Among those with acquired immune deficiency syndrome, salivary gland pathology and other less common signs of human immunodeficiency virus (HIV) seropositivity are emerging. Generally speaking, lymphoepithelial lesions of the parotid gland are uncommon with a reported incidence of 0.6%, but they are beginning to overtake other oral lesions such as candidiasis as predominant oral manifestations of clinical HIV infection. Here, we describe a patient with a known history of neurocysticercosis with presumed extracranial cysticercosis as demonstrated by the clinical manifestation of bilateral parotid gland swelling and a ring-enhancing, hypodense lesion of the left parotid gland on CT. He was found to have a lymphoepithelial cyst arising in a lobe of the left parotid gland per pathological evaluation after left superficial parotidectomy, and this served as the initial sign of HIV positivity, which was confirmed by serological studies. He was found to have a lymphoepithelial cyst arising in a lobe of the left parotid gland per pathological evaluation after-left superficial parotidectomy, and this served as the initial sign of HIV positivity, which was confirmed by serological studies.Among those with untreated acquired immune deficiency syndrome (AIDS), salivary gland pathology and other less common signs of human immunodeficiency virus (HIV) seropositivity are emerging.3 first introduced the term “lymphoepithelial cyst” in 1958 as a replacement for what had formerly been called “branchial cleft cyst,” as they concluded that the parotid lesions they observed had originated from parotid inclusions within lymph nodes rather than embryonal remnants. The origin of these cysts still remains a topic of controversy, and several theories have been posed.4 It is hypothesized that the development of lymphoepithelial cysts as benign lymphoepithelial lesions (BLEL) may represent a localized manifestation of persistent generalized lymphadenopathy, which is commonly seen with HIV infection and autoimmune entities such as Sjögren’s syndrome and sarcoidosis. Alternatively, this parotid pathology may be secondary to lymphatic infiltration of salivary parenchyma, which promotes basal cell hyperplasia of the striated ducts and formation of the lymphoepithelial lesion.1Bhaskar and Bernier2 These lesions may be solitary or multifocal, cystic, solid, or heterogeneous lesions that may occur either unilaterally or, as is more frequently described with concomitant HIV, bilaterally.5 Parotid swelling is most frequently accompanied by cervical lymphadenopathy in BLEL.6 Although benign and painless, these parotid lesions enlarge over time and may become disfiguring if not treated appropriately.5BLEL are commonly associated with HIV infection, although they are not necessarily considered precursors to fulminant AIDS.1 Lymphoepithelial cysts may also arise secondary to pathogenetic processes independent of HIV infection.4Still, the presence of these indolent lesions has frequently been described as the first sign of HIV infection. BLEL have been documented in 5–10% of HIV-positive patients and in up to 20% of patients with AIDS.4 In addition, the viral load in HIV infection shows linear association with gland enlargement secondary to BLEL.1BLEL of the parotid salivary gland were first described in 1985.6 may be employed for the evaluation of BLEL of both cyst and mixed types. The majority of cyst-type lesions are round or ovoid in morphology, with scattered isolated internal echoes. Compression of these lesions tends to suggest fine, filamentous septations. Mixed-type lesions tend to have more irregular, lobulated morphologies. They demonstrate moderate echogenicity to hyperechogenic matrix with solid component predominance, resembling a solid tumour. However, with compression, the motion of internal echoes indicate at least partial fluid composition. Vascularity, as assessed with colour Doppler, is variable across these two subtypes; some are relatively avascular, while others demonstrate hypervascularity. Overall, sonographic appearance of BLEL closely resembles that of parotid glands in Sjögren’s syndrome or Warthin’s tumour, although parotid cysts in Sjögren’s syndrome tend to be smaller and more numerous. BLEL may be differentiated from Warthin’s tumour by the presence of cervical lymphadenopathy.6Initial imaging evaluation of BLEL is most commonly conducted with ultrasonography and CT scan. The lesions may range from nearly completely fluid-filled masses to those of mixed composition with solid components. For the sake of simplicity, a dichotomous sonographic classification scheme according to echogenicity, as proposed by Martinoli et al,6 Findings typical of BLEL, as seen on contrast-enhanced CT scan, include numerous mixed cystic and solid lesions in either one or bilateral parotid glands. Rarely, a solitary cystic lesion may represent a lymphoepithelial cyst. A rim of peripheral enhancement usually encircles the hypodensities, suggestive of cystic components, while heterogeneous enhancement comprises solid components. In addition, the neck should be surveyed for non-necrotic cervical lymphadenopathy as well as tonsillar hypertrophic changes, as these strengthen the case for BLEL.7As the spectrum of sonographic findings of BLEL are non-specific, especially with regard to heterogeneous solid cystic lesions, contrast-enhanced CT scan offers more diagnostic power.T1 weighted images, and high signal intensity on T2 weighted images. In addition, T2 hyperintense bilateral cervical adenopathy is often present. Similar to CT scan, prominence of the Waldeyer's lymphatic ring with high T2 signal intensity is also suggestive of BLEL in the setting of the intraparotid findings. Contrast enhancement demonstrates thin enhancing rims of intraparotid cystic lesions with variable enhancement of solid lesions.8MRI findings include intraparotid cystic lesions with low heterogeneous signal intensity on 9Differential diagnosis includes parotid cysticercosis, lymphoma, metastatic disease, Warthin tumour, parotid sarcoidosis and Sjögren’s syndrome. Sjögren’s syndrome and sarcoidosis can appear identical to BLEL on imaging, and attention is paramount to the aforementioned ancillary findings. Warthin tumours lack the ancillary findings of tonsillar hyperplasia. Lymphoma often has additional clinical symptoms or multifocal findings. Metastatic disease is uncommon bilaterally. On CT scan and MRI, parotid cysticercosis appears as a cystic structure with surrounding oedema in the colloidal stage, and a thickened retracted cyst with decreased oedema in the granular stage, both appearing similar to BLEL. The wall of the cyst will enhance with contrast administration, although often there is nodular/marginal enhancement secondary to the scolex.5Among large lymphoepithelial cysts, similar immunohistochemical profiles with regard to expression of S100 have been observed, regardless of HIV seropositivity. BLEL related to HIV infection may be further delineated by positivity for p24 expression.10The diagnosis of BLEL is made by pathological evaluation. Histopathologically, BLEL are characterized by follicular hyperplasia, ectasia of terminal striated ducts and lymphocytic infiltration. Fine needle aspirate cytology of BLEL reveals a characteristic triad of heterogeneous lymphoid aggregates, foamy macrophages of varying number and distribution, and superficial and/or enucleated squamous cells. In addition, the presence of multinucleated giant cells within lymphoepithelial cysts has been described.The patient is a 44-year-old immigrant male who presented to our institution with multiple masses in bilateral parotid glands, left greater than right. He had a history of neurocysticercosis, presumably owing to ingestion of uncooked pork in Mexico and had undergone a previous craniotomy with removal of the brain mass in Mexico. The patient had been vaccinated against tuberculosis, and subsequent work-up for systemic tuberculosis was negative. This new onset of bilateral parotid masses since his emigration to USA was presumed to represent cysticercosis lesions. A CT scan of the neck with i.v. contrast demonstrated bilateral parotid masses with a dominant, ring-enhancing, hypodense lesion in the left superficial parotid gland, which measured 3.0 × 2.9 cm . A left 9 in 2008. Hence, recognition of BLEL as potential harbingers of HIV infection through imaging is essential for diagnosis and subsequent timely and effective treatment.BLEL, which were originally thought to be infrequently occurring lesions of the parotid gland characterized by lymphoid hyperplasia and concurrent cyst formation, have become more prevalent oral lesions in the setting of untreated HIV infection. Usually manifesting as painless enlargement of one or both parotid glands, BLEL have been described as a common index lesion for the diagnosis of HIV infection, and therefore must be entertained in the differential for cystic and mixed parotid pathology seen on imaging of any patient. The patient described had no diagnosis of HIV/AIDS upon presentation. His parotid swelling was initially thought to be a solitary, extracranial focus of known cysticercosis, but was ultimately found to be a pathologically proven lymphoepithelial cyst. Further serological analysis revealed HIV positivity, and the lymphoepithelial cyst served as the impetus for evaluation. Of note, a parotid focus of cysticercosis could also appear as a solitary, ring-enhancing lesion, but would exhibit predominance of neutrophils and macrophages on fine needle aspiration cytology as described by Veena et alLymphoepithelial cysts should be a differential diagnostic consideration if cystic or solid bilateral intraparotid lesions are discovered in a patient with HIV, or where HIV is suspected.Ancillary findings of cervical lymphadenopathy and proliferation of Waldeyer's ring should raise suspicion for BLEL in patients with bilateral parotid lesions.Consent is received from all patients at our teaching institution.


