Mullerian adenosarcomas are rare and often low-grade mixed tumors that typically respond well to optimal surgical resection. However, adenosarcoma with sarcomatous overgrowth (ASSO) is a high-grade mixed tumor commonly associated with invasion, metastasis, and a poor prognosis. The health care providers herein report a case study of a patient diagnosed with ASSO who has maintained remission status for 19 months following radical surgical resection alone. The patient, a 24-year-old Caucasian female without significant medical history, initially complained of abdominal fullness, pelvic pressure, altered menses, and unintentional weight loss. A necrotic cervical mass was present on the exam; mass biopsy revealed spindle cell sarcoma with rhabdomyosarcomatous differentiation. The patient underwent exploratory laparotomy, total abdominal hysterectomy, bilateral salpingectomy, radical tumor debulking, and pelvic and periaortic lymph node dissection. Histopathological diagnosis of the resected specimen was consistent with ASSO, limited to 0.7 cm out of 2.0 cm of myometrial thickness, with negative lymph node and parametrial tissue, consistent with Stage IB disease. She did not receive adjuvant chemotherapy or radiation and has remained disease-free to date. Because of the rarity of ASSO and lack of abundant case study reports, uniform clinical guidelines for treatment following surgical resection of a high-grade adenosarcoma remain unclear. However, the case study below may suggest that radical surgical debulking of this disease with negative margins in young patients with early-stage disease can be sufficient in treating high-grade ASSO, despite their typical aggressive nature. She also noted altered menses with new-onset menorrhagia, difficulty with urination, and unintentional weight loss of 25 lb over the preceding 8 months. The patient's OB-GYN history was notable for menarche at 12 years old; the typical cycle length was 28 days with 7 days of bleeding. Her medication list included Nortrel 1/35 without side effects. Pelvic examination revealed a black, necrotic mass with foul-smelling yellow discharge in the vaginal canal . MRI of A mass biopsy resulted in spindle cell sarcoma with rhabdomyosarcomatous differentiation. Mayo Clinic concurred with this diagnosis, noting that it was unclear if this was a pure sarcoma or part of a biphasic malignancy. Cleveland Clinic then reviewed the pathology slides and reported atypical spindle cells, suspicious for sarcoma. Spindle cells were hyperchromatic, along with scattered bizarre tumor cells showing frequent mitotic activity. Spindle cells were negative for desmin, CD10, myogenin, ALK1, and WT1. There was diffuse strong positivity for p16 and patchy strong expression for MyoD1, supportive of rhabdomyosarcomatous differentiation. Ki67 labeling index was approximately 80%. Features and staining increased suspicion for underlying sarcoma of some sort; however, the sample was too small for definitive diagnosis.The surgeons subsequently performed an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingectomy, radical tumor debulking, staging, pelvic and periaortic lymph node dissection, bilateral ureterolysis, cystoscopy, and bilateral ureteral stent insertion. Intraoperatively, the ovaries appeared to be completely within normal limits and were left in situ. A complete lymphadenectomy was also performed given suspicious findings on PET and MRI, some lymph nodes were noted to be enlarged intraoperatively but this appeared reactive in nature. The lower uterine segment and cervix measured at least 11 cm in diameter and approximately 15 cm in length. The uterus and cervix were removed and a partial radical vaginectomy was performed without transecting the tumor. The final surgical pathology was consistent with adenosarcoma with sarcomatous overgrowth (ASSO) from the posterior endometrium, approximately 21 cm in greatest dimension, including intrauterine and extrauterine components. There was 0.7 cm invasion out of 2.0 cm myometrial thickness. Bilateral parametria, lymphovascular space invasion (LVSI), pelvic washings, and 9/9 lymph nodes were negative for malignancy. The ER and PR testing were both < 1% staining. Mayo Clinic reviewed the final pathology; their findings were consistent with stage IB high-grade Mullerian ASSO.Given the patient's young age and lack of clear benefit of adjuvant chemotherapy, the patient was routinely followed with alternating CT chest/abdomen/pelvis and PET every 3 months post-operatively. The patient had a questionable recurrence in the lung six months post-operatively on routine PET. Fine needle aspiration was performed, and there was a concern for metastatic disease of Mullerian origin. However, malignancy was ruled out on final lung wedge resection pathology. At present, 19 months after her initial surgery, the patient remains in remission and continues to undergo routine surveillance with imaging and physical exams.2Uterine adenosarcoma (AS) is a low-grade malignancy that is rare in premenopausal women. AS is a biphasic tumor with a benign epithelial component and a low-grade malignant sarcomatous component. Two histologic characteristics appear to have a poorer prognosis in AS: sarcomatous overgrowth (SO) and the presence of heterologous elements. These characteristics are found in about 10–15% of cases . The strThe presenting symptoms of AS can vary, although abnormal vaginal bleeding is the most common in the literature review. Presently, there is no reliable way to differentiate a benign versus malignant uterine leiomyoma before histopathologic review. MRI findings that may suggest AS of the uterus include marked enlargement of the uterus, myometrial thinning, polypoid mass protruding from the endometrial cavity, and mass that contains solid components with high signal intensity . EndometIn a review of risk factors for uterine AS, a few proposed are currently discussed, including a history of pelvic irradiation, hyperestrogenism, and history of tamoxifen use . Mubeen The mainstay of treatment of AS involves surgical resection; however, the role