A giant intermuscular lipoma, an exceedingly rare occurrence, constitutes a non-malignant neoplasm originating from the mesodermal germ cell layer, with dimensions surpassing 10 cm. Its differentiation from liposarcoma and other malignant tumours is imperative. We present a case involving a 75-year-old woman who initially raised suspicions of liposarcoma due to pronounced enlargement and fullness in the upper quadrants of the left breast. After comprehensive imaging evaluations, the identification of a sizable BI-RADS 4a lesion positioned between the major and minor pectoral muscles of the left breast was found. The definitive diagnosis of an exceedingly rare giant intramuscular lipoma was validated solely subsequent to the surgical excision of the lipoma, through histological analysis. Lipomas are defined as non-malignant neoplasms that arise from the mesodermal germ cell layer, which most commonly occur in specific anatomical regions, including the thigh, shoulder, and trunk.Here we present a rare case of a giant intermuscular lipoma (IL) situated between the major and minor pectoral muscles.l-Thyroxine, Nebilet, and Triplixam. The blood tests conducted fell within the normal range.A 75-year-old woman with a 3-month history of discomfort due to a lump in the left breast was admitted to the Department of Plastic and Reconstructive Surgery at Vilnius University Hospital. The patient underwent thorough examination at the National Cancer Institute, revealing breast asymmetry, with pronounced enlargement and fullness in the upper quadrants of the left breast . PalpatiMammography revealed a heterogeneous tissue composition within the breasts. No discrete lesions or microcalcifications were observed on the right side; however, a cluster was observed adjacent to the nipple. On the left, a partially encompassing fatty density structure of ∼13 cm was noted along the chest wall. Three intramammary lymph nodes were identified in the upper-outer quadrant of the left breast. The skin and nipples exhibited no discernible alterations .Figure 2Ultrasonography (US) revealed a substantial lipoma-like structure, measuring ∼13 cm in the lateral left breast. Adjacent to and slightly above the nipple, an area of mixed echogenicity spanning ∼3.5 cm was observed. Normal lymph nodes were up to 1.5 cm long in the left axilla. Conversely, the right breast displayed no discernible formations and the right axilla contained no anomalous lymph nodes.Magnetic resonance imaging (MRI) revealed a mass between the major and minor pectoral muscles, measuring approximately 6 × 8 × 13 cm in the left breast, and contrast-enhancing foci reminiscent of parenchymal tissue were observed bilaterally across the breasts. No enlarged lymph nodes were identified in the axillary regions . FurtherThe patient underwent surgical intervention under general anaesthesia. The lateral border of the major pectoral muscle was accessed and tracked along its contour. Intraoperatively, an elevated major pectoral muscle was observed, with a subjacent soft mass. A 22 × 11-cm soft yellowish lipoma was carefully excised  by disseAn IL arises from the infiltration of undifferentiated, fully mature adipocytes within the interstitial spaces of muscle fibres and myocytes. The precise aetiology of lipomas remains elusive, although one hypothesis posits that the coalescence of endocrine, metabolic, and genetic aberrations could potentially underlie the exaggerated proliferation of mature adipocytes.,,,ILs commonly exhibit a lobular or dumbbell-shaped appearance arising from varying tissue pressures during growth. Giant lipomas measure ≥10 cm, and a lipoma should be considered as a differential diagnosis of such large tumours.,Accurate diagnosis and treatment of an IL necessitate the use of appropriate imaging studies, including US, computed tomography (CT), MRI, and histological examination. US has emerged as the preferred imaging modality for lipomas owing to its cost-effectiveness, non-invasiveness, and convenience. The echogenicity of an IL is determined by the uniformity of the mixture comprising adipose and other tissues within the mass. However, relying solely on US data does not yield definitive results. Therefore, the incorporation of CT and MRI is advised for further assessment of the lesion features. On short T1 and long T2 signal MRI, an IL appears as a mass with signal intensity consistent with adipose tissue. Meanwhile, on CT, an IL typically manifests as a homogeneous low attenuation mass. Conversely, malignant liposarcomas demonstrate low to moderate attenuation on imaging, often presenting an inhomogeneous appearance characterised by intermittently disrupted streaks, primarily attributable to cystic regions containing myxoid material. It is important to note that despite negative imaging findings, a liposarcoma can still possess malignant characteristics.Interventional therapy may be considered unnecessary if an IL does not exhibit symptoms of compression resulting from an excessive volume or a specific anatomical location. In cases of symptom manifestation and vital adjacent organs, tissues, blood vessels, and nerves being subjected to compression, interventional treatment is the primary approach. There are various interventional methods for ILs, including sodium deoxycholate injection, combined steroid and isoproterenol injection, and liposuction. However, surgical resection is the most effective treatment. An IL is characterised by a well-defined pseudocapsule that facilitates surgical dissection.Giant ILs between the major and minor pectoral muscles are rare but cause discomfort, pain, and sensory disturbances. US, CT, and MRI are valuable practical tools for diagnostic confirmation. Complete surgical excision followed by histological analysis is the preferred treatment approach, as it not only provides a definitive diagnosis, but also plays a crucial role in minimising the risk of recurrence.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.None.


Phlegmasia cerulea dolens is a rare and severe form of deep venous thrombosis (DVT), characterized by an extensive thrombus burden and compromised venous outflow. We present the case of a 28-year-old male with a history of bilateral lower extremity DVTs and multiple venous stents who presented with acute-onset pain and swelling in the left lower extremity. Diagnostic imaging confirmed an acute DVT extending throughout the left lower extremity, including the external iliac vein. Given the diagnosis of phlegmasia cerulea dolens, a multidisciplinary approach involving interventional cardiology, orthopedic surgery, and vascular surgery was adopted. Intravascular ultrasound (IVUS)-guided thrombus removal and angioplasty were performed to restore venous outflow and improve limb perfusion. The procedure successfully removed a significant amount of thrombus and improved flow throughout the venous system. The patient exhibited an excellent clinical response, with pain resolution and improved perfusion. This case highlights the challenges and effectiveness of a combined intervention in managing complex phlegmasia cerulea dolens cases with previous venous stents. Phlegmasia cerulea dolens is a rare and severe form of deep venous thrombosis (DVT), characterized by an extensive thrombus burden and compromised venous outflow. It is associated with significant morbidity and mortality if not promptly recognized and managed. Cases with previous venous stents further complicate management due to under-expansion and the potential for thrombus formation within the stented segments .Various interventions have been employed to address phlegmasia cerulea dolens, including systemic anticoagulation, catheter-directed thrombolysis, thrombus extraction, and venous stenting . HoweverIn this case report, we present a challenging case of phlegmasia cerulea dolens in a young male with a complex medical history of bilateral lower extremity deep vein thrombosis (DVT) and multiple venous stents. The patient presented with acute-onset pain, swelling, and signs of ischemia in the left lower extremity. Our management approach involved a multidisciplinary team, including interventional cardiology, orthopedic surgery, and vascular surgery, to address the extensive thrombus burden, under-expanded stents, and compromised venous outflow.A 28-year-old male with a history of bilateral lower extremity DVTs and multiple venous stents presented to the emergency department with acute-onset pain and swelling in the left lower extremity. The patient reported intense pain in the left lower limb, rated at 10/10 in intensity, along with significant edema and purple skin discoloration extending from the foot to the groin. On examination, the patient appeared to be in moderate distress due to pain. The left lower extremity exhibited significant edema, violaceous discoloration, and tenderness upon palpation  to assess the situation, revealing a substantial thrombus burden within the iliac and femoral venous stents, leading to compromised outflow. The stent at the entrance of the inferior vena cava (IVC) exhibited under-expansion, potentially causing the obstruction, along with telescoped and under-expanded segments. The IVUS also indicated a reduced luminal area on the left side where the stent was positioned, suggesting an associated outflow issue responsible for the obstruction. To address the situation, an 8-French sheath was introduced into the contralateral right popliteal vein, enabling IVUS evaluation using a 12-French sheath. To capture any existing clots, an INARI system disc  was deployed in the IVC.We advanced to the left popliteal vein and deployed a 20-French flow retriever device. Through multiple passes across the popliteal, superficial femoral vein, femoral, iliac, and IVC, we successfully extracted a significant amount of thrombus and clot throughout the entire left leg using the INARI system (Figure Following these interventions, we observed restored outflow. To address the primary concern of poor outflow, we performed transluminal angioplasty using a 12 mm × 40 mm Atlas balloon along the entire stent. This procedure aimed to improve stent size and caliber, thereby enhancing outflow. After thrombus removal and angioplasty, the angiogram demonstrated a notable improvement in flow throughout the entire venous system. Post-intervention, the swelling and pain slowly resolved, and the posterior tibial and dorsalis pedis pulses were palpable. The violaceous discoloration also improved, and the patient showed an excellent clinical response Figure . The patPhlegmasia cerulea dolens is a rare but critical condition that requires urgent intervention to restore venous outflow and prevent limb-threatening complications. The management of phlegmasia cerulea dolens has evolved over the years, and various interventions have been employed to address the complex pathophysiology and thrombus burden associated with this condition .Systemic anticoagulation is the cornerstone of initial treatment for phlegmasia cerulea dolens. It aims to prevent thrombus propagation and reduce the risk of pulmonary embolism. However, anticoagulation alone may not be sufficient in cases with an extensive thrombus burden and compromised venous outflow . In suchCatheter-directed thrombolysis has been used in the management of phlegmasia cerulea dolens to rapidly dissolve the thrombus and restore venous patency. It involves the infusion of thrombolytic agents directly into the affected vein under radiological guidance. However, this approach carries the risk of bleeding complications, especially in patients with recent surgery, active bleeding, or coagulopathy . HoweverThrombus removal using mechanical devices or aspiration techniques has emerged as an alternative or adjunct to thrombolysis in cases of phlegmasia cerulea dolens . The useAlong with thrombus removal, angioplasty plays a crucial role in restoring venous outflow and optimizing limb perfusion in phlegmasia cerulea dolens. Transluminal angioplasty using balloons of appropriate size and length can effectively expand under-expanded stents and improve venous flow . In our In cases of phlegmasia cerulea dolens, which is characterized by underlying venous stenosis or insufficient vascular diameter, venous stenting has been employed. Stents serve as structural support to prevent vessel collapse and maintain venous patency. However, in instances of extensive thrombus burden, the presence of under-expanded stents can further compromise venous outflow and contribute to the development of phlegmasia cerulea dolens . In our In our case, we utilized the INARI system, a mechanical thrombectomy device, to facilitate thrombus extraction. The INARI system comprises a retrievable nitinol disc designed to capture and remove thrombus from the venous system. It has demonstrated successful outcomes in various venous thromboembolic disorders, including phlegmasia cerulea dolens. By deploying the INARI system in the inferior vena cava, we achieved effective thrombus capture and extraction.Phlegmasia cerulea dolens is a rare condition, and the available literature on its management is limited. However, various case reports and small studies have reported successful outcomes using different interventions, such as catheter-directed thrombolysis, thrombus extraction, angioplasty, and stenting . The selIn conclusion, phlegmasia cerulea dolens is a rare and severe form of deep venous thrombosis that requires prompt recognition and a multidisciplinary approach for effective management. Our case highlights the challenges associated with previous venous stents and the successful utilization of intravascular ultrasound-guided thrombus removal, angioplasty, and the INARI system in restoring venous outflow and improving limb perfusion. Further research and larger studies are warranted to optimize treatment strategies and enhance outcomes in this uncommon and severe condition.