Zinner syndrome represents a rare congenital malformation of the urinary tract. It comprises a constellation of Wolffian duct anomalies and is almost exclusively encountered as a classic triad of seminal vesicle cysts, ejaculatory duct obstruction and renal agenesis. Patients can be either asymptomatic or symptomatic. Recently, minimally invasive surgical techniques have emerged, superseding traditional surgery for select symptomatic cases. Our case highlights the finding of a rare clinical syndrome that was incidentally detected during a routine mass screening of military recruits in the Greek Armed Forces.Herein, we present a case of a 19-year-old male who reported having a solitary right kidney when examined in a military training center of Northern Greece. No additional clinical information was available; thus, referral to a tertiary urology department for further investigation ensued. Imaging studies, namely, computed tomography and magnetic resonance imaging, revealed left renal aplasia, multiple left seminal vesicle cysts, and ejaculatory duct obstruction. Laboratory values and urinalysis were within normal range. Semen analysis was significant for cryptozoospermia. Our patient remained asymptomatic during the entire hospitalization. Long-term follow-up was recommended. Nevertheless, he declined further investigation and sought treatment in a private practice setting.This article aims to present the incidental diagnosis of a rare syndrome in a military setting. Population screening conducted in the armed forces permits the identification of undiagnosed diseases that warrant further investigation. To the best of our knowledge, this was the first report of Zinner syndrome in a military recruit and the second case cited of a Greek patient in the published literature. Regular follow-up is the key to timely intervention in conservatively managed cases. Zinner syndrome, which was first described in 1914, comprises a rare congenital malformation of the genitourinary tract , 5, 6. PA 19-year-old male was referred to our department after initial physical examination to a military training center as part of his compulsory enlistment for the Greek military service. During the examination, he reported having a solitary right kidney but was otherwise asymptomatic with no further relevant details. Neither a history of trauma nor previous surgeries were reported. Physical examination was unremarkable. He had no scars on the trunk and normal external genitalia. No pain provoked or masses felt during palpation. A digital rectal examination was not performed due to the patient’s preference. No sexual intercourse was reported at the time. Furthermore, no previous notable medical history for hereditary or acquired diseases was mentioned. The whole blood count and urinalysis results were within normal limits.3 vertebral body was revealed with a length of approximately 16 cm  and magnetic resonance imaging (MRI) of the pelvis were performed. CT depicted only a right kidney with absence of left kidney Fig. . Additio1-weighted, T2-weighted, T2-weighted with fat saturation (FS) and T1-weighted FS images on axial, sagittal and coronal planes. Finally, images on T1-weighted FS sequence after intravenous administration of contrast medium (gadolinium) were added.MRI was performed with a Siemens Magnetom Avanto (1.5 Tesla) MRI unit  using a pelvic phased-array coil. The imaging protocol comprised T2-weighted FS and high signal on T1-weighted images, corresponding to a dilated seminal vesicle cyst (SVC). The high signal intensity on T1-weighted sequences was strongly suggestive of proteinaceous or hematic content. In contrast, the normal right seminal vesicle exhibits high and intermediate signal intensity on T2-weighted FS and T1-weighted images, respectively, corresponding to fluid , which might have assisted in clarifying the ejaculatory duct obstruction.Semen analysis revealed cryptozoospermia  of obstructive origin. Nevertheless, fertility was not the patient’s primary concern. He declined further management, although he was made aware of the rarity of the syndrome and the possible future need of surgical management. Despite detailed analysis of the importance of cryopreservation and fertility maintenance, he had no intention to cryopreserve sperm or undergo microsurgical sperm retrieval at the time. Therefore, annual semen analysis was recommended for as long as he presented with altered fertility status. Although SVCs are generally benign and rarely symptomatic, cases of malignant transformation and late diagnosis due to the absence of warning symptoms have been described . Thus, wZinner syndrome is characterized by the triad of renal agenesis, ipsilateral SVC and ejaculatory duct obstruction , 8. It iThe majority of patients typically remain asymptomatic until the second or third decade of life , 6. NeveIn our case, it is important to emphasize the incidental and prompt diagnosis of the syndrome in an otherwise asymptomatic young adult during enlistment screening. The initial screening, which was performed from the onset of the enlistment process, ensures health promotion and enhances the soldier’s future capacity for the remaining period of compulsory military service. The Greek armed forces implement an annual screening protocol for permanent staff, i.e., commissioned officers and enlisted soldiers, allowing for the identification of undiagnosed diseases that necessitate further investigation.Although the syndrome was diagnosed in the context of the regular enlistment process, the investigation employed was neither typical of a mass screening nor could it be adopted as such. Our patient was investigated for a reported solitary kidney, which was subsequently proven to be left renal agenesis. Every step of the detailed imaging work-up was indispensable with regard to the fixed setting of the armed forces in which every disease reflects on the patient’s physical activity status. To the best of our knowledge, this is the first case of Zinner syndrome described in a military setting and the second case with a Greek patient in the current body of literature since the first report by Kyriakidis et al.  in 1995.Our diagnostic approach was based on physical examination, abdominopelvic CT and pelvic MRI. Both CT and MRI are reliable diagnostic modalities , 5. The 2-weighted and intermediate on T1-weighted sequences, without enhancement after intravenous gadolinium administration. However, increased T1-weighted and decreased T2-weighted signal intensity may also be encountered, which is attributable to hemorrhage or increased concentration of proteinaceous fluid, as noted with our case [On MRI, SVCs may appear with variable signal intensity. Their content exhibits signal intensity generally analogous to fluid, i.e., high signal on Tour case . Occasioour case . In seleour case .The differential diagnosis includes a variety of entities from prostate and ejaculatory duct cysts to bladder diverticula and ureterocele , 5. ContSurgery is the gold standard approach for symptomatic cases , 4–6. CuIn conclusion, screening the population employed in the Greek Armed Forces may lead to the early diagnosis of rare diseases. Urologists should consider adding Zinner syndrome in their differential diagnosis when they encounter a solitary kidney in an otherwise asymptomatic patient. A thorough radiologic investigation of the abdomen and pelvis, including CT and MRI, is warranted in the presence of kidney anomalies. Moreover, radiologists should be aware of this rare entity and maintain a high index of suspicion when encountering analogous imaging findings. The need for long-term follow-up in patients presenting with this pathology assures prompt surgical intervention when clinical symptoms emerge or fertility is affected.


T1 and T2 weighted, and gradient recalled echo MRI sequences, the low signal intensity may mimic giant cell tumour of the bone, gout or synovial amyloid deposits, thus posing a diagnostic dilemma for the imagers and the treating clinicians. We present a pathologically confirmed case of PVNS of the cervical spine in a 49-year-old female, detailing her imaging work-up, describing histopathological correlation and highlighting the lesion location and involvement of the joint space as useful imaging discriminators for diagnosing PVNS of the cervical spine.Pigmented villonodular synovitis (PVNS) is an uncommon, benign, but locally aggressive lesion characterized most commonly by synovial proliferation of the appendicular large joints, but occasionally involving a bursa or the tendon sheath. PVNS of the spine is rare, typically involving the posterior elements. The lytic radiographic appearance and fludeoxyglucose avidity of PVNS may mimic malignant bone lesions, including metastatic disease or myeloma. On  We detail her imaging workup, including the CT-guided biopsy technique, describe the histopathological correlation, and highlight the useful imaging discriminators for differentiating PVNS of the cervical spine from other mimicking pathologies. A 49-year-old Caucasian female with no pertinent past medical or antecedent trauma history presented to neurosurgery with a 2-year history of progressive neck stiffness and spasm, left-sided jaw pain and left-sided headache. Her symptoms initially started with toothaches in the left jaw, for which a dental examination was normal. She subsequently developed worsening neck spasm and shooting pain extending from the superoposterior left neck into the left occiput, which was typically worse in the morning and aggravated by leaning to the left.Physical examination revealed full range of motion of the neck with forward flexion to 90°, extension to 30° and lateral rotation to 90°. She had moderate tenderness of the paraspinous muscles bilaterally, a negative Lhermitte sign and Spurling test, and no neurological  deficits. The neck disability index score was rated at 8/50 (16%), in keeping with mild disability.T1 and markedly T2 hypointense, faintly enhancing trans-spatial mass involving the dens and right lateral mass of C2, the anterior arch and right lateral mass of C1, and the prevertebral component of the perivertebral space  heterogeneously al space . Contrasal space . No inteal space . Whole-bal space , revealiBased on the findings of the MRI, CECT and FDG-PET/CT, the differential diagnosis remained broad, including neoplastic , metabolic (brown tumour or amyloid) and inflammatory .To definitively characterize the lesion, a CT-guided transfacial biopsy was performed with the patient in the supine position and under moderate sedation . UtiliziTissue cultures  were negative for growth and Gram stain was negative for organisms or polymorphonuclear leukocytes. Haematoxylin and eosin preparation slides demonstrated numerous histiocytes  with several multinucleated giant cells, foamy macrophages and pigmented macrophages also present . A panelGiven the precarious location, risk of operative morbidity and the patient’s desire to preserve range of motion, a decision was made by our institution’s multidisciplinary tumour board to pursue conservative medical management with imatinib mesylate , rather than resection and occipitocervical fusion. Given the paucity of literature regarding long-term treatment of PVNS with imatinib, the treatment plan was to continue this cytostatic medical therapy and make future changes in treatment based upon clinical and imaging findings. Short-interval MR and CT imaging demonstrated a small reduction in lesion size, particularly the prevertebral soft-tissue component . The pat4 in 1941, PVNS is an uncommon disorder of tumefactive synovial proliferation. It shares common histological features with a related condition called giant cell tumour of the tendon sheath (GCTTS), which demonstrates localized , intra-articular and diffuse subtypes.5 PVNS is differentiated from GCTTS based upon clinical and imaging manifestations.9 PVNS most commonly involves the knees (reported as high as 80% of cases), and less commonly the hips, ankles, shoulders or elbows. In contradistinction, the most common subtype of GCTTS  typically affects the hand, usually the volar aspect of the first three fingers.First described by Jaffe et alPatients with PVNS typically present with non-specific musculoskeletal complaints, including several months of monoarticular joint pain and swelling that may be progressive or wax and wane without specific laboratory or serological findings.3 Furlong et al10 and Motamedi et al,6 as well a few case reports. The true incidence of PVNS involving the spine remains unknown. Involvement of the cervical spine is reported most commonly (50–73%), with thoracic (7–25%) and lumbosacral (20–25%) involvement less common.6Among all sites of PVNS involvement, spinal disease is incredibly rare with the literature description limited mainly to small case series by Giannini et al,6 PVNS has been reported across a wide age range (11–84 years), but it is most commonly seen in the third and fourth decades of life.While there is some variability between the reported case series, no sex predilection for PVNS in general has been established.6 reported spine PVNS to almost always involve the facet joint (93%) and the paraspinal soft tissues (93%), as well as frequently involving the neural foramina (73%), pedicles (67%) and lamina (67%); in their series, PVNS uncommonly involved the vertebral body (27%) or the spinous process (7%). Similarly, our case showed involvement of the neural arch and prevertebral soft tissue extension. Extension into the epidural space is frequent, reported in up to 70% of cases in a small series that evaluated 10 patients for epidural involvement.3 Additionally, invasion of the foramen transversarium and involvement of the vertebral artery was reported in multiple patients by Motamedi et al,6 although the small number of patients in this series limits the scope of applicability of this finding.Based upon data from the three aforementioned series, PVNS of the spine almost invariably (> 90%) involves the posterior elements.6 CT scan findings of PVNS include a lytic soft-tissue mass that is isodense to skeletal muscle (100%), has a defined margin (83%) and typical homogeneous attenuation (67%).6 Internal calcification is not expected and its presence should direct one to consider alternative diagnoses.T1 (100%) and intermediate (67%) or low (33%) T2 signal intensity.6 Heterogeneity of signal intensity is common. As with PVNS of the large joints, susceptibility artefact from haemosiderin on T2* GRE sequences is suggestive of the diagnosis, but may be variably seen owing to the relative amounts of lipid, fibrous tissue, cystic content, cellular constituents and haemosiderin within individual lesions.8 Diffuse and moderate-to-marked contrast enhancement is typical.Spinal PVNS lesions are best evaluated with cross-sectional imaging, with complimentary information obtained from CT scan and MRI. A CT scan is often the first-line imaging modality for these patients and is useful for evaluating the margins, matrix, zone of transition and lesion centre. As reported by Motamedi et al,As aforementioned, PVNS and GCTTS share common histopathological features of histiocytes, giant cells, pigmented (haemosiderin laden) and foamy (lipid laden) macrophages, with the diagnosis dependent upon specific clinical features.3 As with appendicular disease, local recurrence of PVNS following surgery is not uncommon, reported in approximately 20% of cases.16 However, there remains a paucity of literature providing long-term guidelines for the use of imatinib as treatment of PVNS. Additionally, the use of post-operative external-beam radiation following complete or cytoreductive surgery has been reported as an effective means of preventing recurrent PVNS; despite the potential risks of impaired wound healing, radiation-induced neurological damage and the small risk of radiation-induced neoplasia inherent with all radiation therapy, modern treatment planning and typical doses between 30–50 Gy allow for improved control rates of PVNS relative to surgical management alone and confer a minimal risk of neurological injury.Gross-total surgical resection is the recommended first-line therapy for PVNS of the spine.PVNS of the spine is a rare, benign, but locally aggressive lesion that can mimic malignancy, metabolic or inflammatory disease on CT scan, MRI and FDG-PET, resulting in a diagnostic dilemma for imagers, clinicians and pathologists. As reported in this instructive case of PVNS of the cervical spine, location of the lytic soft-tissue lesion about the joint space is a critical diagnostic discriminator.PVNS of the spine is rare, but when it does occur in the cervical spine, it typically involves the posterior elements.PVNS classically has a lytic radiographic appearance and demonstrates low-to-intermediate signal intensity on all MR pulse sequences. The intra-articular location of PVNS can be used to differentiate it from GCTTS, which involves the tendon sheath or is found more focally within the joint space.