and benefit of adjuvant chemotherapy remain undefined in ASSO. Currently, there are no clear treatment guidelines for patients with ASSO and localized disease. Most notably, in their single-center review of treatment responses in 7 patients with AS, Nannini et al. discussed a 60-year-old female with stage IB uterine ASSO with less than 50% myometrial invasion and no LVSI. She did not receive any adjuvant therapy, similar to this presented case. She was without evidence of disease at 84 months . There wAdditionally, Tanner et al. performed a retrospective analysis of 31 uterine AS patients, 19 of which were treated from their initial diagnosis. Five out of 19 patients demonstrated SO. All 5 ASSO patients received surgical resection, three of the 5 cases contained heterologous elements, and two of 5 had myometrial invasion. Both of the patients with myometrial invasion had a recurrence. However, two of the remaining 3 patients without myometrial invasion also recurred . The autUltimately, the patient presented in this paper did not receive adjuvant therapy and has been in remission since the initial surgery for 19 months. Although there is no clear consensus on adjuvant treatment based on the staging of disease, it should be considered that early-stage ASSO may be amenable to radical surgical resection alone in young patients. Some studies of cervical ASSO report impressive disease-free survival in young patients with confined disease who only received surgical resection . In contFurther studies must be conducted on uterine ASSO to determine the appropriate course of postoperative treatment dependent on the stage and histologic elements. Nonetheless, given the aggressive nature of ASSO, early diagnosis and treatment are mandatory for a favorable outcome. In patients such as the one examined in this paper who have solely undergone radical surgical resection in early-stage disease, achieving negative margins is mandatory. Additionally, close surveillance with imaging and exams seems a reasonable option as she remains disease-free for 19 months.D.D. Dowding: Writing – original draft, Writing – review & editing, Project administration. L.M. Wayne: Writing – original draft, Writing – review & editing. A.S. Guirguis: Conceptualization, Writing – review & editing, Supervision.The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


Introduction: Cardiac tumors are significantly rare, with pericardial lymphangiohemangioma amongst the rarest cardiac tumor types, with very few reported cases in the literature. Clinically, lymphangiohemangiomas are generally deemed unresectable due to their proximity to the myocardium and the uncertainty of the outcomes following subtotal resection.Case Report: Herein, we report a case of a 40-year-old man diagnosed with a pericardial mass, dull thoracodorsal pain, and over a 10-years history of palpitation. Notably, the pericardial mass in the present case was found extended within the myocardium. Thus, a maximal safe resection was deemed preferable to a total resection. The surgically resected specimen showed pathological characteristics of a lymphangiohemangioma. After surgical resection of the lymphangiohemangioma, the patient was free of any tumor-related symptoms. Also, there was no evident tumor progression after a 4-year post-operative follow-up.Conclusion: To the best of our knowledge, the present case study is the first in the literature to report on a long-term post-operative outcome following subtotal resection of a pericardial lymphangiohemangioma. Tumors of the heart are significantly rare, with pericardial lymphangiomas amongst the rarest cardiac tumor types, with very few reported cases in the literature –3. ThougThese benign neoplasms constitute a distinctive form of cardiac tumor primarily discovered in patients below 10-years old , 5, 6; hCardiac lymphangiohemangiomas may present in both intrapericardial and pericardial spaces, though benign, cardiac lymphangiohemangioma may cause arrhythmia, tamponade, heart failure, and myocardial ischemia. Therefore, complete excision of cardiac lymphangiomas is recommended if possible . It shouHere we describe a 40-year-old man with pericardial lymphangiohemangioma who underwent a subtotal resection and was free of tumor-related symptoms and tumor progression after 4-years of follow-up.A 40-years-old man was admitted to our institution with a 10-years history of chest pain and palpitation without significant weight loss. No headaches, fever, or cough was dictated with a body temperature of 36.3°C and blood pressure of 132/92 mmHg at resting conditions. Physical examination revealed that the pulse was 78 bpm. No additional heart sound or significant heart murmur was heard over the chest wall. There were no other notable clinical findings and family history of cardiovascular disease following medical history and physical examination. Also, laboratory tests were unremarkable.Electrocardiography revealed sinus bradycardia without nodal block. No significant abnormalities were found following chest X-ray examination. Transthoracic echocardiography demonstrated normal ventricular function with a left ventricular ejection fraction of 69%; however, a saccular mass of 32 × 66 mm in size was discovered within the pericardial cavity, which extended toward the left atrioventricular groove. The saccular mass margins were well-defined and close to the left atrium and ventricle. The abnormal structure was heterogeneous with fractional acoustic enhancement and had gelatinous materials , arrow. The operative team evaluated the patient before the surgical excision of the tumor; afterward, a standard median sternotomy incision is performed. Following exploratory pericardiotomy, a botryoidal saccular mass was discovered below the left atrial appendage and above the left pulmonary vein with the size of about 10 × 8 × 6 cm. Due to the saccular mass size and its botryoidal texture, the patient's aorta was cannulated. Separate cannulas were placed in the superior vena cava and inferior vena cava. After full heparinization, cardiopulmonary bypass (CPB) was applied. The