical examination, it was a soft, mobile, compressible, nontender, nonreducible, and nonfluctuant mass measuring around 4 × 4 cm in the left parotid region with no bruit on auscultation .Ultrasonography suggested tortuous and dilated vascular channels in the left parotid region. FNAC yielded 6 to 7 ml fresh blood and suggested benign vascular swelling. MRI showed a well-marginated mass lesion involving whole of the left parotid gland, hyperintense on the T2W sequence with few lobulated hypointense foci within it, closely abutting the left-sided retromandibular vein suggestive of arteriovenous malformation .The patient was taken up for total conservative parotidectomy. The retromandibular vein was found to be the draining vessel and ligated. The facial nerve and its branches were identified and preserved. Postop period was uneventful.Gross examination showed tiny cystic and hemorrhagic areas on the cut section and microscopically showed fibrous-connective and mature adipose tissue showing admixture of malformed blood vessels comprising arteries and venules suggestive of arteriovenous malformation .Blood vessels have a single layer of endothelial cells, and vascular anomalies are the result of defects at the level of vasculogenesis. For the first time, the vascular lesions were classified into haemangiomas and malformation based on endothelial characteristics .To understand the vascular anomalies better, the ISSVA  recently came up with a classification system that differentiates vascular tumors (lesions with manifestations of cell proliferation and increased mitotic activity) from vascular malformations . The latter are classified according to the type of vessel involved, i.e., capillary, venous, lymphatic, and arteriovenous malformations .Another way of classifying these lesions is by their vascular flow characteristics into low-flow  and high-flow lesions .High-flow malformations are further separated into arteriovenous fistulas (AVFs) and arteriovenous malformations. The fistulas are functionally open embryological residues between the arteries and veins and are present at birth. Local trauma, partial surgery, psychological stress, and endocrine event  may cause the fistulas to grow and form malformation , 4.Vascular malformation of the parotid gland is an extremely rare condition with around 50 reported cases in the literature. A case study conducted by Beahrs et al. showed only 0.5% of the cases from 760 parotid tumours to be vascular malformations, and similarly, a study conducted by Byars et al. showed 0.6% of malformation cases from 460 reported cases of parotid tumours [Mostly, the superficial lobe of the parotid gland gets involved in vascular malformation, but in our second case, deep lobe involvement was seen. Also, such patients present with complaints of painless slow-growing swelling on the face in the parotid region with no other associated complaints. Clinically, turkey wattle sign may be present which is the prominence or enlargement of swelling on tilting the head to the ipsilateral side or doing the Valsalva manoeuvre. This sign is considered pathognomonic of vascular malformation, but has been seen in only 3 out of 23 previously reported cases of adult parotid vascular malformation. Basically, the turkey wattle is a red vascular structure in the neck of the male turkey that can increase in size when filled with blood . This siT2-weighted signals with the presence of phleboliths and marked enhancement, as in our cases [Although clinical examination clinches the diagnosis, MRI is considered the gold standard. It shows contrast uptake and hyperintense ur cases .On ultrasound, arteriovenous malformation appears as a heterogeneous lesion with feeding vessels. Colour Doppler displays high vessel density and high systolic flow and multiple sites of arteriovenous shunting, and arterialization of all veins, i.e., pulsatile flow, is always seen in AVM .FNAC (fine needle aspiration cytology) is not very beneficial in diagnosing arteriovenous malformations. Achache et al. obtained cytological samples with needle aspiration in 30% of patients and were noncontributory in all cases. They may be beneficial or be considered gold standard in diagnosing parotid tumours .Angiography is useful in residual lesions after surgery to show the major vessel and embolize if necessary. The angiographic features of AVMs are dilatation and lengthening of afferent arteries with early opacification (shunting) of enlarged efferent veins.The definitive diagnosis is made on the histopathological report. The classical appearance is benign vascular proliferation of endothelial cells in the vessel walls. The vessel lumen is mainly filled with thrombus or calcifications (phleboliths). Arteriovenous malformations have poorly defined margins with thin walls and variable-sized vessels. Direct communications between vessels are also observed. Stroma shows the presence of adipose tissue, lymphoid follicles, and smooth muscles. Immunohistochemistry (IHC) shows positivity to vascular markers such as CD31 and CD34, not carried out in our cases due to cost issues [Surgical resection is the gold standard treatment, as being performed in our cases, but other options include laser, cryotherapy, embolization, and corticosteroids .Embolization or sclerotherapy can also be performed prior to surgical resection to reduce the size of the lesion. It involves injecting a chemical into the AVM to shrink it and reduce the bleeding intraoperatively, but not performed in our cases to avoid damage to the facial nerve, passing through the lesion. Complications include mucosal injury, anaphylaxis, and/or nerve injury , 11. AloThe world literature suggests arteriovenous malformations of the parotid gland are extremely rare in adults. The clinical examination and MRI do help in provisionally diagnosing the disease, but histopathological examination is confirmatory. Surgical resection such as superficial or total conservative parotidectomy with preservation of the facial nerve remains the mainstay treatment till date.


Postoperative chemotherapy during pregnancy after first trimester is essential for patients with initial disease stage 1, grade 2 ovarian immature teratoma and it associates with lower disease progression and recurrence. Postoperative chemotherapy during pregnancy after first trimester is essential for patients with initial disease stage 1, grade 2 ovarian immature teratoma and it associates with lower disease progression and recurrence. Considering the difficulties of surgery and adjuvant chemotherapy in high‐grade cases during the pregnancy, its management is controversial. Here, two cases of grade‐II ovarian immature teratomas in two pregnant women along with their management and outcomes were presented.Ovarian germ cell tumors are considered as a rare female malignancy accounting for less than five percent of all ovarian tumors with an incidence rate of 0.34 per 100,000 women per years.The immature ovarian teratomas of ovaries are originated from the three germ layers . Histological tumor grades, based on the amount of immature neuroepithelium within the tumoral tissue, determine the prognosis of patients directly.In nonpregnant patients, treatments mainly depend on the surgical resection of the primary tumor with adjuvant chemotherapy in high‐grade tumors. However, during the pregnancy, the management of these tumors is controversial considering the difficulties of surgery and also adjuvant chemotherapy in this situation.Here, two cases of grade‐II ovarian immature teratoma in two pregnant women along with their management and outcomes were presented.22 days 1–5 plus cisplatin 20 mg/m2 days 1–5) while the pregnancy continued. In addition, for comprehensive staging, the MDT recommended that complete surgical staging by a fertility‐preserving approach be considered after delivery.The patient was a primigravid 21‐year‐old woman whose complaint was abdominal pain in the sixth week of pregnancy. She has no specific past medical history, and the familial history was negative. The patient underwent abdomen‐pelvic ultrasonography showing a live embryo at the age of 6–7 weeks along with a right ovarian cyst at the size of 140 × 150 mm consists of the solid component. Laboratory assessments were normal. The patient underwent laparotomy and right ovarian cystectomy. Pathological assessment of the ovarian tumor revealed a yellow 150 × 140 mm solid cystic lesion on the right ovary that microscopically there were embryonic tissues from all three cell lines including adipose, cartilage, and neurogenic connective tissues, confirming the diagnosis of grade‐II immature teratoma  due to gestational hypertension followed by eclampsia. During C/S, a complete surgical staging by a fertility‐preserving approach using the complete right oophorectomy and peritoneal fluid sampling was performed. Pathologic assessment of right ovary and peritoneal fluid was negative for malignancy. The general condition of the patient was good, but unfortunately, a male newborn died due to immaturity. Thereafter, the patient followed in our clinic every 2–3 months. After 2 years of follow‐up, all clinic and paraclinical evaluations including tumor markers, imaging of chest, and abdomen‐pelvic are normal. Besides, she got pregnant 15 months after the completion of treatment and now she has a 1‐year‐old baby.3The patient was a multigravid 2, live 1, 29‐year‐old woman complaining of abdominal pain in the second month of pregnancy. The abdomen‐pelvic ultrasonography reported an intrauterine 7‐week live embryo along with an 82 × 64 mm left adnexal cystic mass consisted of a 58* 41 mm solid component Figure . The pat2) + carboplatin (AUC = 5) every 3 weeks. After 4 years of diagnosis, the patient was progression‐free.The CXR was normal. However, the patient revoked her consents to perform adjuvant chemotherapy until the delivery. At the delivery, she underwent C/S, resection of the abdominal mass, and a male baby was born. The surgeon reported multiple tumoral implants in the bowel meso and omentum along with hemorrhagic ascites. Pathologic assessment of the resected mass revealed "metastatic immature teratoma, grade 2 with positive ascites fluid for the presence of malignant cells." After the surgery, there were an increased level of alpha‐fetoprotein (AFP); 117 U/ml (0.2–8.5) and cancer antigen 125 (CA125); 146 U/ml (0–35) with a normal human chorionic gonadotropin (BHCG). Three cycles of chemotherapy based on BEP regimen were administered, and postchemotherapy evaluation showed a significant decrease in the level of AFP and CA125 . A whole‐body CT scan showed evidence of peritoneal seeding and implants around the liver and in sub‐diaphragmatic, paracolic, and pelvic regions and also two bilateral supra‐diaphragmatic lymphadenopathies. The chemotherapy was continued for two more cycles, and the laboratory assessment showed a continuum reduction of AFP and CA125 . Despite the reduction of tumor markers, the CT scan showed evidence of radiologic disease progression. Based on MDT recommendation, the patient underwent a needle biopsy from supra‐diaphragmatic lymphadenopathies showing mature teratoma on the pathological evaluation. Considering the low tumor marker salvage surgical resection of all tumoral masses was recommended by MDT; however, the surgeon reported diffuse tumoral seeding in the abdominal wall and considered the lesion inoperable. Subsequently, the patient received six cycles of paclitaxel (175 mg/m4Pure immature teratomas, first characterized by Norris et al. in 1976, are rare germ cell tumors that involve three germ layers with at least one of the components having an immature appearance.Young et al. showed that the prognosis for ovarian malignancies was not complicated by concurrent gestation if adequate treatment is administered timely.Based on NCCN guideline, patients affected by stage 1, grade 1 ovarian immature teratoma, have an excellent prognosis without adjuvant chemotherapy; therefore, these patients only observe postoperatively. The patients with stage1 and grade 2 or 3 of immature teratoma and the patients with stages 2 or 3 at any grades need postoperative chemotherapy by BEP regimen for 3–4 cycles every 3 weeks. After completion of chemotherapy, if you have complete clinical responses in laboratory and imaging studies, observation is recommended. But if the imaging studies reveal residual disease with normal tumor marker level, both surgery and observation are advisable and ultimately, if the tumor marker level is high and the imaging studies reveal residual disease too, salvage or high‐dose chemotherapy is recommended.Although there is limited experience for using this regimen during pregnancy,5Based on our two mentioned cases and the other data, it seems that postoperative chemotherapy during pregnancy after the first trimester is essential for patients with initial disease stage 1, grade 2 ovarian immature teratoma and it associates with lower disease progression and recurrence.Published with written consent of the patient.