Primary heart sarcomas are exceedingly rare tumors. Among primary cardiac sarcomas, synovial sarcoma is one of the rarest, involving cardiac cavities or pericardium.Two cases of synovial sarcoma are presented with the clinical course and therapy. Both cases were treated with surgery and chemo/radiotherapy. Interestingly, one of the patient, a 52-year-old male with an intracardiac synovial sarcoma, undergone a SynCardia total artificial heart implantation, but died for multiple pulmonary metastases waiting for transplantation.Complete surgical resection of cardiac synovial sarcoma is the gold standard of therapy, though rarely possible. Although guidelines for the treatment are not well established, due to limited number of cases reported, chemotherapy and radiotherapy are frequently administered and seem to prolong mean patient’s survival. Cardiac transplantation could be considered in selected cases. Synovial sarcoma is a rare soft tissue malignant tumor, usually affecting children and young adults . PrimaryPatients present with nonspecific symptoms, including dyspnea, thoracic pain and pericardial effusion. Complete resection, when feasible, is the treatment of choice, with additional chemotherapy and/or radiation therapy, when necessary , 5–7. HoA 52-year-old male presented with severe headache and elevated blood pressure (180/120 mmHg). Color Doppler echocardiography revealed a large, solid right intraventricular mass of 7.1 × 2.2 × 4.7 cm, originating from the middle-inferior ventricular septum and extending into the right atrium, with systo-diastolic fluttering Fig. . A cardiA cardiac biopsy via catheterization was not conclusive regarding the precise nature of the lesion. After informed consent, the patient underwent surgery. Intraoperatively, a whitish mass, 3 × 4 cm, non-adherent to the wall, was found in the right atrium after median sternotomy and atriotomy Fig. . ThroughComputed tomography scan (CT) and cardiac MRI, performed one month after surgery, prompted administration of chemotherapy  due to increase of the residual ventricular tumor. However, since at the end of this treatment a (18) F-fluorodeoxyglucose positron emission tomography/CT revealed an increased ventricular mass, a SynCardia total artificial heart was implanted in the patient. Unfortunately, waiting for transplantation, the patient developed multiple pulmonary metastases and rapidly died, one and half year after initial surgery.A 39-year-old man presented with signs of cardiac tamponade. The echocardiography revealed abundant pericardial effusion with a large intrapericardial mass. The patient underwent surgery after informed consent. At surgery, a pericardial tumor (9.8 × 6.1 × 3.2 cm) adherent to the anterior wall of the aorta, superior vena cava and right atrium, was extensively removed. No deep infiltration of the myocardium was apparent and aortic reconstruction was unnecessary. Since intraoperative histological examination did not reach a conclusive diagnosis, further surgery was not performed. Definitive histology revealed a monophasic (spindle cell) synovial sarcoma carrying an SYT-SSX2 transcript.Postoperatively, echocardiography, cardiac MRI and CT did not show residual tumor. Owing to a diagnosis of synovial sarcoma, the patient underwent chemotherapy , remaining in healthy conditions for the following 8 months, before the onset of tachycardia and dyspnea. Echocardiography, cardiac MRI and CT revealed a recurrent pericardial tumor extensively involving both atria and left pulmonary veins. Because further surgery was considered unfeasible, an additional course of chemotherapy was started , followed by intensity-modulated radiotherapy (DT 54 Gy/25 sessions/2 months). After a temporary improvement of the clinical conditions, CT documented an extensive regrowth of the tumor, with involvement of the heart, great vessels, left bronchus and esophagus, with mediastinal lymphadenopathy. The patient died 32 months after surgery.Cardiac synovial sarcoma is a very uncommon cardiac sarcoma. There is a lack of accurate guidelines for the treatment of CSS owing to the heterogeneity and scarcity of clinical information, derived from a limited number of cases reported on the topic, mainly as single case report. In a recent extensive analysis of 60 CSS patients derived from 54 articles present in the literature, Wang et al. found that overall survival of CSS patients was affected by patient’s age (less than 30 years) and chemotherapy . HoweverIndeed, a complete resection of the cardiac tumor is frequently impossible to perform, because of the infiltrativeness and extension of the neoplasm. On the other hand, it is difficult to establish precise chemo-radiotherapy guidelines, due to different adjuvant therapy options in the few cases hitherto described. Hence, the choice of optimal treatment in the single patient is challenging. Therefore, it seems worth investigating both optimal surgical procedures and additional chemo-radiotherapy protocols in order to improve patient’s survival. As a matter of fact, total artificial heart implantation, as performed in one of our patients, could offer a temporary solution looking forward to heart transplantation . AlthougBergh et al.  found thThus, as little information on CSS treatment is available in literature, analysis of a major number of cases is necessary in order to establish appropriate therapy protocols. Although limited, our contribution provides further data on the management of this rare malignant tumor.


The present report describes one of the rarest complications of cesarean section, uterocutaneous fistula, diagnosed on magnetic resonance imaging (MRI). A 37-year-old female with history of lower segment caesarean section (LSCS) four years previously presented with a chief symptom of discharge from the right end of a Pfannenstiel incision and on further evaluation was found to have uterocutaneous fistula arising from the LSCS scar to the right end of the abdominal incision. Uterocutaneous fistula is a rare delayed complication of LSCS and MRI plays a definitive role in the accurate diagnosis and delineation of the tract. The present case highlights that although rare, uterocutaneous fistulae must be kept in mind in patients presenting with discharge from the abdominal incision site and MRI evaluation should be performed in such cases for appropriate delineation of the tract. PRECIS: To identify the possible risk factors for postpartum urinary retention.. Uterocutaneous fistulae, being a rare condition, need appropriate diagnosis for which magnetic resonance imaging (MRI) plays the most important role, thereby helping in the proper delineation of the tract to guide the appropriate management. The most common cause of uterocutaneous fistula is incomplete closure of the cesarean section wound. Earlier diagnosis can be made using fluoroscopic or cross-sectional modalities. Surgical repair is the treatment of choice with preoperative gonadotropin administration showing better outcomes, but few cases may result in hysterectomy. We describe this case of uterocutaneous fistula, which occurred 4 years after a caesarean section and was diagnosed using MRI.Uterine fistulae usually occur between the uterus and bowel or bladder  with uterocutaneous forming the rarest variety of uterine fistulae. The fistulae occur due to postoperative injuries or infections, use of drains, and incomplete closure of incisionA 37-year old female with a history of lower segment cesarian section (LSCS) performed four years previously presented with the chief symptom of discharge from the incision site. MRI of the pelvis was performed, which revealed an enhancing fistulous tract originating from the site of the lower segment of the cesarean section and traversing through the parietal wall opening at the right edge of abdominal incision scar . The dia,5.During the era of classic cesarean section, a number of cases of uterocutaneous fistula were reported but with the advent of LSCS, the frequency has decreased to a large extent. Most uterocutaneous fistulae owe their origin to infections  complicating uterine or abdominal scars. Besides LSCS, the causes of uterocutaneous fistulae include post septic abortion, placement of drain, missed uterine perforation following a diagnostic laparoscopy,7. Other rare causes that may result in the formation of uterocutaneous fistula are patients with multiple abdominal myomectomies, history of hysterectomy, and as a primary presentation in underlying gynecologic malignancy such as endometrioid adenocarcinoma, which can predispose the weak cesarean scar to fistula formation,7,8. The blood leakage from the incision site during menstruation has been described as pathognomonic of the uterocutaneous fistula.Cases of post-partum hemorrhage wherein B-lynch sutures are placed have high risk of scar dehiscence, which may result in uterocutaneous fistula,3. CT imaging helps in the proper delineation of the tract after the injection of the contrast agent through the abdominal site, but soft tissue resolution of CT scans is less compared with MRI,11. MRI provides good soft tissue resolution, avoids radiation , and helps in proper delineation of the fistulous tract and its relation to the surrounding viscera. The assessment of other pelvic organs with greater spatial resolution is possible. Intravenous contrast administration shows the enhancement of the granulation tissue along the fistulous tract, which gives a clue about the active status of the fistula. Further, contrast agent can also be administered via the abdominal site as in other investigations to check the patency of the.The various modalities that can be used in the diagnosis of uterocutaneous fistula include fistulography with injection of contrast through the skin site, hysterosalpingography with injection of contrast via cervix, computed tomography (CT) scanning, and MRI. Fistulography and hysterosalpingography define the fistulous tract but cannot provide details of its communication with the other intra-abdominal visceraHence, MRI forms the best investigative modality in the assessment of uterocutaneous fistulae and other pelvic post-operative complications.