International Journal of Surgery Case Reports with great interest [We read the article by Dr. Almodumeegh and colleagues titled “The potential pitfalls of synovial sarcoma mimicking intraneural ganglion cysts: A case report and literature review” in the interest . The stu1Magnetic resonance imaging (MRI) plays a pivotal role in the differential diagnosis of soft-tissue tumors and is characterized by the presence or absence of contrast effects which is beneficial for differential diagnosis. Additionally, MRI with contrast is beneficial for differentiating cystic lesions from solid tumors . In gene2In the present case, we recognized the type of operation as an unplanned excision. Usually, the main reason for unplanned excisions is the absence of a pathological examination followed by a pathological diagnosis. We understand that the differential diagnosis from MRI in this case was difficult and limited, therefore, needle biopsy or open biopsy was essential as the next step. SS is defined cytogenetically as the presence of the SS18::SSX 1/2/4 fusion gene . In addi3An unplanned excision often occurs in cases with a superficial location and small size . The rec4In the diagnosis of some sarcomas, including SS, atypical symptoms and imaging results with pitfalls are occasionally encountered. In such cases, we should return to the principles of diagnosis to complete the process of biopsy, followed by pathological examination.Although it is important and valuable to avoid missing the tail sign, we must keep the Jaffe's triangle composed of surgeons , radiologists (various image findings), and pathologists  in mind.Not applicable.None.All authors contributed to the study concept and design, data collection, data analysis and interpretation, and manuscript writing.Jiro Ichikawa.Not applicable.The authors have no conflicts of interest to disclose.


A 21-year-old woman presented with 1 year history of progressive dorsal right foot pain with no recollection of trauma. The physical exam did not reveal any unusual appearance to the plantar or dorsal skin surfaces. Specifically, no scars were seen. Foot radiographs were unremarkable. The presumed etiology after Doppler ultrasound (US) and MRI was a likely venous or venolymphatic malformation. She received 2 rounds of sclerotherapy 12 months apart with transient symptomatic clinical improvement. After the second sclerotherapy treatment, repeat MRI revealed dorsal extension of the lesion with skin involvement, prompting referral for surgical intervention. At surgery, a 3 cm wood splinter was found surrounded by granulation tissue. After showing the patient the resected splinter, she recalled stepping on a wooden broomstick that punctured the bottom of her foot in her childhood. She stated she also remembered pulling a splinter out of her foot. This case demonstrates the unusual and rare appearance of a chronic retained foreign body creating a cystic lesion in the foot presumed to be a low-flow vascular malformation on US and MRI. Venous malformations are a rare form of congenital vascular malformation present in approximately 1-2 per 10,000 births A 21-year-old woman presented to her primary care physician with right foot pain, functional impairment, and a soft, mobile, nodular mass on the dorsum of her right foot. The US revealed 2 irregularly shaped cystic lesions with intralesional echogenic soft tissue with an arterial and venous flow on Doppler . For furInterventional Radiology was then consulted as the US and MRI features best fit with a low-flow vascular malformation. The lesion morphology was very unusual, and the patient was specifically asked during the Interventional Radiology consultation if she had any history of trauma to her right foot in the past, which she denied. Physical exam was notable for an approximately 1 cm fluctuant nodular lesion of the dorsum of the foot between the second and third metatarsals that was mildly tender to palpation. There was no skin color change or scar about this lesion nor the plantar aspect of her foot opposite the dorsal nodule.The patient underwent sclerotherapy with 1.8 mL of doxycycline at 10 mg/mL. The associated venography demonstrated serpentine filling of the lesion with minimal venous outflow . She repA second sclerotherapy treatment was performed 1 year later with an additional 1.8 mL of doxycycline, with follow-up exams demonstrating a similar pattern of partial symptom resolution followed by recurrence . Follow-Additionally, after further detailed inspection of the plantar aspect of her foot in the operating room, a faint skin defect suspicious for a remote puncture site was identified. Discussion with the patient of the findings following surgery prompted her to recall an event from her early childhood in which she experienced intense pain after stepping on a broomstick but was unaware a portion of the wooden splinter that penetrated her foot was not completely retrieved.Several aspects of this case complicated the correct diagnosis. The most critical information not revealed until surgery was a history of penetrating trauma to the foot at the site of her lesion. The length of time this foreign body was present in the foot without significant symptoms, the presentation on imaging, and the partial response to sclerotherapy also confounded the correct diagnosis.The US and MRI with contrast were concordant but not classic in appearance for a low flow vascular malformation. While multiple rounds of sclerotherapy are frequently required to achieve symptom resolution, it was unusual that her symptoms were so poorly controlled after 2 treatments. This, coupled with the imaging findings of interval increase in size and dorsal migration led to surgical exploration. The wood splinter likely penetrated the plantar skin and migrated dorsally between the second and third metatarsals where it was clinically dormant for many years. The orientation and location of the splinter were likely parallel to the tendons, masking its appearance. The chronic granulomatous foreign body reaction and resultant neovascularization ultimately led to the clinical and imaging findings of this case.This case demonstrates the importance of a comprehensive history and physical exam when dealing with an unusual appearing low flow lesion on US and MRI. Re-assessing the history and physical after no considerable improvement in symptoms from sclerotherapy treatments could have helped differentiate a chronic foreign body reaction mimicking a low flow vascular lesion.Written informed consent was obtained from the patient to publish this case report.


Stafne defect also known as Stafne bone cavity is a lingual surface depression generally found in the posterior mandible. This entity is usually unilateral and asymptomatic, found during routine dental radiographic evaluation. Stafne defect appears as a well-defined, oval, corticated entity located below the inferior alveolar canal. These entities are the inclusion of the salivary gland tissues. In this case report, we present the case of a bilateral Stafne defect that was asymmetrically located in the mandible and was incidentally detected on the cone-beam computed tomography scan taken for implant treatment planning. This case report highlights the significance of three-dimensional imaging and correctly diagnosing the incidental findings within the scan. Stafne defect or Stafne bone cavity (SBC) is classically described as an asymptomatic, unilateral depression on the lingual surface of the posterior mandible located between the inferior alveolar canal and the inferior border of the mandible. Several variations and atypical locations have been reported. In this case report, we present one such case of a bilateral Stafne defect which was asymmetric and presented in atypical locations and highlight the importance of imaging in the diagnosis of this entity. In 1942, Edward Stafne described an entity found unilaterally in the posterior region of the mandible, which was a radiolucent cavity between the mandibular angle and the third molar and below the inferior alveolar canal and referred to it as SBC . The purA 62-year-old male visited the University of Florida, College of Dentistry with a chief complaint of missing teeth in his upper and lower left jaw regions. He had no significant medical history and was completely asymptomatic. On routine panoramic radiographic examination, we found a well-defined, partly corticated radiolucency in the left mandible superimposed over the inferior alveolar canal at the level of the edentulous space of 19 Figure . The mulThe Stafne defect is a rare anomaly found as a depression along the lingual surface of the mandible. It is believed that the cavity could be developmental as the literature reports the inclusion of tissues of the submandibular salivary gland or even fatty tissues. These bone cavities are incidentally identified during routine dental evaluation as these patients are generally asymptomatic ,5. AlthoThe literature has reported varied prevalence of SBC; however, most studies have reported a lower range of around 0.10% to 0.48%, with a higher incidence in the male population . AlthougThe etiopathogenesis of SBC is not well studied and documented in the literature. However, several different theories have been proposed to understand the development of the cavity, which include the possibility of it being developmental in origin with failure of proper ossification versus salivary gland inclusion versus variation in anatomy at birth considering it congenital by nature .The diagnosis of the Stafne defect is usually based on radiographic imaging. Routine dental imaging including panoramic radiograph(s) is usually enough for a preliminary radiographic diagnosis; however, Stafne defect can occur at atypical locations, including the anterior mandible , the ramVery few cases with bilateral SBC are published in the literature, with only three other case reports reported so far according to a PubMed search. A study by Queiroz et al. reported a bilateral Stafne defect in the anterior mandibular region apical to canine and incisors . AnotherSBCs do not require any treatment. The most common management recommendation is observation. Periodic radiographic imaging with conventional modalities may be performed to monitor the size and shape of the defect over time .SBC can be present bilaterally with asymmetric sizes and at different locations; therefore, careful radiographic evaluation is imperative in reaching the correct diagnosis. Three-dimensional imaging modality can play a significant role in the differential diagnosis when multiple such defects are present, and when there is a need to rule out any other pathology in the area.