Skin cancer is the most common cancer in Ireland. Our patient presented for removal of a cutaneous lesion on his ear. The histopathological diagnosis was malignant epithelioid hemangioendothelioma. It is very rare for this to present primarily as a cutaneous lesion. Here, we discuss the management of this patient and the surveillance he required. It is important to consider alternative histological diagnoses in patients presenting with cutaneous lesions and how this affects management and prognosis. Skin cancer is the most common cancer in Ireland, with 11 000 new cases diagnosed each year. Nonmelanoma skin cancer (NMSC) accounts for >90% of skin cancer diagnoses . The strMalignant epithelioid hemangioendothelioma (EH) is a tumor of vascular origin, which is exceptionally rare, and it is highly unusual for it to present as a cutaneous lesion. This case report outlines the diagnosis and management of a patient who presented with a lesion on his pinna and was post-operatively diagnosed with EH.An 80-year-old gentleman was referred by his general practitioner with a lesion on his left ear . He undeThe excised specimen revealed a 13-mm tumor which invaded down to elastic cartilage with ulceration and focal necrosis. The tumor was composed of sheets of epithelioid cells with eosinophilic hyaline cytoplasm and intracytoplasmic vacuoles .The mitotic index was up to 6/10 high-powered fields (HPF). No lymphovascular invasion was observed. The peripheral margin was 1.9 mm and the deep margin was 2 mm. Immunohistochemistry showed positivity for CD31 and the tumor cells were strongly positive for ERG. The pathologic features supported a diagnosis of malignant EH.The patient’s case was discussed at the skin cancer multidisciplinary team meeting and he underwent imaging of the area for further evaluation, given the histological diagnosis. Computed tomography (CT) neck showed a 1.3 × 1.2 × 1.3-cm ill-defined soft tissue enhancement above the left external auditory canal involving the subcutaneous tissue and overlying skin . There wThe patient underwent further wide local excision which revealed negative margins. Positron emission tomography CT after re-excision of margins showed no FDG avid areas, indicating no remaining disease. The patient went on to have adjuvant radiotherapy.Malignant EH is a rare neoplasm of vascular origin. It can present in a wide variety of anatomical locations; however, it most commonly affects the liver, lungs and bones. Its prevalence is approximately one in a million globally .The histological and immunohistochemical findings demonstrated in this case are typical of EH. The microscopic features of epithelioid cells with eosinophic hyaline cytoplasm and intracytoplasmic vacuoles are common features of EH . Both ERet al. [Deyrup et al.  proposedWhen EH presents elsewhere, its appearance in visceral organs can mistakenly lead to a wide range of differential diagnoses. It is exceptionally rare for EH to occur on the skin, with few reported cases . Our patThe ear is one of the most anatomically challenging areas to reconstruct. The location of the lesion on the patients concha limits reconstructive options. In this case, options for reconstruction include a full thickness skin graft or a local flap.A full thickness skin graft involves a skin graft taken from a donor site such as the pre-auricular area, the donor site closed primarily and the graft inset over the defect. The limitation of this option is that is does not reconstruct the cartilage.The trap door flap provides a reliable way to reconstruct conchal defects. This flap was first described in 1972 by Masson . It is rDue to the high-risk nature of the lesion, the patient had a follow-up CT neck 3 months after completion of radiotherapy. He will continue to undergo close surveillance with clinical examination and imaging.This case demonstrates a rare neoplasm which unusually presented as a cutaneous lesion. The location of the lesion on the ear is a challenging area for the surgeon to reconstruct. Local flaps, such as the trap door flap as used in this case, provide adequate reconstruction and are cosmetically acceptable. Malignant EH requires aggressive management, given its potential to metastasize to lymph nodes, and it requires continued surveillance after treatment. It is important to consider alternative histological diagnoses in patients presenting with cutaneous lesions and how this affects management and prognosis.None declared.None.Informed consent was obtained from the patient for publication of this article.


Small bowel adenocarcinomas are rare malignant tumors that account for less than 2% of gastrointestinal tumors. Despite a thorough history, physical examination and complete diagnostic workup, the correct diagnosis of small intestinal neoplasm has been established preoperatively in only 50% of cases. Due to the rarity of this disease, there are very few established guidelines for its management and it has been primarily treated the same way as colorectal cancer, even though patient’s prognostic outcome is worse. With new guidelines in 2020, we review a clinical case of a 64-year-old male patient with adenocarcinoma of the jejunum treated in our institution. Small bowel adenocarcinomas (SBA) are rare malignant tumors that account for less than 2% of gastrointestinal tumors . AdenocaWith new guidelines in 2020, we review a clinical case of adenocarcinoma of the jejunum .This work was reported in line with the SCARE criteria .The patient was a 64-year-old Caucasian male presenting at our emergency room (ER) with progressive asthenia, adynamia and paleness for about 1 month. Gastrointestinal bleeding was not reported.Past relevant medical history included colonic polypectomy 2 years prior. The patient had no symptoms at that time, total colonoscopy was performed as part of colorectal cancer screening program and revealed two polyps—tubular adenomas with low grade dysplasia. Relevant family history included one brother with gastric neoplasia.Laboratory tests revealed severe microcytic anemia (Hemoglobin 4.8 g/dl).After stabilization, the patient underwent upper gastrointestinal endoscopy and colonoscopy, both with no alterations.Therefore, a capsule endoscopy was obtained which revealed an ‘obstructing, large, friable and hemorrhagic tumor of proximal jejunum’ and subsequent single-balloon enteroscopy was performed but it was not possible to reach the neoplasia.The staging computed tomography (CT) revealed a ‘nodular image with 48 × 57 × 44 mm, close to the anterior abdominal wall’  with actThe patient underwent urgent laparoscopic segmental intestinal resection and omental lymph node resection. The lesion was a 5-cm mass at proximal jejunum without serosal invasion. Enterectomy was performed with 5 cm proximal and distal margins and end-to-end small intestinal manual anastomosis.No complications were reported and the patient was discharged one week after surgery.Histopathology examination confirmed invasive adenocarcinoma moderately differentiated with no evidence of metastasis on five lymph nodes examined—pT2N0M0, stage I disease—and free surgical margins .Due to the lack of clinical guidelines at that time (2015), the team decided on clinical management similar to colon adenocarcinoma.No genetic testing was performed since there were no clear guidelines.The patient was followed up for a period of 5 years during which his medical history was collected with physical examination, CEA and CA 19.9 were evaluated every 6 months and chest, abdominal and pelvic CT scans performed every year. A total Colonoscopy with Ileoscopy was performed 3 years after surgery and a 6 mm sigmoid polyp was removed—tubular adenoma with low grade dysplasia.No complications or remission signs were found, lung nodules remained stable and the patient remained asymptomatic .SBA is extremely rare. Despite a complete diagnostic workup, the correct diagnosis has been established preoperatively in only 50% of cases .The diagnosis is delayed because symptoms are non-specific and small intestine is not totally accessible to endoscopic examination. It usually manifests through complications like occlusion (40%) or bleeding 24%) [4% [2].Since SBA is often diagnosed at advanced stages, the outcome is generally poor with a 5-year survival of less than 30% and median survival of 19 months [Historically, management of SBA has been extrapolated on the treatment approach for large bowel adenocarcinoma . Risk faThe understanding of this rare cancer warranted the establishment of specific guidelines, like French intergroup clinical practice guidelines 2018) and NCCN Clinical Practice Guidelines in Oncology (2019). Both agree in surgical resection as the curative treatment, depending on factors related to the tumor and the patient. For adenocarcinoma located in the jejunum, segmental resection with lymph node dissection is recommended 8 and NCC. In stagNo studies have evaluated adjuvant therapy after resection. A prospective international Phase III study comparing adjuvant chemotherapy vs observation is currently underway  , 5.NCCN guidelines recommend MMR or MSI testing for all patients with SBA because it can function as a prognostic and/or predictive marker and can help identify patients who should be tested for Lynch syndrome .NCCN recommendations for adjuvant therapy are applied to stage II and III. In stage II, the adjuvant therapy is dependent on MSI-H or dMMR and high-risk features .Patients with locally unresectable disease may undergo neoadjuvant therapy and should be routinely monitored for conversion to resectable disease.The main sites of distant metastasis are liver and peritoneal cavity. It should be considered metastasectomy in resectable metastasis, surgical citoreduction with hyperthermic intraperitoneal chemotherapy and systemic therapy , 5.Follow-up is done during the first 5 years and includes clinical history, physical examination, CEA and/or CA 19–9 measurement and chest, abdominal and pelvic CT scans.The diagnosis of SBA is challenging and frequently delayed.Symptoms are non-specific and it cannot be totally accessed without the use of capsule endoscopy or double-balloon enteroscopy.According to National Comprehensive Cancer Network (NCCN) Guidelines version 1.2020, for local (stages I–III) SBA, like in our clinical case, primary treatment consists of surgical resection with en bloc removal of the regional lymph nodes. These recent guidelines also recommend MMR or MSI testing for all patients with SBA.Recent specifics guidelines provide us uniformization of clinical care.None declared.


