Cardiac myxomas are rare with reported incidences of less than 0.03%. Cardiac myxomas are most commonly observed in the left atrium. Their clinical manifestations vary and most are non-specific to the diagnosis. The most common extra-cardiac manifestations are thrombo-embolic infarcts from tumor embolization. A previously healthy 55-year-old man presented with findings suggestive of acute arterial limb ischemia. Following surgical treatment of his acute presentation, a left atrial mass was found on echocardiography suggesting that the embolization was secondary to a cardiac myxoma. The patient was discharged without complication. Embolic myxoma should be included in the differential in younger, previously healthy patients presenting with acute arterial limb ischemia without obvious cause. Our patient’s dramatic presentation with a single acute event, however, prompted immediate treatment and resulted in a functional recovery with minimal complications. Cardiac myxomas (CMs) are the most common primary cardiac neoplasms, accounting for 50% of all cardiac tumors. These benign tumors are rare, with reported incidences of less than 0.03% . The vasA 55-year-old previously healthy man presented with a two-hour history of abrupt, severe pain and paresthesia of the right lower extremity (RLE) after playing basketball. This is the first time he had experienced this, but he had experienced intermittent episodes of dizziness and palpitations over the past two months. He denied any history of trauma, smoking, alcohol abuse, and illicit drug use. Vitals were as follows: blood pressure (BP) from the right arm - 151/83 mmHg; heart rate - 102 beats/min; respiratory rate - 16 breaths/min; temperature - 36.3° C, and oxygen saturation - 100% on room air. On examination, his RLE was cold, pale, and insensate. There were no palpable femoral, popliteal, or pedal pulses in the RLE. Distal pulses were also negative by Doppler ultrasound. He had conserved 5/5 motor strength in both feet. Examination of the left lower extremity (LLE) was normal. Cardiopulmonary exam was unremarkable, with no murmurs or audible extra heart sounds. All other physical exam and laboratory findings were unremarkable. Computed tomography angiography (CTA) of the RLE revealed an occlusion of the right common femoral artery (CFA). There was also a second occlusion at the proximal popliteal artery at the level of the knee joint with no opacification of the leg vasculature distal to the popliteal artery , the patient’s troponin and creatinine kinase levels were elevated. A CTA of the chest revealed an irregular mass in the left atrium (Figures , the patMyxomas arise from mesenchymal stem cell dysregulation resulting in over-proliferation. These stem cells have the capacity to differentiate into smooth muscle cells, fibroblasts, and endothelial cells, whose roles are vital in the cardiovascular system -4. HistoThe majority of CMs are sporadic, but up to 7% of CMs are familial with an autosomal dominant inheritance pattern with multiple families being linked to chromosome 2p16 -6. AlthoThe clinical manifestations of LAMs are heterogeneous and up to 20% of patients are completely asymptomatic .The pathophysiology by which the penduculated LAMs arise make them very friable and therefore susceptible to detachment. Consequently, almost half of all LAMs result in peripheral embolization with the cerebral circulation being the most common site of embolization, followed by the pulmonary and coronary vasculature . In theoLaboratory findings suggesting inflammatory patterns are well reported in cases of CMs and have been associated with anemia and increased expression of interleukin-6, and elevated troponins and globulin can also be observed . A 28-yeEchocardiography is the mainstay of diagnosis with histological evaluation for definitive confirmation. To our knowledge, there is no evidence of conservative medical management to decrease tumor size or for complete ablation . TreatmeRecurrence has been reported in up to 5% of sporadic CMs and up to 20% in familial cases and is more likely within the first 10-years postoperatively -9. Thus,Acute arterial limb ischemia secondary to a cardiac myxoma is rarely reported, but our literature review suggests that it is not infrequent. A high index of suspicion should be prompted when younger patients present with acute arterial limb ischemia in the setting of constitutional symptoms or obstructive manifestations in the absence of known cardiovascular risk factors. Echocardiography is standard for diagnosis, although a definitive diagnosis is only arrived at through histology. Timely diagnosis is vital, and outcomes are favorable with prompt surgical management. Our patient’s dramatic presentation with a single acute event prompted immediate treatment and resulted in functional recovery with minimal complications.


A 13-year-old girl presented with irregular vaginal bleeding for 2 years and aggravated 10 days. Anal examination showed a hard vaginal mass. On the MRI, a 7.1 cm×6.3 cm×5.5 cm mass at the upper part of the vagina was seen, the relationship with cervix was unclear. The biopsy revealed a malignant tumour with massive necrosis, tended to be rhabdomyosarcoma or the other sarcoma. Because the clinical stage was International Federation of Gynecology and Obstetrics IIA, neoadjuvant chemotherapy took before the radical operation.http://virtualacp.com/JCPCases/jclinpath-2018-205612_1/ and http://virtualacp.com/JCPCases/jclinpath-2018-205612_2/ and consider your diagnosis.Review the high-quality, interactive digital Aperio slides at Mesonephric adenocarcinomaSerous carcinomaMalignant mixed Müllerian tumoursMüllerian papilloma malignant transformation to endometrioid carcinomaBenign Müllerian papillomaThe correct answer is after the discussion.Müllerian papilloma of the genital tract is rare. It mainly occurs in prepubertal girls ranging from 1 to 9 years old, and the major symptom is non-cyclical vaginal bleeding or discharge. Since JamesThis is the first case of Müllerian papilloma malignant transformation in a prepubertal girl, the endometrioid carcinoma differentiation has not The cervix and the vagina were coinvolved in our case. Both masses had the benign to malignant region and were isolated from each other with a distinct boundary. We couldThe Müllerian papilloma malignant transformation had not been reported in the prepubertal girl, there may be some relationship with the development abnormity as this case was combined with uterine hypoplasia.D. Müllerian papilloma malignant transformation to endometrioid carcinoma.The Müllerian papilloma malignant transformation is rare; the existence of benign Müllerian papilloma, borderline and malignant transformation may be the main diagnostic standard.The Müllerian papilloma malignant transformation can perform all kinds of adenocarcinoma of the female genital tract as the Müllerian papilloma gland epithelium is primitive.Useful immunohistochemical markers are helpful to diagnose and determine the differential diagnoses.The Müllerian papilloma malignant transformation is available in both the old and prepubertal woman, we should take attention to the vaginal bleeding of prepubertal girl for early detection and treatment.


Extraosseous Ewing’s sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing’s sarcoma of the parapharyngeal space is even rarer. To the best of our knowledge, only three cases of EES of the parapharyngeal space have been reported in the literature.We report a rare case of EES of the parapharyngeal space in an 8-year-old girl. She presented with complaints of earache, difficulty in breathing and swallowing and bleeding from the mouth. Investigations revealed a large parapharyngeal mass causing narrowing of the nasopharyngeal and oropharyngeal airway with skeletal and lung metastasis. Biopsy from the parapharyngeal mass was suggestive of malignant small round cell tumor. The patient was treated with chemotherapy and radiotherapy, but developed brain metastasis and succumbed to disease approximately 1 year after diagnosis. Herein, we describe the characteristic clinicopathological features and treatment with a comprehensive review of the literature.EES in this unusual location behaves aggressively, with a high rate of recurrence and distant metastasis. Aggressive multimodal treatment comprising of multi-agent chemotherapy, surgical resection if feasible, and radiotherapy should be considered. Extraosseous Ewing sarcoma (EES) is histologically and molecularly similar to Ewing sarcoma of the bone. These neoplasms are composed of small round blue cells of neuroectodermal origin, and about 85% of Ewing sarcomas are associated with translocation t11;22)  was suggestive of a large heterogeneous enhancing soft tissue density mass in the left parapharyngeal space with extension to the oropharynx, laryngopharynx, nasopharynx, prevertebral space, paravertebral region and reaching superiorly up to the base of skull . The masBiopsy of the mass showed sheets and nests of round-to-oval tumor cells with hyperchromatic nuclei and scant cytoplasm . Brisk mitosis and large areas of tumor necrosis were seen. Immunohistochemically, the tumor cells were positive for CD99 and negative for smooth muscle actin (SMA), desmin and chromogranin immunostains. Weak periodic acid-Schiff (PAS) positivity was also noted . A CECT of the chest and abdomen revealed multiple lung nodules with lytic lesions in the dorsal vertebra suggestive of metastasis. Hence, a final diagnosis of EES of the parapharyngeal space with lung and skeletal metastasis was made.In view of the disseminated disease, chemotherapy was started with vincristine, doxorubicin and cyclophosphamide at standard doses. The patient received six cycles of chemotherapy, and showed improvement in symptoms. Repeat CECT showed persistence of the parapharyngeal lesion with skeletal metastasis, although resolution of the lung nodules was noted. The patient was then given palliative local radiotherapy of 36 Gy in 20 fractions over 4 weeks. However, 1-month post radiotherapy, she presented with complaints of headache, vomiting and medial deviation of both eyes. Subsequent brain CECT revealed an intensely enhancing mass in the left frontoparietal region with mediastinal shift and uncal herniation. Following the CECT brain findings, palliative whole-brain radiotherapy of 25.2 Gy in 14 fractions was given at a rate of 1.8 Gy per fraction. The patient deteriorated further with rapid progression of local and systemic disease. Despite the best medical efforts, the patient succumbed to her disease 1 year after diagnosis.EES of the head and neck region is a rare neoplasm, and EES of the parapharyngeal space is even rarer. EES is equally prevalent in males and females and predominantly affects adolescents and young adults. Allam et al. reported a median age at diagnosis of 16.5 years and a male to female ratio of 2.4:1. Biswas To the best of our knowledge, only three cases of EES of the parapharyngeal space have been reported in the literature –9. The fShe expired 14 months after excision due to an intra-cerebral hemorrhage, and the autopsy revealed no recurrent or residual disease. Our patient had disseminated disease at presentation, for which she was started on chemotherapy and subsequently given radiotherapy to palliate local symptoms. However, due to the progressive nature of disease, she could not be saved. The treatment of EES is multimodal, comprising of surgery, chemotherapy and radiotherapy. However, the parapharyngeal space is difficult to reach and it is difficult to perform surgery most of the times due to the advanced presentation of the disease, anatomic complexity and close proximity to the surrounding structures. Siegal et al. reported that patients with biopsy only or complete resection had significantly better survival rates when compared with patients with incomplete resection . In a retrospective analysis of 24 patients with Ewing sarcoma of the head and neck region reported by Allam et al. , most paIn the present case, the patient was treated with both chemotherapy and local radiotherapy due to the advanced stage of the disease; however, she ultimately succumbed to her very aggressive disease.EES of the parapharyngeal space is an extremely rare occurrence with only three cases reported in the literature to date. Herein, we report the fourth case of EES of the parapharyngeal space. Diagnosis is based on tissue biopsy. Aggressive multimodal treatment should be considered due to the aggressive nature of the disease. It is difficult to comment on prognosis based on four cases. More reports are needed to determine the nature and prognosis of this rare neoplasm.