Lipofibromatosis is a recently recognized slow growing rare pediatric tumor. Paucity of its cytological description in the literature leads to its pre operative misdiagnosis and further incomplete management. A twelve-month-old female presented with a rapidly progressive mass in the right thigh and buttock region. On examination, the mass was huge and involved the medial, posterior and lateral aspects of the thigh. The cytological smears showed mature adipocytes with few spindled out cells. FNA was reported as a lipoma, corroborating with the radiological presumptive diagnosis. However, histopathological and immunohistochemical features favoured a diagnosis of Lipofibromatosis. The cytological smears were reviewed and a cyto-histo correlation was established. The diagnosis of Lipofibromatosis rests upon classical cytological features in a clinically and radiologically suggestive picture. An early and accurate diagnosis if established can help the surgeon plan excision with wider margins as incomplete excision is associated with a high rate of recurrence. Lipofibromatosis (LF) is an uncommon pediatric soft tissue tumour, more often seen in males M: F -2.7:1) (.7:1 (2).A twelve-month-old girl presented with a rapidly progressive painless mass over the right gluteal and upper thigh region. The mass was seen at birth in the right buttock and was of the size of a lemon to begin with; however, it had rapidly grown over the last six months and now extended over the thigh. The patient gave a history of incision and drainage of that site in a private hospital in view of it being reported as a gluteal abscess with mild vascularity on Ultrasound. On examination, the mass was diffusely involving the medial, posterior and lateral aspect of the right upper thigh extending into the gluteal region, measuring 17 x 14 cm with an ulcer on the overlying skin . No thriFine needle aspiration cytology (FNAC) was done and showed a moderately cellular lesion composed of large fragments of mature adipose tissue admixed with singly scattered and groups of plump to spindle cells . No lipoLipofibromatosis was first described in the year 2000 by Fetsch et al. in a series of 45 cases, which were previously reported as infantile or juvenile fibromatosis of the non-desmoid type . This tuThe lesion is known to occur from birth to early second decade of life but can also be seen congenitally. Though the median age of presentation in the two large series is 1 year, the majority of the cases usually present by 3 years of age and 18% of these lesions are congenital ,3. Our cThe etiopathogenesis of LF is still unclear. No recurrent genetic alteration has been detected. Al-Ibraheemi et al. in their study on 20 cases of LF found recurrent FN1-EGF gene fusion in four of their cases. This genetic aberration was found to be characteristic feature of calcifying aponeurotic fibroma. Based on this, they suggested that some cases of LF actually represent early stages of calcifying aponeurotic fibroma with no calcification. Their study found 8 other genetic fusions which are known to activate the PI3K-AKT-mTOR pathway . A singlRadiological imaging can help distinguish LF from other entities, though not always diagnostic. Ultrasound is usually nonspecific. MRI is the imaging modality of choice, which shows LF as a well-defined mass with hyperintense signalling that is isointense to fat on T1- and T2- weighted images. These signals however vary depending upon the proportion of adipocytic and fibroblastic components. Hence, MRI is only suggestive and therefore partially helps in pre-operative planning. The cytological features of this entity are not reported often in the literature and the definitive diagnosis rests upon histopathology. The tumor is associated with high recurrence rates of about 72% . No metaThe cytological indicators of LF are presence of skeletal muscle fibres, mature adipose tissue and benign appearing spindle cells clusters in close proximation. The background may be lipidaceous or myxoid. A few stromal fragments may also be noted. No true lipoblast is seen. There is absence of nuclear pleomorphism, necrosis and mitosis. Grossly, LF is unencapsulated with poorly defined margins. The cut section is yellow with whitish streaks. The microscopic sections show abundance of mature adipose tissue (> 50%) traversed by a fibroblastic component. The latter is comprised of bland spindle shaped cells and sometimes may show myxoid change. Small univacuolated cells, which are actually degenerating adipocytes or lipid rich fibroblasts, are seen at the interface of fibroblast and adipose tissue. These may be mistaken for lipoblasts. No nuclear atypia or necrosis is seen. Mitotic figures were not seen in our case but have been reported by Fetsch et al. in one third of the cases ,4,6. ImmOur experience with the current case has led to the conclusion that Lipofibromatosis may be congenital, can occur in females and can present as a large mass involving the extremities. The diagnosis can be made with near precision on FNAC if classical cytological features are present along with clinical and radiological suspicion. A pre-operative diagnosis of this rare entity can help the surgeon plan complete resection with wider margins to prevent future recurrence.


A 5-year-old girl presented with jaundice and loss of appetite. Physical examination revealed that she had a palpable liver 4 cm below the right costal arch. There were no ascites, cervical adenopathy, or splenomegaly. Laboratory values showed elevated liver enzymes and direct bilirubin.After ultrasound and magnetic resonance cholangiopancreatography examinations, parasites and sludge were considered. Endoscopic retrograde cholangiopancreatography (ERCP) was initially performed, and when adequate patency was achieved after sphincterotomy, the procedure was continued with a transnasal scope. The first pathology report showed an epithelial fragment of the intestinal mucosa and necrobiotic material. Cholestasis improved rapidly after the procedure.The common bile duct was cannulated with a transnasal scope, biopsies were taken, and new stents were inserted during the second ERCP. Abdominal computed tomography and magnetic resonance imaging were unremarkable . After Video 1 Use of endoscopic retrograde cholangiopancreatography in a pediatric case and consideration as a diagnostic tool .RMS is an unusual tumor of the bile duct in children. Intermittent jaundice, fever, and anorexia are typical symptoms. The radiologic appearance of the lesion resembles that of a congenital choledochal cystEndoscopy_UCTN_Code_CCL_1AZ_2AN


Paratesticular cystadenomas remain a very rare entity, typically presenting as a painless mass, often indistinguishable from the testicle. As such, the predominant management seems to be complete excision via various approaches, which often proves curative. Given its rarity, post-operative surveillance has not been standardized; most patients and providers elect a more conservative surveillance approach. Based on the available literature, this seems appropriate, given the lack of morbidity or recurrence associated with these types of tumors. Cystic structures of the testis and epididymis are often benign and vary in etiology. The most common benign primary tumor of the epididymis is the adenomatoid tumor, followed by papillary cystadenoma . The litA 65-year-old male with a history of medically managed atrial fibrillation incidentally discovered a firm right para-testicular mass that resulted in intermittent right groin discomfort for six months. Physical examination was notable for normal and descended testicles bilaterally, a right palpable para-testicular mass that was mildly tender on palpation, a cyst of the left epididymal head, and no other obvious testicular or scrotal lesions. The patient ultimately underwent a scrotal sonogram demonstrating what was originally suggested to be a 2cm right-sided spermatocele and a 1.5cm left epididymal cyst (Figure Fewer than 10 identifiable case reports comment on para-testicular serous cystadenomas, which is not to be confused with papillary cystadenoma as it lacks the proper ovarian stains. Our patient represents another case to further display the variable presentation and management strategy of this rare tumor type. The most common presentation is of a testicular mass , one bilThe differential for masses of the genitalia is broad, especially, within various benign histologic types and this is further limited by an inability of imaging to differentiate solid benign masses from malignant. Our case brings forth yet another unique histologic type of a para-testicular mass, which, in this instance was associated with the epididymis. Given the rarity of these tumors, as well as most of their treatment being based around surgical excision there is not a well-defined management strategy for the undifferentiated patient at the time of original presentation. Fortunately for this population, in our review of a few case reports that do exist, it would seem that regardless of specific location, the ovarian serous cystadenoma sub-type maintains a benign course following surgical excision. Therefore, the need for close postoperative follow-up does not seem to be paramount, however, more cases would need to be established with increased follow-up to confirm this observed trend.


Large-cell calcifying Sertoli cell tumor (LCCST) is a rare, testicular sex cord, gonadal stromal tumor that belongs to the histological subgroup of Sertoli cell tumors. LCCSTs may involve malignant potential. However, metastasis is a rare phenomenon. We describe a case of benign late-onset LCCST with testis-sparing surgery. Modern imaging techniques were useful for considering organ-sparing surgery.The ultrasound of a 37-year-old man disclosed a sharp demarcated and strong hyper-echoic lesion sized 1.5 cm, with broad dorsal acoustic shadowing. Testicular tumor markers, including lactate dehydrogenase (LDH), alpha-fetoprotein (AFP), and Beta-human chorionic gonadotropin (ß-HCG) did not reveal any pathological finding. Contrast-enhanced MRI of the pelvis showed a ring-shaped tumor with a strong contrast medium enhancement. Sections of the tumor showed a hard mass with a white calcified ring. A frozen section examination of the testicular tumor did not indicate malignancy. Histologic examination revealed a prominent and noticeable calcification of approximately 3 mm thickness. Tumor cells presented in the form of solid nests, tubules, and cords.Our present case differs from previously reported LCCST cases because the tumor was unilateral, smaller in size, and presented in an older patient.  Testicular germ cell tumor (GCT) is a rare disease, accounting for no more than 1.5% of all neoplasms in males but representing the most common tumors in adolescents and young men in Western countries .Radical inguinal orchiectomy is the standard treatment for malignant testicular neoplasms. However, benign tumors can safely be managed with testis-sparing surgery (TSS). A thorough preoperative evaluation of a testicular mass is thus mandatory for considering conservative surgery. Small lesion size is a characteristic feature of benign tumors. A systematic review by Henriques et al. on small testicular masses showed the percentage of benign tumors to be 58.3% overall, 55.8% and 68.8% in testicular masses < 2.5 cm, and <1 cm, respectively .,7.2,7].Proppe and Scully provided the first description of LCCST cases in 1980 .SCSTs encompass 4% of all testicular tumors, and SCT comprises 1%, respectively . Other SLCCST malignancy is a rare phenomenon that is characterized by two criteria, size >4 cm, extra-testicular growth, necrosis, vascular space invasion, and high mitotic index . MalignaGiglio et al. divided LCCSTs into two clinical subgroups, early-onset and late-onset LCCST . Early-oA large clinicopathological study revealed that median patient age and size were 15.5 years and 1.9 cm, 19 years and 1.6 cm, and 28.5 years and 2.3 cm, for benign, ambiguous, and malignant tumors, respectively .Our case represents a typically late-onset LCCST that is associated with malignancy in many cases. However, malignant behavior was neither documented histologically nor clinically. In particular, the histologic findings of tumor size 7 mm and Ki-67 index of 2% are fully consistent with benign nature. We report a case of unilateral benign late-onset LCCST in a 37-year-old patient without any genetic disorders. LCCST is histopathologically associated with the presence of massive calcification; tumor cells have abundant eosinophilic cytoplasm and prominent nucleoli. There is a unique sonographic feature of the neoplasm, for example, hyper-echogenicity with acoustic shadowing, due to the calcified tissue. A differential diagnosis of malignant testicular calcified masses, such as teratomas, has to be made. However, calcifying non-seminomatous germ cell tumors are usually larger and likely heterogeneous in echogenicity with calcification .Scrotal sonography in our patient clearly showed a round calcified lesion with acoustic shadowing, as well as peripheral vascularization. This unique feature was not consistent with malignant germ cell tumors and thus prompted considering TSS, as also advocated by the current German clinical practice guideline for the management of germ cell tumors . FurtherOur present case is consistent with previously reported LCCST cases, with respect to histopathological features and ultrasonographic appearance. Minor differences to previous cases relate to unilateral presentation, smaller size, and older patients’ age. MR imaging of LCCST has been reported only exceptionally to date but this imaging technique proved useful in the present case to consider organ-sparing surgery.SCTs encompass around 1% of all testicular neoplasms. LCCSTs represent one rare subtype of SCTs that are further subclassified into early-onset and late-onset LCCSTs, with the latter usually representing malignant potential. Radical inguinal orchiectomy is the gold standard for malignant LCCSTs while organ-sparing surgery with intraoperative frozen section may be a good option for patients with benign LCCSTs. Preservation of remaining active testicular parenchyma may allow for maintaining endocrine function and fertility. The unique feature of LCCST is a circumscribed hyper-echoic lesion with acoustic shadowing upon ultrasonography.