Primary cardiac tumors (PCTs) are rare and represent a heterogeneous group of tumors, potentially arising from various parts of the heart. The majority of these tumors are benign (90%), with myxoma being the most common subtype. Cardiac hemangiomas are rare vascular tumors that constitute 1-2% of all benign heart neoplasms. We present a rare case of a 79-year-old woman presented with multifocal embolic brain infarcts secondary to cavernous hemangioma of the mitral valve (MV). Cavernous hemangioma was successfully resected with follow-up imaging at six months, demonstrating no regrowth. There are no pathognomonic signs or findings to suggest cavernous hemangioma of the MV on clinical examination or imaging studies. Surgical resection and histopathologic analysis remain the gold standard for diagnosis and treatment, respectively. Following complete resection, the prognosis is generally favorable with a low recurrence rate, but periodic echocardiography is recommended to detect any potential recurrence. Primary cardiac tumors (PCTs) are rare, with an incidence of between 0.0017% and 0.27% at autopsy, and in most cases are histologically benign . It is hA 78-year-old Caucasian woman presented with acute-onset dysarthria for 2-3 hours. Physical examination was unremarkable except for dysarthria. Vitals signs were normal. She had a past medical history of type 2 diabetes, coronary artery disease, chronic kidney disease, hypertension, and hyperlipidemia. She was admitted four months ago with symptoms of perioral numbness and underwent a full neurologic and cardiologic workup for stroke, which was negative for any significant findings. Subsequent transesophageal echocardiogram (TEE) revealed two MV pedunculated masses, clinically labeled as fibroelastomas. The patient was started on medical management with dual antiplatelet therapy (DAPT) instead of surgery. ECG obtained revealed normal sinus rhythm. The CT scan with and without contrast was negative for acute mass effect or bleeding. The TEE revealed two pedunculated masses that arise from the annulus by the medial side of P2. The larger mass measured 1.0 x 0.7 cm and the smaller one measured 0.5 x 0.5 cm (Figure The patient underwent open thoracotomy for resection of MV masses. During surgery, an inspection of the MV showed 1.5 x 0.5 cm and 0.5 x 0.5 cm calcified lesions along the annulus. These lesions were excised and sent for pathology. Interestingly, the histopathology examination reported it as a cavernous hemangioma Figure . The patPCTs are rare and often found incidentally on echocardiography or chest imaging. It is generally difficult to differentiate between different types of intracardiac mass on imaging. Common differentials include benign papillary fibroelastomas, myxomas, intracardiac thrombus, rhabdomyoma, fibromas, hemangiomas, teratomas, lipomas, cardiac hamartomas, intracardiac pheochromocytomas and paragangliomas , 2. CardHemangiomas are vascular tumors composed of blood vessels lined by endothelial cells. Cardiac hemangiomas are sporadic vascular tumors representing 1-2% of all detected benign heart neoplasms . CardiacCardiac hemangiomas are classified as capillary, cavernous, and arteriovenous. Predominantly intramural hemangiomas are arteriovenous or cavernous, whereas endocardial hemangiomas are capillary. The gross appearance of hemangiomas generally depends on their location. Most intramural hemangiomas appear to be poorly circumscribed masses with a hemorrhagic or congested discoloration , 5. UponCavernous hemangioma involving the MV is an infrequent phenomenon. We report a case of MV cavernous hemangioma presenting as a thromboembolic phenomenon causing multiple brain infarcts. Although cavernous hemangioma is a histopathological finding, it can present as thromboembolic events, heart failure due to obstruction, arrhythmia, pseudo-angina, or mimicking infective endocarditis. Surgical resection of the mass involving the MV is usually therapeutic. Recurrence of cavernous hemangioma has been reported in the medical literature. It is recommended to do periodic echocardiography to look for possible recurrence.


Simultaneous bilateral aneurysms of the extra-cranial internal carotid artery (E-ICA) in a patient are one of the rarest lesions. Here, we report the case report of a 19‑year‑old male with bilateral E-ICA aneurysms. His left-sided, expanding aneurysm of E-ICA at skull base was successfully treated with surgical resection and interposition prosthetic graft placement. Successful surgical repair in this challenging field is safe and can be undertaken with an acceptable perioperative complication rate with excellent results. Aneurysms of the extra-cranial internal carotid artery (E-ICA) are rare lesions. Schechter described only 853 cases in a literature review from 1687 to 1977 . E-ICA aBesides gold standard conventional angiography, multiple non-invasive diagnostic modalities such as CT or magnetic resonance angiography (MRA) are available to assess the exact size and the extent of the aneurysm with the degree of cerebral circulation completion throughout the Circle of Willis’. The preferred method of repairing these types of aneurysms is to excise the diseased vessel part and do exact end-to-end anastomosis if feasible; otherwise, an interposition graft is required to maintain distal cerebral blood flow. E-ICA can be ligated but carries approximately 30% or higher risk of stroke, depending on the contralateral flow . DespiteA 19‑year‑old right‑handed, average-built male was admitted to our hospital because of a pulsatile mass at the left side of his neck. The patient reported that he had noticed this painless, continuously expanding mass one month ago without any provocative trauma. At the same time, he also developed difficulty in swallowing, which was associated with initially solid food but now progressively has increased, even with liquids. The patient also developed hoarseness of voice with left-sided mild ptosis within the last one week. There was no relevant history of any nearby infective lesion, head and neck trauma, or any family history. On clinical examination, a pulsatile, firm, non-tender mass in the left side of the neck was found. On examination, there was also a sensorineural type of hearing loss which was unnoticed by him. No other obvious neurologic symptoms were present and an assessment of the cranial nerves showed no anomalies.After a screening duplex scan revealed a left-sided internal carotid arterial aneurysm, urgent detailed computed tomography angiography (CTA) was done, which showed the presence of aneurysms of the bilateral internal carotid arteries, measuring five centimeters in diameter in the left E-ICA Figure  and threConventional angiography was performed with digital subtraction angiography (DSA) and three‑dimensional (3-D) reconstruction to further define the aneurysms and the status of cerebral circulation. The DSA images confirmed bilateral aneurysms of both E-ICAs. The left E-ICA aneurysm measurements were 25 mm × 30 mm, with a cranial‑caudal length of 50 mm  and segmental classification, the aneurysm was initiated in segment two and extended up to segment three of the E-ICA Figure .The right E-ICA aneurysm was saccular in shape, measuring 30 mm x 25 mm at segment two. No intracranial aneurysmal disease was identified. In view of simultaneous bilateral aneurysmal disease, the case was thoroughly discussed by a multidisciplinary team consisting of neurosurgeons, vascular surgeons, head and neck surgeons, and intervention neuroradiology. We also suspected a potential connective tissue disorder or vasculitis as a possible cause of this, but relevant blood markers reports were inconclusive. It was decided to do staged procedures for the patient. The left E-ICA aneurysm was large, thrombosed, progressive, angulated and causing pressure symptoms; therefore, urgent surgical intervention was planned. The right E-ICA aneurysm was scheduled for endovascular treatment later in an elective manner, as it was relatively smaller and seems amenable for endovascular intervention.The operative procedure was performed under general anesthesia. A standard accepted incision in the neck along the anterior border of the sternocleidomastoid muscle was performed, which extended up to the mastoid  for 72 h in standard doses. Swallowing and feeding were possible on the third postoperative day. Further postoperative recovery was uneventful without any neurologic dysfunction and proper wound healing with a total hospital stay of 21 days.The patient was discharged on an oral single antiplatelet drug  with a successful outcome. He is in regular follow-up for the last two months after discharge and now is scheduled for the elective endovascular intervention of his right-sided aneurysm.The E-ICA aneurysm is an infrequent and dangerous lesion. The etiology may be trauma, inflammation, idiopathic, and rarely iatrogenic. Vascular lesions involving the E-ICA at the base of the skull in the petrous segment are extremely rare, as the petrous part of ICA resides in well-protected housing of bone within the carotid canal without vessel branching in this portion. Moreover, it has been also postulated that the petrous part of the ICA is devoid of atherosclerotic changes. However, the anatomic arrangement of the cervico-petrous junction of the ICA may predispose to injury from blunt trauma. As the relatively free cervical portion of the ICA enters the carotid canal, the vessel gets fixed to the skull base, which may be subjected to stretching forces resembling a whiplash-type injury, commonly seen with excessive head movement around the atlantooccipital joint region. This may lead to a dissection of the cervical part of E-ICA and may also have the potential to form a pseudoaneurysm due to a minor leaking effect within the vessel wall. A pseudoaneurysm develops