saccular tumor was filled with dark red liquid and a fibrous membranous network structure. Furthermore, the abnormal structure was found closely connected to the left atrium and left ventricle.Notably, complete resection of the saccular structure could not be achieved following intraoperative findings; hence, partial resection of the mass was performed. The patient tolerated the procedure, and the heart was beating in sinus rhythm. After adequate hemostasis and closing the wound in layers, the patient was carefully transferred to the intensive care unit (ICU) in a stable condition. Pathological results of the surgically resected specimen confirmed the diagnosis of lymphangiohemangioma with the proliferation of fibrous tissue, hyalinosis, and lymphocyte infiltration. The results also indicated that the cells were positive for Ki-67, Vim, SMA, MC and partially positive for CR, CK5/6, CD34, CD31, D2-40, and WT-1, but negative for CK, EMA, MOC31, and Des . The patPrimary cardiac tumors are sporadic, and they have often been diagnosed post-mortem . PericarNonetheless, the mass was generally unresectable in the case herein due to its significant invasion of surrounding tissues , 2, 8, 9Interestingly, Daubeney et al. report an intrapericardial lymphangioma with spontaneous regression 16-months after a pericardial window formation and biopsy of the mass . Given tWith the increased risk of bleeding and the lack of a pathologic diagnosis, we ruled it unsafe to attempt a total resection of the mass and instead carried out a partial resection. Notably, the patient's symptoms significantly improved after surgery. During a 4-years follow-up study of the patient, the tumor showed no evidence of progression nor regression, which was in contrast to the report by Robillard et al.  and DaubIn conclusion, we report a rare case of primary pericardial lymphangiohemangioma located in the left atrioventricular groove, close to the left posterior atrial wall and the left ventricle. Subtotal resection of the tumor is a satisfactory method to relieve symptoms. To the best of our knowledge, the present case study is the first in the literature to report on the long-term post-operative outcome following subtotal resection of a pericardial lymphangiohemangioma.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.CF drafted the manuscript. CF and LS designed the study and responsible for the collection of data or analysis. LS and CD revised 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.


Thymomas are slow growing, evolve with metastasis and commonly present as ipsilateral pleural involvement along with the primary tumourof the mediastinum. Early-stage recognition and treatment with surgical resection and postoperative chemoradiotherapy may offer a better10-year survival rate. Thymomas areslow-growing neoplasms that may show aggressive characteristicsinvolving invasion of adjacent mediastinal structures and metastasisto the pleura and pericardium.[3]Thymoma is a malignant neoplasm that arises from the epithelialcells of the thymus gland, a lymphoid organ situated in the anteriormediastinum. It is one of the most commonly reported primarytumours of the anterior mediastinum.A 40-year-old female patient was referred to our service from adistrict hospital. She complained of left-sided infraclavicular painfor 4 months. A chest radiograph revealed a homogeneous opacityin the left upper zone, with multiple pleural-based mass lesionsA subsequent computed tomography (CT) scan revealed alarge lobulated soft-tissue anterior mediastinal mass (measuring 60Hounsfield units (HU)), with multiple pleural nodular masses A soft-tissue mass of 3 cm in diameter was also seen in the laterallimb of the left adrenal gland. The patient’s previous images capturedon the local healthcare system’s radiology archive were reviewed andit was noted that a chest radiograph performed 4 years earlier alsoshowed the mass lesion in the anterior mediastinum [4]An ultrasound-guided Tru-cut needle biopsy of the mass lesion wasperformed. Histopathological examination revealed epithelioid cellsgrowing in sheets, composed of bland oval cells with oval nuclei thatwere euchromatic without nucleoli. On further immunohistochemicalstaining, the cytokeratin marker MNF116 was positive in epithelioidcells and CD3 and terminal deoxynucleotidyl transferase were positivein the lymphoid component, which confirmed the diagnosis of a typeB2 thymoma. She was thus staged as IVB thymoma B2 based on theMasaoka-Koga staging system.[5] The incidence of thymoma increases with age and peaks in the fifth decade of life, with menand women equally affected. Approximately 50% of nearly all typesare detected incidentally in asymptomatic patients on chest imagingexamination.[6] When symptoms are present, they are usually due tolocal effects of the malignancy, including compression and invasionof adjacent structures, which can result in dysphagia, diaphragmaticparalysis or superior vena cava syndrome. It has been reported thatnearly a third of patients report chest pain, dyspnoea or cough.Patients may also present with paraneoplastic syndromes owing tothe frequent association of immune-mediated systemic diseases, ofwhich myasthenia gravis is the most common (seen in 30% - 50%of patients with thymoma). Hypogammaglobulinaemia and purered cell aplasia are the other significant paraneoplastic syndromesassociated with thymoma.[7]Thymic epithelial neoplasms are rare malignant tumours that arisefrom the thymic gland. These neoplasms include thymoma, thymiccarcinoma and neuroendocrine tumours of thymic origin, ofwhich thymoma is the most common.[8]Thymomas are composed of neoplastic epithelial cells andlymphocytes that are non-neoplastic in nature and exhibit markedvariations in histology. According to the recent histologicalclassification system by the World Health Organization consensuscommittee, thymoma is classified into five subtypes: A, AB, B1, B2and B3. The committee also noted that several subtypes can coexist inthe same tumour, which poses a challenge to classification; however,this has no clinical implication.The clinical course and decisions are based on the stage of the disease.Staging of thymoma has conventionally been based on either the Masaokaor the Masaoka-Koga staging systems, with the latter recently beingrecommended by the International Thymic Malignancy Interest Group.