Branchial fistulas and cysts are uncommon anomalies of embryonic development that involve soft tissues of the neck. According to Bailey-Proctor classification, second BCCs are classified into four types: Type-I cysts are situated along the anterior border of the sternocleidomastoid muscle beneath the superficial cervical fascia. Type-II ones are the most common and lie just laterally to great vessels beneath enveloping fascia of the neck. Type-III ones pass between internal and external carotid arteries. Type-IV cysts are situated in the pharyngeal mucosal space just deep to the palatine tonsil and medial to great neck vessels, often extending upward towards the skull base. Most second BCCs comprise the first three types, while type-IV cysts are extremely rare.17 years old male patient from Baghdad/Iraq, single, a student, and living with his family.The patient presented to Al-kindy Teaching Hospital for general surgery consultation due to the presence of a lump in the upper third of the anterior border of the sternocleidomastoid muscle for several years ago, the lump was painless but gradually increase in size with discomfort but without fever, anorexia or weight loss. There were no relieving factors. Regarding the review of systems nothing positive and the history was negative also patient had no past drug history and no psychological illness.Physical examination of the lump showed a smooth, non-tender, fluctuant cyst located at the upper third of the anterior border of the left sternocleidomastoid muscle about 7 × 4 cm and there were no enlarged lymph nodes. Regarding examination of the other systems there was nothing positive.Laboratory and radiological investigation showed that the cystic lesion mostly was a branchial cyst, so the operation is done for the patient by complete excision of the cyst with its tract which was located between external and internal carotid vessels. A histopathological study revealed a squamous epithelium-lined cyst with lymphoid infiltration, consistent with a branchial cleft cyst. The patient was discharged without any complication or any evidence of recurrence for 14 months follow-up.Branchial anomalies remain asymptomatic and can present later in life. They can be misdiagnosed. CT scans and MRI neck are helpful in the diagnosis of the cyst and its anatomical extensions. A proper history and physical examination are required to look for other anomalies like craniofacial syndromes. The treatment of branchial cyst is complete surgical excision to prevent recurrence and removal of these lesions at an earlier stage will increase the quality of life of the patient. In addition, since they are rarely malignant, more successful results will be obtained with early diagnosis and treatment. •Branchial fistulas and cysts are uncommon anomalies of embryonic development.•Second BCCs are classified into four types.•Most second BCCs comprise the first three types, while type-IV cysts are extremely rare.•Branchial anomalies remain asymptomatic and can present later in life.•The treatment of branchial cyst is complete surgical excision to prevent recurrence. The sixAccording to Bailey-Proctor classification, second BCCs are divided into four types: Type-I cysts are located along the anterior border of the sternocleidomastoid muscle beneath the superficial cervical fascia. Type-II ones are the most common and located just laterally to great vessels beneath enveloping fascia of the neck. Type-III ones pass between internal and external carotid arteries. Type-IV cysts are located in the pharyngeal mucosal space just deep to the palatine tonsil and medial to great neck vessels, often extending upward towards the skull base. Most second BCCs comprise the first three types, while type-IV cysts are extremely rare .This work has been reported in line with the SCARE criteria .217 years old male patient from Baghdad/Iraq, single, student, living with family.The patient presented to Al-kindy Teaching Hospital for general surgery consultation due to a lump in the upper third of the anterior border of the sternocleidomastoid muscle for several years ago, the lump was painless but progressively enlarged with discomfort but without fever, anorexia or weight loss. There were no relieving factors.The review of the system was anything but positive, and the medical history was negative. In addition, the patient had no history of drug use or psychiatric illness.Physical examination of the lump revealed a smooth, non-tender, fluctuant cyst approximately 7 × 4 cm in the upper third of the anterior border of the left sternocleidomastoid muscle, with no enlarged lymph nodes. There is nothing positive about the research on other systems, Laboratory tests were normal, and FNA showed a large number of lymphocytes, foamy histiocytes, and cystic accumulations of lymphoid tissue without malignancy.With regard to radiology, U/S showed a cystic lesion without evidence of infection, whereas CT scan showed a well-circumscribed hypodense cystic lesion displacing the vascular capsule, independent of the deep parapharyngeal space.The operation is performed on the patient under general anesthesia through an elliptical incision over the cyst and complete removal of the cyst with its duct located between the external and internal carotid arteries in order to ligature the end of the duct and close it in layers . Histopa3Clinically, the remainder of a branchial cleft, which accounts for the majority of branchial cleft anomalies, can present a variety of morphologic patterns, such as a cyst, a sinus, or a fistula. The branchial cleft cyst most commonly occur between the second and fourth decades of life . A brancThe Bailey classification divided anomalies of the second branchial arch into four groups . Type I The type III patient referred to in this article lies between the internal and external carotid arteries. A history of a positive transillumination reaction and variable edema character to exclude the possibility of cystic hygroma, as well as solid nodules such as tumors of various neck tissues and cervical lymphadenitis and other vascular malformations should be excluded . FNAC prThe typical ultrasound image of abranchial cyst has been described as a well-defined anechoic cyst, while computed tomography (CT) and magnetic resonance imaging are helpful in evaluating the cyst. Well-circumscribed, homogeneous cystic lesion that may be unilocular or detach in case of secondary infection, as usually seen on CT . The fin4Branchial anomalies remain asymptomatic and may appear later in life. They can be misdiagnosed. It is mandatory to confirm the extent of the tract before surgery is considered. Computed tomography and magnetic resonance imaging of the neck are helpful in diagnosing the cyst and its anatomical extensions. Adequate history and physical examination are required to detect other abnormalities such as craniofacial syndromes. Treatment for a branchial cyst is complete surgical removal to prevent recurrence. Early removal of these lesions improves the patient's quality of life. Because they are rarely malignant, early diagnosis and treatment can have better outcomes.The proposal of the study was discussed and approved by scientific & ethical committee in the author teaching hospital. The agreement of health authority in author institution approved before collecting data.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.N/A.N/A.


Syringocystadenoma papilliferum is a rare, benign adnexal neoplasm most often found on the scalp, head, and neck region as a solitary hairless plaque, verruca, or nodule. Lesions may arise sporadically or from a pre-existing sebaceous nevus. We report the case of a 56-year-old female who presented with an indurated multilobulated cystic mass in the left buttock region. Excisional biopsy demonstrated the histological picture of syringocystadenoma papilliferum. The interest of this case report lies in the rarity of syringocystadenoma papilliferum and its unusual presentation in the left buttock region. Syringocystadenoma papilliferum is a rare benign adnexal neoplasm most seen in childhood or adolescence. Lesions may arise sporadically or from a pre-existing nevus sebaceous. It is seen most often in the scalp, head, and neck region as a solitary hairless plaque, verruca, or nodule that may be exudative. Cases have also been reported in the extremities and rarely in the groin and anogenital regions - 4. HoweWe report the case of a 56-year-old female with no significant past medical history who presented with a large tri-lobulated mass to the left buttock. Pathology revealed an atypical squamous proliferation with verrucous features and indeterminate margins measuring 16 x 13 x 5 cm.The patient first noted intermittent swelling and tenderness of the gluteal area in January 2022; however, she did not seek care until two months later when she experienced an episode of acute pain in her left buttock region. In March, the patient sought out care from an urgent care clinic and was prescribed doxycycline and topical bacitracin with no improvement of symptoms. Ten days later, the patient presented to the emergency department complaining of pain and redness to the left buttock.Workup in the emergency department showed no fever and normal white blood cell count. A computed tomography (CT) abdomen/pelvis with intravenous (IV) contrast demonstrated a complex cystic mass in the subcutaneous left buttock soft tissue superficial to the left gluteus musculature Figure . The larPreoperatively, the patient was given IV fluids and IV antibiotics while awaiting incision and drainage with possible excision of left buttock soft tissue to treat the overlying infection prior to surgical excision. A complex cystic trilobed mass was excised. Postoperatively, the pathology report showed an atypical squamous proliferation consistent with well-differentiated squamous cell carcinoma with verrucous features and indeterminate margins, arising in association with syringocystadenoma papilliferum Figure . The groGiven the pathology, re-excision of the left buttock wound to assess for the margins was indicated. Re-excision pathology reported benign skin with evidence of recent manipulation including ulceration, inflamed granulation tissue, fibrosis, and negative findings for residual squamous cell carcinoma.Syringocystadenoma papilliferum is a rare hamartomatous tumor arising from pluripotent cells of apocrine or eccrine glands . This caIn Figure Additionally, the findings from this patient’s pathology indicated a highly hyperplastic epidermis, which could indicate reactivity to syringocystadenoma papilliferum, resembling pseudoepitheliomatous hyperplasia Figure . The patIn 2019, Konstantinova et al. performed a report on 16 cases which provided insight into sporadic syringocystadenoma papilliferum located in the buttock and anogenital area, highlighting various morphological changes that can occur in these lesions. Some tumors showed features resembling warts, but the presence of HPV was not consistently linked to these changes . In compThe interest of this case report lies in the rarity of syringocystadenoma papilliferum and its unusual presentation in the left buttock region with multilobulations. This case sheds light on a rare surgical pathology finding, demonstrating an atypical squamous proliferation consistent with well-differentiated squamous cell carcinoma with verrucous features. Although benign, it is important to distinguish syringocystadenoma papilliferum from other skin tumors to ensure accurate diagnosis and treatment. The overall risk of malignant contiguous squamous proliferations to appear in syringocystadenoma papilliferum is rare. We hope this provides guidance in the future for patients with syringocystadenoma papilliferum who require surgical excision.