Heterotopic ossification is a rare phenomenon defined by the formation of bone within nonossifying soft tissues. A rare variant of heterotopic ossification is heterotopic mesenteric ossification (HMO), in which there is involvement of the mesentery and surrounding intra-abdominal structures. There are only four previously reported cases of HMO involving an ileostomy. We present a case of HMO affecting an ileostomy which was discovered during elective stoma reversal in a 52-year-old male who required fecal diversion following perineal necrotizing fasciitis. Heterotopic ossification (HO) is a peculiar, benign disease characterized by organized bone formation in nonossifying muscle or soft tissue . OssificThe patient is a 52-year-old African American male with an unwanted diverting loop ileostomy who was scheduled for an elective stoma reversal. Originally, the loop ileostomy was created for fecal diversion after a large perineal defect was made for the treatment of necrotizing fasciitis of the perineum. A high splenic flexure, foreshortened colonic mesentery, and morbid obesity precluded performing a diverting colostomy at that time. Of note, the patient had no family history of HO or abdominal surgical history prior to the ileostomy creation.During the reversal of the loop ileostomy, a circumferential incision was made at the mucocutaneous junction. Dissection was carried towards the serosal surface of the small bowel with electrocautery. There were significant adhesions between the small bowel and surrounding soft tissue which was much denser than expected. At that time, the base of the ostomy was reached and a dense structure was identified by palpation. It was circumferential in both limbs of the loop ileostomy. It was hard and fixed with a bone-like consistency. We encountered difficulty in freeing the small bowel from this circumferential dense structure, and this, combined with the patient's body habitus, rendered it difficult to take the stoma down safely through the peristomal incision. As a result, we converted the operation to an open procedure using a lower midline incision to reverse the ileostomy. After significant lysis of adhesions, the ileostomy was reached from the midline incision, and the hard structure was excised circumferentially using a combination of sharp dissection with Metzenbaum scissors and Bovie electrocautery. Upon gross inspection, it was observed that the structure was seated at the level of the rectus abdominis muscle and formed a complete circle around the ileostomy. A portion appeared to have the consistency of thin connective tissue, while the majority of the specimen appeared osseous. The specimen grossly consisted of three irregular portions of ragged skeletal muscle and bone measuring 5.5, 7, and 12.5 cm in the greatest dimension . The skeThe patient had follow-up visits after ileostomy reversal at weeks 2, 3, 7, 10, and 16. He developed transient fecal incontinence given the extensive nature of his perineal wound, which was successfully treated with pelvic floor biofeedback therapy by week 10. His ileostomy and midline wounds healed without abdominal pain, hernias, or signs of recurrence.Heterotopic ossification is a rare, benign disorder in which osteoblasts organize into discrete bone layers and deposit in nonskeletal tissues . The pheWe describe a case of HO involving an ileostomy site, which we theorize could represent a rare subtype of intra-abdominal heterotopic mesenteric ossification (HMO). It appears that stoma involvement of HMO is a rare presentation of an already rare phenomenon, as only 4 other reports have specifically noted involvement of an ileostomy –5. The mTo date, there have been approximately 50 cases reported of HO , 9. In 2Heterotopic ossification must be differentiated from other phenomena, such as dystrophic calcification and malignant bone tumors , 7. DystACVR1/ALK2 gene, which codes for a bone morphogenetic protein type I receptor [There are three forms of HO: myositis ossificans progressiva (MOP), myositis ossificans circumscripta (MOC), and myositis ossificans traumatica (MOT). Myositis ossificans progressiva, also called fibrodysplasia ossificans progressiva or stone man disease, is an autosomal dominant connective tissue disorder caused by mutations in the receptor . At birtreceptor . This direceptor . Myositireceptor . MyositiWhile MOT typically describes the phenomenon of HO in tissues adjacent to skeletal muscle, there have been reports of intra-abdominal MOT following abdominal surgery and/or trauma . In thesDue to the rare nature of HMO and its nonspecific radiological appearance, it may be challenging to diagnose preoperatively without a biopsy. Imaging modalities may not be able to accurately distinguish between differential diagnoses such as contrast leakage or osseous neoplasia. This difficulty with radiologic identification is compounded by the only mild increase in soft tissue density that can be observed in early stages of heterotopic ossification . LaboratThe exact natural history of heterotopic ossification regarding onset may depend on the location. The time from initial injury to development of ossification has been reported as early as 4 weeks following burn injury and as early as 3 months following elbow injury , 28. OneThe benign pathology of this condition lends credence to a conservative management if the lesions are asymptomatic, but a symptomatic case  may be an indication for surgical excision . ExcisioHeterotopic ossification must be distinguished from other pathologies that may present similarly. As in our case, the surgical team may initially confuse HO with a foreign body due to the shape and location of the ossification. This is an important consideration because a surgeon may choose to simply leave ossification behind or at most perform an incisional biopsy, as opposed to removal if it is a foreign body. Furthermore, a tissue capsule usually forms around a foreign body making its removal more straightforward. Knowledge of such cases may deter the surgeon from performing further dissection due to a benign pathology such as HMO . HoweverWe report a rare case of HMO that was identified at loop ileostomy takedown. A thorough literature review revealed only four previous cases involving an ileostomy. Heterotopic mesenteric ossification can result in serious complications, including but not limited to small bowel obstruction, fistula, sepsis, and death . However