[6]A CT scan is the modality of choice for the evaluation of thymoma.It usually presents as an anterior mediastinal mass of 1 - 10 cm, roundin shape and with well-defined margins and occasional calcification.Nearly a third of all thymomas are potentially invasive, infiltratingthrough the capsule into the nearby structures. Advanced-stagethymomas exhibit pleural spread or ‘drop’ metastases, which showup as one or multiple pleural nodules and are usually ipsilateral to theprimary tumour.[9] After primary treatment of resectablethymomas, surveillance for recurrence should include six-monthly chestCT scans for 2 years and then annually for 10 years. Given the currenttreatment recommendation, we administered multiagent inductionchemotherapy for our patient  to relieve tumour-related symptoms.The 10-year overall survival rates for stage I, II, III, IVA and IVBthymomas are 84%, 83%, 70% ,42% and 53%, respectively. The treatmentstrategy is based on tumour resectability; completeness of resection isthe most important predictor of outcome. Postoperative radiotherapyis recommended only for incompletely resected thymomas, whichshould be administered by the three-dimensional conformal techniqueto reduce damage to surrounding normal tissue and coupled with theuse of intensity-modulated radiotherapy. Induction chemotherapyis recommended for locally advanced thymomas, followed by anevaluation for surgery; postoperative radiotherapy can be consideredafter resection of the primary tumour. In those with solitary metastasisor ipsilateral pleural metastases, treatment options include inductionchemotherapy or surgery. In the case of unresectable disease,radiotherapy with or without chemotherapy is recommended. Althoughsix different combination regimens are available in the NationalComprehensive Cancer Network algorithm of 2018, a combinationof cisplatin, doxorubicin and cyclophosphamide is regarded as theregimen of choice for thymoma.


Infantile digital fibromatosis (IDF), also called inclusion body fibromatosis is an uncommon benign tumour occurring in the digits of young children. In about a third of cases, it is congenital and the diagnosis is based on the presence of peculiar intracytoplasmic inclusions on histology. Recurrence rate post-surgery is high. However, spontaneous regression has been reported. We present a case of a 5-month-old infant who had excision of a right second toe mass, which has been present from birth. Histological examination revealed this to be infantile digital fibromatosis. To the best of our knowledge, no report of this has been made in Nigeria. It is important that this diagnosis be entertained in young children with masses on the digits as this will influence the management instituted. Infantile digital fibromatosis is an uncommon fibroblastic and myofibroblastic tumour occurring in the digits of young children.The patient is a 5-month-old female infant who presented with a painless round swelling on the second toe of the right foot, which had been present from birth. The swelling was located on the dorsum of the toe proximal to the nail and gradually increased in size to obliterate the nail bed. The parents brought the child to our facility on account of increasing size of the mass. Physical examination revealed a pedunculated, non-tender soft tissue mass, measuring 4.5 x 3 cm, with the base located on the middle phalanx of the right second toe, spreading over the nail bed . RadioloThe mass was excised with primary closure of the wound. There was initial primary hemorrhage which was controlled with pressure dressing and the child was subsequently discharged from the hospital. The wound healed satisfactorily without any adverse sequelae.At the histopathology laboratory, we received a piece of cystic tissue measuring 2x2x1 cm. Sectioning, revealed greyish white solid tissue and an empty cystic cavity. Microscopic examination revealed skin tissue with intact epidermis, proliferating fibroblasts arranged in bundles, forming a poorly defined nodule within the dermis on Haematoxylin and Eosin (H&E) stain. Also seen were characteristic perinuclear eosinophilic inclusions on high power .Masson's trichrome (MT) stained the intracytoplasmic inclusions red . The hisInfantile digital fibromatosis is also called Reye's Tumour and inclusion body fibromatosis. It is a benign tumour first extensively described by Reye as recurring digital fibrous tumour in 1965.7The tumour may be single or multiple, firm or gelatinous in consistency7About a third of cases are congenital as occurred in this patient, while most will present within the first year of life. A few cases have been described in adults. There is no sex predilection3Radiographs show a non-specific soft tissue mass, the underlying bone may be involved with either erosion or invasion but only rarely13Histological features are unique, interdigitating fascicles of spindle cells and collagen fibres make up the tumour which is located in the dermis and may extend into the subcutaneous tissue.15Due to the benign nature of the tumour, recent studies recommend a conservative management with regular monitoring.7Complications are rare and include deformity, functional impairment and ulceration7. Spontaneous regression without scarring occurs, although the exact rate is not known.21Congenital Infantile digital fibromatosis is a rare benign tumour occurring predominantly in infants. Preoperative diagnosis is uncommon because it is often not considered in the differential diagnosis of nodular digital tumours in children. It is important for surgeons to consider it as a diagnosis as the current management of IDF is non-surgical. However, in this environment, surgical management is advised because patients often end up with untrained caregivers and quacks that will worsen the condition when left untreated.