A 55-year-old man was referred to our institute after total gastrectomy and partial esophagectomy for a gastroesophageal tumor. Esophageal reconstruction included the right colon and the last loop of the ileum with esophageal–ileal, colonic–jejunal, and jejuno–jejunal anastomoses (Roux-en-Y reconstruction). Following the initial surgery, a computed tomography scan showed a suspected anastomotic leak with the development of intrathoracic/intra-abdominal collections, so the patient underwent a second surgical procedure with placement of a thoracic/abdominal drain.The patient’s condition subsequently worsened and he was referred to our institute, where gastroscopy showed a wide (approximately 280°) dehiscence of the esophageal-ileal anastomosis at 20 cm from the oral cavity, with fluoroscopic examination showing extraluminal diffusion of contrast medium into the mediastinum, which was filled with purulent/oily secretions . The reVideo 1 Video showing the endoscopic treatment of a post-surgery dehiscence of an esophageal–ileal–colonic reconstruction performed after surgical resection of a gastroesophageal tumor. The management included double fully covered self-expandable metal stent placement and fixation through endoscopic sutures and metal through-the-scope clips.The patient was clinically stable for 3 days and imaging showed the assembled system remained in place . UnfortEndoscopy_UCTN_Code_TTT_1AQ_2AG


The epithalamus region contains the tiny, functionally endocrine pineal gland, which has the shape of a pinecone. Less than 1% of adult primary intracranial malignancies are pineal parenchymal tumors, which are incredibly uncommon brain tumors. A rare variety of pineal parenchymal tumors are those with intermediate differentiation. These tumors, whose namesake refers to a malignant pineal parenchymal tumor, are intermediate between pineoblastomas and pineocytomas .A female patient, age 13, who had been experiencing terrible headaches on and off for a month, went to the emergency room. Along with the headache, she experienced nausea, vomiting, dizziness, and blurred eyesight. A nonenhanced computed tomography scan was used for the initial brain neuroimaging, which showed a hypodense mass posterior to the midbrain and superior to the cerebellum. A heterogeneous bulk was visible on MRI.The headache, vertigo, visual disturbance, nausea, and vomiting have all improved, according to the patient. Both postoperative MRIs with and without contrast revealed the resolution of the obstructive hydrocephalus and the absence of any residual enhancing mass. The patient was followed up for 2 months without any complications or adverse events.One should carefully investigate a headache as the early symptom of many illnesses and rule out any other potential causes. This would therefore enable us to create a management structure for such a very unusual malignancy. HIGHLIGHTSPineal parenchymal tumors are rare and account for less than 1% of all central nervous system neoplasms.Patients with pineal parenchymal tumors of intermediate differentiation may present with headache and diplopia.The pineal parenchymal tumors of intermediate differentiation may be large enough to compress surrounding structures such as the cerebral aqueduct, resulting in hydrocephalus with or without signs of elevated intracranial pressure such as ataxia.Pineal tumors account for 0.5% of all central nervous system (CNS) tumors in adults, 1% in young adults aged 20–34 years), and 2.7% in children (aged 1–12 years)4 years, A 13-year-old female patient presented to the emergency department complaining of recurrent attacks of severe headaches for 1-month duration. The headache was associated with dizziness, blurred vision, nausea, and vomiting. Despite the chronic mild headache, the patient asserted that she had never experienced pain similar to this before. The patient reported no personal and/or family history of cancer, any acute, repeated, or discontinued medications, any allergies, any genetic or psychosocial issues, and had a free past surgical history. There was no history of trauma. Upon admission, physical assessment and routine laboratory evaluation, including a complete blood count and metabolic panel, were unremarkable. The initial brain neuroimaging was performed with a nonenhanced computed tomography scan and revealed a hypodense mass located superior to the cerebellum and posterior to the midbrain. MRI showed a heterogeneous mass demonstrating low T1, high fluid-attenuated inversion recovery (FLAIR)/T2 signal, and no definite diffusion restriction measuring 3.5×2.5×3 cm, resulting in compression over the cerebral aqueduct, which appears effaced with no cerebrospinal fluid (CSF) flow through the occluded duct. MRI also revealed a moderate supratentorial ventricular dilatation with periventricular edema , a rare SMARCB1-mutant tumor devoid of histological indications of malignancy, has been added to WHO CNS5 . Pineal tumor behavior is still a subject of much debate, and histological grading standards for PPTID, papillary tumors of the pineal area, and desmoplastic myxoid tumor, SMARCB1-mutant have not yet been establishedNeoplasms in the pineal area can develop from parenchymal pineal cells, leftover stem cells, or surrounding glia. Pineal parenchymal tumors, which make up about 27% of tumors in the pineal area, are so named because they develop from these cellsLong known as malignant pineocytomas, pineocytomas with anaplasia, and atypical pineocytomas, these pineal parenchymal tumors are intermediately malignant between pineocytomas and pineoblastomasThe clinical manifestation of a PPTID seems to be similar to that of other lesions in the pineal region. Diplopia and headache are the most common symptoms. Parinaud’s syndrome  is another frequent finding. If large enough, PPTID can cause hydrocephalus; obstructive  hydrocephalus, due to obstruction of the Sylvian aqueduct by the tumor, is the main underlying mechanism of the presenting symptoms in these patientsFor pineocytomas, surgery is frequently curative, but for pineoblastomas, severe chemotherapy (and frequently stem cell transplantation) is necessary. There is no agreement on how to treat PPTID, and many treatment paradigms that fall somewhere between these two extremes of aggression have been observed. The choice to utilize chemotherapy and radiation must be decided following the maximum safe surgical resection. The extent of the resection as well as the presence of spinal or CSF metastases, frequently play a role in this choice. The probability of craniospinal recurrence is intended to be decreased by systemic chemotherapy and more thorough ventricular radiation regimens. On the other hand, radiation and chemotherapy’s long-term harmful effects must be taken into account. These factors are weighed against the highly varied survival and recurrence risk associated with PPTID. In comparison to high-grade PPTID, low-grade PPTID has an estimated 5-year overall survival rate of 74%Fifteen individuals first received surgical resection in Helsinki, Finlandet al.et al. suggest surveillance with radiation saved for recurrence for large complete resections without spinal and/or CSF dissemination. Local radiation is pursued in situations of subtotal resections without spinal and/or CSF dissemination. Mallick et al.After conducting a thorough analysis of 29 studies involving 127 patients with PPTID, Mallick We present the case of a 13-year-old female patient who had a big PPTID that was blocking her ventricular system and causing obstructive hydrocephalus. Physicians will be reminded of its prevalence and presentation by this case. One should carefully investigate a headache as the early symptom of many illnesses and rule out any other potential causes. This would therefore enable us to create a management structure for such a very unusual malignancy.Our institution has exempted this study from ethical review.Written informed consent was obtained from the patient and family for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.None.O.N.S. and A.W.M.J.: writing the manuscript; H.O., O.N.S., T.S., and M.A.: imaging description; O.N.S., N.S., Y.A., A.K., H.D., and S.H.: reviewing and editing the manuscript.There are no conflicts of interest.Name of the registry: none.Unique identifying number or registration ID: none.Hyperlink to your specific registration (must be publicly accessible and will be checked): none.Oadi N. Shrateh.Not commissioned, externally peer-reviewed.