Myxopapillary ependymomas are a slow-growing, grade I type glial tumor in the lumbosacral region. More rarely, they can present as extradural, subcutaneous sacrococcygeal, or perisacral masses, and it is under these circumstances that they are more likely to spread. Here, we report the presentation of a sacrococcygeal mass in patient that was initially resected confirming extradural myxopapillary ependymoma. At initial resection, multiple small pulmonary nodules were detected. This mass recurred 2 years later at the resection site with an interval increase in the previously imaged pulmonary nodules. Resection of both the post-sacral mass and largest lung metastasis confirmed recurrent myxopapillary ependymoma with oligometastatic spread. Because these tumors are rare, with extradural presentation being even more infrequent, to this date there are no definitive therapeutic guidelines for initial treatment and continued surveillance. For myxopapillary ependymoma, current standard of care is first-line maximal surgical resection with or without postoperative radiotherapy depending on the extent of disease and extent of resection. However, there remains insufficient evidence on the role of radiotherapy to oligometastatic foci in providing any further survival benefit or extending time to recurrence. Thus, prospective studies assessing the role of upfront treatment of oligometastases with local resection and adjuvant radiotherapy are needed for improved understanding of extradural myxopapillary ependymoma. Myxopapillary ependymomas (MPE) are a rare, slow-growing, well-circumscribed grade I ependymoma . They arMagnetic resonance imaging (MRI) remains the preferred imaging modality of choice, as it identifies the tumor and its relation to the surrounding soft tissues and, if intradural, the terminal spinal cord elements. MRI is also best suited for detection of leptomeningeal spread along the spinal axis . ManagemHere we report the initial presentation of a sacrococcygeal mass in a young patient. This mass was found to be an extradural MPE. Given the rarity of MPE, with extradural presentation being even more infrequent, there is currently limited understanding of the best measures to control disease and to prevent recurrence. The initial management of extradural MPE, surveillance, and treatment strategy at the time of recurrence are discussed herein.A previously healthy, active 30 year-old Caucasian male presented with fatigue and dyspnea on exertion and was found to have a pulmonary embolism. The patient was a rare smoker with no previously known risk factors. A thorough oncologic history revealed that he had been living with a small, asymptomatic “bump” on his lower coccyx that had been stable for years. It was first noted to appear shortly after falling from the bed as a small child. At the time of presentation, the patient denied any back pain, discomfort, gait instability, or changes in bowel or bladder function.CTNNA1, a gene that codes for alpha-catenin of the cadherin family. No other clinically significant alterations or rearrangements were identified.The mass was fine needle biopsied showing a myxoid and epithelioid population, thought to be a low-grade sarcoma. The patient then continued on 8 months of oral anticoagulation until the mass was ultimately resected. Pelvic imaging of the asymptomatic mass prior to initial resection revealed an expansile, non-eroding mass in the post-sacral soft tissues . PatholoExtensive imaging at the time of resection additionally revealed small indeterminate bilateral pulmonary nodules in the right and left lower lobes, with one right lower lobe nodule measuring 4 mm in maximum diameter. Pelvic imaging was negative for significant lymphadenopathy but did show visible iliac nodes. No adjuvant therapy was initiated, and the patient was followed with surveillance CT scans.Surveillance imaging with a CT of the chest, abdomen, and pelvis 2 years following resection of the sacrococcygeal mass showed enlargement of the right lower lobe nodule to 8 mm in diameter, and an enlarged left lower lobe nodule measuring 2 mm. Other left and right lower lobe nodules were both stable at 3 mm . Pelvic Upon referral to the academic center, staging studies were performed, including a negative brain MRI without any abnormal enhancement and normal flow voids and a normal MRI of the cervical, thoracic, and lumbar spine. MRI of the sacrum revealed two avidly enhancing right internal iliac lymph nodes measuring 14 × 11 mm and 17 × 12 mm. The post-sacral mass was irregular, multilobulated, and enhancing, extending through the soft tissues from the first through fourth coccygeal segments. A thin plane of fat was interposed between the tumor and coccyx without evidence of coccygeal invasion .After referral to the academic center's medical oncology, neuro-oncology, and thoracic and orthopedic oncology specialists, both the lung nodule and post-sacral mass were resected with thoracoscopic wedge resection and coccygectomy in the same operative setting. The lung nodule measured 1.1 cm pathologically; it was positive for metastatic tumor, consistent with MPE, with a focus of lymphatic spread. The resected coccygeal mass and pelvic lymph node again demonstrated strong GFAP and S100 expression, and stained negative for brachyury, P40, smooth muscle actin (SMA), calponin, D2-40, and 34bE12. There was weak, focal positivity for pancytokeratin. Both pathologic specimens supported the diagnosis of MPE, and resection margins were negative for tumor in both specimens.The patient tolerated surgery well. An MRI 5 weeks later showed a stable post-operative resection cavity . There wAn extradural MPE should be considered in the differential of a slow-growing soft tissue mass in the pre- or post-sacral region. The mechanisms by which these ependymal tumors arise in the extradural space are not well-understood, but three mechanisms are thought to be responsible: (1) via direct extension through the dura, as has been characterized from small spinal dysraphism , (2) viaen bloc or gross total resection (GTR) with the addition of early adjuvant radiation therapy (RT). The use of RT should be considered especially if sub-total resection (STR) can only be achieved. Retrospective studies for spinal MPE have found an increased risk of recurrence following STR compared to en bloc or GTR   –47 and c breast) , 49. GivThe role of systemic therapy in the treatment of MPE remains largely unknown. There is limited evidence that systemic therapy can be curative. Some studies, however, suggest that tyrosine kinase inhibitors (TKI) can halt disease progression. One case report described the use of imatinib following temozolomide (TMZ) failure based on the tumor's positivity for platelet-derived growth factor receptor, and the patient remained without disease progression for 11 months . AnotherLong-term survival data for spinal MPE suggests that treatment of the primary site with surgery and adjuvant radiation offer the best rates of local control and PFS . HoweverExtradural MPE, although rare in incidence, have a common tendency to spread to the lymph nodes, liver, and lung. In the current case, a slow growing and rather indolent extradural MPE warranted invasive intervention when it expanded in size, the time at which this tumor likely spread to the lungs. From case report experience, one critical early intervention for control of this tumor is primary resection and early adjuvant radiation. The primary site was treated with surgery, of which the extent of the initial resection was unfortunately unknown. Molecular sequencing on the original tumor revealed a low mutational burden but showed a loss of alpha-catenin expression potentially favoring a greater metastatic potential. Within 2 years, the primary tumor recurred with a more indicative presentation of both nodal and pulmonary metastases. This begs the question if the natural history of disease recurrence can be altered by adjuvant radiation to the primary site. In this case upon disease recurrence, the decision was made to treat with GTR of the oligometastatic focus in the lungs along with GTR and adjuvant IMRT to the pelvis. Post-treatment monitoring is not well-defined for extradural metastatic MPE. Clinicians encountering this situation agree that certainly frequent and prolonged post-operative follow up is necessary, as late recurrences can occur several years after the primary tumor is removed. For this reason, the case reported here will be monitored frequently with pelvic scans and whole-body PET, given the proclivity of this tumor to spread to extradural sites. The manner in which this rare type of MPE presented reflects how distinguishable these extradural MPE may behave. As such, this unique case highlights the multi-disciplinary approach required to manage both primary site and oligometastases by neuro-oncology and medical oncology communities.A written informed consent was obtained from the patient for the publication of this case report.KP, RR, BT, WE, and JK were involved in patient management with regards to initial presentation, diagnosis, treatment, and follow-up. BT and WE assisted with obtaining the subsequent tissue diagnoses. RM, PP, and CT contributed to the histopathologic diagnosis, immunohistochemistry, and secondary review of all specimens presented. KB collected and interpreted the patient data. KB, KP, and RR wrote the manuscript. All authors read and approved the final manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


We present a case of a 54-year-old man with primary angiitis of the central nervous system (PACNS) who was initially admitted to a psychiatric clinic with a diagnosis of delirium. We discuss the difficulty in establishing the diagnosis of PACNS and provide the reader with some recommendations on how to promptly and correctly diagnose this disease in order to avoid potentially lethal outcomes. Primary angiitis of the central nervous system (PACNS) is a vasculitis of unknown origin confined to the brain and spinal cord . PACNS wAll the vasculitides that affect the central nervous system (CNS) are characterized by inflammation and necrosis of blood vessels and may be primary or secondary. They may cause tissue ischemia, which can have a variety of clinical presentations. Isolated CNS vasculitis is a known, rare disease entity with a variety of presentations; headache, dementia, lethargy, seizures, or multifocal neurological deficits . IncidenThe clinical presentation is highly unspecific ranging from psychiatric to neurological symptoms, therefore, frequently delaying a prompt diagnosis and treatment with potentially lethal consequences. We describe a patient initially admitted to a psychiatric hospital, but diagnosed subsequently with PACNS. In addition, we provide a brief review of the literature.A 54-year-old Caucasian man presented with agitation and confusion. Glasgow coma score (GCS) was 14 out of 15. The patient was reported to be in his baseline state  when his family last saw him one week prior. The initial working diagnosis was delirium. The clinical picture worsened rapidly when the patient developed hypertension, tremor, meningism, and an increase in C-reactive protein , without fever. MRI FLAIR—sequences presented abnormal but nonspecific bilateral hyper‐intensities supra‐ and infratentorially in the gray and white matter , the basal ganglia, the thalamus, the middle cerebellar peduncle, and in the brainstem Figures . A lumbaDespite a large volume of literature on PACNS definitive diagnosis is frequently delayed resulting in potentially lethal consequences. One major reason is the unspecific clinical presentation ranging from recurrent headaches, altered consciousness, seizures, stroke, and a lack of high clinical suspicion  multi‐focal symptoms associated with encephalopathy . Vasculi


Background: Congenital infantile fibrosarcoma (CIF) is a rare malignant soft tissue tumor that predominantly occurs in children under 1 year of age. CIF is frequently misdiagnosed with other conditions like hemangioma of infancy, infantile fibromatosis, or kaposiform hemangioendothelioma. Disseminated intravascular coagulopathy (DIVC) is rarely reported to be associated with CIF.Case presentation: We describe an infant who presented with a large mass over the right arm. She was initially treated conservatively as hemangioma but was later confirmed by tissue histopathological examination to have CIF as the mass rapidly increased in size. She developed massive intra-tumoral bleed with DIVC whilst receiving neoadjuvant chemotherapy requiring multiple blood products transfusion. An urgent near-total resection of the tumor was performed in view of life threatening bleeding despite multiple blood transfusions. Post-operatively, she received further adjuvant chemotherapy. Subsequently, she remained in complete remission 32 months off-treatment and has full function of the affected limb.Conclusions: CIF is an important condition to be considered in infant who has large mass over the extremity. DIVC could be associated with large CIF and when it occurs can be life-threatening. Whenever feasible early surgery should be performed in very young patients with large CIF to prevent mortality from bleeding. Pre-chemotherapy assessment revealed normal cardiac, renal and liver function. Her initial full blood count also showed normal hemoglobin and platelet count. During the 2nd week of chemotherapy, she developed massive intra-tumoral bleed with DIVC; the mass size had increased markedly. A repeat MRI showed increased mass size to 10.3 × 11.3 × 12.3-cm with intra-tumoral hemorrhage. The coagulation parameters showed prothrombin time of 18.3 s with INR ratio of 1.57, activated prothrombin time (APTT) of 61.2 s with APTT ratio of 1.58, fibrinogen level of 0.6 g/l, and D-dimer of 19.21 ug/ml. She required multiple platelet, fresh frozen plasma (FFP), cryoprecipitate and red blood cells transfusions. Despite multiple blood products transfusion the DIVC parameters did not improve significantly and the hemoglobin level dropped to lowest of 5.7 g/dL and platelet of 21 × 10s of CIF . SubsequCIF is a rapidly growing tumor, and in a few weeks or months could become highly vascular. The extremities are affected in most cases of CIF, followed by the head, neck, and trunk. Despite being locally aggressive, CIF is associated with a high long-term survival rate. Surgical excision is the primary treatment modality and, neoadjuvant chemotherapy should be considered in unresectable cases or to reduce morbidity from surgery given the chemosensitive nature of the tumor . BleedinBleeding or DIVC is a rare manifestation of CIF and it could be associated with a large mass in the extremities, trunk, intrathoracic, or intraabdominal. Whenever feasible early surgery should be performed in very young patients with large CIF at risk of life threatening bleeding to prevent mortality.All datasets generated for this study are included in the article/supplementary material.The National University of Malaysia (UKM) Research Ethics Committee has approved the study. The Research Ethics Committee, The National University of Malaysia operates in accordance to the International Conference of Harmonization Good Clinical Practice Guidelines. Parental written consent has been obtained allowing inclusion of material pertaining to the patient. The patient's parents were informed that no identifying information will be published and this has been acknowledged by them.HA acquired the clinical data and drafted the manuscript. HA, AA, SL, C-KL, JS, and SI were responsible for the clinical management of the patients. RZ and YC were responsible for the pathological diagnosis. ME was responsible for the molecular test and diagnosis. HK and ME were responsible for interpretation of the molecular diagnosis and critical revision of the manuscript. All authors have read and approved the final manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Intramuscular myxoma (IM) is a rare mesenchymal neoplasm with the incidence of one per 1 000 000 people and is reported greater among women. Our case reports an IM of a deltoid muscle in a young man. Differential diagnosis with sarcoma is important. Surgery is the treatment of choice usually without recurrence. Intramuscular myxoma (IM) is a rare mesenchymal neoplasm with the incidence of one per 1 000 000 people and is reported greater among women. Our case reports an IM of a deltoid muscle in a young man. Differential diagnosis with sarcoma is important. Surgery is the treatment of choice usually without recurrence. The current study reports a case of a 34‐year‐old swimmer. Imaging showed an ovoid mass in the deltoid muscle. The differential diagnosis of low‐grade fibromyxoid sarcoma is important when diagnosing IM. Surgical excision is the treatment of choice with a low risk of recurrence.Myxoma is a benign connective tissue tumor found usually in the heart. The extracardiac manifestations of the tumor range from intramuscular and superficial cutaneous to odontogenic and juxta‐articular myxoma. Thus, intramuscular myxoma (IM) is a type of extracardiac manifestations of myxoma. IM has an incidence of one per 1 000 000 people between the fifth and seventh decades of one's life and occurs more frequently in women with 60% predominance.The diagnostic imaging options include magnetic resonance imaging (MRI) and ultrasonography, but the definitive diagnosis is histopathological. IM is characterized by bland spindle‐ and/or stellate‐shaped cells embedded in a hypovascular abundant myxoid stroma. The nuclei are small and show no or minimal nuclear atypia.A comprehensive literature review was performed by searching the PubMed‐National Center for Biotechnology Information database, using the keywords “deltoid AND myxoma.” The search yielded 6 case reports published prior to July 2018. Of these, four included cases of solitary myxoma in the deltoid muscle and thus were included in our literature review Table 2A 34‐year‐old otherwise healthy male swimmer presents with the shoulder pain during swimming. MRI was performed in an effort to differentiate between a solid tumor and a rotator cuff tear. The images showed no rotator cuff injury, but instead a 40 × 40 mm polycystic tumor with solid aspects of the deltoid muscle, which was possibly malignant Figure . Due to 3Intramuscular myxoma is a somatic mosaic disorder occurring as a sporadic isolated lesion, although it can be part of Mazabraud's syndrome (fibrous dysplasia with multiple IM’s) or McCune‐Albright syndrome , which are caused by mutations in the GNAS gene.Histologically, IM is composed of bland spindle‐ and stellate‐shaped cells in an abundant myxoid stroma. Some IM may have areas of hypercellularity and hypervascularity. In this case, a differential diagnosis between myxoid liposarcoma, low‐grade myxofibrosarcoma, and low‐grade fibromyxoid sarcoma should be taken into consideration. Immunohistochemically, IM typically stains negatively for S‐100 protein, whereas myxolipomas and liposarcomas stain positive.Soft tissue sarcomas (STS) are a heterogeneous group of rare malignant mesenchymal tumors with incidence of two to three cases per 100 000.Magnetic resonance imaging is the most sensitive imaging option diagnosing IM. It reveals a well‐defined ovoid mass displaying low signal intensity on T1‐weighted images and high signal intensity on T2‐weighted images. A peripheral and patchy enhancement of the signal after gadolinium injection may be observed. A surrounding fat rim on an MRI is suggestive of IM.In this context, we emphasize the role of the radiologist and the pathologist within the differential diagnosis of IM, in order to avoid inappropriate treatments.4MRI is the most sensitive examination for diagnosing tissue injury and tumors. Core needle biopsy may be very helpful for differential diagnosis between benign or malign tumor prior to treatment. Surgical excision is the treatment of choice, but the diagnosis is defined by the histological findings. In the differential diagnosis, reactive lesions and low‐grade myxoid sarcomas should be kept in mind when planning the treatment. Surgical excision has shown to be curative in the vast majority of cases, with minimal recurrence rates.None declared.SA and STM: had the idea for this case report; SA: wrote the first draft of the manuscript; AT, STM, BE, and AM: revised the final manuscript. All authors have read and approved the manuscript.