Gastrointestinal stromal tumors (GISTs) quite frequently occur synchronously with other malignancies, with most cases being adenocarcinomas. GISTs and liposarcomas are both of mesenchymal origin, and their coexistence is extremely rare.  We conducted a review of the current literature regarding the synchronous occurrence of GISTs and intra-abdominal liposarcomas. An electronic search of the literature was undertaken using MEDLINE (database provider PubMed). Furthermore, we present the first described case of an 86-year-old male with a GIST and synchronous liposarcoma, both located in the stomach, as well as a 66-year-old male with a gastric GIST and concurrent retroperitoneal liposarcoma.  A total of 5 cases of synchronous GIST and intra-abdominal liposarcoma have been reported in the literature to date, with the most recent cases included in the present study.  Further research is required to explain any possible correlation in the coexistence of these different neoplasms of the same origin. Meanwhile, R0 resection of both tumors remains the treatment of choice. Although rare and accounting for only 0.1–1% of all malignancies of the gastrointestinal (GI) tract, gastrointestinal stromal tumors (GISTs) are the most common nonepithelial neoplasms of the GI tract . They arConversely, liposarcomas are one of the most common soft-tissue sarcomas, usually affecting the limbs, retroperitoneum, and trunk . GastricHerein, we present, for the first time in the literature to date, a patient with a GIST and synchronous liposarcoma both located in the stomach and a second case of a patient with a gastric GIST and a synchronous retrocecal liposarcoma. Furthermore, a review of the literature was conducted aiming to identify all cases of concurrent GIST and liposarcoma which have been reported to date.An 86-year-old male patient presented to the emergency department with a two-week history of fatigue and tarry stools. There were no significant findings based on physical examination. His past medical history included diabetes mellitus, hypertension, and chronic usage of acetylsalicylic acid. From routine laboratory exams, the patient's hematocrit was extremely low (HCT = 19%), while all the other parameters were within normal limits. An emergency upper gastrointestinal endoscopy was performed and revealed a submucous tumor in the gastric antrum, without present bleeding. The patient received a blood transfusion and intravenous iron therapy and remained hemodynamically stable. A computed tomography (CT) examination was performed which revealed a hypodense and well-defined soft-tissue mass (5.5 × 3.5 cm) in the antrum of the stomach . No hepath postoperative day.Two tumors were identified intraoperatively. The larger tumor was detected in the antrum and the smaller tumor in the gastric body. No distant metastasis or nodal involvement was noted. Distal gastrectomy, Billroth II reconstruction, and Braun enteroenterostomy were performed. The patient had an uneventful recovery and was discharged on the 9d = 11 mm, R0 resection) was a low-risk GIST (1-2 mitoses/50 hpf). Immunohistochemistry displayed strong staining for c-Kit, CD34, and DOG1. The second tumor in the antrum  was a well-differentiated liposarcoma  with a diameter of 4 cm. The risk of progressive disease was low (4–6/50 hpf), and immunohistochemistry displayed strong staining for c-Kit, CD34, and S-100 protein. Histopathological findings for the retrocecal tumor revealed a 5.9 cm well-differentiated sclerosing-type liposarcoma with immunohistochemistry S-100 protein and vimentin positive. Cancer cells were present microscopically at the resection margin (R1 resection) of the liposarcoma. Thus, adjuvant chemotherapy  was introduced.At the 6-month follow-up, the patient remained disease-free; however, unfortunately, 13 months after surgery, the patient suffered a severe myocardial infarction and died 2 days later in the intensive care unit.The main clinical and pathological features of all 5 patients with two synchronous mesenchymal tumors are summarized in Although gastrointestinal stromal tumors (GISTs) are rare neoplasms, they represent the most common mesenchymal tumors of the gastrointestinal tract. GISTs originate from the cells of Cajal, which are responsible for autonomous gastrointestinal (GI) movement , 14, 15.Liposarcoma is the most common soft-tissue sarcoma, accounting for 15%–20% of all mesenchymal malignancies. Based on morphological and histopathological features, they are classified into 4 types: (1) well differentiated; (2) dedifferentiated; (3) myxoid/round cell; and (4) pleomorphic . Well-diGISTs commonly coexist with other primary tumors that can involve either the GI tract or other extra-GI sites. According to a recently published review, the synchronous occurrence of GISTs with other malignancies varies from 8% to 38%, while the most frequent localization of GIST-associated malignancies is in the gastrointestinal tract. Interestingly, GISTs of small size and low risk of malignant transformation are usually found in patients with other malignancies, supporting the theory that the GIST itself in these patients has little effect on the overall survival and patients' prognosis . It is nAs yet, the clinical importance of this concurrence has not been clarified. In a recent case series study of survival analysis comparing the overall survival, no statistically significant difference was found between patients with a single or a synchronous GIST. However, patients with synchronous GISTs and other primary tumors had a statistically significant increased possibility for relapse. In addition, they proved that patients with a single GIST have larger tumors than patients with synchronous tumors, and the difference in the mean age of appearance was not statistically significant in the two groups .Both GISTs and liposarcomas are of mesenchymal origin, and their coincidence requires specific expertise related to diagnostic and therapeutic management . PossiblWe identified only three previously reported cases in the literature. The first case reported by Diamantis et al. was a 46-year-old patient with synchronous gastric GIST and retroperitoneal liposarcoma, treated with excision and adjuvant chemoradiotherapy due to microscopically positive margins in the liposarcoma specimen . The secThe synchronous occurrence of GIST and liposarcoma is very rare, with only three cases described in the literature worldwide. Here, we report two additional cases, one of which is the first case of synchronous GIST and liposarcoma both located in the stomach. Although coincidence may be the answer, the hypothesis of gene mutations or a carcinogenic agent resulting in two tumors of the same origin cannot be excluded. Nevertheless, further research is required to explain the possible causation of the synchronous occurrence of two mesenchymal tumors and define the best treatment strategy. Until then, the treatment of choice is radical excision of both tumors.