Cardiac tumors (CTs) and intracardiac masses are rare, with an incidence of 1 per 2 million people annually. We present a case of an intracardiac mass where the patient exhibited progressive lower extremity swelling, night sweats, and diarrhea. Computed tomography of the chest with intravenous contrast revealed a sizable intracardiac mass with mixed attenuation and signs of metastatic lesions, suggesting a malignant process. This case stands out due to its uncommon presentation, considerable size, and extension from the right atrium into the right ventricle and the inferior vena cava. Although the exact etiology remains unclear because of the absence of a biopsy, it was presumed to be a type of sarcoma. Owing to significant cardiac obstruction, the patient's condition worsened rapidly, culminating in a fatal outcome mere days after the initial presentation. While there are multiple approaches to identify and treat CTs, their propensity to grow quietly until they reach a size large enough to cause fatal symptoms restricts opportunities for early detection and treatment. Intracardiac masses are most commonly caused by thrombi, hamartomas, structural abnormalities, and infectious vegetations. Neoplastic causes are rare and can arise from metastatic disease or a primary etiology. The most common primary neoplastic masses are myxomas/papillary fibroelastomas (PFEs) in adults and rhabdomyomas and fibromas in the pediatric population . While m3, hemoglobin 10.2 g/dL, hematocrit 32%, sodium 130 mEq/L, lactic acid 2.3 mg/dL, and a brain natriuretic peptide 1120 pg/mL. Chest X-ray showed multiple bilateral pulmonary nodules and small bilateral pleural effusions with atelectasis. Chest CT with intravenous contrast revealed a large mass extending from the inferior vena cava (IVC) into the right atrium and right ventricle. It measured up to 4 cm in the right atrium and 7 cm in the right ventricle and is of mixed attenuation. There were also numerous pulmonary and hepatic parenchymal nodules up to 1 cm in size suspicious for metastatic disease. The patient was admitted to the medical intensive care unit on empiric intravenous antibiotics, pressors, and diuretics. Over the next three days, she deteriorated with presumed obstructive shock and multiorgan failure. Given the size of the tumor, extension, and metastasis, the tumor was deemed inoperable. The patient's family subsequently withdrew life-sustaining support. She passed away four days later. An autopsy was not performed.A 69-year-old female with a 40-pack-year smoking history and asthma presented to the emergency room with complaints of progressive bilateral lower extremity edema, diarrhea for three weeks, and night sweats for a month. She denied any fever, chills, chest pain, shortness of breath, nausea, or dysuria. She had a chronic cough that was unchanged from baseline, which was occasionally productive without hemoptysis. She denied any known history of cardiac or gastrointestinal disease. She had not been receiving any routine medical care for the past 10 years due to being uninsured. She was not on any medications at the time. On presentation, she was ill-appearing and had 4+ bilateral pitting edema. Her vitals were tachycardic to 109 beats per minute but otherwise stable. Initial lab work showed leukocytosis of 17,900/mmCertain radiological features of intracardiac masses can help distinguish their etiology. This includes size, location (left vs right), morphology, attachment, contrast enhancement, margins, invasion, metastasis, history of pericardial effusion, and calcification. The details of these features are featured in Table In this particular case, the mass is large  CardioOncology state-of-the-art review classifies the role of surgery between different types of CTs. For primary benign CTs, such as lipomas and rhabdomyomas, surgical intervention is only recommended in severely symptomatic cases. Certain primary benign CTs are prone to other complications, including embolic events in myxomas and papillary fibroelastomas, sudden cardiac death in infants with fibromas, and catecholamine surges in paragangliomas. These complications warrant surgical resection where possible. Primary malignant CTs, such as leiomyosarcomas, rhabdomyosarcomas, angiosarcomas, osteosarcomas, undifferentiated sarcomas, and mesotheliomas, are prone to invasion metastasis and paraneoplastic complications; thus, they warrant surgical resection when possible. One notable exception is primary cardiac lymphoma, which is treated with anthracycline chemotherapy and anti-CD20 monoclonal antibody treatment rituximab. Metastatic tumors within the cardiac chambers are typically indicated for surgical removal due to their potential for local invasion and obstructive tendencies . RBesides primary cardiac lymphoma, a glaring issue in many of the studies involving CT response to chemotherapy is the lack of adequate sample size and power. Pediatric rhabdomyosarcoma, which benefits from a combined regimen of surgery, systemic chemotherapy , and radiotherapy, accounts for 4%-7% of cardiac sarcomas, but are also highly invasive and often fatal within a year [Survival rates of CTs are generally poor, regardless of origin. A retrospective study on the incidence and survival of primary CTs found a five-year survival rate of 14% . CTs are rare diagnoses with poor prognosis secondary to obstructive shock and fatal arrhythmias. The use of multiple imaging modalities such as TEE, MRI, CT, and 3D echocardiography has played a large role in identifying tumors and guiding the approach to therapy. Treatment options include surgical resection, systemic chemotherapy, and radiation. While small CTs may be clinically benign, larger tumors can be fatal, without much improvement in survival after multifactorial treatment. With this case, we hope to add to the existing literature on these rare tumors, and further longitudinal studies are needed to study and describe their behaviors.


Histologically benign splenic cysts (SCs) resemble splenic sacs. SCs are rare. Here, we present and discuss a new case of bilateral endometriotic cystic ovaries with massive SCs. A 26-year-old single female visited the hospital with left lower quadrant discomfort and suprapubic pain for three months, accompanied by anorexia, weight loss for these three months, and persistent dysmenorrhea for two years. Splenic examination revealed a soft abdomen with left hypochondria, suprapubic tenderness, and a lump in the upper left quadrant. All laboratory results were normal, except for two cancer antigens (CA-125 and CA 19-9). Therefore, magnetic resonance imaging was used to make the definitive diagnosis, which revealed bilateral ovarian endometrioma with a left upper abdominal cystic mass of splenic origin. When CA-125 and CA-19-9 readings are high, physicians should investigate endometriotic and SCs. Imaging aids diagnosis. Histopathological results are essential. Tools and follow-up should rule out malignancy, and surgery is the best treatment option. Ovarian endometrioma (OEM) is a benign estrogen-dependent cyst in women of reproductive age. OEMs can cause infertility and subfertility, possibly because of oocyte number and quality. "Chocolate" endometriotic cysts were present in 10% of women aged 25-40 . SCs areA 26-year-old single female, who lived with her family, visited the hospital with left lower quadrant (LLQ) pain and suprapubic pain for three months, associated with anorexia, weight loss throughout these three months, and chronic dysmenorrhea for two years. There was no history of any chronic disease, any drug use, only analgesics used during the menstrual period, no history of trauma or surgery, no smoking or alcohol consumption, or a family history of malignancy. There was no contact with farm animals.On clinical examination, she had a soft abdomen, but left hypochondria and suprapubic tenderness, and a mass in the left upper quadrant could be felt on splenic examination.Her left ovary contained two unilocular thick-wall cystic masses with numerous small papillary projections, and vascularity was only detected in the wall of the largest mass (75×76×60 mm). Her right ovary contained a single unilocular thick-wall cyst measuring 77×60 mm, and her abdominal and pelvic ultrasounds showed a well-defined regular outline solid mass measuring 136×112×114 mm with obvious vascularity and pushed up the spleen. Magnetic resonance imaging (MRI) was requested for further details.MRI revealed a thin-walled unilocular cystic mass measuring 13×12 cm in size in the left upper abdomen on T1- and T2-weighted fluid signal intensity, no blood signals, and no blood diffusion restriction. The post-contrast scan showed marginal enhancement with stretched splenic parenchyma over part of it, which displaced the pancreatic tail superiorly and compressed the left kidney posteriorly; no enhancing solid component was observed  and left (70×65×60 mm), with high T1 signal, T2 weight low (shading sign) with diffusion restriction of the fluid content, both located posterior to the uterus and adjacent to each other =13.8 g/dL, and renal function tests were normal in which urea was 20.5 mg/dL and creatinine was 0.72 mg/dL (0.5-0.9 mg/dL). The serum concentrations of both cancer antigens CA-125 and CA19-9 were 204.1U/mL and 70.64 IU/mL, respectively, showing a high concentration of these markers.Her complete blood count (CBC) tests were unremarkable including RBC=4.55×10A group of general surgeons and gynecologists with 10 years of surgical expertise decided to perform surgical intervention through a midline laparotomy. Each ovary contained a large cyst (6×7 cm). Cystectomy was performed, and the adhesion between the cysts and the posterior uterine wall was removed. After checking bilaterally, ova were present in both ovaries, and good hemostasis was achieved.In splenic exploration, a very large cyst lesion in the spleen was present, which occupied all spleen parenchyma Figure , so the Histological results confirmed that both ovarian sac lesion cysts were hemorrhagic endometriotic cysts with benign behavior. The histology of the splenic lesion shows a huge solitary cyst of the spleen rimmed by a normal attenuated layer of splenic white and red pulp, and most lining of the cyst has been lost through repeated bouts of hemorrhage but there are intact spots where a single layer of cuboidal epithelial cells with benign nuclear features are supported by a thick layer of hyalinized fibrous stroma and organized fibrin.Following the predetermined postoperative follow-up period, the patient's recovery was smooth and uneventful. Feeding began two days after surgery and continued until regular bowel movements were produced. On the second postoperative day, the patient was discharged without adverse events.Endometriotic cysts are prevalent benign gynecological disorders linked to high CA-125 and CA-19.9 levels. However, excessively elevated markers and an adnexal mass suggest ovarian cancer, making it difficult to distinguish between the two diagnoses . CA-125 SCs can be primary or secondary. Primary cysts are either parasitic (60%) or non-parasitic. Congenital non-parasitic cysts are secondary cysts. Young people are more likely to develop cysts in the upper pole of the spleen -15.Few cystic malignant splenic tumors have been identified in the literature, and most real SCs are epithelial and are thought to contain embryonic inclusions of epithelial cells from neighboring organs . As in tLater, the preferred imaging modalities for diagnosing SCs were US, CT, and MRI ,17. ThesA previous study proved the correlation between the SC and the tumor marker CA19-9; the elevated level will return to normal after cyst removal .Most patients had no symptoms unless the tumor was large enough to compress the surrounding organ . The patTreatment is recommended for symptomatic or complicated large SCs. Large cysts are more likely to rupture and lead to other problems, and previous reports have stated that cysts >5 cm in diameter require surgical treatment [However, partial splenectomy was successful. If the cyst is tiny and one splenic pole has an intact parenchyma, partial splenectomy may be considered. This preserves some splenic function and may minimize overwhelming post-splenectomy infection . Due to Since elevated levels frequently imply malignancy, the association between tumor markers CA-125 and CA19-9 and these benign conditions complicates differential diagnosis. To make an accurate diagnosis and start the right course of treatment in such cases, a thorough and comprehensive evaluation is essential. Additionally, this case highlights the importance of tumor markers in benign cystic diseases as well as malignancies, giving important new information about their function in clinical practice. When cysts cause symptoms and are >5 cm in size, the recommended treatment is partial or complete splenectomy. US and MRI are effective diagnostic modalities, though surgical intervention is still the mainstay of treatment for such symptomatic and large cysts.