The ossification of the ligamentum flavum (OLF) was characterized by the replacement of the ligamentum flavum by ectopic new bone formation. Although OLF is well known as one of the causes of thoracic myelopathy through the compression of the spinal cord from the posterolateral side, contiguous multilevel OLF is quite a rare cause of myelopathy. Severe ossifications were reported that only present in individuals aged over 25 years. External triggers of OLF might likely be increased as a consequence of biomechanical stress to the effect of posttraumatic ossification. The authors described one rare case of an enormous contiguous multilevel OLF in a 20-year-old female's thoracic spine after mild back trauma, to the extent that it was misdiagnosed as an old epidural hematoma initially. This female with obesity presented with a 6-month history of progressively worsening dorsal cord disorders. Resection or floating of the enormous OLF could be successfully achieved using O-arm-based navigation, and sensory loss, numbness, and gait disturbance were improved after operation. The ligamentum flavum may undergo various pathologic changes including hypertrophy, calcification, cyst formation, hematoma, and ossification , 2. The Here, the authors described one case of an enormous contiguous multilevel OLF after mild back trauma, to the extent that it was misdiagnosed as an old epidural hematoma initially. Despite the high rate of perioperative complications in OLF resection, resection or floating of the enormous OLF could be successfully achieved using O-arm-based navigation in the present case.2) presented with a 6-month history of progressively worsening paresthesia of the lower limbs and gait disturbance. Moderate muscle weakness, severe spasticity in the lower limbs, a superficial hypoesthesia below the Th6 level, and severe dorsal cord disorders were noted on the first physical examination. There was a history of a contusion in the back following the drop from 2 meters height, treated with conservative therapy 2 years previously. She showed no feature of skeletal dysplasia. Laboratory data revealed no abnormality.A 20-year-old female with obesity (body mass index: 33.3 kg/mMagnetic resonance imaging (MRI) showed a dorsally located epidural lesion (Th6-Th9) which seemed a heterogeneous mass hypointense on both T1- and T2-weighted images . These cThoracic pedicle screw insertions in Th5, 6, 10, and 11 levels preceding thoracic cord decompression and the surgical excision of ossified lesion or floating of ossified lesion with the slight adhesion to dura matter were performed under O-arm-based navigation system . When Th6-Th10 laminectomy was performed, the posterior epidural space was filled with an ossified mass. Between Th6-Th9 laminas and the ossified mass, there was a part of denatured flavum with abundant blood vessels. In particular, these ossified lesions developed in Th7-Th8 levels, where this mass adhered to bilateral pedicles of Th7 and Th8 . To floaOssification of the ligament tissue was observed in the resected tumor. Contiguous multilevel OLF was diagnosed and confirmed by pathology . After oThe onset of OLF-related symptoms with consecutive surgical treatment ranges between 55 and 65 years, as the prevalence increases with age. The formation of OLF in the population is strongly considered due to degenerative wear-and-tear. There are reports on OLF manifestations in relation to diabetes mellitus, activity patterns , body weight , fluorosis, increased bone density, hyperparathyroidism, diffuse idiopathic skeletal hyperostosis (DISH), ankylosing spondylitis, acromegaly, and achondroplasia . ExternaOLF can significantly contribute to a spatial reduction of the thoracic spinal canal, resulting in slowly progressive or acute paraplegia after trauma to the back. Given a 2-year-ago history of trauma and her young age, the formation of OLF in the present case could be attributed to the effect of posttraumatic ossification, in addition to the influence of intrinsic high-BMI values. Because of the difficulty in clinically monitoring contusion in the back without fracture or neurological deterioration when we take the attributed risks of imaging radiation exposure and medical expenses into consideration, the exact incidence of epidural hematoma and much more epidural hematoma ossification is unknown. Although the posttraumatic or postoperative ossified epidural hematomas in cranial, especially in children, were discussed in a few reports –11, the According to the previous classification on axial CT scan and T2-weighted sagittal MRI image , we coulThoracic myelopathy secondary to OLF can be treated with laminectomy. Further, laminectomy with partially internal fixation is safe and effective in treatment of patients with symptomatic multilevel OLF . The oveThe authors described quite a rare case of an enormous contiguous multilevel OLF in young female thoracic spine after mild back trauma. Asymptomatic chronic epidural hematomas even in young adult spine might undergo large ossification, and enormous OLF could prevent natural absorption, leading to cause myelopathy. If there is a chronic spinal epidural mass with ossification, we recommend surgery before irreversible change has been completed. The use of O-arm-based intraoperative 3D scans for navigation can make drilling and resection of an enormous contiguous OLF reliable.


McCune-Albright’s syndrome (MAS) is a rare disorder that is characterized by café-au-lait macules, fibrous dysplasia of the skull and endocrinopathies like excessive secretion of growth hormone by a hyper-functional pituitary adenoma (PA).We describe the case of a 43-year-old male with history of Gigantism in 1990 secondary to a GH-secreting pituitary macroadenoma that was treated via microscopic transsphenoidal surgery at that time.He was reported as asymptomatic for 26 years until he developed headache and right temporal hemianopia with left amaurosis. Also ptosis and proptosis was found caused by a re-growth of the tumor on the follow up MRI.A second surgical procedure was performed via a dorsolateral craniotomy. Gross total resection was also achieved with a Neuropathology report of a pituitary adenoma tissue accompanied by extended dystrophic calcification and bone formation.This is a rare case of MAS. Gigantism within the context of a pituitary calcification raises special diagnostic and therapeutic challenges. The cause of the excessive secretion of GH in MAS is not well understood concluding that it seems to be a different etiology of patients with Acromegaly and Gigantism in non-MAS patients. The hIdentifying dystrophic calcifications in a PA in the preoperative imaging studies may represent a key role on the surgical decisión, since the scope in achieving a gross total resection can be more difficult to accomplish in the occurrence of extensive calcification ,11. MediWe reported a rare case of a 43 year old male with gigantism due to an invasive PA in association of MAS that showed extensive dystrophic calcifications that calcified the pituitary tumor. The present case has been reported in accordance with the SCARE guidelines for case reports .22.1A 43-year-old man with pituitary gigantism from the age of 16 secondary to a GH-functional PA. He underwent microsurgical trans sphenoidal surgery for resection of a PA in 1990 and adjuvant radiotherapy given in 2011 . In 2016, almost 27 years after the initial surgery, he presented progressive decrease in vision on the left eye for six months. He also complained of excessive perspiration and sweating and had a past history of carpal tunnel syndrome. He was found to have gigantism features as extreme physical size (2.07 mts height) and pigmentation around eyes, neck and flexures.In ophtalmologic examination, a left ptosis with both exophthalmos and upward/inward limitation on left eye movements was achieved, and in the Goldmann’s test perimeter an ipsilateral amaurosis and right hemianopia  .Fig. 1GoRandom serum GH level was 0.071 ng/ml (0.003–0.97 ng/mL) with a normal GH concentration during the Oral Glucose Tolerance Test (OGTT) with a GH-nadir of 0.13 ng/ml (the gold standard for diagnosis is that GH excess fails to suppress serum GH level to less than 1 ng/ml after OGTT). The Insulin-like Growth Factor-1 (IGF-1) 84.3 ng/ml (64–210 ng/mL); the rest of hormones are listed and shown in On brain Magnetic Resonance-Imaging (MRI) (Siemens 3.0 T magnetic resonance scanner and a 32-channel head coil) revealed a large policystic selar lesion with extensive osteophytic reaction and invasion of the ipsilateral orbital apex associated with fibrous dysplasia see A and B w2.2A second surgical resection was performed in 2016 by a left orbitozygomatic approach for tumor removal with no acute complications A–C. Thre2.3After the informant consent was obtained, brain autopsy was performed. Neuropathologic examination of the pituitary lesion removed in 2016 (first neuropathology report was unavailable) was characterized macroscopically by a 40 × 40 mm mass with grayish outer surface and inner yellowish-irregular zones thickened by “sand-like” calcifications A and B. 3The MAS has been thought as a consequence of postzygotic mutations in GNAS1, located on 20q13.3, a complex gene that uses several promoters to produce gene products like the ubiquitously expressed α-subunit of the Gs stimulatory protein (Gsα) . This inRecently, some molecular and genetic etiologies of pituitary gigantism have been revealed to produce several genetic diseases like Multiple Endocrine Neoplasia (MEN) type 1 and 4, MAS, Carney complex, familial isolated PA or in association to defects in the familial succinate dehydrogenase-genes and the recently identified X-linked acrogigantism (X-LAG) syndrome ,17.The biological activities of Bone Morphogenetic Proteins (BMPs) in the endocrine system have been revealed. For example, the BMP-4 plays a key role in the initial development of the adenohypophysis and is also active and functionally involved in the differentiation of pituitary tumors, including prolactinomas and Cushing's disease but not in GH-releasing hormone PA .Specifically in the primary pituitary tumors, one of the main neurosurgical cases that represents 10% of intracranial tumors and 10% in autopsy series as well ,20. The It is known that the presence of calcification in a PA is more frequent with prolactinomas, because the ossification derived from the osteo-metaplasia from the mesenchymal fibroblasts due to the effects from secondary ischemia by the tumoral outgrowth and the autocrine effects of prolactin hormone (Prl) ,18.Only eleven cases of a calcified PA have been described, comprising 5 reports with prolactinomas, 3 patients with a GH-secreting adenomas, 2 patients due to a so-called “TSHomas” and one patient with a gonadotrophin-secreting tumor ; but theAdditionally, matrix proteins are considered to be essential for biomineralization and to be important factors in cranioharyngioma calcification, like the Osteopontin (OPN) as a noncollagenous, acidic bone-matrix glycoprotein, which binds tightly to hydroxyapatite and appears to form an integral part of the mineralized matrix, probably important to the integrity of cell-matrix interactions .Since the surgical management in craniofacial deformities is complicated in particular by the frequent post-operative FD re-growth, the treatment should be focused on the correction of functional deformities and notably in the optic nerve decompression in patients with objective vision loss . Convers4This is a rare case of gigantism with micro-calcifications in a GH-releasing PA associated to MAS. There has been a recognized association between Gigantism and MAS, however the presence of dystrophic calcifications in PA is rare and has to be recognized in the preoperative imaging as an important finding to consider on the surgical decision-making, since it may determine the complexity of its resection caused by struggling technical issues in the trans sphenoidal surgery due to thickening of the skull base ,12. FurtAll authors report no conflict of interest.None funding.The internal review board of the National Institute of Neurology and Neurosurgery does not require ethical approval for case reports when informed consent was taken, if required by the journal it could be granted.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.Miguel Vega Arroyo: Writing the paper, analysis, design and reviewing literature.Martha Lilia Tena-Suck: Data collection and data analysis.Celia Teresa de Jesús Álvarez Gamiño: Translating, design and reviewing literature.Salinas-Lara Citlaltepetl: Data collection and data analysis.Gómez-Amador Juan Luís: Concept of paper, getting approval and interpretation.Not apply.Miguel Vega Arroyo.Not commissioned, externally peer reviewed.All the authors have contributed substantially to the design, realization, analysis and presentation of the products of this work, in such a way that they have taken responsibility for it. Each author believes that this article represents the communication of a valid, true and ethical work, and each author has reviewed the content of this writing and has approved it for publication, this authorization being dictated by the author of correspondence, but whose responsibility is delegated to it, without conflicts, by each of the authors.In addition, all authors attest to the precision with which the analysis were made, to the point where human error not noticed allowed to achieve. The authors declare that this work has never been published, either partially or totally, in another scientific journal, and that it is not being considered anywhere else for publication. On the other hand, there is no affiliation with any organization with a direct or indirect monetary or ethical interest with the substance of what is discussed in this scientific paper; therefore all the authors declare that there is no conflict of interest that affects the design and the report of the results of the study.All relevant data are within the paper.