Oesophagostomum in the intestinal walls of many species, including ruminants, pigs, humans, and nonhuman primates. Although great apes appear to tolerate the parasite in the wild, they can develop a clinical form that can lead to death in captivity and the natural environment. At the Primatology Centre of the International Centre for Medical Research in Franceville (CIRMF) in Gabon, we recorded 4 deaths of chimpanzees (Pan t. troglodytes) caused by Oesophagostomum spp. between 2015 and 2019. In each case, coprological analysis was positive for strongylid eggs and abdominal ultrasound revealed nodules about 4 cm in diameter on the intestinal and abdominal walls. Albendazole treatments administered by mouth in two doses of 400 mg six months apart resulted in the disappearance of the parasite in coprological samples but the chimpanzees still died. Autopsies carried out on all four chimpanzees revealed a rupture of the cysts and a discharge of pus into the abdomen in each case. We report surgical management involving the removal of Oesophagostomum spp. cysts from a chimpanzee following coprological analysis and abdominal ultrasound examination. Surgical exploration confirmed the fragility of the cystic walls, the rupture of which we avoided. This 5th new case of Oesophagostomum ssp. nodules recovered without complications following the operation and could rejoin his group. We suggest that surgical intervention should be considered in similar cases in captive primates, especially chimpanzees.Oesophagostomosis is a zoonotic disease caused by nematodes of the genus  Oesophagostomum genus, transmitted by ingestion of L3 larvae found in soiled food or earth. In the infected host, the ingested L3 larvae develop into adult worms whose fertilized females lay eggs that are eliminated in the feces. Sometimes the L3 larvae form nodules in the intestinal wall. The nodule protects the parasite from medical treatment and is the cause of clinical symptoms [Oesophagostomum species  are common to primates, including humans [Oesophagostomum spp. in humans are characterized by symptoms of weight loss, abdominal pain, and diarrhea [Oesophagostomosis is a disease caused by parasitic nematodes belonging to the symptoms . This pasymptoms –3. Severg humans –6. Infecdiarrhea –9 and spdiarrhea , 10. Mandiarrhea , 12, infdiarrhea , 13. Thidiarrhea , 14.Oesophagostomum spp. caused 4 deaths of chimpanzees (Pan t. troglodytes) between 2015 and 2019 despite the systematic use of recommended medical treatments and protocols. Here, we report surgical management involving the removal of Oesophagostomum spp. cysts from a chimpanzee following coprological analysis and abdominal ultrasound examination.Of various medical protocols, albendazole appears to be the treatment of choice for oesophagostomosis , 10, 15.Oesophagostomum spp. nodular abscessation, the chimpanzee was anaesthetised, blood samples were taken from the femoral vein to test various hematological and biochemical parameters, and surgery was performed.Cabinda, a 19-year-old male chimpanzee, was captive-born and weighed 51.3 kg before his illness. He lived at the Primatology Centre of the International Centre for Medical Research in Franceville (CIRMF) in southeast Gabon in a multimale and multifemale group of 21 individuals. In September 2019, Cabinda started to lose weight with episodes of diarrhea and loss of appetite. He was isolated to collect feces. Coprological analyses using sedimentation and flotation methods  revealedμg/kg  and ketamine hydrochloride 1 g at 1.0 mg/kg . A dose of 1.1 ml of Domitor® and 4 ml of ketamine 1 g ® was administered. Anesthesia was supplemented with Domitor® (0.14 μg/kg) and ketamine 1 g  to maintain an optimal anesthetic plane. Clinical examination consisted of measuring parameters including general condition, weight (43.1 kg), temperature (37.1°C), oscillation, palpation, and heart rate (94 bpm). Abdominal ultrasound scan revealed 3 large nodules of 4.2 cm x 2.1 cm on mesenteric tissue  using a combination of medetomidine hydrochloride 1 mg at 27.4 c tissue , 4.1 cm c tissue , and 3.7c tissue . Some noc tissue .After the preparation phase, Cabinda was rehydrated by infusion with alternating solutions of Ringers lactate® and Glucose® 5% (250 ml/h over 2 hours) administered using an 18G catheter. The surgical procedure consisted of removing the nodules after a midline abdominal incision from the pubis to the lower edge of the sternum. Also known as the laparotomy incision, this method allows access to the abdominal organs. The three main nodules were removed due to their large size. The first nodule, located on the mesenteric tissue, was removed by dissection of the tissue and blood vessels ligated with resorbable polyglactin 910 no. 2 thread . The second nodule in the wall of the descending colon was removed by incision around the cyst. The wall of the colon was sutured with resorbable polyglactin 910 no. 2 suture  to avoid adhesions during healing. It was checked that the contents of the colon did not fall into the abdomen. The third nodule was removed from the abdominal wall by dissection.Nodule walls were necrotic and fragile  and the μg/kg body mass. Relay therapy was administered 48 hours later with amoxicillin/clavulinic acid (Augmentin®) 40 mg/5 mg/kg daily in three doses for one week and ibuprofen 400 mg (Advil®) in two doses for three days. Cabinda recovered well and no complications were noted. After two days of liquid food, he resumed normal eating and presented a good general condition. At a check-up, two months later, he weighed 53.20 kg.Cabinda's postoperative management included antibiotics  at 15 mg/kg