when a leak in the vessel wall dissects within the wall; thereby, creating a swollen, fibrous adventitial tissue capsule. This swelling has the potential to thrombose later. Such injury may result from several mechanisms, including trauma, infections, or inflammatory conditions. Although most often resulting from blunt trauma, E-ICA aneurysms have also been described as rare sequelae of iatrogenic vessel injury during pituitary and sinus surgery as well as from traumatic gunshot wounds. The lack of structural integrity of the arterial wall of a pseudoaneurysm often permits rapid expansion, causing early various neurological problems and eventual fatal rupture. Other reports of rapidly enlarging aneurysms in children and young age groups have been documented with the associated anomalies or idiopathic reasons. Such cases may suggest congenital disorders, including connective tissue diseases such as Marfan’s syndrome and fibromuscular dysplasia. However, the involvement of the E-ICA has not been documented in this age group -7.Differential diagnosis of these aneurysms includes; angular curvature or looping of the ICA, CCA or subclavian artery, carotid body tumour, angioma, cervical lymph nodes enlargement, peritonsillar abscess, inflammation and tumours of the salivary gland, lateral cervical cyst, neoplastic lesions of adjoining structures as palatine tonsil, pharynx, thyroid gland, skin and subcutaneous tissue. Usual symptoms associated with E-ICA aneurysms include headache, nasal congestion, and midface pressure and pain. A ruptured E-ICA aneurysm or dissecting aneurysm may present with otorrhagia, epistaxis, and cranial nerve compression leading to a neurological deficit. Sudden sensorineural hearing loss has also been reported with aneurysms due to compression and dysfunction of the eighth cranial nerve. The presence of an aneurysm in this region may be demonstrated on CT or MR angiographic images after a primary Doppler duplex study. Conventional digital subtraction angiography (DSA) remains the gold standard investigation for diagnosing such vascular lesions -8.The management of petrous E-ICA aneurysms is very challenging. The primary treatment goals for these aneurysms are minimizing the risks of hemodynamic compromise to the cerebrovascular circulation, thromboembolism with stroke prevention, and fatal bleeding. The safety of adequate treatment depends on the etiology, anatomy of the aneurysm, adequate collateral flow to the brain, and the patient's age. Treatment of this kind of aneurysms has been initially limited to rare instances amenable to surgical ligation versus conservative management. Earlier, most case reports favour conservative management or direct ligation of the artery due to complex surgical territory. Open surgical repair and reconstruction of these lesions are advised and documented by Malikov et al. in a series of 13 patients in 18 years. In their entire series, all cases had a unilateral ICA aneurysm . Rosset Our patient presented with a rapidly growing pulsatile mass, hearing loss, and early features of Horner syndrome with adjoining area pressure symptoms. There was no history of head and neck trauma in our patient, so we initially suspected vasculitis in light of bilateral presentation. Considering his younger age, we suspected connective tissue disorders, especially fibromuscular dysplasia, but there was no typical image of "string of beads" on angiographies. In our case, treatment options are discussed by a multidisciplinary team. As our patient had a large, expanding aneurysm with acute angulation in E-ICA and possible leakage outside, an urgent open surgically repair was chosen. We faced surgical challenges while exploring the neck region to preserve cranial nerves; also, we had to translocate the facial (seventh) cranial nerve while dissecting the ICA in the middle ear. The arterial exposure in petrous bone was problematic as we elected to cut through the middle ear. The mandibular angle was dislocated and later restored to gain control of the distal arterial end due to this large aneurysm with compression of the vessel's distal flow. However, it was challenging to surgically repair this aneurysm in the “Well" of the petrous bone at the skull base. Still, with proper exposure and multispecialty team help, we were able to repair it successfully.An E-ICA aneurysm is an uncommon entity and may be manifested with no or varying symptoms. Safety and long‑term results of appropriate treatment are still evolving due to their rarity and warrant clear indications because of the risk of possible embolization and fatal rupture of these aneurysms. An E-ICA aneurysm at the skull base is a challenging territory for getting distal control and performing the anastomosis during open surgery, which requires various other associated maneuvers. Endovascular therapy may also be considered when expertise is available and in very selected cases only. Surgical reconstructive treatment is feasible with an acceptable risk of stroke and cranial nerve injuries, especially when an aneurysm is located in this challenging territory.


The diagnosis of Bezold’s abscess can be challenging especially when craniofacial malformations imply facial and cervical morphological asymmetries. In addition, craniofacial malformations might predispose to the occurrence and atypical diffusion pathways of suppurative processes originating from abnormally developed temporal bone structures.A 30-year-old female presented with a left laterocervical swelling, worsening over time. The female was affected by Goldenhar syndrome. CT and MRI were performed.CT revealed a dysmorphic os tympanicum and a deep cervical abscess in continuity with its cavity. Drainage of the cervical abscess was performed but a subsequent brain MRI detected a large cholesteatoma that was removed with left lateral petrosectomy.Radiology has a crucial role in the diagnosis and planning of the treatment of Bezold’s abscesses, particularly in syndromic patients. MRI, in this case, helped in diagnosing the presence of the cholesteatoma and consequently appropriately approach the surgical removal. The resulting phenotype is considerably heterogeneous: common features are auricular abnormalities  and hemifacial microsomia.6 The most severe cases also present with eye or spine involvement .7 The spectrum of abnormalities might encompass the central nervous system, cranial nerves and ipsilateral inner ear, internal carotid artery and salivary glands.8–10 Because of external ear malformation, these patients might be more vulnerable to regional infections, especially when a congenital cholesteatoma is present.11 In the most severe forms of facial asymmetry,6 local signs of inflammation, tympanic membrane bulging or hyperemia and otorrhea might not be evaluable or partly hindered by the altered anatomy of the temporal bone. Consequently, infectious processes might be easily overlooked, and severe complications might arise.11Oculo-auriculo-vertebral spectrum 12 due to erosion of the medial mastoid tip at the level of the digastric groove, determining the diffusion of the suppurative process into the posterior cervical space.13 First described in 1881 by the German otologist Friedrich Bezold, this complication has become less common most probably because of early management of mastoiditis with antibiotics as well as with modern imaging tools.14 Acute otitis media, cholesteatoma or necrotizing external otitis are the most common causes of mastoiditis associated with Bezold’s abscess.14,15 Typical clinical presentation is a floating laterocervical swelling frequently but not constantly accompanied by pain, fever, hearing loss, otorrhea and retroauricular swelling.14 Radiology has a crucial role in identifying the suppurative process, in delimiting its extension in the cervical spaces and in recognizing possible features, such as a coexistent cholesteatoma, mastoid bone erosions or peculiar anatomical characteristics that condition the subsequent surgical management.Bezold’s abscess is a well-defined through extreme rare complication of mastoiditisHerein, we report on a patient affected by Goldenhar syndrome who developed Bezold’s abscess as a complication of an os tympanicum cholesteatoma.A 30-year-old female was admitted because of left laterocervical swelling, worsening over time despite protracted large spectrum antibiotic therapy. The female was affected by Goldenhar syndrome, with left craniofacial microsomia, aural atresia, microphthalmia and multiple cervical vertebral anomalies . Her medAt admission, head and neck CT  showed s−2) tissue inside the os tympanicum with milder peripheral contrast enhancement compared to the residual abscess’s walls  cholesteatoma. They present a partially subverted anatomy, and their facial asymmetry might interfere with the identification of the laterocervical swelling leading to a delay in the diagnosis.6 As the association of cholesteatoma and mastoiditis might become an impending condition, in craniofacial malformation patients early imaging with bone CT and diffusion-weighted MRI sequences are pivotal for a prompt, tailored and adequate treatment.These observations strengthen the diagnostic role of MRI, both in characterizing cervical soft tissue involvement and in recognizing the typical DWI hyperintensity of the cholesteatomatous tissue. A proper MR and CT imaging protocol appears to be pivotal, especially in patients with craniofacial malformations, where cholesteatomas are more common and often implicated in suppurative processes.The presence of craniofacial malformations might impair the early detection of a neck abscessA Bezold’s abscess may arise from the os tympanicumAn underlying cholesteatoma might favour Bezold’s abscess formation.For the best surgical management, the diagnostic work-up should include both bone CT and diffusion-weighted MRI.