Isolated tuberculous epididymo-orchitis is a rare manifestation of the vast extrapulmonary tuberculosis (EPTB) disease spectrum, especially in developed nations, making it prone to delayed diagnosis or misdiagnosis and inadvertent orchiectomy. Several observational studies and case reports have been reported with the successful use of fine-needle aspiration cytology (FNAC) in diagnosing tuberculosis orchitis, thus avoiding inadvertent orchiectomy. Because tuberculous epididymo-orchitis can mimic testicular neoplasm, the use of FNAC is not prevalent in developed countries for fear of the seeding of tumor cells and there is a lack of consensus on the use of FNAC for diagnostic purposes in such patients.We report a case of a 27-year-old man with an atypical presentation of genitourinary tuberculosis (TB) and its management. The case report also reviews the literature to discuss the available evidence and tries to answer the long-standing question on the role of FNAC in the diagnosis of tuberculous epididymo-orchitis. The currently available literature has demonstrated the safety and efficacy of FNAC in diagnosing TB epididymo-orchitis and, based on our review, the benefits of differentiating TB epididymo-orchitis from testicular malignancy using FNAC exceeds its minimal risk and must be considered to minimize clinical diagnosis error and unnecessary orchiectomy in low-risk patients. Tuberculosis (TB) is a widely prevalent disease worldwide, especially in developing countries. The incidence of tuberculosis is decreasing worldwide. The US TB incidence in 2018  represented a 1.3% decrease from 2017; the rate among non-U.S.-born persons was >14 times that in US-born persons [A 27-year-old Hispanic male from Guatemala, with no past medical and family history, was brought to the hospital by the border patrol for the complaint of right-sided scrotal pain and gradually increasing swelling started about one month before presentation. The patient denied any abdominal pain, dysuria, sexually transmitted disease, cough, weight loss, night sweats, or any other symptoms. On arrival, he was febrile with temperature 101.9 F, with other vital signs being unremarkable. Only significant findings on the physical exam were marked swelling and induration, with mild tenderness of the right testis and epididymis. Lab workup included a normal complete blood count and normal renal function. Urinalysis was unremarkable with negative chlamydia and gonorrhea urine polymerase chain reaction (PCR).A testicular ultrasound showed an irregular heterogeneous echotexture of the right testicle with multiple hypoechoic masses with septation, microcalcification, and increased hypervascular flow, as shown in Figure CT scan of the chest with contrast showed pleural thickening with bilateral pleural effusion, compressive atelectasis, and multiple bilateral mildly enlarged hilar lymph nodes. Further investigation with tumor markers, including alpha-fetoprotein (AFP), beta-human chorionic gonadotropin (hCG), and lactate dehydrogenase (LDH), was unremarkable. Human immunodeficiency virus (HIV) was also ruled out. Three successive sputum samples for consecutive three days were negative for acid-fast bacilli (AFB) stain. For the findings of the CT scans showing lymphadenopathy, a right indurated testicular mass, and poor response to antibiotics, a right radical orchiectomy was done for a suspected diagnosis of right testicular neoplasm with secondary infection. Biopsy and FNAC of the testis were not done to avoid causing the seeding of tumor cells to the inguinal lymph nodes because of suspicion of the presence of a cancerous right testicular mass.Testicular tissue histopathological examination and AFB stain showed caseating granulomas and acid-fast bacilli, as shown in Figure Urogenital TB is the third most common presentation of EPTB after lymph node and pleural effusion, accounting for 2% to 20% of total cases of TB epididymo-orchitis accounts for 10%-50% of the total cases of EPTB .The differential diagnosis in patients with testicular epididymo-orchitis can be broad. Symptoms localized to the single scrotum can be present in testicular neoplasm, bacterial epididymo-orchitis, genitourinary sarcoidosis, and hernia with hydrocele. Many times, an atypical presentation can cause either delayed diagnosis or misdiagnosis and can lead to orchiectomy, as it mimics testicular tumors. Diagnostic tools to diagnose genitourinary TB include urine acid-fast stain, mycobacterial culture, and polymerase chain reaction (PCR) for Mycobacterium tuberculosis and radiographic studies -5. PositIn our case, the patient’s presentation with only fever and unilateral scrotal swelling prompted a diagnosis of the more common bacterial epididymo-orchitis. After an inadequate response to appropriate antibiotic therapy, further imaging studies were obtained. For findings of lymphadenopathy and pleural effusion on imaging, the patient also underwent bronchoscopy and sputum AFB stain, which was negative, and cultures only became positive after one week. Right orchiectomy was carried out for concern of underlying testicular malignancy. Although the tumor markers were negative in our case, studies have shown that 25% of seminoma testicular neoplasm can have beta-hCG elevation without AFP elevation in contrast to non-seminomatous germ cell tumor where beta-hCG and AFP can be elevated in 80%-85% cases -8.Many times, an atypical presentation can cause either delayed diagnosis or misdiagnosis and can lead to inadvertent orchiectomy, as it mimics testicular tumors. Kinnear et al. reported a case where the patient with a similar presentation initially diagnosed with bacterial epididymo-orchitis and treated with antibiotics first . Later, Handa et al. and Gupta et al., in their retrospective analysis of a combined more than 250 patients, found FNAC useful in 90% cases for a diagnosis of tuberculous orchitis -15. ViswNonetheless, in our opinion, based on a literature review, FNAC Is a cost-effective and less invasive procedure. It is easy to perform and has a low risk of adverse effects and complications. The diagnostic accuracy of FNAC is increased when a cytopathologist is involved in the interpretation of the smear cytology. FNAC is also very quick, and the results of the smear can be obtained rapidly to guide surgical necessity by the urologist. It can serve as an essential tool in the diagnosis of a suspected case of tuberculous epididymo-orchitis, with the benefit of avoiding inadvertent orchidectomy. FNAC should be considered if epidemiologic risk factors for TB are present and especially if tumor markers are negative.Our case report illustrates the diagnostic challenges associated with an atypical and rare presentation of the EPTB. The condition has been long-recognized and well-illustrated in the medical literature. However, it remains mostly unknown, especially to physicians trained in developed nations given the low prevalence of tuberculosis, causing delayed diagnosis and putting patients to grave risks of further complications. It also highlights the challenge of immigrant refugee health where a diagnosis delay or misdiagnosis may lead to catastrophic results because of poor access and follow up to appropriate health care services. It serves as a learning lesson for keeping a high suspicion for the patients with risk factors and the appropriate use of FNAC in a patient with less suspicion for an underlying neoplasm in the absence of elevated tumor markers that can minimize clinical diagnosis error and unnecessary orchiectomy. Currently, available literature has demonstrated the safety and efficacy of FNAC in diagnosing TB epididymo-orchitis. We believe that the risk of the spread of tumor cells is overestimated. Hence, the benefits of differentiating TB epididymo-orchitis from testicular malignancy using FNAC exceeds its minimal risk. Further clinical studies focused on the diagnostic yield of FNAC and complication rates like seeding of tumor cells can provide more clarity and establish it as a standard of care.


Primary hyperparathyroidism is characterized by hypercalcemia and elevated or inappropriately normal serum levels of parathyroid hormone. Brown tumor of bone is a rare non-neoplastic lesion resulted from abnormal bone metabolism in hyperparathyroidism. However, nowadays, skeletal disease caused by primary hyperparathyroidism is uncommon. We report a case of brown tumor in the mandible as the initial exhibition of primary hyperparathyroidism associated with an atypical parathyroid adenoma.99mTc-MIBI SPECT-CT scintiscan and pathological examination were used to confirm the diagnosis of brown tumor. The patient’s symptom improved after parathyroidectomy.The patient was a 49-year-old female, she had a pain mass on the right mandible a year ago and was treated with root canal therapy and marginal resection. After seven months, the mass recurred and enlarged. Enhanced CT scan, laboratory examination, Ultrasonography, 99mTc-MIBI SPECT/CT scintigraphy is a highly sensitive examination of the localization diagnosis of hyperparathyroidism. Brown tumors should be considered in the differential diagnosis of osteolytic lesions to avoid unnecessary and harmful interventions. Primary hyperparathyroidism (PHPT) is a common endocrine disorder that is characterized by hypercalcemia and elevated or inappropriately normal serum levels of parathyroid hormone . Single We report a case of brown tumor in the mandible as the initial exhibition of PHPT associated with an atypical parathyroid adenoma.A 49-year-old female was referred to our hospital for a recurrent right mandible mass one year after primary excision. One year ago, she had a painful mass on the right mandible, which was first treated with root canal therapy and then by marginal resection of the right mandible at another medical institution. The postoperative pathology suggested giant cell granuloma. Seven months after surgery, the mass recurred and gradually enlarged.99mTc-MIBI SPECT-CT scintiscan demonstrated increased radiotracer uptake at the site of the left parathyroid, which was suggestive of parathyroid adenoma (Fig. On admission, an enhanced CT scan of the patient revealed a 3.6*2.1 cm lesion on the right mandible (Fig. In this case, the combination of hyperparathyroidism and the existence of giant cells in the mandibular pathology as well as gradual degradation of the tumor after operation supported the diagnosis of brown tumor.Hyperparathyroidism may be primary, secondary and tertiary. Primary hyperparathyroidism is characterized by excessive PTH production and hypercalcemia, most frequently due to parathyroid adenoma. Secondary hyperparathyroidism is usually caused by vitamin D deficiency, malabsorption, or hypercalciuria. Low serum calcium levels resulted from primary diseases brings about redundant secretion of PTH. Tertiary hyperparathyroidism, in most cases, developed from secondary hyperparathyroidism and evolves into a more severe circumstance with autonomous PTH secretion . As in oNowadays, with the increasingly available measurement of biochemical examination, the detection rate of hyperparathyroidism has been increased and PHPT has evolved into a typically asymptomatic disease, especially in developed countries where serum calcium levels are routinely measured , 2, 6. ABrown tumors are giant cell lesions caused by abnormal bone metabolism in hyperparathyroidism. Increased circulating PTH aggravates osteoclastic bone absorption, then leads to diffuse osteopenia, fractures or multiple circumscribed lytic lesions , 8. Ther99mTc-MIBI SPECT/CT scintigraphy is a highly sensitive examination of the localization diagnosis of hyperparathyroidism [The most prominent aspect of this case was the discovery of a brown tumor in the mandible, which was the initial exhibition of an atypical parathyroid adenoma. However, the confirmed diagnosis was made after the surgery in the pathological findings, how to obtain certain diagnosis before the parathyroidectomy surgery remains to be explored. yroidism . We sugg