body mass and an analgesic  at 15 Oesphagostomum spp. infections between in wild and captive animals.Oesophagostomosis is a disease described in several primate species including humans , 17. In Oesophagostumum infections [Mandrillus sphinx (N = 26), Cercopithecus cephus (N = 4), Cercopithecus nictitans (N = 6), Macaca sp. (N = 13), and chimpanzees (N = 19)), we have recorded 4 cases of death in 6 chimpanzees with large Oesophagostumum spp. nodules detected using ultrasound when coprological analyses carried out after treatment were negative for strongylid eggs. In contrast, 13 chimpanzees presenting pea-sized cysts regained their health following treatment. No other infected species developed large cysts. These observations confirm (i) the sensitivity of chimpanzees to Oesophagostumum spp. infection and show that albendazole is effective on nonencysted parasites and small cysts but ineffective on parasites protected in large nodules. Medical treatment alone would therefore not be sufficient when the nodules are large. (ii) At an advanced stage of development, it is possible for the parasite to die in the nodule but the walls of the nodules are so fragile that they rupture even after the death of the parasite. This hypothesis is supported by the observations that the adult worms we found in Cabinda's cysts were dead , we havThus, in the case of Cabinda, although the coprological analyses were negative after treatment and hematology showed only mild changes, there was a risk of nodule abscessation and rupture; therefore, surgical treatment was elected. The combination of medical and surgical treatment was successful in achieving full clinical resolution. Treatment by surgery was motivated by the fact that, in humans, the treatment of oesophagostomosis is also surgical in cases of Dapaong tumors . Thus, uOesophagostumum spp. infections are common at the CIRMF primatology center and 5 cases of nodular pathology, including 4 deaths, have been registered since 2015. A preventive schedule consisting of systematic treatment with albendazole 400 mg  every 6 months [Oesophastomum spp. in the centre and above all prevent nodular pathology.6 months  has beenOesophagostumum nodules should be performed in addition to medical treatment when the nodules are large. Furthermore, because of the zoonotic nature of the infection and the close phylogenetic relationship between humans and great apes that result in opportunities for parasite exchange [This experience leads us to recommend that for captive primates and chimpanzees, in particular, surgical extraction of exchange , an ultr


Craniopharyngioma is a rare intracranial tumor, with a high morbidity rate due to its common refractiveness to conventional treatments. BRAF V600E mutation has recently been identified as the principal oncogenic molecular driver of papillary craniopharyngiomas (PCP), one of the two main variants of craniopharyngioma.A 49-year-old man with recurrent craniopharyngioma, harboring BRAF V600E mutation, has been treated with targeted therapy based on a combination of a BRAF-inhibitor, dabrafenib , and a MEK-inhibitor, trametinib . Before starting treatment, the patient was symptomatic: he lamented confusion, dysphasia, and intense fatigue, that did not allow him to work normally. After just one cycle of treatment, the patient showed an important clinical improvement, reporting a progressive regression of the basal symptoms, hinting at a rapid and dramatic response, which was confirmed at the first radiological assessment. Thus, treatment was continued and at the time of writing, the treatment is still ongoing  and it is well tolerated, with very good quality of life: the patient has no limitations in daily activities and he has even been able to restart to work.The use of targeted therapies—as a clinical practice or in clinical trials—represents an important therapeutic alternative and a great evolution for patients' prognosis vs. the standard of care, historically represented by unselected chemotherapies. The discovery of the BRAF V600E mutation in patients with PCP is very rare, resulting in a lack of data on the efficacy of the combination of dabrafenib and trametinib. Craniopharyngiomas (CPs) are rare primary brain tumors, that are originated from the embryonic remnants of Rathke's pouch (an invagination at the roof of the developing mouth that evolves into the anterior pituitary gland). CPs can be located either in the sella turcica (intra-sellar CPs) or above it (supra-sellar CPs). They show high levels of morbidity, as they arise in the proximity of critical brain structures and can often compress or infiltrate vital neurological areas . Visual defects, endocrine deficiencies, namely, panhipopituitarism, cognitive deficits, memory loss, headache, morbid obesity, hyperphagia, and personality changes are common complications caused either by tumor growth and the consequences of treatment with surgery, radiation, or both. Careful analysis of tumor extension and symptoms vs. long term and often irreversible adverse effects is important when considering therapeutic strategies , 2.There are two histopathologic variants of craniopharyngioma: adamantinomatus craniopharyngioma (APC) that occurs in both children and adults  and papillary craniopharyngioma (PCP) that occurs almost exclusively in adults, typically between the age of 40 and 55 .Both histopathologic variants have similar clinical presentation and response to standard treatment, represented by surgery and/or radiotherapy, but there is a high tendency to relapse and worsening of the quality of life. Intracystic chemotherapy  is used in the monocystic subtype