Intussusception has a low incidence rate in adults. Many cases in adults are caused by tumors. Intussusception results from conditions other than tumors are uncommon. This is the first case report about intussusception that occurred after removing a long intestinal tube (LT).A 69-year-old female complained of “recurrent abdominal pain with reduced flatus passage and frequency of bowel movement for 10 days” was admitted to the hospital. Plain abdominal radiography and abdominal CT upon admission showed intestinal obstruction. The patient’s abdominal pain was not relieved after symptomatic treatments, which involved fluid and electrolyte replacement, LT placement, spasmolytic agents, and analgesics. Hence, surgical exploration was carried out. The patient had a good recovery postoperatively. No abdominal pain or bloating developed after food intake. The patient passed flatus and had bowel movements later. On postoperative day 9, the LT was removed. On the 10th day, the patient suddenly developed abdominal distension and acute abdominal pain. Emergency abdominal CT showed small bowel intussusception. Surgical exploration was then performed. Severe small bowel dilatation located at 1.5 m from the ligament of Treitz was found during the procedure. Intussusception at the site was observed. No color change of the intestinal wall was detected, suggesting that no necrosis was present. So, a manual reduction was done. The patient was discharged on postoperative day 6.This case serves as a warning that the simple action of pulling out the LT might also cause serious complications, which should be given more attention. Unlike intussusception in children, the incidence of intussusception in adults is extremely low, and most of them are secondary . In prevA 69-year-old female patient complained of “recurrent abdominal pain with reduced flatus passage and frequency of bowel movement for 10 days” was admitted to the hospital. Physical examination: slight abdominal distension, hyperactive bowel sounds (about 6 times/min), no tenderness or rebound pain, shifting dullness, no high-pitched bowel sounds, or gurgling. The liver and spleen under the rib cage were not palpated. Laboratory results: WBC: 5.9 × 10E9/L, Neutrophile (%): 66.9, Hemoglobin: 134 g/L, C-reactive protein (CRP): 10.53 mg/L, Carcinoembryonic antigen (CEA): 19.30ng/ml, CA 125: 92.90 U/ml, CA 72: 96.64 IU/ml. Radiological findings: Plain abdominal radiography: obvious pneumatosis in the abdominal cavity with intestinal dilatation  was placed on September 30, 2020 Fig. . AbdominThere are two main reasons why we did not pull out the LT before the surgical intervention: (1) the LT helps with gastrointestinal decompression, which prevents postoperative intestinal dilatation due to poor intestinal function (2) the tube helps with intestinal splinting, which reduces the recurrence of bowel obstruction postoperatively. Postoperative pathology: moderately to poorly differentiated adenocarcinoma. Considering the patient’s past medical history and immunohistochemistry results, the patient’s condition met the diagnostic criteria of colonic metastasis of cardia adenocarcinoma Fig. . GeneticIntussusception mainly occurs in infants and children. Typical manifestations of intussusception involve abdominal pain, bloody stool, and abdominal mass . Adult iEarly diagnosis of intussusception in adults is difficult, and it might be misdiagnosed in an emergency easily. The patient in this case report presented with abdominal pain, reduced flatus passage, and frequency of bowel movement. So, it is difficult to make a definite diagnosis based on the signs and symptoms. CT plays an important role in the diagnosis of intussusception in adults. Intussusception can be diagnosed according to specific signs detected on the image of a CT scan, such as “concentric circle sign”, “comet tail sign” or “kidney-shaped sign”. These signs represent anatomic relationships between the layers of the intestinal wall, the intestinal lumens, and the mesentery. In this case, a “concentric circle sign” in the CT imaging was found, which greatly confirmed the working diagnosis .This case of intussusception is due to the removal of the LT, and there is no relevant literature published at present. We hypothesized that the main causes of the intussusception, in this case, are: (1) The speed of tube withdrawal was too fast. (2) The water in the balloon of the LT was not evacuated completely.Therefore, we suggest that the following steps should be done before removing the tube: (1) During the process of removal, the speed of tube withdrawal should be slow. The tube could even be withdrawn by a section of length each day until it is completely removed. (2) Before removing the LT, the health care professional should make sure to empty the water balloon completely. The purpose of these instructions is to ensure the intestines have sufficient time to adapt to the removal process and to prevent intussusception.Once the diagnosis of adult intussusception is confirmed, surgical treatment is recommended: (1) If there was no necrosis, a manual reduction should be carried out. After the reduction, the bowel should be carefully examined for tumor, polyp, diverticulum, focal necrosis, and other types of lesions. (2) If necrosis of the intestine is found, the manual reduction is not recommended. An intestinal resection should be performed promptly. (3) To avoid squeezing, which can cause the spread of cancer cells to the intestine or bloodstream, patients with suspected malignant tumors should not receive the manual reduction. Intestinal resection and lymph node dissection should be performed instead. After the lesion is removed, the small intestine can be directly anastomosed. In this case, the patient’s intestine was not necrotic. So, a manual reduction was performed .Medical risks are ubiquitous. Health care professionals should be cautious with every step in the medical procedure. This way, the incidence of iatrogenic complications will be minimized. This largely prevents patients from unnecessary suffering.


Echinococcus granulosis is the most common causative agent, whereas Echinococcus alveolaris is rare. Three patients, two females and one male, with primary echinococcosis of the skeletal muscles are described in this report. Mean patient age was 33.3 ± 14.6 years. The disease occurred as soft tissue masses in all three patients, and no hydatid foci were detected elsewhere in the patients. Skeletal muscle echinococcosis should be considered in the differential diagnosis of limb masses, especially in endemic countries.Hydatid cysts are rarely detected in the muscle tissue, even in endemic countries.  Echinococcus granulosus is the most common causative agent, while Echinococcus alveolaris is rare. In humans, hydatid cysts commonly affect the liver (55%-70%), followed by the lungs (18%-35%). The lungs and liver are simultaneously affected in 5%-13% of the patients,Hydatid cysts are parasitic diseases transmitted to humans through dog feces; they are prevalent in countries wherein agriculture and livestock breeding are practiced. Hydatid cysts are rare in patients presenting with muscle swelling or muscle pain without swelling. This should be considered in the differential diagnosis for such patients. The diagnosis of a hydatid cyst should be ruled out, particularly in patients from endemic regions, before undergoing biopsy-aspiration to evaluate a possible abscess, hematoma, or tumor in the muscle. The present study aimed to present the findings of magnetic resonance imaging (MRI) to diagnose hydatid cysts and emphasize the need to consider hydatid cysts in the differential diagnosis of patients presenting with muscle swelling and pain.Case 1: A 33-year-old male patient was admitted to the hospital with complaints of pain and swelling on the lateral side of the right calf. Complete blood count and biochemical tests revealed no abnormal findings. Her medical history was unremarkable, and there were no specific characteristics of the mass. MRI with contrast enhancement was performed for a pre-diagnosis of malignancy/lipoma, which revealed a 95 × 60-mm cystic lesion within the soleus muscle. The lesion appeared hypointense on T1-weighted images and hyperintense on T2-weighted images, suggesting the presence of multiple daughter cysts. Post-contrast-enhanced images demonstrated peripheral contrast uptake. Imaging results indicated a hydatid cyst diagnosis pathologically confirmed after surgical excision . No hydaCase 2: A 26-year-old female patient was admitted to the hospital with swelling in the posterior region of the right thigh. Laboratory tests revealed no abnormal findings. Contrast-enhanced MRI revealed a 145 × 85-mm cystic lesion with membranous structures in the adductor brevis muscle. The lesion was hypointense on T1-weighted images and hyperintense on T2-weighted images. Peripheral contrast agent uptake was detected in post-contrast-enhanced MRI images, indicating a hydatid cyst diagnosis, which was confirmed with histopathological examination after surgical excision . No hydaCase 3: A 41-year-old female patient was admitted to the hospital with complaints of difficulty in walking. She complained of pain and swelling in the left thigh. Laboratory tests revealed no abnormal findings. Contrast-enhanced MRI was performed for the differential diagnosis of lipoma. MRI revealed a 200 × 130-mm cystic lesion with multiple daughter cysts in the adductor brevis, sartorius, and semitendinosus muscles. The cystic lesion was hypointense on T1-weighted images and hyperintense on T2-weighted images, with peripheral contrast agent uptake in post-contrast-enhanced images. Imaging characteristics indicated a hydatid cyst. The definitive diagnosis of a hydatid cyst was pathologically confirmed after surgery . There wE. granulosus in 99% of cases and E. multilocularis in 1% of cases. Hydatid cysts are common in Southern Europe, Asia, Australia, Africa, and the Middle East. Animals such as dogs, wolves, and foxes are definitive hosts, and humans are intermediate hosts. The disease occurs after the consumption of food contaminated with live parasite eggs. Although hydatid cysts can be observed in almost all organs, they less commonly affect muscle structures. The thoracic wall muscles and pectoralis major, sartorius, psoas, quadriceps, and gluteus muscles are possibly affected by hydatid cysts. Psoas muscle involvement is reported as the most common intramuscular form of hydatid cystsHydatid cysts are parasitic diseases caused by ,The pathogenesis of intramuscular hydatid cysts is not clearly understood. Two different theories have been proposed. The first theory suggests direct implantation owing to a dog bite, and the second theory suggests transportation through systemic circulation from the intestines to the skeletal muscle. Iatrogenic contamination of hydatid cysts can be considered if the musculoskeletal system and adipose tissues are involved after surgeryThe rare occurrence of hydatid cysts with primary muscle involvement is explained by the high lactic acid concentration in the skeletal muscles owing to its high activity, which prevents settling of the organism at this siteThe primary clinical sign of muscle hydatidosis is a localized and palpable mass. Additionally, patients can present with pain and restricted movement. Routine hemogram and biochemistry results are often normal. Although eosinophilia is anticipated in parasitic infections, it may not be observed in all patients.The use of imaging methods such as ultrasonography (USG), computed tomography (CT), and MRI for the diagnosis of slow-growing cystic masses in the musculoskeletal system is important for accurate diagnosis. USG is the primary modality used to diagnose such patients. USG is a widely available, noninvasive, inexpensive and can be repeated. Cysts are classified according to the Gharbi criteria on USG (8). According to this classification, type 1 is defined as a pure cystic lesion, type 2 is a detached germinative membrane within the cyst, type 3 is a multi-cystic lesion separated into septate, type 4 is a degenerated cyst with a pseudo-solid view, and type 5 is a calcified pseudo-solid cyst. Daughter cysts, separated membranes, and double-line signs are the most characteristic features of hydatid cysts on USGThe presence of hydatid cysts should be considered when evaluating the lesions involving the muscles. Slow-growing tumors, hematomas, myositis, and abscesses should also be considered for differential diagnosis.Diagnostic biopsy and aspiration should be avoided in cases of suspected hydatid cysts to prevent rupture and spread. Total cyst excision is the primary treatment method. Disease relapse is prevalent after surgical treatment. Postoperative mortality has increased from 0.95% to 3.5% owing to repeated surgical operations and accompanying disordersIn conclusion, a hydatid cyst must be considered in the differential diagnosis of a cystic mass with well-defined margins refractory to medical therapy in the extremities of individuals from endemic regions.