Broad ligament herniation is a very uncommon cause of small bowel obstruction. Surgery permits reduction of the hernia and closure of the defect to prevent recurrence.We report a rare case of a 35 year old woman admitted in our hospital for recurrence of a small-bowel obstruction, secondary to internal hernia. The patient underwent intestinal resection in 2018 for herniated left broad ligament of the uterus strangulated.This time, the computed tomography revealed a double junctional syndrome with ileal dilation localized in right side of uterus. Emergency surgery confirmed internal hernia with passage of ileal loops through the right broad ligament. After reduction of the hernia, closure of the defect was performed to prevent recurrence. For the first time, a check of other classic abdominal hernias was carried out.Internal hernias are responsible for less than 5% of mechanical bowel obstruction. Broad ligament hernias represent only 4–7% of these hernias.The diagnosis is often delayed, because of its rarity and lack of surgical history. Surgery confirms the diagnosis, treats the occlusion and prevents recurrence.The presence of several internal hernias has never been studied and no recommendation has been made on this topic.This case demonstrate the importance of a systematic exploration of other internal abdominal hernias during the surgery, especially a contralateral broad ligament hernia. The most common causes of intestinal obstruction include adhesions, neoplasms and incarcerated inguinal hernias ,2. The oThe Broad Ligament Herniation (BLH) is responsible of 4–7% of all cases of internal abdominal hernia .The preoperative diagnosis is very difficult and abdominal Computed Tomography (CT) represents the diagnostic tool of choice. The surgery represents the last diagnostic tool and the first therapeutic modality .The operative procedures are the reduction of the hernia and the closure of the defect to avoid the recurrence, but the research for other internal hernia is not classical proposed.In this current case, we report a recurrence of small bowel obstruction due to BLH but on the other side.This case has been reported in line with the SCARE criteria .2We present a case of a 35 year old woman admitted through the emergency department of our hospital, for acute abdominal pain with nausea and vomiting. The symptoms started 4 h before the admission. She already underwent an intestinal resection in 2018 for herniated left broad ligament of the uterus strangulated.Physical examination showed tachycardia, no fever and abdominal distension with diffuse tenderness, and no bowel sounds. The pain on palpation was diffuse and was similar to that which appears of last year.3).Laboratory studies were normal with the exception of leukocytosis , transmesenteric (8%), Winslow hiatal (8%), intersigmoidal (6%), supravesical and pelvic (6%) and transomental (4%) .Internal hernias originating in broad ligament defects are very rare, comprising 4%–7% of all internal hernias. The first case was reported at autopsy by Quain in 1861 .Cilley et al.  proposedType II, corresponds to a defect through the mesovarium and mesosalpynx above the round ligament.Type III, which corresponds to a defect through the meso-ligamentum teres, between the round ligament and the remainder of the broad ligament.Our patient had a complete fenestration through the broad ligament (type I), it is the most common type.The cause of the defect in the broad ligament of the uterus may be congenital or acquired. Congenital cases have an embryologic explanation due to a developmental peritoneal defect around the uterus. In nulliparous patients, such defect may result from spontaneous rupture of cystic structures .Acquired defects are secondary to surgical trauma, pregnancy and birth trauma, perforations following vaginal manipulation and inflammatory pelvic disease ,15.The defect of the broad ligament is often unilateral but can occasionally occur bilaterally, as in this case.We did not find any case of the literature reporting a bilateral BLH complication.It is normally considered as a severe condition due to the risk of strangulation and perforation of the hernial content. In most cases, the herniated viscera is the ileum, although hernia of the colon, ovary and ureter has been reported .BLH are difficult to diagnose preoperatively, due to non-specific symptoms. The diagnosis is often uncertain, delayed due to the few cases and lack of surgical history. BLH is often revealed by small bowel obstruction but a delay in diagnosis may lead to strangulation and an increased risk of serious complications.The preoperative diagnosis is very difficult but CT represents the best medical investigation, especially, multidetector CT with 3D reformatted images which provides significant advantages to evaluate the small bowel and surrounding structures, and shows its superiority to identify site, level and causes of small bowel obstruction including BLH.Treatment is always surgical. The management of internal hernia through the broad ligament is two fold:First, incarcerated contents reduction, and if necessary, non-viable bowel resection. Second, defect closure to prevent recurrent small bowel obstruction (using a suture or clip).The laparoscopic approach is actually the treatment in the management of internal hernia for uncomplicated cases ,18. HoweIn our case, we preferred the laparotomy approach because the patient had an anterior surgery and bowel ischemia was suspected. This open surgery allowed a complete exploration, which is important due to the presence of other internal hernias.The possibility of internal herniation through a defect in the broad ligament should be considered whenever a patient presents small bowel obstruction without obvious classical cause.Because of a possible contralateral BHL, a systematic exploration on the other side must be carried out.The surgery should be include the reduction hernia, the closure of defect and a complete abdominal exploration.4The occurrence of internal hernia through defect in the broad ligament is a very rare cause of small bowel obstruction.The recurrence of small bowel obstruction on BLH but on the other side is exceptional, like in this case.The surgeon, performing this procedure, should detect of possible other internal abdominal hernia, after the conventional treatment.Systematic exploration of a contralateral BLH should be recommended.This study did not receive any funding.Written informed consent was obtained from the patient for publication of this case report.This case report is exempt from ethical approval by our institution.Informed consent was obtained from the patientJ. Zemour: study concept, data collection, drafting of the paper and editing of the paper.X. Coueffe: Literature review.H. Fagot: Drafting of the paper.As this was a case report and not a clinical trial, this study does not require registration.Johanna Zemour is the guarantor for this study.Not commissioned, externally peer-reviewed.The authors declare that they have no conflict of interest.