Primary intraosseous meningioma is a subset of extradural meningioma that arises in the bone, and only a few cases have been reported to date.An 80-year-old man presented with decreased hearing on the right side accompanied by a disturbance of balance 10 months prior to admission. Magnetic resonance imaging revealed an 8 × 7 cm osteolytic mass in the right posterior fossa related to the petrous bone, with extension to the cervical region. During surgery, the tumor was found to be located extradurally, with no invasion of the dura. The tumor was removed entirely, apart from a small portion around the jugular foramen to avoid lower cranial nerve injury.The final diagnosis was primary intraosseous osteolytic meningioma with atypical pathology. Here, we report a rare case of an osteolytic skull lesion in the skull base not invading the dura and with extensive bone destruction.The online version of this article (10.1186/s12883-019-1392-5) contains supplementary material, which is available to authorized users. Meningiomas are common intradural lesions that arise from the arachnoid cap cells of the arachnoid layer. In contrast, primary extradural meningioma is a relatively rare entity, accounting for less than 2% of all meningiomas , 2. TheyAn 80-year-old man presented with a progressive decrease in hearing on the right side accompanied by dizziness and disturbance of balance 10 months prior to admission. The neurological examination revealed right hypoglossal nerve palsy. Audiometry documented complete sensorineural hearing loss on the right side.Skull x-ray and cranial computed tomography (CT) scans showed a large osteolytic lesion with bone destruction, including the temporal bone, occipital bone, clivus, jugular foramen, and hypoglossal canal Fig. a and b. The patient underwent surgery to obtain a pathological diagnosis and for complete removal of the mass. A C-shaped postauricular skin incision was made that extended to the neck. The scalp was reflected anteriorly, and the mass infiltrating the subcutaneous tissue was exposed. The lesion appeared as a firm gray mass that had destroyed the temporal and occipital bones. The dura was intact with no invasion, and the lesion was easily peeled off. For the cervical part of the tumor, the major vessels were secured inferiorly, and the mass was removed up to the skull base. The tumor was removed entirely, except for a small portion around the jugular foramen to avoid lower cranial nerve injury. Finally, the large empty space was filled with a sternocleidomastoid muscle flap Fig. . There wHistopathological studies confirmed a WHO  grade II atypical meningioma with up to 6 mitoses per 10 high-power fields Fig. . The Ki-Primary intraosseous meningiomas are rare cranial lesions that arise from the bone, and they represent the most common type of primary extradural meningiomas –3. The mPrimary extradural meningiomas are most commonly found in skull convexities, the paranasal sinus, and the middle ear but rarely in the skull base. Liu et al. reported 170 cases of extradural meningiomas in the head, and only 5.8% were located in the skull base . NotablyThe exact origin of extradural meningiomas is unclear, but several theories have been proposed. Their unusual locations are assumed to be the result of the aberrant differentiation or misplacement of undifferentiated mesenchymal stem cells . AlternaThe osteolytic subtype of intraosseous meningiomas is often misdiagnosed as a primary or secondary bone tumor due to its radiological appearance. The differential diagnosis of a solitary osteolytic skull lesion includes hemangioma, chondroma, chondrosarcoma, eosinophilic granuloma, epidermoid cyst, giant cell tumor, myeloma, and metastatic skull tumor , 23, 33.Primary extradural meningiomas were practically classified according to their location by Lang and colleagues Table  3]. The. The3]. Histopathological features are also important factors affecting tumor recurrence and prognosis. Recurrence was noted in 22% of benign intraosseous meningiomas in the literature, while it was reportedly 33% in cases of tumors with atypical or malignant pathology. In addition, aggressive atypical or malignant meningiomas had a significantly higher mortality of 29% compared to tumors with benign features 4.8%) . In prev% . In prWide surgical excision is the main treatment for extradural meningiomas, and it is potentially curative if complete resection is achieved , 7. In tIn conclusion, we performed surgical treatment for a rare case of primary osteolytic intraosseous meningioma in the skull base with extension to the cervical area. The histopathologic diagnosis was atypical meningioma. If possible, complete resection is the treatment of choice, and serial follow-up should be done to confirm recurrence or progression.Additional file 1:Figure S1. Immunohistochemical staining results. The tumor showed a wild-type p53 pattern  and exhibited strong cytoplasmic expression of β-catenin . Some tumor cells exhibited weak membranous expression of EGFR . The tumor was negative for Bcl-2 . The tumor shows membrane and cytoplasmic immunopositivity for EMA  and negative for S-100 protein . Vimentin is diffusely expressed in the cytoplasm of tumor cells . The Ki-67 proliferation index is estimated to be approximately 15% . Scale bar = 100 μm. (DOCX 8214 kb)


Intraductal papillary-mucinous neoplasms (IPMNs) are potentially malignant intraductal epithelial neoplasms that sometimes penetrate into other organs. To the best of our knowledge, no report has yet described a case with penetration into the spleen. We recently encountered a case of IPMN with penetration of the stomach and spleen that was successfully treated by total pancreatectomy.A 70-year-old female visited our hospital with a complaint of fever and abdominal pain. Contrast-enhanced computed tomography (CT) revealed dilatation of the main pancreatic duct in the entire pancreas and penetration into the stomach and spleen. Upper gastrointestinal endoscopy revealed mucin extruding from four openings of the fistula in the stomach. No malignancy was detected based on cytology of the mucin. Inflammation markers and tumor markers  were elevated in the blood. The pre-operative diagnosis was IPMN of main pancreatic duct type penetrating into the stomach and spleen. A total pancreatectomy and splenectomy were performed, combined with distal gastrectomy including resection of the fistulas between the pancreas and stomach. No postoperative complications were noted. Histopathological examination of the resected specimen revealed atrophy of the pancreatic parenchyma, and the main duct of the pancreas was filled with mucin. Mucin-producing malignant tumor cells were detected in the epithelium of the main pancreatic duct with no signs of invasion. No malignancy was found at the fistulas between the pancreas and stomach or spleen. The patient was finally diagnosed with non-invasive intraductal papillary-mucinous carcinoma (IPMC) of main pancreatic duct type. Mechanical penetration was suspected as a mechanism of the penetration. The patient remained disease-free without evidence of recurrence more than 15 months after the operation.Though IPMNs sometimes penetrate into other adjacent organs, penetration into two organs, including the spleen, is rare. The rare case of IPMC penetrating into the stomach and spleen presented here was treated successfully by total pancreatectomy. In 1982, intraductal papillary-mucinous neoplasm (IPMN) was reported by Ohashi et al. as a mucus-producing pancreatic carcinoma characterized by a favorable prognosis [Kimura et al. initially reported nine cases of IPMN penetrating into other organs, such as the common bile duct, or developed fistula formation . KobayasA 70-year-old woman was admitted to our hospital because of upper abdominal pain. Her medical history included appendicitis at 20 years old. Upon physical examination, left hypochondriac pain and tenderness in the upper abdomen were noted. The laboratory examinations revealed elevated inflammatory markers  and biliary enzymes . The levels of tumor markers were also elevated . Pancreatic tumor markers were not elevated . Contrast-enhanced computed tomography (CT) revealed a markedly dilated main pancreatic duct (MPD) 55 mm in length in the whole pancreas, and the whole pancreatic parenchyma was thinning with atrophy Fig. . In addiDuring the last three decades, an increasing number of reports of IPMN of the pancreas have been published , 4, 5. TWe experienced a case of IPMN of the pancreas penetrating into the stomach and spleen that was successfully treated by total pancreatectomy. This case could contribute to improving our understanding of this type of neoplasm.