Background: Aggressive angiomyxoma is a rare entity within mesenchymal cell neoplasms, especially in pregnant women. Its main characteristic is the ability to infiltrate neighboring structures and to recur. Case Presentation: We present the case of a pregnant woman who debuted with a genital prolapse in the second trimester of pregnancy. She was diagnosed with bilateral ovarian teratomas and a pelvic mass of which the diagnosis could not be established until delivery. The route of delivery used was cesarean section since the genital prolapse behaved as a previous tumor. After the puerperium, the patient was referred for consultation to complete the study of the mass. The extension study was carried out with a negative result. The patient underwent surgery for tumor exeresis. Hormonal treatment was not administered according to the patient’s preferences. Conclusions: Aggressive angiomyxoma is a benign neoplasm that should be considered in the differential diagnosis of pelvic tumors in women. In pregnant women, the vaginal route of delivery is not contraindicated as long as the tumor does not obstruct the birth canal. The definitive treatment is surgery, preferably performed in a second stage after delivery. Aggressive angiomyxoma is a rare benign mesenchymal neoplasm characterized by its infiltrative capacity. It was first described in the literature in 1983 .The most frequent locations are mainly the vulva, vagina, female pelvis and perineum. It usually has a soft, rubbery consistency and can sometimes be confused with other benign tumors, such as lipomas, Bartholin’s gland cysts, etc. ,4,5,6. IThe objective of this work is to describe a clinical case of aggressive angiomyxoma in a pregnant patient who came to a follow-up of the pregnancy and in whom the diagnosis of angiomyxoma was established after completing the study and excision of the mass in the postpartum period, and to carry out a bibliographic review of the cases published in the literature.The case of a 36-year-old pregnant woman is presented. She was referred to our center in week 8 of her pregnancy for follow-up in a high-risk obstetric clinic because she had had a history of an acute psychotic outbreak and was undergoing psychiatric follow-up. Initially, the patient was asymptomatic, and at this moment the patient took olanzapine 2.5 mg/24 h.In the 12th week ultrasound, both ovaries were enlarged at the expense of cystic formations of mixed content with predominantly dense areas that presented low-level echogenicity and echorefringent areas that suggested the presence of fat and bone tissue. These tumors had measurements of 78 × 63 mm and 63 × 44 mm. The rest of the ultrasound findings were within normal limits  and the combined screening for chromosomal diseases in the first trimester obtained a low-risk result. Tumor markers were requested and the result was negative. This, added to the fact that the patient was totally asymptomatic and the appearance of the images was highly suggestive of teratomas, ruled out the possibility of exploratory laparoscopy.The ultrasound examination at week 20 found fetal findings within normality, with female fetal sex. The previously described tumors were visualized without changes in terms of measurements and ultrasound characteristics. The Doppler of the uterine arteries was pathological, so a follow-up was scheduled at week 26. A magnetic resonance imaging (MRI) of the pelvis was requested which described, in addition to the already known and stable lesions, a complex tumor located in the 7 × 3 × 3.5 cm vesico-vaginal space that suggested the presence of a pedunculated myoma from the cervix as a diagnostic option .At week 26, the patient returned to consultation for control of the Doppler study of the uterine arteries, which were still pathological, for which the sFlt1/PIGF ratio was requested, with a normal result. At this time, the patient reported a prolapsed mass through the vagina, of a soft and rubbery consistency, not painful. This mass was easily reductible, but prolapsed again when standing and during the Valsalva maneuver. That is why, initially, the doubt was raised that it could be a cystocele and a gynecological pessary was inserted for containment until the end of the pregnancy and treatment of the pelvic floor in a second stage if necessary.The patient required hospital admission at week 34 to control a new psychotic outbreak that stabilized and was discharged 10 days later to continue the outpatient pregnancy control. At this moment, a course of corticosteroids was administered.She had a marfanoid phenotype and was referred to Cardiology for evaluation 4 for this reason and due to a sibling’s history. The study of Marfan syndrome was negative, the maternal echocardiography was normal, and the connective tissue disease study was also negative.Elective caesarean section was scheduled at week 37 due to the prolapsed vaginal mass, which was a previous tumor . During A female was born, who weighed 2880 g and whose Apgar score was 9 at the first minute and 10 at 5 min. The postoperative period passed normally, and the patient was discharged 4 days after admission.Subsequently, the patient was referred to the gynecology office after the puerperium, where a control transvaginal ultrasound was performed in which the previously described mass of heterogeneous appearance persisted in a difficult-to-delineate vesico-vaginal space. The internal genitalia were sonographically normal. A new pelvic MRI was requested, in which a complex, high-density 7 × 7 × 7.5 cm mass was observed, which did not seem to depend on any pelvic organ . The extAfter completing the study, surgery was scheduled. Initially, a vaginal exploration was performed under anesthesia in the operating room, and, for the excision of the lesion, the abdominal approach was chosen, carefully dissecting the vesico-uterine plica on the anterior aspect of the vagina until the mass was located to perform the enucleation of the same. Subsequently, a redundant vaginal rhombus was cut and the vagina was fixed to the uterine isthmus-anterior cervical lip .The anatomo-pathological study confirmed the diagnosis of ulcerated aggressive angiomyxoma that affects the deep margin opposite the vaginal mucosa .Regarding the immunohistochemical study, the tumor had the following characteristics: Vimentin (+), Actin (+/− vessels), CD34 (+/− vessels), S-100 (−), Desmin (−), Estrogenic receptors (+) , ProgestIn the present work, a case of aggressive angiomyxoma is described, which constitutes a rare entity within benign mesenchymal tumors, in a pregnant patient, which makes it even more exceptional .Our case also turned out to be complex and unique to our knowledge due to the type of presentation in the form of a genital prolapse and the concomitant existence of two adnexal tumors that made the interpretation of the findings difficult since the origin of said prolapsed mass could not be correctly established in the vagina, generating confusion about whether it was due to said mass, the adnexal tumors themselves or a pelvic organ prolapse .The clinical presentation of aggressive angiomyxoma depends on its location and size, and it is often asymptomatic. In the current case, the patient did not present any symptoms prior to 12 weeks of pregnancy, at which time the tumor was detected for the first time. The differential diagnosis is important, and it should take into account benign tumors, such as lipomas, Bartholin’s cysts, pelvic organ prolapses, etc., as well as with other tumors, such as histiocytoma .This type of tumor is described as “aggressive” due to its ability to infiltrate neighboring structures and its ability to recur. Distant metastatic disease, however, is rare. There are only two cases described in the literature with pulmonary metastases and six mediastinal lymph nodes ,8,9,10. The most appropriate imaging tests for the study of this type of lesion are ultrasound 146 and MRI. At resonance, they present a laminated pattern in T2. In T1, alternating hyper and hypointense linear areas are seen. Rarely, cystic degeneration or intratumoral vessels are seen. In the present case, both complementary tests were performed. Computed tomography is generally used as an extension study and, in the case of our patient, it was negative .The main characteristic of aggressive angiomyxoma in the pathological study is the presence of hypocellular tissue with myxoid stroma in which stellate or spindle cells with elongated cytoplasm with little mitosis and abundant blood vessels are distinguished; this agrees with the anatomopathological findings of the present case.The immunohistochemical study is characterized by a positive result for vimentin, hormone receptors (estrogens and progesterone) positive in up to 80% of cases, S100 (−) , and low Ki. In our case, the sample was positive for vimentin, hormone receptors were positive, S100 was negative, and Ki 67 was less than 1% .Treatment consists of surgical excision of the mass, trying to achieve free edges to avoid recurrences that occur in about 30% of cases. Patient’s follow-up is extremely important since the recurrence rate is high even in surgeries with free surgical margins. In our case, the deep margin of the lesion was affected .Hormone treatment with gonadotropin-releasing hormone (GnRh) agonists can be considered as adjuvant since angiomyxoma has positive hormone receptors. However, it is not clear that long-term treatment is curative or that it prevents recurrences of the disease once it is stopped .During pregnancy, there seems to be an exponential growth due to hormonal influence and, although vaginal delivery is not contraindicated, if it acts as a previous tumor, a cesarean section and surgical treatment of the tumor should be performed in a second stage due to the risk of massive bleeding that it entails if it is carried out in the same act, as it was carried out in the present case .In the present case, the patient was summoned to a consultation for revision and explanation of the attitude to be followed according to the decision of the expert committee. The committee, made up of gynecologists, medical oncologists, radiotherapists, radiologists and pathologists, decided an expectant attitude and follow-up with imaging tests  and markers every 6 months. In the first radiological control, recurrence versus tumor bed with low metabolic uptake was observed. Following new presentation on committee, treatment with GnRh analogues and control at 3 months were decided. The patient was advised and informed of both the benefits and the disadvantages of adjuvant treatment and accepted adjuvant hormonal treatment.Aggressive angiomyxoma is a rare entity, although it must be considered for the differential diagnosis of pelvic tumors detected during pregnancy for which we do not know its etiology.As a general rule, the maternal–fetal results are good and, in principle, the route of choice of delivery would be vaginal unless it exerts as a previous tumor since, in this case, a cesarean section would have to be performed as in our case.The treatment of choice is surgery and close follow-up after surgery is important in order to identify possible recurrences although the most important thing of all is to assess the patient with her peculiarities and carry out an individualized treatment.