Desmoid tumors are a rare entity, histologically benign (fibroblastic proliferation) but with an infiltrative growth that gives them a local aggressive behaviorThey affect the abdominal wall by 50%, retroperitoneum by 9% and the mesentery by 40%We present the case of a 76-year-old man with no pathological history. admitted in the emergency room due to a 5-day evolution fever associated with abdominal distension and a palpable mass in the hypogastrium. Hemodynamic instability with BP of 90/50 and tachycardia with good response to initial resuscitation with 2000 ml of physiological solution and antibiotic therapy (Metronidazole+Ceftriaxone). The exploration showed indurated and mobile formation in hypogastrium, without signs of peritoneal irritation. Blood analysis showed leukocytosis with immature cells, and CRP increased with normal lactate.Abdominal CT presented large supra-bladder pelvic mass of 12 cm with central necrosis and hydro-aerial level compatible with abscess formation in the tumor, presence of hepatic intra-portal gas in relation to the patient’s septic process .Streptococcus anginosus associated with mixed anaerobic flora. Percutaneous biopsy was negative for malignant cells; acute inflammatory component was associated with intestinal perforation.Pig tail drainage was placed obtaining purulent liquid, admission to the intensive care unit requiring noradrenaline (0.15 μg/kg/min). Improvement of the septic pattern occurred in the first 48 h with withdrawal of vasoactive drugs and decrease in inflammatory parameters. Cultures of blood and abscess liquid were positive for At 72 h, orotracheal intubation was required due to progressive respiratory insufficiency, without any increase in inflammatory parameters. Thoracoabdominal CT demonstrated respiratory distress, abdominapelvic free fluid and completely drained intra-tumoral abscess.Urgent surgical intervention showed a large tumor of 15x15 cm affecting the jejunum ; intestinal resection was performed with free margins and lateral-lateral mechanical anastomosis was done. During the post-operative period, the patient recovered progressively and was discharged after 13 days.Anatomopathological examination revealed mesenchymal proliferation on the intestinal wall without mucosa infiltration, constituted by a proliferation of elongated cells without pleomorphisms arranged forming bundles.Immunohistochemical analysis was negative for CD117, DOG1, ALK1, S100, CD34, desmin and actin and positive for vimentin and beta-catenin, desmoid tumor dependent on the jejunal wall, free neoplasic intestinal margins .Intra-abdominal desmoid tumors are frequently associated with familial adenomatous polyposis or Gardner’s syndrome. There are fewer than 100 published cases of sporadic intra-abdominal desmoid tumorsThe diagnosis of these tumors is made by immunohistochemistry.,The cells usually have a poorly circumscribed pattern with spindle cell proliferation forming long beams or spiral patterns; the cells do not show nuclear atypia or hyperchromasia are strongly positive to vimentin staining and the immunoreactivity to beta-catenin is expressed in the 67-80% of casesImaging exams are useful in establishing size, extension and anatomical relationship. One of the differential diagnoses to be taken into account are gastrointestinal stromal tumors (GIST) that share a stroma of common origin but are histologically, genetically and biologically different, so their treatment differs substantiallyThe therapeutic decision requires the approach of a multidisciplinary team. Surgery is considered the treatment of choice whenever possibleOur case was presented as a complication that required urgent surgical treatment. However, whatever the treatment of choice, recurrence rates are high (30-40%)


The synchronous colorectal malignancy is well described in the literature but combination of pancreatic incidentaloma with sigmoid cancer has not been well described and the association has not been described in syndrome.A 65-year-old man from the Indian subcontinent with a history of abdominal pain with loss of appetite, and with a history of bleeding per rectum and altered bowel habits presented to our hospital. An abdominal examination revealed a palpable mass in the region of his epigastrium and left hypochondrium, and a rectal examination was normal. A work-up included blood investigations, an abdominal contrast-enhanced computed tomography scan, a colonoscopy, and a positron emission tomography/computed tomography scan. He was managed by simultaneous distal pancreaticosplenectomy and radical sigmoidectomy. The final histopathology results were suggestive of moderately differentiated adenocarcinoma of the sigmoid colon with serous cystadenoma of the pancreas.The synchronous sigmoid colon cancer and pancreatic cystic incidentaloma is a rare presentation, which, to the best of our knowledge, has not been reported in the literature. We report the surgical management of this case and present a review of the literature. Genetic studies may be conducted to find out whether there is common genetic mutation resulting in these two malignancies, and may be helpful in screening programs. AssociaA 65-year-old man from the Indian subcontinent presented to our hospital with complaints of a mild, dull, aching left-sided abdominal pain for the past year, loss of weight and appetite for 6 months. There was a history of per rectum bleeding and recent history of altered bowel habits. He had no history of malignancy in the family. There was no other clinically significant history.A general examination of our patient was within normal limits. There was no significant lymphadenopathy.On abdominal examination, a 7 cm × 6 cm size lump was palpable in the epigastric region extending up to the left hypochondrium; it was nodular, nontender, firm in consistency, with a well-defined border, and not moving with respiration. The rest of his abdomen was unremarkable. A rectal examination was normal.Routine laboratory investigations including a complete blood count, an international normalized ratio, liver function tests, and renal function tests were within normal limits. Ultrasonography of his abdomen and pelvis was performed, and a multicystic lesion in the distal body and tail of the pancreas measuring about 7 × 7 cms was seen; no lymph nodes were seen. The rest of his pancreas was normal, and the proximal pancreatic duct was dilated, his liver was normal, and no free fluid was seen. Abdominal and pelvic contrast-enhanced computed tomography (CECT) was performed and revealed a multicystic lobulated mass arising from the distal body and tail of the pancreas with proximal pancreatic duct dilatation, a normal liver, no free fluid, an irregular mass in the sigmoid colon with mild narrowing of the lumen, with no proximal dilatation of the colon. Tumor marker tests showed carcinoembryonic antigen (CEA) test results of 10.48 ng/ml , and a CA19–9 test result of 7.19 U/mL (< 37 U/mL). A colonoscopy revealed ulceroproliferative, nonobstructing growth in the distal sigmoid colon with small polyps nearby. Colonoscopic biopsy result showed well-differentiated adenocarcinoma. A positron emission tomography/computed tomography (PET-CT) scan was performed and showed metabolically active circumferential thickening in the distal sigmoid colon with a maximum standard uptake value (SUV max) 7.0. and a multicystic lobulated mass with low metabolic activity arising from the distal body and tail of pancreas.Endoscopic ultrasound confirmed the picture of microcystic serous cystadenoma with no vessel involvement or lymph nodes. An endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS- FNA) was done, his fluid amylase levels were within normal limits, and his fluid CEA levels were not elevated. Cytology showed only benign cells Figs.  and 2.FiDistal pancreaticosplenectomy and radical sigmoidectomy with end-to-end colorectal anastomosis was performed.His postoperative course was uneventful. The histopathology report showed microcystic (benign) serous cystadenoma of the pancreas, and moderately differentiated adenocarcinoma of the sigmoid colon with lymph node metastasis 0/37. Angiolymphatic invasion was positive, and perineural invasion was absent.Our patient was followed up every third month for 1 year and was evaluated. Blood reports and imaging studies were unremarkable and did not detect any recurrence Figs. , 4 and 5This case report deals with a rare association of a pancreatic incidentaloma (a large pancreatic serous cystadenoma) with a symptomatic sigmoid colon cancer, which was dealt by simultaneous distal pancreticosplenectomy for the pancreatic serous cystadenoma and radical sigmoidectomy for the sigmoid colon cancer. Such a combined resection, to the best of our knowledge, has not been reported in the literature. The distal pancreaticosplenectomy was completed first to facilitate and avoid a theoretical possibility of contamination of the pancreatic area with colonic contents; also the colonic resection was facilitated by resecting the large pancreatic tumor impeding the left colon mobilization. Subsequently the sigmoid resection was completed, with an end-to-end descending colon to rectum anastomosis.et al. [Pancreatic incidentaloma (PI) has been defined as a mass that is incidentally discovered during an imaging study for symptoms other than the ones of the mass itself or the organ affected. The term was first described by Ho, et al.  and Kostet al. .Left-sided cystic pancreatic incidentalomas are uncommon neoplasms, but a significant number of the patients harbor early stages or frank malignancy, hence an aggressive approach may be required .et al. [Management of a cystic pancreatic incidentaloma (PI) is a complex problem as there is a lot of debate in the management of cystic lesions of the pancreas. Gore et al.  have recLarge (> 4 cm) serous cystadenomas are more likely to be symptomatic. Although the median growth rate for this neoplasm is only 0.6 cm/year, it is significantly greater in large tumors. Whereas expectant management is reasonable in small asymptomatic tumors, resection for large serous cystadenomas is recommended, regardless of the presence or absence of symptoms .Serous cystadenoma are generally considered benign cystic tumors of the pancreas. Very rarely, they are malignant (< 1% incidence) , 6, 7.et al. [et al. [In a multi-institutional retrospective study of 398 patients, Le Borgne  [et al.  found onet al. [et al. [The role of F-18-fluorodeoxyglucose positron emission tomography (FDG-PET) has been discussed Tseng et al.  and Sper [et al. . FDG-PETEndoscopic ultrasound is increasingly being used in characterization of cystic pancreatic lesions because it provides the highest resolution of the lesion and guided fine-needle aspiration can be done for analysis of the fluid for cytology, viscosity, amylase levels, tumor marker levels, and molecular analysis that may contribute significantly in management of the case .In the literature, there is not much data on concomitant occurrence of serous cystadenoma and colorectal malignancy, but colorectal cancers are the most common extrapancreatic malignancy associated with intraductal papillary mucinous neoplasm (IPMN) in studies performed in Western patients ranging from 3 to 12% , 12, whiThis case report deals with a rare combination of a large cystic pancreatic incidentaloma, a benign serous cystadenoma, with a symptomatic sigmoid colon cancer, which was successfully managed with a simultaneous distal pancreaticosplenectomy and radical sigmoidectomy, in that sequence. To the best of our knowledge, such a surgical procedure in a case of cystic pancreatic incidentaloma has not been reported. Genetic studies may be conducted to find out whether there is common genetic mutation resulting in these two malignancy and may be helpful in screening programs.