Sialolithiasis is the most common disease of the salivary glands. Sialolithiasis usually develops in the major salivary glands, and rarely in the minor salivary glands, with only 2% of all cases of sialolithiasis occurring in the minor salivary glands and sublingual glands. Sialoliths in the minor salivary glands result in few or no clinical symptoms and are seldom identified on imaging.We report herein our experience with a case of minor salivary gland sialolithiasis in a 67-year-old woman. On examination, an elastic soft, mobile, and well-circumscribed mass was palpable within the left upper lip. Ultrasound examination revealed a hypoechoic mass with heterogeneous internal echoes. The mass was excised under local anesthesia. Based on histopathological findings, a diagnosis of minor salivary gland sialolithiasis was established.Diagnosis of minor salivary gland sialolithiasis is challenging due to the difficulty of detecting sialoliths on imaging. A well-circumscribed mass was detected in the upper lip, and ultrasound examination revealed a round lesion, raising the suspicion of a benign tumor. Other diseases that can develop at the upper lip are calcified lymph node, phlebolith, fibroma, pleomorphic adenoma, myxoma, vascular malformation, salivary gland tumor, non-specific sialadenitis, and malignant tumor. Surgical excision is the favored approach for confirming a diagnosis of intramucosal nodular lesions. Sialolithiasis is a common disease of the salivary glands and can be attributed to factors such as ductal obstruction, inflammation, and foreign bodies. Sialolithiasis mostly occurs in the major salivary glands, and only rarely in the minor salivary glands –3. SialoA 67-year-old woman presented to the Department of Oral and Maxillofacial Surgery at Nagoya Ekisaikai Hospital with a chief complaint of a mass in the left upper lip. The patient had become aware of left upper lip discomfort in early August 2017, but had not sought medical attention as she was otherwise asymptomatic. She visited a local dentist for a routine checkup, who referred her to our department for further examination. In terms of medical history, she had hypertension that was adequately controlled by oral medications. Facial appearance was symmetrical, with no swelling of the lips  with heterogeneous internal echoes, but without calcification Fig. . The proHistopathological examination showed a mildly dilated salivary duct surrounded by granulation tissue and fibrous connective tissue with infiltration of inflammatory cells Fig. . No evidSialolithiasis is a condition in which a sialolith forms within a salivary gland or excretory duct and represents the most frequent disease of the salivary glands. Sialoliths form most often in the submandibular glands (92%), followed by the parotid glands (6%). The incidence is low for minor salivary glands and the sublingual glands: 2% for both sites combined .There are at least two potential reasons for the infrequent occurrence of sialoliths in minor salivary glands . First, Although the precise etiology of sialolith formation is unknown, two distinct phases are thought to be involved –9. DurinPatients with minor salivary gland sialolithiasis typically present with painless swelling or mass formation and lack specific clinical symptoms –3. The cHistopathological findings of minor salivary gland sialolithiasis include a dilated duct in minor salivary gland tissue, acinar atrophy, and periductal inflammation. These findings are essential for distinguishing the disease from mucus-secreting, low-grade mucoepidermoid carcinoma . A long-The sialolith in the present case was small and somewhat amorphous, and was thus considered relatively new. In addition, the lesion showed only inflammatory granulation tissue and fibrous connective tissue with inflammatory cell infiltration, ruling out a diagnosis of neoplastic disease. Sialolithiasis in a minor salivary gland is difficult to diagnose accurately based on clinical findings alone and clinical and histopathological diagnoses show poor consistency , 8, 9. TDiagnosis of sialolithiasis in minor salivary glands is challenging, and the number of cases diagnosed may underestimate the true incidence of the disease. Because salivary calculi of the minor salivary gland are small, they are only rarely discovered on imaging. As detecting a sialolith via imaging is difficult, surgical excision is the best approach to confirm the diagnosis of intramucosal nodular lesions.


Introduction: Chylous ascites is a rare form of ascites characterized by milk-like peritoneal fluid, rich in triglycerides. Clinical signs and symptoms include abdominal distention, pain, nausea, and vomiting. In gynecology, the most common cause for its occurrence is lymph dissection leading to impairment of major lymphatic vessels. There are only a few reported cases of chylous ascites arising after operations for benign diseases. Case report: We report a case of a 46-year-old female patient, who underwent laparoscopy for a myomatous node with chylous ascites occurring on post-surgery Day 2. The ascites was conservatively managed. The exact cause of the chyloperitonitis could not be determined. Conclusion: Although extremely rarely, chylous ascites may also occur in operative interventions for benign diseases in gynecological surgery. Chylous ascites (CA) is a rare form of ascites, which represents milk-like peritoneal fluid, rich in triglycerides ,2. The iWe present a case in which chylous ascites occurred after performing a myomectomy. In our review of the literature, we did not find any similar cases described.We present a 46-year-old, gravida 0 para 0 Caucasian woman with heavy, painful menstrual bleeding and dyspareunia for three months. No previous diseases, surgeries, or comorbidities were reported. The general physical examination detected no abnormalities. The gynecological examination found a tumor mass located on the left posterior uterine wall, of 4 × 4 cm size. Transvaginal ultrasonography demonstrated an intramural/subserous myoma of 40 × 41 mm. It was classified as Type 6 according to the International Federation of Gynecology and Obstetrics (FIGO) . LaparosWe closed the myometrium with interrupted sutures and extracorporeal knots. We decided to use a pelvic drain. Blood loss of 100 mL occurred. The pathology report of surgical specimens revealed fibroleiomyoma.On post-surgery Day 1, the patient felt well and had normal peristalsis, diuresis, and hemoglobin levels. The patient was afebrile, and there was an output of 300 mL from the drain with a small amount of blood. On post-surgery Day 2, 400 mL of milky-white fluid was noticed in the drain. Vital signs of the patient were stable with no abdominal pain and normal peristalsis .Suspecting chylous ascites, an analysis of the liquid was performed, which revealed high levels of triglyceride: 13.00 (high) mmol/L (1150 mg/dL). Laboratory characteristics of ascitic fluids revealed: glucose 3.57 (low) mmol/L; total protein 43.7 (low) g/L; albumin 24.4 (low) g/L; lactate dehydrogenase 668 (high) mmol/L; amylase 56 U/L; creatinine 84 mmol/L; and urea 4.9 mmol/L. We sent the fluid for cytological examination to determine the prevalence of erythrocytes, neutrophils, mature lymphocytes, and absence of tumor cells. No abnormalities of blood urea and creatinine of the patient were detected. A computed tomography (CT) was performed in her case. The purpose of the CT scan was to detect any unknown diseases, causing chylous ascites. There were no pathological findings. The patient initiated a low-fat oral diet with medium-chain triglycerides. During the next three days, 100 mL of milky white fluid was found in the drain. On Day 6, the drain fluid became serous (100 mL). On the next day, the drain was removed, and the patient was discharged from the hospital. Ten months later, the patient was free of symptoms and in a good health condition. The patient signed an informed consent form for the publication of anonymous clinical data.Chylous ascites is a rare form of ascites. Clinical findings are nausea, vomiting, abdominal distention, and pain ,12. The The chylous ascites has a various etiology and may be either atraumatic or traumatic  3,4]. I,4. I3,4]The basic causes of chylous ascites in developed countries are abdominal malignancies and cirrhosis , while iParacentesis or peritoneovenous shunting can be considered as options for patients who are poor candidates for surgery ,37,38.Iatrogenic surgical chyloperitonitis can be divided into two types after surgery: chylous ascites after oncogynecological operations or after operations for benign gynecologic pathology.It is most common after retroperitoneal oncogynecological operations . Ulas SoChylous ascites is an extremely rare complication in gynecological operations for benign pathology. There are very few cases that have been reported in the literature.Miller reported a case of chylous ascites after modified radical hysterectomy for placenta accreta, and he concluded that it is a potential complication . Zhang eWe did not find a report of chylous ascites after myomectomy. In our case, we had evidence of CA (triglycerides level of 1150 mg/dL), but we could not find a cause for it. Based on the examinations performed on this patient, nothing abnormal was detected. There were no data of tuberculosis or other infectious diseases. Because the ascites occurred immediately after the operative intervention and disappeared after conservative treatment of several days, we considered that the myomectomy was its cause. We cannot explain its mechanism of occurrence, but we consider that it is possible to occur after such types of surgery.Although extremely rare, chylous ascites may also occur in operative interventions for benign diseases in gynecological surgery, and physicians must be ready to deal with such a complication.


Xanthomas within the gastrointestinal tract occur secondary to a mucosal insult. When enough cells accumulate, their appearance can range from small nodules studding the intestinal mucosa to bandlike infiltrations to pseudotumor-like masses within the intestine with fibrosis and inflammation resembling malignancy. When large enough, they can produce symptoms of obstruction such as vomiting, abdominal pain, distention, and dysmotility. This case demonstrates the epidemiology, clinical presentation, diagnosis, and treatment of duodenal xanthomas. Xanthomas are commonly limited to the skin and rarely occur within the gastrointestinal tract. They are caused by an exaggerated response to a past mucosal insult, the lipid laden debris consumed by histiocytes that persist as foamy macrophages. Duodenal xanthomas have a wide spectrum of presentation from whitish speckling of mucosa to larger masses within the small intestine causing obstruction -2. This A 75-year-old Japanese female with gastroesophageal reflux disease (GERD), dyslipidemia, hypothyroidism, and osteoporosis, presented with a two-month history of intermittent dysphagia to solid food associated with bloating and unintentional weight loss of seven pounds over two months. The dysphagia was aggravated with the consumption of starchy foods and alleviated with drinking water.Esophagram findings demonstrated mild to moderate nonspecific esophageal dysmotility. An esophagoduodenoscopy (EGD) demonstrated gastric erythema and erosions with areas of desquamation in the antrum and prepyloric region Figures -2.Biopsies were taken from the bulb and second portion of the duodenum which were positive for duodenal xanthoma. The patient was recommended for a repeat procedure for further resection of the xanthoma only if the dysphagia persisted. However, it resolved and the patient was recommended the procedure if the symptoms returned.In terms of intestinal obstruction, duodenal xanthomas are on the far end of the spectrum of differentials. Xanthomas are considered to be an exaggerated response to a past mucosal insult, the lipid-laden debris consumed by histiocytes that persist as foamy macrophages. With a predilection for skin, they are rarely seen in the gastrointestinal tract. They tend to occur more commonly in the stomach and then in the esophagus (based on literature) with the duodenum as a distant third -2. When Diagnosis is made by endoscopy and biopsy with immunohistochemistry (IHC) staining confirming foamy histiocytes distributed through three layers , positive for cluster of differentiation 68 (CD68), decreased smooth muscle, and occasionally accompanied by fibrosis Figure .Electron microscopy demonstrated foam cells with fatty cytoplasm, as seen in Figure Despite being an uncommon differential in intestinal obstruction, Xanthomas should be considered when patients with insults from chemotherapy, radiation, or/and CMV colitis present. Xanthomas range in presentation and size, sometimes requiring surveillance, but may also require intestine-preserving surgery. Due to this rare occurrence and wide range of presentation, duodenal xanthomas are not a dismissive matter, rather they require endoscopists' attention.