Fetal lung interstitial tumor (FLIT) is a rare primary lung mass in neonates. Classical incisions, such as posterolateral thoracotomy or median sternotomy, do not provide optimal exposure of the operative field for the resection of pediatric thoracic giant tumors. Herein, we report a rare case of a FLIT in a full-term male neonate, with complete resection achieved using a hemi-clamshell approach, which provided the required visualization of the operative field. The neonate was transferred to our hospital because of mild respiratory distress, which developed 18-hour after normal vaginal delivery. A mass in his right chest, without a midline shift, was observed on chest radiographs. Computed tomography showed a well-circumscribed solid anterior cervicothoracic mass, with a uniform density and no apparent cysts, diagnosed as a primary thoracic giant tumor. Once the patient was clinically stabilized, we proceeded with right upper lobectomy, using a hemi-clamshell approach, full sternotomy, and anterolateral thoracotomy, on postnatal day 22.Histopathologic examination revealed an 8.5 × 6.5 × 4.0 cm solid mass within the right upper lobe, which was diagnosed as a FLIT. His postoperative recovery was uneventful. The patient was followed up for 1 year, with no complaints or symptoms and no postoperative shoulder dysfunction. Gross total resection of primary thoracic giant tumors can be accomplished in neonates with optimal exposure of the chest cavity using a hemi-clamshell approach. For thoracic giant tumors in pediatric patients, isolated posterolateral thoracotomy and median sternotomy incision approach provide insufficient exposure to achieve complete resection.The patient was a male neonate . He developed tachypnea and mild respiratory distress 18 hour after delivery. A chest radiograph showed a mass in the right chest without a midline shift to the left, which had not been identified on prenatal routine ultrasound. The neonate was transferred to our neonatal intensive care unit for assessment and treatment at Christmas time.n-DPAP), which improved his condition. All serum levels were within the normal range. The patient's alpha fetoprotein , human chorionic gonadotropin (<0.1 ng/mL), urinary vanillylmandelic acid (6.4 µg/mg Cr), homovanillic acid (13.2 µg/mg Cr), and neuron-specific enolase (35.1 ng/mL) levels were not abnormal. Computed tomography (CT) imaging under sedation used midazolam, andn-DPAP showed a well-circumscribed solid anterior cervicothoracic mass of uniform density, 6.6 × 4.6 × 4.8 cm in size, and with no apparent cysts  was observed to be continuous with the right upper lobe (5.0 × 2.5 × 2.0 cm), with moderate adherence to the surrounding tissues at the cervicothoracic junction. Finally, we performed a right upper lobectomy and completely excised the tumor without spillage . The cuThe neonate's postoperative recovery was uneventful. He required minimal ventilator support and was extubated on postoperative day (POD) 2. Oral feeding was resumed on POD 5, and the neonate was discharged on POD 15. Over the subsequent 1-year follow-up, the infant presented no symptoms or observable complaints, with no occurrence of shoulder dysfunction .A recent retrospective cohort study of pediatric patients with cervicothoracic tumors reported outcomes of clamshell or trapdoor thoracotomy.The diagnosis of the mass in our patient was difficult at first due to the absence of remarkable features on the first preoperative CT image. This is why we first suspected other primary lung tumor of neonates or a mediastinal tumor. After careful consideration, we diagnosed congenital primary lung tumors. They are rare, and their prevalence is still largely unknown.In the absence of a clear diagnosis at the time of surgery, surgical resection of the entire tumor was essential. It was very important to avoid spillage of tumors at the time of resection, especially in tumors like pleuropulmonary blastoma, which were relatively resistant to chemotherapy and radiotherapy.In cases with poor overall visualization of the full giant tumor and of the lung hilum, optimal exposure of the operative field is recommended. A posterolateral thoracotomy provides limited visualization and access to the mediastinum.Hemi-clamshell incisions were originally applied for the management of mediastinal tumors or trauma of subclavian vessels.In 1994, Bains et alLimitations of this report should be acknowledged. The incidence of complications about the hemi-clamshell approach in pediatric patients has not been evaluated. In this approach, we need to be careful about the risk of developing musculoskeletal deformities, including chest wall deformities, scapular anomalies, and scoliosis. The standard thoracotomy approach in neonates may lead to injury of the long thoracic nerve and consequent atrophy of the serratus anterior muscle, resulting in many of the musculoskeletal sequelae associated with thoracotomies.As the surgical injury associated with the hemi-clamshell approach may be an etiologic factor in the development of spinal deformity, the use of techniques, such as minimally invasive surgery, which have a low reported rate of spinal and cosmetic problems, might be encouraged.However, with adequate surgical preparation, this approach should be useful to provide optimal exposure for complete resection of a giant tumor.In our case, the hemi-clamshell incision provided good exposure to the entire mediastinum and chest cavity, which allowed us to easily perform the right upper lobectomy with complete resection of the mass.FLIT is a newly identified lung lesion in infants. It is characterized by its morphologic features, including immature airspaces and interstitium, a fibrous capsule, and abundant cytoplasmic glycogen deposition in epithelial and interstitial cells.FLITs are rare tumors in neonates that are difficult to diagnose before surgery due to their nonspecific findings and the fact that they are indistinguishable from lung tumors. Our case suggests that a hemi-clamshell approach should be considered in providing optimal exposure for complete resection of a giant tumor with the possibility of malignancy.


The patient and her baby survived with neither recurrence nor complications until 5 years. Aggressive management for proximal-type epithelioid sarcoma, such as early termination of pregnancy and operation, can improve maternal outcomes.Vulvar proximal-type epithelioid sarcoma during pregnancy is extremely rare; only two reports are available to date. Herein, we describe a 36-year-old woman who presented with a pigeon-egg-sized solid mass with cystic component on the left labia majora at 18 weeks of gestation. The patient underwent tumor resection at 23 weeks of gestation and was diagnosed with epithelioid sarcoma, proximal-type. At 29 weeks of gestation, elective cesarean section, radical local resection of the vulva and vagina, and inguinal lymphadenectomy were performed. After surgery, she underwent six courses of adjuvant chemotherapy (doxorubicin 60 mg/m Particu22 and cisplatin 50 mg/m2; six courses) was commenced every four weeks. The patient and her baby survived with neither recurrence nor complications until 5 years.A 36-year-old healthy woman, gravida 2 para 1 had presented to another hospital with a pigeon-egg-sized cystic tumor on the left labia majora at 18 weeks of gestation in 2016. She had noticed a soybean-sized elastic mass on the left vulva six months previously. The tumor gradually grew to a pigeon-egg size and was diagnosed as Bartholin’s gland cyst. A 5-cm asymptomatic smooth elastic mass was found on the left labia majora and did not extend to the vagina. The tumor was resected at 23 weeks of gestation at Kanoya Medical Center. Moreover, a histopathological diagnosis of PES, proximal-type, was made. The tumor was a solid mass with hemorrhagic cyst, 50x30x10 mm in size. Microscopically, the tumor consisted of a multinodular proliferation of epithelioid or polygonal tumor cells with hemorrhage and necrosis within the nodules . Immunoh3Cancer during pregnancy is relatively rare; approximately 0.1–0.2% of women have cancer during pregnancy . ParticuPES is an extremely rare soft-tissue tumor of mesenchymal origin . It pres2 and ifosfamide 1.5 g/m2). Despite these therapies, she died 5 months after delivery  was administered every 4 weeks. In ES cases, single doxorubicin or doworubicin with ifosfamide (or with other cytotoxic drugs or single agents) is commonly choosen (SMARCB1/INI1 gene located on chromosome 22q11.2 acts as a tumor suppressor gene and play a crucial role in ES (Only two vulvar PES cases during pregnancy have been reported in the English literature . One casdelivery . Anotherdelivery . In our delivery . In casedelivery . Additiodelivery . Regardidelivery . Genitaldelivery . However choosen . However choosen . Recentl choosen . Since, le in ES . Gene thIn vulvar PES cases, it is often misdiagnosed initially as Bartholin’s gland cyst. During pregnancy, misdiagnosis might happen more easily due to rarerity of vulvar PES and concerns over possible maternal-fetal adverse effects of surgical treatment. However, early-stage diagnosis and aggressive approaches such as early termination of pregnancy, surgical resection, and adjuvant therapy can improve maternal outcomes. Moreover, accurate diagnosis and management according to gestational age are important.Yuji Orita: Conceptualization, Writing – original draft. Masaki Kamio: Project administration, Writing – review & editing. Akio Tokudome: Resources, Writing – review & editing. Ikumi Kitazono: Resources, Writing – review & editing. Fumino Ichihara: Resources, Writing – review & editing. Hiroaki Kobayashi: Supervision.The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


Introduction The congenital anomaly of omphalocele, cloacal exstrophy, imperforate anus, and spinal abnormalities (OEIS complex) is rare but well recognized. Hindgut duplications are also uncommon and are not known to be associated with OEIS. We describe a neonate with OEIS who was found to have fully duplicated blind-ending hindguts.Case Report A premature infant boy with OEIS underwent first-stage closure on day of life 6, which included excision of the omphalocele sac, separation of the cecal plate and bladder halves, tubularization of the cecal plate, hindgut rescue with end colostomy, and joining of the bladder halves. Cecal plate inspection revealed two hindgut structures that descended distally, one descended midline into the pelvis along the sacrum and the second laterally along the left border of the sacrum. Both lumens connected to the cecal plate and had separate mesenteries. In an effort to maximize the colonic mucosal surface area, the hindgut segments were unified through a side-to-side anastomosis, creating a larger caliber hindgut. The cecal plate was tubularized and an end colostomy was created. Bowel function returned and he was discharged home on full enteral feeds.Discussion This case represents a cooccurrence of two extremely rare and complex congenital anomalies. The decision to unify the distinct hindguts into a single lumen was made in an effort to combine the goals of management for both OEIS and alimentary duplications. The hindgut is abnormal in OEIS and should be assessed carefully during repair. The management of the collection of congenital malformations including omphalocele, cloacal exstrophy, imperforate anus, and spinal abnormalities (OEIS complex) has evolved over the past decades. Once carrying significant mortality risk,Alimentary tract duplications have been described at all locations in the gastrointestinal tract and are found most frequently in the small bowel. Colonic and rectal duplications are relatively uncommon, accounting for approximately 16 and 4% of enteric duplications, respectively.Here, we describe a neonate born with OEIS complex with duplication of the entire hindgut. The duplicated hindguts