Gastrointestinal stromal tumor (GIST) of the ileum is not a common differential to consider in the management of acute right iliac fossa (RIF) pain and tenderness. Finding of a normal-looking appendix intraoperatively should arouse the surgeon to explore further and look for other unanticipated pathologies. We present a case, clinically diagnosed as acute appendicitis and intraoperatively found to be an ulcerated ileal GIST.  A 28-year-old female without previous comorbidities presented to the emergency unit with sudden pain around the umbilicus that later migrated and localized to the RIF for one day. There was associated intermittent fever and anorexia without urinary symptoms. Abdominal examination revealed guarding and rebound tenderness at RIF. Examination by 2 senior surgeons at different points of time, the same day, made a clinical diagnosis of acute appendicitis. Ultrasonogram (USG) was inconclusive. At laparotomy through Lanz incision, the appendix was found to be normal and no other pathology was identified on walking bowel up to 3 ft proximal to ileocecal junction (ICJ). Just when closure was thought of, an ulcerated lesion could be seen through the medial aspect of the incision. On further exploration, a 7 × 5 cm ulcerated lesion arising from the antimesenteric border of the ileum was noted with localized interloop hemoperitoneum and inflammatory exudates. Ileal segmental resection anastomosis was done with peritoneal toileting. The lesion was subsequently reported to be an ulcerated malignant GIST.  The commonest cause of RIF pain with localized peritonitis is an acutely inflamed appendix. Dilemma arises when the appendix is found to look normal. Further exploration is indicted to not miss other findings. The term “GIST” was first introduced by Mazur and Clark in 1983 to include the nonepithelial tumors of digestive tract that lack ultrastructure of smooth muscle cells and immunohistochemical properties of Schwann cells. GISTs are known to arise from the interstitial cells of Cajal that are regarded as the pacemaker cells, constituting a part of the autonomic nervous system of the gut and controlling intestinal peristalsis [A 28-year-old female with insignificant past medico surgical history presented with one day of acute onset pain in the periumbilical region that later migrated and confined to the RIF. She had associated intermittent fever, nausea, and loss of appetite. She did not have any urinary symptoms, bowel irregularities, or gynecological complaints. Abdominal examination was performed by two senior surgeons at two different occasions; the same day had findings of guarding and rebound tenderness at RIF. Hematological tests showed polymorphonuclear leukocytosis with left shift. Biochemical tests and urinalysis were normal. Urinary pregnancy test was negative. Abdominal radiographs were unremarkable. USG could not visualize appendix and was inconclusive except for probe tenderness in RIF. CT scan of the abdomen could not be done due to unavailability. A clinical diagnosis of acute appendicitis was made assigning an Alvarado score of 9/10. Laparotomy was performed using the Lanz incision in RIF. Intraoperatively appendix was found to be normal without evidence of inflammation or infection in RIF. In view of symptoms and signs, a possibility of other pathology was thought. Walking the bowel proximally up to 3 feet (1 m) did not show a Meckel's diverticulum or any other small bowel lesions. There were no obvious mesenteric lymph nodal enlargement and pelvic organs looked pristine. Approaching closure, just when the medial edge of the incision was retracted superomedially, a hemorrhagic lesion seemed to appear little deeper in the mid abdomen. Therefore, the incision was extended transversely from the medial edge to explore further. Entire bowel was explored and this revealed an ulcerated lesion measuring 7 × 5 cm arising from the antimesenteric border of the ileum 8 feet (2.5 m) from ICJ with localized interloop hemoperitoneum and inflammatory exudates as shown in Histopathologically, gross examination confirmed the operative findings, and the cut section revealed a nodular lesion protruding out of the serosal surface measuring 7 × 5 cm along with 2 lymph nodes each measuring 2 × 1 cm.Microscopically, the growth from the ileum had villous lining epithelium with focal ulceration. The submucosal region had a circumscribed nodule with proliferation of loosely cohesive spindle cells; some of which were arranged in vague storiform pattern and others in long fascicles. There were areas with epitheloid cells forming small anastomosing nests and cords. The areas in between these showed skenoid fibers along with focal areas of hemorrhage, infarction, and congestion as shown in Based on tumor size and mitotic activity, possibility of a malignant GIST was suggested along with immunohistochemical analysis (CD117 and CD34) for further confirmation. The patient had an uneventful recovery and was discharged on the 8th postoperative day. She was advised to review a week later at the outpatients but failed to report. All possible contacts were used to trace her, but she remained inaccessible and lost to follow-up.It is a common clinical situation to have a patient presenting with periumbilical pain subsequently localizing to the RIF associated with vomiting with or without nausea and fever. The classical symptom complex called Murphy's triad is often observed and tends to occur in the same sequential order . The finThe annual global incidence of appendicitis is reported to be 11 cases per 10,000 population . In one In essence, an ulcerated malignant GIST of the ileum masquerading as acute appendicitis is a common presentation of an uncommon diagnosis. Disproportionate symptoms and signs inconsistent with a normal-looking appendix on table should alert the surgeon to suspect other possible causes no matter how remote. Negative appendectomy should not be taken lightly and mandates thorough exploration of the entire length of bowel. Definitive diagnosis is possible on histopathological evaluation aided by IHC. Resection with negative margins and further therapy based on IHC panel forms the backbone of management. The awareness of the clinical presentation and good pathological expertise are important adjuncts in the diagnosis. Surgery is the mainstay of treatment in the acute presentation.


Non-Hodgkin lymphoma (NHL) is a type of blood cancer and 25% of NHL patients present with a primary extranodal tumor. Primary NHL of the vagina is extremely rare with only a few cases reported. We present the case of a 79-year-old, grand multipara, Peruvian woman with an eleven-month history of symptoms of pelvic and vaginal pain and vaginal bleeding. Vaginal examination revealed an exophytic tumor that involved the labia, right vaginal wall, cervix, parametrium, and pelvic bone. A computed tomography (CT) image showed a 10 x 10 x 9-cm solid tumor in the pelvic cavity with irregular edges in the pelvic floor, parametrium, and the perineal soft tissues. Bilateral pelvic and inguinal adenomegalies were found. No signs of metastases were found. Marrow and bone biopsy studies were negative. The diagnosis was NHL type diffuse large B-cell lymphoma (DLBCL) of vagina stage one. The patient was treated with six courses of CHOP-R . She showed a complete response and remained in remission in follow-up control visits. NHL of the female genital tract is rare. CHOP-R is the first line of treatment for this type of cancer. However, less is known about the follow-up protocol and relapse management. Vaginal lymphoma has an extremely low prevalence, and collaborative studies are required to study the same. Non-Hodgkin lymphoma (NHL) is a group of lymphoid malignancies, which typically develop in the lymph nodes but may occur in almost any tissue. Twenty-five percent of NHL patients present with a primary extra-nodal tumor . Ninety Hematologic malignancies rarely present as a primary gynecologic problem. They usually occur as a result of the systemic dissemination of the tumor cells rather than the primary tumor. Extranodal NHL often involves the gastrointestinal tract and the bone marrow. Less than 0.5% of all extranodal NHLs involves the female genital tract . PrimaryA 79-year-old Peruvian woman presented with 10 months of vaginal bleeding and a vaginal tumor that was exophytic involving the cervix and extending to the right vaginal wall. There was a right tumoral mass that involved the sub-epithelium of the labia to the pelvic bone. The right parametrium was invaded until the pelvic bone. Computerized tomography (CT) scans showed a large solid tumor mass (10 x 10 x 9 cm) in the pelvic cavity with irregular edges that infiltrated the pelvic floor, parametrium, and the perineal soft tissues . The patient completed six courses of CHOP-R chemotherapy. The last clinical evaluation showed a complete clinical response Figure . The patNHLs are a large group of lymphoid neoplasms, with different morphologic, immunophenotypic, and genetic characteristics. NHL of the female genital tract is rare . The priPrimary vaginal lymphomas have been reported in women of age 26 to 66 years . This lyDue to its rarity, there is no established treatment for primary NHL of the vagina. The first line of treatment for NHL is chemotherapy since NHLs are extremely responsive to it . The treVaginal lymphoma has an extremely low prevalence. CHOP-R is the first line of treatment for this type of cancer. However, little is known about the follow-up protocol and relapse management. More collaborative studies are required to study vaginal lymphoma.


We report a case of a 56-year-old woman who simultaneously presented adrenal and spleen tumors. Computed tomography imaging revealed a 7-cm enhancing adrenal and 2-cm solitary spleen masses. The patient simultaneously underwent left adrenalectomy and splenectomy. The pathological findings revealed the presence of synchronous adrenal and spleen angiosarcomas. Remarkably, she is disease-free since postoperative 18 months. Angiosarcoma, a malignant endothelial-derived soft-tissue sarcoma, develops from blood or lymph vessels, with the most common locations being the head, neck, breast, and extremities . AlthougA 56-year-old woman visited our hospital presenting a left adrenal tumor; during examination, a spleen nodule was incidentally diagnosed. Computed tomography revealed a 7.4-cm-long enhanced left adrenal tumor and 1.8-cm-long enhanced nodule within the spleen Fig. a. The spAngiosarcoma, a rare disease developing from the endothelium of blood or lymph vessels, constitutes 1–2% of all sarcomas . This tuPrimary adrenal angiosarcoma is extremely rare and was first reported by Kareti et al. in 1988 . Since tIn our case, differential diagnosis was adrenal carcinoma and spleen metastasis, because the adrenal tumor size was > 4 cm. According to the report from Alfonso et al., the tumor size more than 4 cm indicates malignancies , althougTo the best of our knowledge, this is the first report of angiosarcomas simultaneously occurring in the left adrenal gland and spleen. The diagnosis was readily established with appropriate H&E and immunochemical panels. The patient underwent surgery and survived for 18 months. Hence, we believe that surgery should be considered when patients are tolerant undergoing surgery.