Unlike borderline ovarian tumors, paratubal borderline tumors are extremely rare gynecologic tumors. They occur in reproductive-aged females at an earlier stage of disease and have a good prognosis. A 61-year-old woman, gravida 3 para 3, presented with progression of ovarian cyst. Computed tomography revealed a 6-cm simple cystic lesion without enhancing papillary projections. The patient underwent total laparoscopic hysterectomy and bilateral salpingo-oophorectomy. Frozen specimens showed that the tumor was benign, thereby confirming a final diagnosis of paratubal borderline serous tumor. The patient refused comprehensive surgical staging and opted for close follow-up. The patient remains asymptomatic with no evidence of recurrence at the 24-month follow-up. To the best of our knowledge, this is the first reported case of paratubal borderline serous tumor in a postmenopausal patient. The findings of this study and those of other case reports can contribute to the understanding, diagnosis, treatment and prognosis of these rare tumors. In asymptomatic postmenopausal women, most adnexal cysts are benign and simple cysts with a prevalence of 15%-20%. Of these, paratubal cysts have a prevalence of 5% . To dateA 61-year-old woman, gravida 3 para 3, presented with an increased size of ovarian cyst. Her history revealed a diagnosis of left ovarian cyst 3cm in diameter 3 years previously. Her serum cancer antigen (CA) 125 and CA 19-9 levels were within the normal ranges, and she was followed up at 6-month intervals at a private hospital. The ovarian cyst size had not changed in size and structure on biannual transvaginal ultrasound (TVS). However, she was referred to our institution in July 2016 with progression of ovarian cyst to 6cm on a pelvic ultrasonogram 2 weeks ago. The patient underwent menopause at the age of 48 years, with an uneventful past menstrual cycle. She had no medical history except the benign thyroid nodule, no relevant surgical history and had not received hormonal therapy. Routine blood chemistry and serum tumor marker analysis showed that the levels of carcinoembryonic antigen, CA 19-9, CA 125, and human epididymis protein 4 were all within the normal ranges. Uterine cervical cytology at the time of admission was normal. TVS showed a left ovarian unilocular cyst, size 6cm × 5cm × 4cm, with diffuse low-level internal echoes, thin walled, smooth margined, no septa and no papillary projections . The uteParatubal cysts are common disorders, representing approximately 10% of all adnexal masses . In asymNine of ten PBTs were described as having papillary projections on the image modalities or intraoperative findings, but only the present case showed no papillary projections. There were no cases of elevated serum CA 125 level in the ten cases and three of the eight frozen biopsy results were misdiagnosed as benign cysts. TVS is the most useful diagnostic tool to assess the risk of malignancy in adnexal masses and shows the high sensitivity but relatively low specificity in the diagnosis of paratubal cysts . While tPBSTs can occur in postmenopausal women without papillary projections on image studies and intraoperative findings. Reports of these rare tumors need to be shared to better understand diagnoses, treatments, prognoses, recurrences and metastases using data accumulated through case reports. Our findings contribute to the literature on PBT treatments and further long-term follow-up and additional case reports are required to elucidate the clinicopathological characteristics of the PBSTs.The author declare no competing interests.


Splenic torsion is an uncommon condition becoming clinically apparent when thespleen twists or rotates around the organ’s vascular pedicle. In the caseof a wandering spleen the organ is only attached to an elongated vascularpedicle while the peritoneal attachments are absent. However, splenic torsioncould also occur in patients with abnormal laxity of the splenic peritonealattachments. We report a case of a splenic torsion due to absence of splenicligaments with pancreatic volvulus and partial involvement of descending colonin a 9-year-old boy. Physical examination revealed a mobileabdominal mass. No prior history of chronic constipation and no history of abdominaltrauma or prior surgery was reported by the parents. An initial abdominal ultrasound (US) revealed a well defined homogenous and echogenic mass of 21 cm averagediameter, which was interpreted as splenomegaly in expected position with poorly,defined fluid in the surrounding tissues. US examination was limited by heavymeteorism, therefore, further imaging was indicated. Laboratory results showednormal level of platelet count of 156.0 × 10T1 weighted study was interpreted as global splenic infarction. Thesplenic enlargement and a rather hyperintense T2 weighted signal was considered as a sign of edema and venouscongestion due to the twisted vascular pedicle preventing blood drainage andsubsequently leading to an enlargement of the spleen. There were also signs ofpoorly defined fluid in the surrounding tissues in the former splenic lodge as wellas in the lower abdomen and pelvis.Further diagnostics were conducted by magnetic resonance imaging (MRI) to assess theenlarged organ and other intraabdominal organs and to rule out malignancies. MRIrevealed an enlarged and wedge-shaped spleen . No enhaAnother relevant finding revealed by the MRI was an involvement of the pancreas. MRIshowed an involvement of the pancreatic tail with a focal dilatation of thepancreatic duct, suggesting an obstruction of the main pancreatic duct . FurtherEmergent laparotomy was performed immediately after MRI examination. Access to thespleen was gained by an upper transverse abdominal laparotomy. The spleen was25 cm in length and was translocated from the original location towards themidline of the abdomen directly underneath the abdominal wall . All susThe splenic vascular pedicle was twisted several times with thrombosis as well asdilatation of the splenic artery and vein with a diameter of at least 1 cm . The orgThe patient recovered fast after surgery with regular bowel movements from the secondday after surgery and without any digestion problems. The boy was discharged on day9 after surgery. He received a postoperative vaccination against Streptococcuspneumoniae, Haemophilus influenzae Type B and Neisseria meningitides on day 14 aftersplenectomy. The recovery period was uneventful and the follow-up examination after2 months was inconspicuous.3 In addition to that, a long vascular pedicle predisposes to acute or chronicintermittent torsion with or without infarction depending on whether congestion ofthe organ occurs or not. This condition is also referred to as “wanderingspleen” with two peaks of incidence in children aged less than 10 years, asin this reported case, and in females of childbearing age.4,5 This condition also accounts for 0.2–0.3% of all splenectomies.6,7 The involvement of other organs are rather rare. Only a few cases reported apancreatic involvement.2,8,9 To the best of our knowledge there has been only one report about a splenictorsion with involvement of pancreas and descending colon.1Splenic torsion is a rather uncommon condition associated with the absence,underdevelopment or hyperlaxity of splenic suspensory ligaments, which causes anincreased mobility of the spleen.3,6 A splenic torsion can be a reason for recurrent abdominal pain, mostlylocalized in the left upper abdomen. The patients describe a colic pain lasting fora few moments to several hours with complete remission. Recurrent torsions withspontaneously detorsions of the spleen are in discussion for this rare clinical finding.11Patients with a wandering spleen can highly vary in their clinical presentation,ranging from being asymptomatic with an incidental palpable mass or present with anacute abdomen due to splenic infarction.12 However, the US examination depends highly on the examiner’s skillsand it is often limited by meteorism.12 In this presented case, US was inconclusive mainly due to heavy meteorism asdescribed, and hence, further imaging with MRI was needed.Imaging plays a major role in establishing the diagnosis. US with color-Dopplercontrol may reveal an abnormal location of the spleen and detect a mass, usuallylocated in the midline of the abdomen. US can deliver valuable information in thepreoperative workup of splenic torsion, i.e. for the assessment of viability of thespleen, involvement of other organs and the “whirl sign”. Therefore,US should be considered as a primary diagnostic and preoperative imaging tool.whirl sign”can be described, which is considered to be a sign of the torsion of the splenic pedicle.13 Contrast-enhanced MRI is able to assess splenic viability. Theabove-mentioned pathology was found on MRI.Tomographic examinations such as contrast-enhanced CT or MRI examinations may addvaluable information in the course of diagnostic workup and can be included in caseof an inconclusive or incomplete ultrasound examination. The most common findingsinclude an empty splenic fossa and a translocated spleen. Additionally, intomographic examinations the so-called “14 If, however, the spleen is substantially infracted, a partial subtotalresection or splenectomy should be considered.5 After splenectomy it is recommended to perform vaccination against capsulatedpathogens like pnemococcus, H influenzae and meningococcus.15Surgical treatment options of wandering spleen depend on the organ’sviability. If the spleen shows proper reperfusion after resolving the splenictorsion, either open or laparoscopic splenopexy may be offered, due to thespleen’s physiologic importance, especially in children, and the risk ofpost-splenectomy sepsis.Splenic torsion with the involvement of neighboring anatomical structures isan uncommon condition.However, it should be considered in differential diagnosis of an acuteabdomen, especially in pediatric patients.Therefore, accurate preoperative imaging is mandatory. Contrast-enhanced MRIis a suitable method to deliver valuable preoperative information regardingthe spleen’s viability and the possible involvement of otherneighboring organs. However, due to limited availability of MRI, US shouldbe the first choice in the diagnostic path.


Identification of any variant anatomy prior to surgery is as essential as having knowledge of normal anatomy. These surprises bring on many challenges along with as they can be fatal. We encountered a case of patient who succumbed down to an unrecognized rare mesenteric vasculature variant known as “Arc of Buhler” (AOB) which is a persistent embryonic ventral anastomosis between the Celiac trunk and the Superior mesenteric artery. It is usually asymptomatic and found incidentally after evaluation for other pathologies. We herein report a pseudoaneurysm of Arc of Buhler being surgically managed after massive retroperitoneal hemorrhage. Unfortunately, the patient did not survive the procedure and passed away. AOB aneurysms present formidable risks to patients and diagnostic and therapeutic challenges to physicians. They are rare and require high index of suspicion on radiographic imaging. Present case reports underscore the importance of identifying it and treating it regardless of the size.  The gastrointestinal system is mainly supplied by celiac trunk, superior mesenteric artery (SMA), and inferior mesenteric Artery (IMA) . There aA 55-year-old man presented to the emergency department (ED) with acute onset abdominal pain started insidiously approximately 1-hour prior to presentation. He was discharged only a week before from the same hospital when he was admitted with acute abdominal pain when he underwent a CT-angiogram and a digital subtraction angiography (DSA) of the abdomen, which showed incidental finding of Arc of Buhler . There wThere are several procedures, both surgical and interventional, that require detailed knowledge of the hepatic and celiac trunk variations for example hepatic and pancreatic surgery, liver transplantation and intra-arterial infusion of chemotherapy procedures. The AOB was first described by Buhler and Tandler in 1904 . TandlerThis condition is usually detected incidentally in asymptomatic patients undergoing imaging for other reasons as well as in symptomatic patients with celiac trunk stenosis related to arterial sclerosis or median arcuate ligament syndrome . This vaKugai described aneurysmectomy of 3-cm saccular AOB in a patient with a celiac artery occlusion. Surgery was performed without revascularization of the Celiac artery after attempts at failed embolization . CloselyIn conclusion, AOB aneurysms present formidable risks to patients and diagnostic and therapeutic challenges to physicians. They are rare and require high index of suspicion on radiographic imaging. Present case reports underscore the importance of identifying it and treating it regardless of the size. None declared.