developed as two distinct structures, with individual mesenteries, and travelled distally along opposite sides of the pelvis. To preserve colonic surface area, we created a longitudinal side-to-side anastomosis between the two structures to create a single functional hindgut, which was brought up to the abdominal wall as an end colostomy. This is a rare combination of two uncommon malformations.3/7weeks showed similar findings. Fetal echocardiography performed at 324/7weeks demonstrated situs solitus without structural defects, valvar, or ventricular dysfunction. The mother established obstetric care at a nearby facility with labor and delivery services and in discussion with urology and general surgery, a plan was established to take the pregnancy to term if possible.The infant's mother is a 28-year-old G3P1, who was referred to our fetal center for evaluation and counseling due to concern for OEIS complex based on prenatal ultrasound. She was initially seen at 22 3/7 weeks of gestation, and underwent magnetic resonance imaging (MRI) and ultrasound, confirming the diagnosis . At tha4/7weeks in preterm labor with leakage of fluid for over 24 hours. She received one dose of betamethasone and was delivered by cesarean section for breech position. At birth, the infant emerged limp, cyanotic, and apneic with the umbilical cord wrapped around his trunk. Apgar's score at 1 minute was 1, and improved to 9 at 5 minutes, after stimulation and positive pressure ventilation. A Replogle tube and an IV were placed, and he was started on a 10% dextrose solution at a rate of 120 mL/kg/day. Empiric perinatal sepsis coverage was initiated with administration of ampicillin and gentamicin. Birth weight was 2.41 kg.She presented at 33Physical examination was consistent with OEIS ; he hadPostnatal echocardiogram on day of life (DOL) 2 was notable for a large patent ductus arteriosus (PDA) with bidirectional flow, patent foramen ovale with left-to-right flow, as well as mild right ventricular dilation and hypertrophy. At DOL 6, repeat echocardiography showed that the PDA had become small with continuous left-to-right flow. Perinatal blood cultures remained negative and antibiotics were stopped after 48 hours. Deemed stable for surgery, he was brought to the operating room on DOL 7 for first-stage cloacal exstrophy repair. The planned procedures included excision of the omphalocele sac, separation of the cecal plate and bladder halves, tubularization of the cecal plate, hindgut rescue with end colostomy, and joining of bladder halves.In the operating room, two ureteral openings were identified on the bladder plate and ureteral stents were placed. A laparotomy was performed by fully excising the omphalocele sac, allowing better inspection of the peritoneal cavity and understanding of the gastrointestinal anatomy. The terminal ileum was identified by its relation to the cecal plate where it prolapsed externally. Two structures were found descending from the cecal plate, one descended into the pelvis and the second tracked laterally into the left paracolic gutter. They were approximately 8- and 9-cm long. They appeared to emerge from either side of the cecal plate, each with its own orifice, as well as its own mesentery and pair of appendices . The mePostoperatively, the patient was extubated to room air 48 hours after surgery. He remained on parenteral nutrition while awaiting return of bowel function, which occurred on POD 10. Small volume oral breastmilk feeds were initiated and advanced to full volume over several days; feeds were then fortified until he demonstrated appropriate weight gain and was discharged home on POD 30.Subsequent follow-up has been notable for oral feeding intolerance and failure to thrive. During placement of his gastrostomy tube, he was found to have an odd appearance to the liver. Further imaging and workup demonstrated an extrahepatic portosystemic shunt with associated hyperammonemia. This has been addressed by closure by our interventional radiology colleagues. He subsequently underwent tethered cord release for a terminal myelomeningocele, and second-stage exstrophy repair at 14 months of age including epispadias repair, bilateral osteotomies, creation of penoscrotal hypospadias, and abdominal closure. At 2 years of age, he was at 20th percentile of weight for age.This case represents a rare association of two complex congenital anomalies. To date, only one prior report exists of a colonic duplication in the setting of OEIS complex.Enteric duplications are uncommon, and hindgut duplications represent a small subset of this anomaly. Resection is generally recommended for both symptom management and risk of malignant transformation. The true risk of malignancy in duplications is unknown as most of them are discovered in childhood and resected. The rate of malignant transformation of persistent duplications is likely small, but cases have been described.Due to the rarity of both hindgut duplications and OEIS complex, there are no clear guidelines of how to approach and manage this patient's hindgut segments. Current management of OEIS includes preservation of the hindgut in continuity with the rest of the alimentary tract, as an end colostomy to aid with nutrition and fluid management.The broad manner of presentations of colonic/rectal duplications and associated anomalies of the reproductive and genitourinary system, in combination with their low incidence rate, make them difficult to study in a systematic manner, and thus there is no strong evidence to support optimal management strategies. The only other reported case describes a patient in whom the duplicated hindguts were excised, and the terminal ileum was anastomosed to the perineum.


Primary mediastinal synovial sarcomas constitute a rare subset of mediastinal tumors. The diagnosis is often delayed at the time of presentation impacting the five-year survival rate due to its highly aggressive natural history. We report a 22-year old female with a monophasic variant of the primary mediastinal synovial sarcoma.A 22-year-old female, with a two-month history of productive cough, fever, breathlessness, was referred to our center in view of persistent right-sided hydropneumothorax despite multiple thoracocenteses. Examination revealed reduced right-sided air entry with a succussion splash. Imaging suggested a well-defined cystic lesion with fluid, air foci, and multiple septations. A provisional diagnosis of a ruptured hydatid cyst was made and exploratory thoracotomy was planned. Intraoperatively, a well-defined cystic lesion with 350 mL of hemorrhagic fluid, densely adherent to the lung and diaphragm, was found. The biopsy revealed a monophasic spindle cell variant of the primary synovial sarcoma. Follow-up positron emission tomography (PET) on postoperative day 20 showed no residual disease and evidence of metastasis. However, the patient was lost to follow up following one cycle of chemotherapy with ifosfamide.Primary mediastinal synovial sarcomas are aggressive tumors that warrant an early diagnosis for prompt treatment. They usually present with non-specific respiratory symptoms. The gold standard of diagnostic modalities is a molecular panel looking for the translocation - t. However, in low-middle income countries, a biopsy may be more practical, as they are cost-effective. The treatment is surgical resection, with combined chemotherapy and radiotherapy if metastases are present.Our case emphasizes the early detection of this lesion, its mimicry with other lesions, and the impact of early diagnosis. Primary synovial sarcomas are aggressive malignant tumors that are a subtype of soft tissue sarcomas. However, the term “synovial” is a misnomer as the cells of origin do not belong to the synovium, their histogenesis still being largely unknown . ConsideWe present a case of a 22-year-old female with primary mediastinal synovial sarcoma (PMSS) mimicking a hydatid cyst.A 22-year-old female, with a two-month history of sudden onset cough with expectoration, breathlessness, and fever, and no known co-morbidities, was referred to our center in view of a non-resolving hydropneumothorax despite multiple thoracocenteses. Sputum was grey, with mucoid consistency. She reported no hemoptysis, loss of weight, and history of tuberculosis. She reported no symptoms of orthopnea, paroxysmal nocturnal dyspnea, palpitations, and chest pain, ruling out cardiac involvement. History was significant for poor sanitation and environmental surroundings.Examination showed reduced air entry on the right side with a succussion splash, indicating a fluid-filled lesion. Other examination findings were unremarkable. Chest X-ray showed right hydropneumothorax Figure  and lateA preoperative diagnosis of a ruptured infected pulmonary hydatid cyst was made and she was counseled regarding the need for surgery - an exploratory thoracotomy. Preoperative investigations were unremarkable. α-fetoprotein and β-human chorionic gonadotropin (β-HCG) were within normal limits. After informed consent, she underwent surgery, and intraoperatively, we found a thick fluid-filled cyst with solid areas and approximately 350 mL of hemorrhagic fluid  showed membrane positivity for cluster of differentiation 99 (CD99) , and cytoplasmic positivity for B-cell lymphoma 2 (BCL2) , cytokeratin (CK) , epithelial membrane antigen (EMA)  and a proliferative index of 70%-80%.Postoperatively, because of the persistent right lung collapse Figure , a broncOn review, she was symptomatically better, postoperative Eastern Cooperative Oncology Group (ECOG) score was 1 (compared to preoperative ECOG score of 2). Chest x-ray showed complete expansion of the right upper lobe, and PET scan showed no evidence of residual disease or metastasis. She was advised chemotherapy, but after one cycle with ifosfamide, was lost to follow up.PMSSs are extremely rare tumors, with a limited number of case reports in the English literature . These eErroneously named “synovial sarcomas,” the cellular origin of this neoplasm remains unknown, with mesenchymal stem cells and myoblasts hypothesized to be probable cells of origin ,4.The tumor is aptly referred to as a translocation-associated tumor as a translocation t is present in 90% of cases. This translocation gives rise to a fusion protein - SS18-SSX which disrupts gene expression due to aberrant transcription . HoweverAt the time of presentation, PMSSs are usually quite big, with symptoms ranging from a productive cough to hemoptysis. An examination is usually significant for reduced air entry and in case of large cystic fluid-filled lesions as in our case, a succussion splash can be elicited. Our case presented findings consistent with existing literature. We considered the diagnosis of a cystic lesion, with our first differential being a ruptured hydatid cyst given the history of poor environmental hygiene and the prevalence of the same in India and the second being a loculated empyema, given the history of fever, and productive cough. We also considered Hodgkin’s lymphoma and mixed germ cell tumor due to age incidence ,7.The laboratory investigation panel must include α-fetoprotein and β-HCG to rule out germ cell malignancies. X-ray imaging yields a picture of round to oval lobulated mass. However, certain cases, such as ours, can present with hydropneumothorax. CT findings commonly show a well-circumscribed heterogeneously enhancing mass, as in our case . MoleculPrimary mediastinal synovial sarcomas are rare, and difficult to diagnose aggressive malignancies with a significant potential for metastasis and a poor prognosis. Early diagnosis is key and the need to consider it in a differential when a cystic thoracic lesion is warranted. Histopathological and immunohistochemical correlation is advised in cases where a molecular diagnosis is not feasible. Complete surgical resection with adjuvant chemotherapy is the goal of treatment.