Mucocele is a slow growing, benign but locally aggressive cystic structure lined by true epithelium. It often results due to obstructed sinus outflow or obstruction of gland-like mucous retention cyst. It can cause bony destruction and might result in orbital symptoms like diplopia, orbital displacement, visual disturbances. Other clinical features are facial numbness, dental problems, etc. Radiological evaluation is the preferred diagnostic modality. Surgical removal is the treatment of choice both endoscopic and open (could well luc) approach or combined approach are preferred. Here we report a very typical case of maxillary mucocele who presented with subtle symptoms of nasal obstruction. The study was done in compliance with SCARE guidelines.[1]We present a very unique case of 24 years man with complaints of nasal obstruction and swelling over the right cheek for 2 years. He had a history of facial trauma two years back. Diagnosis was made on the basis of radiological examination CT (Computed Tomography) scan. He underwent enucleation via Cold well Luc's approach with good postoperative results.Maxillary mucoceles are slow growing benign lesions. However, they are locally aggressive and cause bony destruction resulting into orbital and dental symptoms. Thus early recognistion with regular folllowr up and planning for surgical intervention can help avoid complications. •Maxillary sinus mucoceles are rarer entities as compared frontoethmoidal mucoceles.•We report a case of a giant maxillay sinus mucocele with a history of nasal obstruction and right cheek swelling for 2 years.•Early diagnosis and surgical intervention is the mainstay of management in most of the cases. It is often described as mucous retention cyst or cystlike and cystic with true epithelial linings. When present in paranasal sinuses they are lined with respiratory epithelium  AlthoughAccording to the possible mechanisms postulated by Robert.E. Blasley et al., in 1984, th first mechanism states that a mucocele can arise from an epithelial inclusion within the connective tissue stroma of the sinus lining . Similarly, the second mechanism states that extravasation of mucus into the connective tissue stroma through a ruptured mucous gland leads to reflecting the sinus lining within itself as the gland continues to secrete mucus. This results in formation of mucocele. The third mechanism involves the blockage of a mucous gland. Glands lying within the connective tissue stroma of the sinus lining, as they expand might reflect the normal sinus lining resulting into a mucocele. The fourth mechanism describes the pooling of the mucous discharge from the mucous glands of the sinus due to blockage of the sinus ostium. This mucocele Is lined by the original sinus membrane and will contain mucus. Mechanisms 2 and 3 are the most accepted mechanisms and have been described by previous authors to their studies .The most important diagnostic tools are computerized tomography and magnetic resonance imaging where egg shell bony erosion can be seen in a CT scan ,7 Treatm2A 26-year-old man presented with complaints of nasal obstruction for 2 years. He was having nasal obstruction on and off as was on repeated use of topical nasal drops He also complained of an associated headache on and off which was more localized in the right side. He also complained of swelling of the right cheek for the same duration of time. The swelling was insidious in onset and progressively enlarging in size. There was no history of pain, nasal congestion, facial numbness, or any oroantral surgery in the past. However, he gave a history of blunt trauma over his right cheek 5months back. There was no significant family history relevant to the disease. On Inspection, we could see a diffuse swelling of the right cheek with the mild erythematous change of the overlying skin . On palp3Mucocele is more common in the frontoethmoidal region and rarely involves the maxillary sinus. The most common etiology for maxillary sinus mucoceles are untreated trauma, history of prior sinus surgery, facial fractures . Antral In our case, we performed enucleation via Caldwell Luc approach. The post operative period was uneventful. He was discharged on the 7th postoperative day. There were no signs of complications or disease recurrence on subsequent follow-up. The patient was compliant with the treatment provided.4Long-standing benign inflammatory condition of paranasal sinus and blockage of the osteomeatal complex can result in mucocele. Though bening in nature regular follow up and close observation can be helpful in planning the surgical intervention to avoid complications that usually arise due to the locally aggressive nature of the tumor.As this is a case report ethical approval has been exempted and written informed consent has been taken from the patient.No sources of funding1-Dr.Sudha Shahi – - Study concept or design, data collection, literature search, writing paper, final decision to publish.2-Dr.Anuj Devkota-literature search, final decision to publish.3-Dr.Tika Ram Bhandari- Supervised the writing of the manuscript, final decision to publish.4.Dr.Tridip Pantha: Supervised the writing of the manuscript, final decision to publish.5.Dr.Dipendra Gautam: Supervised the writing of the manuscript, final decision to publish.No conflict of interest.Is a case report.Dr. Sudha Shahi.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 as required.All authors declare that they have no competing interests.Not commissioned, externally peer reviewed.


Sporotrichosis is the most common subcutaneous mycosis. It is caused by the dimorphic fungus Sporothrix schenckii. Ocular sporotrichosis is uncommon and has been rarely reported.We describe a 34-year-old female who presented with a nodule increasing in size near the medial angle of the left eye. Originally, she was misdiagnosed with a dacryocyst space-occupying lesion, and the lesion was removed by surgery.Sporothrix.Findings of fungal structures in the histopathological examination contributed to the diagnosis of Sporothrix dacryocystitis. Further culture of conjunctival secretions and contact lens storage solution was positive for She was treated with oral itraconazole, 200 mg by mouth twice daily.After 3 months of treatment with oral itraconazole, culture of the conjunctival secretions was negative.It is of paramount importance to clinically suspect mycosis, even in unusual locations or in the absence of the typical epidemiological history. It is the most prevalent subcutaneous mycosis in Latin America, with worldwide distribution. Infection is usually associated with traumatic subcutaneous inoculation with contaminated soil, plants, or organic matter contaminated with Sporothrix. Sporothrix mainly affects the exposed skin; the most common location is the upper limbs followed by the face, lower limbs, and chest. Ocular sporotrichosis is a rare condition, and even rarer cases of dacryocystitis caused by Sporothrix have been reported. In this article, we describe a case of Sporothrix dacryocystitis with the secondary involvement of both conjunctivae, probably due to auto-contamination with contact lenses (CLs).Sporotrichosis is a subacute or chronic mycosis, caused by traumatic inoculation of the 2S schenckii after 3 days of incubation  from July 2008 to July 2010, and most of them are from areas prone to infection with sporotrichosis such as Brazil. Although the pathogenesis of dacryocystitis is not completely understood, it typically occurs in the setting of nasolacrimal duct obstruction, and as a consequence, obstruction of the lacrimal ducts develops; therefore, epiphora and discharge are the most common presentations of dacryocystitis. We speculate that our patient was treated at the initial stage of dacryocystitis; therefore, she did not present with epiphora or discharge, and the lacrimal excretory system was unobstructed until just before the surgical procedure. Although less reported, conjunctival sporotrichosis can be seen in some cases.–9 Different from other reported cases of conjunctival sporotrichosis, our patient first presented with a nodule near the medial angle of the left eye and bilateral conjunctival injection. We considered that her bilateral conjunctivae might be contaminated through any one of the following transmission channels: the fungus was transmitted from the nodule to the bulbar conjunctivae during the CL handling, which in turn contaminated the lens solution; or the fungus in the dacryocyst travelled retrograde to the conjunctivae along the lacrimal duct, then contaminated the CLs and lens solution during the CL handling. We are inclined to believe the second transmission channel for Sporothrix was found in the lacrimal sac but not skin surface of the nodule.Sporotrichosis is a chronic infection of the cutaneous and subcutaneous tissues and lymphatics caused by the thermally dimorphic subcutaneous fungus, The lesion's unusual location and the patient's uncommon epidemiological history caused our misdiagnosis and mistherapy, which ultimately led to the loss of her dacryocyst. The polymorphic appearance of sporotrichosis lesions makes the diagnosis difficult on clinical grounds alone. In this particular case, sporotrichosis was definitively diagnosed by detection of rarely observed fungal structures via histopathological examination. Therefore, even in unusual locations or without a common epidemiological history, clinical suspicion of fungal diseases is of the utmost importance. The patient provided written informed consent for her case details, photos, and other images to be used for this report.Conceptualization: Yaru Dong.Data curation: Lixia Sun, Yaru Dong.Formal analysis: Lixia Sun.Funding acquisition: Lixia Sun, Xin Wang.Investigation: Xin Wang, Min Zhang, Baihui Shan.Methodology: Xin Wang, Min Zhang, Baihui Shan.Project administration: Lixia Sun, Xin Wang, Min Zhang, Baihui Shan.Resources: Lixia Sun, Baihui Shan.Visualization: Yaru Dong.Writing – original draft: Yaru Dong.


Cecal volvulus is an extremely rare cause of intestinal obstruction in the pediatric age group and its incidence is unknown.We present a unique case of cecal volvulus in a 3 year old female with congenital dilated cardiomyopathy.Cecal mobility due to malfixation and malrotation is the main cause. Cecal Volvulus usually presents with constipation, abdominal pain and distention. Common complications entail strangulation, ischemia and gangrene. Cecal volvulus has been reported in association with various abnormalities. However, Incidence of cecal volvulus in a child with congenital dilated cardiomyopathy has not been reported. Surgery is the mainstay of management.Cecal volvulus should be kept in mind in a child presenting with signs suggestive of intestinal obstruction. Cecal m29/L, Hemoglobin was 11.1 gm/dl and the platelet count was 343 × 109/L. Abdominal X-ray with contrast was performed , patent ductus arteriosus (PDA) nor pericardial effusion.There is paternal consanguinity and the parents gave a history that the patient’s older sister underwent an operation at the age of 18 months for intestinal obstruction; however the patient records and details of the operation were not available.3Cecal volvulus occurs due to an abnormally mobile cecum due to improper developmental fixation or malrotation of the cecal mesentery ,3. CV isThe clinical presentations of patients with CV vary depending on the extent of involvement and duration of the twist, with chronic constipation, generalized abdominal bloating and vomiting being the usual symptoms. CV can lead to serious complications including strangulation, gangrene, perforation and peritonitis ,4,6. DueCV has been reported as a postoperative complication of right nephrectomy and in association with Cornelia de Lange syndrome . Corneli4Although CV is a rare cause of intestinal obstruction in pediatrics, it should be kept in mind in a child presenting with chronic constipation and abdominal bloating. Early diagnosis is crucial to prevent the potentially fatal complications of CV. The association of congenital dilated cardiomyopathy and CV should also be kept in mind.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Our study is exempted from ethical approval in Institutional Review Board of our hospital.We have written informed parental consent to publish this report.Ahmed Shehata: Literature Review and drafting manuscript.EzzElDien Ibrahim: literature review and drafting manuscript.Mohamed Helal: literature review editing and revising the manuscript.Basma Magdy: operating on the patient and presenting the case.Mohamed Elseoudy: operating on the case.Muayad Shaban: literature search discussion data collection.Heba Taher: Clincal supervision, drafting and revising the manuscript.This is a case report and not a human study. It is exempt from registering.Heba Taher.Not commissioned, externally peer-reviewed.We declare no conflict of interest.