Cross-sectional imaging of the upper abdomen, especially if intravenous contrast has been administered, will most likely reveal any acute or chronic disease harbored in the spleen. Unless imaging is performed with the specific purpose of evaluating the spleen or characterizing a known splenic lesion, incidentally discovered splenic lesions pose a small challenge. Solitary benign splenic lesions include cysts, hemangiomas, sclerosing angiomatous nodular transformation (SANT), hamartomas, and abscesses, among others. Sarcoidosis and tuberculosis, although predominantly diffuse micronodular disease processes, may also present as a solitary splenic mass lesion. In addition, infarction and rupture, both traumatic and spontaneous, may take place in the spleen. This review aims to describe the imaging features of the most common benign focal splenic lesions, with emphasis on the imaging findings as these are encountered on routine cross-sectional imaging from a multicenter pool of cases that, coupled with clinical information, can allow a definite diagnosis. The spleen, often referred to as the forgotten organ, serves in adulthood as a filter for blood cells with additional important immune functions. As it is not essential for the preservation of vital life functions, it often receives limited attention from clinicians. Nevertheless, a wide range of diseases can affect the spleen, which might present as focal lesions on routine cross-sectional imaging, classified into six categories .Incidental splenic lesions, defined as lesions detected by imaging performed for a reason unrelated to the spleen, have not been evaluated in great detail and may equally excite and puzzle the reporting Radiologist. In a population-based whole-body magnetic resonance imaging (MRI) study of 2500 healthy individuals, 31.5% had incidental findings of potential clinical relevance, but only 12 involved the spleen with a reported likelihood of malignancy of only 1% -Fluoro Deoxy Glucose (18F-FDG-PET) shows lower metabolism and tracer uptake of the normal spleen compared to the liver , but alsInflammatory myofibroblastic tumor (IMT), formerly known as inflammatory pseudotumor, is an uncommon benign splenic lesion. It has been described in virtually all major organs with a few exceptions. In the liver and spleen, inflammatory pseudotumor is possibly linked to Epstein–Barr infection [nfection . The prenfection ,15. In tnfection . They prnfection . The dianfection ,17.Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas. One-third of patients presenting with splenic sarcoidosis findings have normal chest radiography [iography . Spleniciography . It may iography . Nodulesiography  , leukemia, or lymphoma. There is a great diversity of mechanisms and etiologies for splenic infarction, but it is a rare event with a reported incidence of only 0.016% of admissions to an academic general hospital over 10 years [10 years . Acute s10 years . The SI Hemangioma, although rare, is the most common splenic neoplasm and is found in up to 14% of patients at autopsy. It is formed by a proliferation of vascular channels, lined by a single layer of epithelium, and filled with blood. Hemangiomas are usually asymptomatic, solitary, or multiple. The natural course of hemangiomas is slow growth, and symptoms or complications, when present, occur late. Splenic hemangiomas may occur as part of generalized angiomatosis, as seen in Klippel–Trenaunay syndrome. Complications include rupture, hypersplenism, and malignant degeneration. Kasabach–Merritt syndrome, which involves the triad of anemia, thrombocytopenia, and coagulopathy, has been reported in patients with large hemangiomas [angiomas . Hemangiangiomas . They apangiomas .Splenic hamartomas are very rare splenic lesions, with only around 200 cases reported since 1861 [nce 1861 . A revience 1861 . They prnce 1861 . On US, nce 1861 . Color-Dnce 1861 . On MRI,nce 1861 . Althougnce 1861 .Littoral cell angioma is another rare vascular tumor that often results in splenomegaly, with less than 150 cases reported in the literature. Due to increasing numbers of littoral cell angiomas described in association with autoimmune disorders  and tumors , an immune system dysfunction has been postulated as a possible crucial pathogenic mechanism [echanism ,39. Multechanism . The lesechanism . The golSclerosing Angiomatoid Nodular Transformation (SANT) is a rare benign vascular disorder of unknown cause, with the same prevalence as inflammatory pseudotumors and approximately 170 cases described in the scientific literature. SANT is almost exclusively described in the spleen, except for one reported in the adrenal gland [al gland . Half ofal gland . SANT isal gland . The nodal gland . On CT, al gland .Pyogenic abscesses are rare (incidence between 0.2 and 0.7% in autopsy series) and frequently unrecognized lesions that can be infective (from hematogenous spread or by direct extension), traumatic, post-infarction, or related to immunodeficiency. They can be solitary or multiple. In the majority of abscesses, streptococcus or staphylococcus is isolated. In the presence of a pyogenic splenic abscess without obvious etiology, it may be helpful to investigate bacterial endocarditis as a possible source of septic emboli [c emboli . On US, c emboli . CT showc emboli .Fungal abscesses affect most commonly immunocompromised individuals. The most common infecting organisms are Candida albicans, Aspergillus fumigatus, and Cryptococcus neoformans [oformans . Concurroformans . The lesoformans . Associaoformans .Splenic tuberculosis (TB) is rare, with few cases reported usually encountered in immunosuppressed individuals, although some case reports have been published on splenic TB in immunocompetent hosts [nt hosts . Splenicnt hosts ,48. Solint hosts . On CT, nt hosts .Hydatid cysts may occasionally involve the spleen, especially in endemic areas. Isolated hydatid of the spleen is rare, even in endemic regions, constituting 0.8–4% of all cases of human hydatid, while it constitutes 5.8% of all cases of abdominal hydatid. The diagnosis of cystic echinococcosis rests mainly on imaging, with immunodiagnostics playing only an ancillary role due to limitations in sensitivity and specificity [cificity . The splcificity . Accordicificity . In a smcificity . On US, cificity . On CT, Benign focal splenic lesions, mostly detected incidentally, are not uncommon. Imaging can often only provide a differential diagnosis rather than a single definite diagnosis. Radiologists should be familiar with the characteristic imaging findings of different benign solitary splenic lesions, which, together with the clinical context, will aid them in giving the correct diagnosis and avoid overtreatment and unnecessary follow-up scans.


Testicular Sertoli cell tumor (SCT) is very rare sex cord-gonadal stromal tumor, and sclerosing SCT (SSCT) is even rarer. So far, no more than 50 cases of SSCT have been reported. 80% of SSCTs are less than 2 cm in diameter, large volume mass is pretty unusual. SSCT is usually benign with very low malignant potential. However, it is easily misdiagnosed as a malignant tumor resulting in the removal of the entire testicle.A 55-year-old Chinese male patient presented with a six months’ history of right testis progressively enlargement and negative tumor markers. The physical examination was nothing special except for swelling in the right testicle. Imaging identified a large mass in right testicle with rich blood. A right radical orchiectomy was performed on suspicion of malignancy. However, the tumor was postoperatively diagnosed as SSCT, which pathologically consisted of a tubular pattern with regular nuclei and embedded in a densely collagenous stroma, as well as diffusely positive for vimentin, β-catenin and synaptophysin. After 7 months of follow up, no evidence of local recurrence and metastasis has been observed.This rare case is helpful to expand the knowledge of the testicular tumor and alert us fully understand the rare variant of SCTs in order to choose the optimal management when they encounter SSCT.The online version contains supplementary material available at 10.1186/s13000-023-01351-7. SCT is very rare sex cord-gonadal stromal tumor, accounting for 0.4–1.5% of all primary testicular neoplasms . Most SCA 55-year-old Chinese male was referred to the urology department of our hospital with a six months’ history of right testis enlargement, which had worsened in the past few days. There were no specific symptoms of fever, dysuria, osphyalgia, hematuria and impotence. He denied history of cryptorchidism, testicular traumas, varicocele, or prior scrotal surgery. On physical examination, there were no lymphadenopathy, anaemia, or gynaecomastia except for the swelling in the right testis. Swelling was non-tender and hard. However, admission laboratory investigations were entirely normal, including tumor markers, such as eta-human chorionic gonadotropin (β-HCG) and alpha fetoprotein (AFP).A scrotal ultrasound (US) examination revealed bilateral testicular hydrocele and a well-defined 4.9 × 3.0 × 4.1 cm hypoechoic, heterogeneous mass in the middle of right testicle with internal rich flow on color Doppler Fig. .The patient underwent a right radical orchiectomy and the tumor measured 4.2 × 3.1 × 2.7 cm, without involvement of the adjacent spermatic cord or epididymis. The cut section was an intratesticular whitish-red firm nodule, mixed with hemorrhage and liquefactive necrosis. Microscopically, the neoplasm presented a tubular pattern with regular nuclei and embedded in a densely collagenous stroma , but in our case, lesion size reach 4.2 cm, and even lesions greater than 7 cm have been reported previously . MacroscTo distinguish SSCT from other potential mimics, the immunohistochemical stains are utilized for an accurate diagnosis. SSCT is characterized by positive vimentin, synaptophysin, CD56 . 60% of Most SSCTs are benign and have a significantly better prognosis than SCT-NOS and large cell calcifying. So far, only 2 malignant cases have been reported. One patient proved clinically malignant, showing bone metastases and died after 27 months after presentation .The otheIn terms of treatment, the majority of the cases reported previously have undergone orchiectomy. However, whether fertility and sexual function are affected after surgical treatment also deserve surgeon’s attention, especially for such a tumor with high survival rate and low recurrence rate. Thus, the testicle-sparing surgery should be strongly recommended for patients with small bilateral lesions. When the intraoperative frozen section analysis is uncertain, the surgeon should reserve the testis and awaits a final pathology consultation. Secondary orchiectomy can be proceeded if the subsequent pathology indicates a non-stromal tumor.SSCT was regarded a separate entity until the 2016 revision of the WHO classification, where it was specified a morphologic variant of SCT-NOS due to the similar clinical features and same molecular features including CCNB1 gene mutation, nuclear staining of β-catenin protein (> 70%) and diffuse positive expression of cyclin D1, in contrast to SCT-NOS , 15. In In conclusion, SSCT is very rare and usually manifests as a palpable mass without malignant behavior in a postpubertal man. Although no specific imaging characteristics for SSCT were reported, and serum tumor markers or hormone levels are normal, we believe the testicle-sparing surgery or a second look surgery are achievable with the collaboration of pathologists and surgeons, which is particularly beneficial for patients with fertility needs.Below is the link to the electronic supplementary material.Supplementary Material 1Supplementary Material 2