Emphysematous pyometra is a rare canine disease characterized by gas-forming bacteria infecting the uterus and causing an accumulation of both gas and infectious exudate in the uterine lumen. While radiological features of emphysematous pyometra have been previously described in dogs, the ultrasonographic appearance has not been reported.A 7-year-old intact female Labrador Retriever was presented because of a 1 day history of vomiting, anorexia, mild polyuria/polydipsia and signs of fatigue. On physical examination the dog had a swollen vulva with a sparse amount of yellow discharge. Lateral and ventrodorsal radiographs showed a dilated predominantly gas-filled tubular structure located in the mid and cranial abdomen traversing from left to right and ending dorsally at the level of the 12th thoracic vertebra. A small intestinal ileus was initially suspected. Following the radiographic examination, abdominal ultrasound was performed. In the left mid and caudal abdomen there were two thin-walled gas-containing tubular structures. One had the typical layered appearance of an intestinal wall and represented the descending colon. The second structure had a similar thickness but homogenously hypoechoic wall and contained gas and echogenic fluid in the lumen. By use of several positional changes of the dog aiming to alter the location of the intraluminal gas, the second structure was traced to the right ovary cranially and the uterine body caudally, confirming that the structure was the right uterine horn. A final diagnosis of emphysematous pyometra was made.Ultrasound can be used as a non-invasive diagnostic method to differentiate between small intestinal ileus and emphysematous pyometra. Emphysematous pyometra in the dog is a rare, life threatening disease, characterized by gas-forming bacteria infecting the uterus and causing an accumulation of both gas and infectious exudate in the uterine lumen . To dateWe describe an unusual case of emphysematous pyometra, predominantly affecting one uterine horn that was in an unexpected anatomical position.9 cells/L, reference 5.05–16.76 × 109 cells/L). A serum chemistry panel identified mild metabolic hypochloremia and respiratory alkalosis and mildly elevated lactate.A 7-year-old intact female Labrador Retriever was presented because of a 1 day history of vomiting, anorexia, mild polyuria/polydipsia and signs of fatigue. The owner had noticed some discharge from the vulva, as well as mucus and helminths in the feces. The dog had been in estrus 2 weeks before presentation but was not mated. The owner reported episodes of vomiting and weakness during the dog’s previous estrus cycles. On physical examination the dog was normothermic, had a swollen vulva with a sparse amount of yellow discharge and showed signs of pain on abdominal palpation. Hematology showed mild leukocytosis  and methadone . The ultrasonographic findings were confirmed on surgery Fig. . The rigEscherichia coli is the most commonly isolated organism [Staphylococcus spp., Pseudomonas aeruginosa, Citrobacter diversus, Clostridium perfringens and Enterococcus avium have been isolated [Pyometra is a bacterial infection that leads to accumulation of pus in the uterus, and commonly affects intact female dogs. Its pathogenesis is related to the repeated exposure of the endometrium to progesterone during the long luteal phase of the estrus in bitches . The proorganism  but a wiorganism , 2, 7–9.isolated –5.The radiological features of emphysematous pyometra have been described in dogs –5, and iIn cases of pyometra, the uterine horns are usually symmetrically affected, but this is variable and only one horn may be affected . The desThe ultrasonographic appearance of a pathologically altered uterus varies, but luminal fluid is commonly found and ultrasound is of great use to detect mild accumulation of fluid, even if differentiation of the type of fluid is not possible .In this case the ultrasound examination was performed to localize the area of and the reason for the suspected obstruction  since this could not be established based on the radiographs. Usually ultrasound gives limited information when a large amount of gas is present in the investigated organ, since gas is a strong reflector and prevents transmission of ultrasound waves, creates reverberation artifacts and inhibits diagnostic information from being obtained . DespiteSevere inflammatory bowel disease can significantly affect the intestinal wall layers, even causing their complete loss, thereby making it difficult to differentiate between an abnormal intestinal segment and a gas-filled uterus. The peristaltic movement can help distinguish intestinal tract from genital tract, but altered motility is often present with severe enteritis, making this parameter less useful for distinguishing between intestines and a gas-filled uterus. Crucial to this case was the continual change in position of the dog during the examination, that allowed both the gas in the uterus to move and the superimposed colon to change position. These positional changes of the dog were repeated until a clear connection of the tubular structure with the right ovary and uterine body was identified, allowing for the definitive diagnosis.In conclusion, as previously stated , radiolo


Harlequin syndrome (HS) is a rare dysautonomia of the sympathetic nervous system leading to asymmetric facial flushing and sweating. In the literature, only a few cases of HS after thoracoscopic tracheoesophageal fistula (TEF) repair are reported. We report on a newborn with TEF who developed HS after thoracoscopic repair. On the first day of life, the girl  underwent thoracoscopic repair of a type C esophageal atresia  without complications. The postoperative course was uneventful, the patient swallowed and thrived well and did not require esophageal dilatations. At 2 years of age, missing facial flushing, transpiration, and warming on the right side of her face during agitation were noticed. As no further intervention was required, the girl and her parents adapted well to the symptoms. Our report shows that the late onset of HS after the surgical procedure is unlikely a direct causal relation to the thoracoscopic operation but rather a shared embryological pathogenesis, like a neurocristopathy. Harlequin syndrome (HS) is a very rare and seemingly benign condition characterized by unilateral loss of facial sympathetic functions. It was first described by Lance, an Australian professor of neurology, in 1988 in adults who showed unilateral flushing and sweating. He hypothesized that these syndromes occurred due to an idiopathic ipsilateral affection of the sympathetic outflow of the third root.Since then, several reports and reviews have been published on this disorder. The cause of HS can be idiopathic and iatrogenic in both adults and children.A Caucasian baby girl of healthy parents was delivered spontaneously in the 41th week of gestation with a birth weight of 3,480 g, height of 50 cm, and head circumference of 37 cm. Postnatal she was diagnosed to have esophageal atresia (EA) type C (TEF). On the second day of life (DOL) and after bronchoscopy, the thoracoscopy was performed in left-sided half prone position. A type C (gross) EA with distal fistula (TEF) was confirmed. The azygos vein was electrically transected and the vagal nerve was preserved. After ligation of the fistula using a polyglactin 4.0 suture, a well-perfused, tension-less primary anastomosis was created with polyglactin 5.0 interrupted stiches and splinted with an 8 Charrière nasogastric feeding tube. A 12 Charrière chest-drain was placed. Operating time was 105 minutes. The girl returned to neonatal intensive care unit (NICU) and the postoperative course was uneventful. On the 2nd postoperative day (POD), nutrition was started via the feeding tube. On the 4th POD, the girl was extubated, and continuous positive airway pressure ventilation was performed until the 7th DOL. Oral feeds were started at the 7th DOL. At the 10th DOL, she was transferred to the pediatric surgical ward. The nasogastric feeding tube was removed at the 22nd DOL. The VACTERL workup revealed no associated malformations. A screening for metabolic diseases was unremarkable. The patient was discharged on the 25th DOL. The further development at home was uneventful. At the age of 1 year, the first endoscopic follow-up was performed and showed a wide esophageal lumen without stenosis. The girl had a normal height (P91) and weight (P81). The second endoscopic follow-up after 2 years was unremarkable too with a pH study showing slightly higher acid exposition without remarkable reflux episodes. At the age of 2 years, the girl presented with no complications, normal ingestion, normal weight, and normal height. However, her parents reported that the right side of her face remained pale and was not sweating during agitation . This wWe report a case of HS that occurred 2 years after thoracoscopic repair of a child with TEF. HS is a repeatedly reported dysautonomia of the sympathetic innervation leading to loss of hemifacial flushing, sweating, and other sympathetic functions. It has to be separated from the Harlequin color change, which happens due to a paroxysmal change of vasomotor condition of the skin in the first DOLs, leading to alternating hemi-sided flushing.To our knowledge, the report presented here describes the third patient of HS in a girl with EA (gross type C) and successful thoracoscopic treatment. This raises the question whether HS is a side effect of the thoracoscopic technique. Studies comparing the long-term complications after thoracoscopic versus open approaches for EA show no differences.The pathogenesis of primary and secondary HS is still not sufficiently understood. The loss of hemifacial flushing and sweating most likely occurs due to a loss of function or dysregulation of the third-order neuron of the vaso- and sudomotor fibers of the ipsilateral sympathetic nervous pathway.The theory of neurocristopathy describes the connection between disturbed migration of neural crest cells and maturational dysautonomia. Maldevelopment of the autonomic nervous system during embryogenesis can result in dysregulated sympathetic innervation. There is evidence for an association of EA and autonomic disturbances such as hyperhidrosis during feeding and hyperthermia.We report the third case of HS in a 3-year-old girl with EA after uncomplicated thoracoscopic repair. The late presentation of the HS in these cases makes a direct causal relation to the surgical procedure implausible. HS may therefore be a (primary) neurocristopathy associated with EA/TEF.


We report the case of a 24 year old woman, without significant past medical history, who consulted for an asymptomatic and pigmented lesion of the breast evolving from infancy. Dermatological examination showed a brown plaque localized on the milk line in the underside of the right breast, measuring 1 cm, firm in consistency, with a non infiltrated base. Dermoscopic examination revealed a peripheral pigmented regular network with a cleft-like appearance in a pigmented homogeneous central area. The rest of clinical examination was normal. Based on the clinical and dermoscopic findings a diagnosis of supernumerary nipple was established. The patient refused the removal of the nipple since it was asymptomatic and without any aesthetic concern. Supernumerary nipples are congenital malformations of nipples that arise in addition to the normal bilateral nipples. Their prevalence is between 0.22% and 5.6%. They most commonly arise along the milk lines, which extend from axillae to inguinal region bilaterally. However, they may also appear in other regions of the body and may be associated with underlying breast tissue. Supernumerary nipples may be associated with other abnormalities of the central nervous, gastrointestinal, respiratory, skeletal or cardiovascular systems and sometimes genetic and chromosomal abnormalities. Nevertheless, the most known is the association with genitourinary tract malformations and malignancies. Given these morbid associations, some authors recommend their systematic search while others do not consider them. Supernumerary nipples do not require any specific treatment. However, surgical excision may be indicated for aesthetic reasons or in case of doubt diagnostic or complications.