Primary pericardial synovial sarcoma is an extremely rare malignant tumor, and affected patients have a poor prognosis. Only a few cases have been reported in the literature.A 34-year-old man was admitted to our hospital with chest tightness and a cough. An echocardiogram revealed a heterogeneous mass with a large pericardial effusion. Further computed tomography (CT) of the chest and cardiac magnetic resonance imaging (CMRI) demonstrated an irregular pericardial mass abutting the left atrium and left ventricle and invading the mediastinal structures. Pathology results showed that the tumor was a monophasic synovial sarcoma. The patient underwent chemotherapy and survived for 17 months.Many cardiac tumors are clinically asymptomatic or nonspecific, and they are frequently detected or diagnosed at an advanced stage of the disease. Multimodal cardiac imaging facilitates the detection and assessment of cardiac tumors. In particular, CMRI is considered as a superior imaging tool, because it provides high tissue contrast and can detect invasion of the myocardium. We describe the clinical details and multimodal imaging features of a rare primary pericardial synovial sarcoma, hoping to provide guidance for the diagnosis of similar cases in the future. Synovial sarcoma (SS) is a kind of aggressive, soft-tissue, and malignant sarcoma. The incidence of SS in soft tissues is about 10%, and it usually arises in the deep soft tissue of children and young adults, especially around limb joints and tendon tissues . Primary9/L, a D-dimer level of 5100 ng/mL, and an N-telencephalon sodium peptide precursor (B-type natriuretic peptide) level of 843.20 pg/mL; however, the level of troponin-T was normal. The patient’s medical history and physical examination showed no significant abnormalities. An echocardiogram revealed a substantial amount of pericardial effusion and a 7.9 × 2.3 cm2 heterogeneous hypoechoic mass located in the left visceral pericardium. The mass was found to have a broad base and to be adjacent to the left ventricle and atrium  testing was positive as an SS18 (SYT) break-apart probe found that the SS18 gene was broken, thereby further supporting the diagnosis of PSS  chromosomal translocation, and about 95% of SS cases involve a t translocation . The diaSS is typically located in the extremities, but it is occasionally found in the head and neck, the eye sockets, the lungs, the chest wall, and the mediastinum. Primary PSS is extremely rare, with only in a few dozen cases reported in the literature . PSS hasPSS on echocardiography usually presents as an irregular, lobulated, inhomogeneous hypoechoic mass; and the tumor is mostly accompanied by pericardial effusion and thickening, usually with a broad base, and only approximately 24.1% of tumors are pedicled . A previCT scanning can provide detailed information about the features of PSS and its relationship with surrounding structures; assess the involvement of the mediastinum, lung, and pleura; and identify lymph nodes and distant metastases. PSS is seen as an equal or slightly higher-density mass with clear or unclear boundaries on CT images. In some cases, wall nodules, necrosis, cystic degeneration, or hemorrhage may be seen in the lesion. The literature has reported marginal calcification being found in SS, which is helpful for the differential diagnosis . Only wiCMRI is a superior noninvasive diagnostic tool: it provides higher resolution for better characterization of details in patients with PSS, and it can detect invasion into the myocardium and other adjacent structures. CMRI can also accurately provide additional information related to cardiac and pericardium about structure or function. PSS on T1-weighted MRI often shows tumor parenchyma as an uneven equal signal, and T2-weighted images show mixed signals dominated by high signals. In other words, multiple T2 signals can appear within the tumor. This may involve “triple signals,” such as a low signal , a slightly higher signal (the tumor’s solid component), and an obviously high signal (liquefactive necrosis or cystic degeneration) . The solIn the present case, CT showed an irregular, cystic, solid mass with unclear boundaries and uneven density. However, no marginal calcification was found within the tumor, which differs from what has been previously reported in the literature . CMRI deThe differential diagnosis of PSS includes metastasis, pericardial mesothelioma, pericardial teratoma, and other subtypes of pericardial sarcomas . The most common cardiac metastases are lung and breast cancer, however, known history of primary neoplasms is the main basis for differential diagnosis. Both primary and metastatic tumors can lead to bloody pericardial effusion. Metastatic pericardial effusion usually develops rapidly, prone to massive pericardial effusion, even causing pericardial tamponade, and easy to relapse after drainage.Patients with PSS have extremely poor prognoses and are prone to relapse. Surgical resection remains the preferred treatment, although it is difficult and carries significant risks. A statistical study showed that the 2-year survival rates of complete and incomplete resection were 75.2% and 55.0% . The surPSS is a rare clinical disease, but the symptoms and presentation are usually cryptic and atypical, such as the case of this study only presented dyspnea and cough. Accordingly, the diagnosis and management of PSS are complex and need multidisciplinary collaboration and multimodal imaging examinations. It is necessary to continuously report and accumulate information about new PSS cases to develop a more comprehensive understanding of this disease’s clinical characteristics, pathological diagnosis, and multimodal imaging features. Multimodality cardiac imaging includes echocardiography, CT, and CMRI, all of which are critical for the diagnosis, management, staging, and prognosis of PSS. In this article, we discuss the role of each imaging modality and the features of PSS in the hope of providing valuable reference information for affected patients in the future.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Written informed consent was obtained from the necessary individual(s) for the publication of any potentially identifiable images or data included in this article.HJ and WZ analyzed the data and image acquisition, and KW designed the study. All authors revised the manuscript. They all commented and approved the final version of the manuscript. They have contributed equally to this work.


The combination of disorder of sex development and canal of Nuck hernia, in which the hernial sac contains the internal reproductive organs (gonads) of both genders, is exceedingly rare. We present a unique case of a neonate who presented with ambiguous genitalia and a lump in the left inguinal region. The child underwent various radiographic procedures and hernial repair. Blood work-up, karyotyping, and histopathological analysis from gonads confirmed the diagnosis of Ovotesticular disorder of sex development. Subsequently, the child had a reconstructive vaginoplasty, and the final decision regarding gender assignment will be made after assessing mental and sexual behavior in early childhood. A 2-month-old baby was brought to our hospital by the parents concerned about the shape of their child’s external genitalia and a lump in the left inguinal region. On physical examination, the child had abnormal external genitalia and labioscrotal folds. A large chordae phallus measured 2.5 cm with an atretic urethral opening at its tip and a small urogenital opening in the perineum. There was a focal non-tender soft lump in the left inguinal region extending toward the left labioscrotal fold. The lump was not reducible, and the overlying skin was normal—no palpable testicles. The family history was negative for a similar condition. The pregnancy and delivery were uneventful. The laboratory tests revealed a high level of 17-hydroxyprogesterone, more than 16 nmol/mL [the normal range in our institution is 0.35–4.13 nmol/L]. The ACTH stimulation test was normal, with a good cortisol response  [normal: more than 500 nmol/L]. Based on the previous laboratory results, congenital adrenal hyperplasia (CAH), the most common cause of DSD, was excluded. Furthermore, the patient had an increased level of testosterone following the HCG stimulation test , indicating the presence of functioning testicular tissue.Ultrasound evaluation showed a defect in the left inguinal region and a hernial sac extending downwards toward the labioscrotal fold. An elongated structure passes through the defect toward the bladder neck, with a central echogenic strip representing a herniated uterus . Three mA high-resolution pelvic MRI confirmed the presence of an elongated herniated uterus with normal zonal anatomy, an ovary , and twoA fluoroscopic genitogram through the single urogenital opening in the perineum showed a urogenital sinus with a short common channel. The endometrial cavity and fallopian tubes were opacified by contrast within the hernial sac .The surgeon confirmed the presence of a second ovary within the hernial sac during diagnostic laparoscopy in addition to the previous contents mentioned by imaging. Karyotype testing revealed 46 XX, and tissue biopsy from gonads was consistent with a separated form of bilobated ovotestis.The differential diagnosis for an inguinal lump is broad and includes inguinal hernia, hydrocele, lymphadenopathy, infection/abscess, inguinal gonad, and benign or malignant neoplasms.Canal of Nuck hernia containing the internal reproductive organs in a child with Ovotesticular-Disorders of sex development (OT-DSD) based on karyotype, laboratory, and histopathology results.The multidisciplinary team discussed the case thoroughly and explained it in detail to the family, including possible future consequences. The parents and the team decided to raise the baby as a female. Therefore, the child had reconstructive vaginoplasty. An elective orchiectomy will be performed at the pre-pubertal age after assessing the child’s mental and sexual behavior. The patient usually develops secondary sexual characteristics incompatible with the assigned gender.The most frequent causes of disorders of sex development in individuals with the 46XX karyotype includes congenital adrenal hyperplasia, maternal androgens, and ovotesticular diseases.Disorders of sex development (DSD) refer to a group of diseases characterized by different pathophysiological changes resulting in abnormal development of gonads, external genital, or both with variant clinical manifestation. Patients with 46 XX karyotype have a broad spectrum of clinical presentations making it difficult to diagnose based on clinical examination and phenotype alone. The diagnostic approach starts with background history, clinical examination, laboratory testing, genetic analysis, and radiological evaluation. If no precise diagnosis is reached using non-invasive methods, surgical intervention and gonadal biopsy are performed.Ovotesticular DSD (OT-DSD) is one of the rarest DSDs in which both internal reproductive organs are present, either separated (ovaries and testes) or integrated (ovotestes).The typical physical manifestation of DSD is ambiguous genitalia. If a penile phallus is present, the urethral opening is usually abnormal in location (hypospadias). Testicles, if present, are more likely to be undescended. While a clear vaginal opening is not always visible by local inspection, it is usually evident on ultrasound, and a hypoplastic uterus is present.Radiology has a critical role in assessing the internal genitalia of DSDs patient. Ultrasound is the first modality of choice in most cases. It is non-invasive, easily accessible, and readily available in most hospitals. It carries no radiation risk or exposure to contrast material. It helps identify the gonads and the presence or absence of the uterus. The adrenal glands and kidneys are also evaluated by ultrasound. The main disadvantages are being operator-dependent and limited intra-abdominal assessment in some cases. High-resolution pelvic MRI has superior soft tissue resolution and multiplanar imaging capabilities. It can clarify the internal abdominal anatomy, localize non-palpable ectopic gonads or streak gonads, and detect the presence or absence of intra-abdominal reproductive organs like the uterus and prostate. Fluoroscopic Genitogram and voiding cystourethrogram have a role in complex cases. They are beneficial in delineating the urogenital sinus if associated with DSD. They are also helpful in identifying male or female urethra, the length of the common channel, and the level at which the vagina opens into the sinus. Sometimes, the hernial sac includes the uterus, fallopian tubes, or ovaries. Herniation of ovaries carries an increased risk of complications such as incarceration and torsion; therefore, urgent surgical intervention is required for such cases.The Canal of Nuck hernia represents the continuation of an outpouching parietal peritoneum through the inguinal canal to labia majora. Usually, the hernia contains a bowel loop or peritoneal fat.DSD is a complex subject, and radiology is essential in diagnosing.The investigation and management of DSD should be tailored case-by-case and requires a multidisciplinary approach.Canal of Nuck hernia is uncommon and should be considered in young females with inguinal/labial swelling.Herniation of internal reproductive organs into the canal of Nuck hernia is rare.