of APC.CPs are characterized by high morbidity due to both hypothalamic and optic chiasm extension, with consequent endocrinological and visual disorders that can usually represent late primary clinical manifestations. CPs presenting as incidental findings are rare ; the diagnosis of childhood CP cases is often late, with a clinical picture characterized by unspecific symptoms connected to increased intracranial pressure, such as nausea and headache .About 1- and 3-year survival rates were found to be better in juvenile patients with small tumors when undergoing subtotal resection and radiotherapy. Conversely, 1- and 3-year survival was worse in the black race , papillary thyroid cancer, hairy cell leukemia, colon cancer in combination with Cetuximab. In this study, we present a real-world case report of the efficacy of targeted therapy in PCPs with BRAF mutation.In October 2006, a 46-year-old man received brain MRI, advised by a family doctor to investigate the persistency of memory loss and headaches unresponsive to pain killers medications. MRI evidenced a previously unknown infra- and supra-sellar mass of 3.5 × 2.6 cm, with high pressure on the third ventricle and on the interpenducular cistern; with MRI contrast, peripheral highlighting and an 8 mm enhanced solid nodule were found; the imaging was consistent with a diagnosis of cystic craniopharyngioma .Subsequently, the patient underwent fronto-temporal trans-cranial excision of the lesion and histological examination confirmed the diagnosis of PCP. In January 2007, the patient was referred by a multidisciplinary committee for discussion of adjuvant radiotherapy (RT), which was confirmed and then administered . Follow-up visits with clinical and radiological evaluations were periodically performed until 2013, every 3 months when the patient decided to discontinue medical examinations.In November 2019, for the onset of vertigo and episodes of lipothymia, the patient accessed the emergency department where he performed computed tomography (CT), which showed an iso-dense infra and supra-sellar solid mass of 19 × 23 mm.Additionally, a brain MRI was also performed, which confirmed the presence of a 25 × 38 × 23 mm lesion that extended to the chiasmatic and inter-peduncular cisterns and the flooring of the third ventricle; mass effect had displaced the pituitary gland to the right and had caused compression of the pons .via endonasal endoscopic approach and the histological examination confirmed the recurrence of craniopharyngioma. The postoperative course was complicated by polyuria, mental confusion, dysphasia, ptosis, mydriasis, and ophthalmoplegia, and partially improved by palliative treatment with prednisolone and mannitol. Late surgery-related complications were anosmia and psychomotor impairment. At this stage, considering the young age of the patient and the clinical aggressiveness of the tumor, not controlled by curative treatments with surgery and RT, molecular analysis on the surgical sample was carried out: real-time polymerase chain reaction (PCR) for BRAF gene mutation and then next-generation sequencing analysis for a panel of >300 genes was carried out and the canonical mutation V600E in the exon 15 of BRAF gene was identified as the only relevant mutation . As previously anticipated, the BRAF mutation had been recognized as a characteristic mutation of PCP, being identified in most, but not all, PCP and never in adamantinomatous craniopharyngioma (ACP). Post-operative brain MRI performed in February 2020 revealed residual tissue with dimensions of 19 × 22 × 19 mm, and after contrast, an inhomogeneous increase in this lesion occurred  in craniopharyngiomas in various stages, since the discovery that PCPs often harbor BRAF gene mutation .The patient started combination treatment with double inhibition of BRAF and MEK with Dabrafenib 300 mg/day and Trametinib 2 mg/day .The patient was consequently evaluated monthly clinically and with a biochemical profile. The treatment was initially well tolerated with no relevant adverse reactions.At 4 months, laboratory results showed a pathological increase intriglyceride and Cholesterol levels, graded 3 on the CTCAE scale, rarely described during treatment with BRAF/MEK inhibitors. Anti-BRAF and anti-MEK were then suspended temporary and the patient was referred to an endocrinologist: a new medical treatment was established with Fenofibrate and Omega 3 with following the reduction in laboratory levels: oncological treatment was then resumed with dose reduction in accordance to European Medical Agency (EMA) recommendation  when levels reached grade 1, according to CTCAE scale. This was the only adverse event reported by the patient and it did not influence his quality of life. At the time of writing, treatment is ongoing .First radiological evaluation with MRI after 4 months of treatment showed not only a significant volume reduction of the tumor (14 × 9 × 5 mm vs. 19 × 22 × 19 mm) but also a reduction of the mass effect on the surrounding tissue, for example, realignment of chiasmatic structure . The lasCraniopharyngiomas are locally aggressive supra-sellar tumors that often reach a large size before a symptomatic presentation. They compress and infiltrate critical structures, as optic nerves, hypothalamus, and pituitary gland, causing a profound neurological deficit. Standard treatment, namely, surgical resection and radiotherapy may achieve local tumor control, but, unfortunately, poor quality of life often follows these aggressive local treatments due to permanent neurological and endocrine deficits. Tumor control rates after radiotherapy with limited surgery are similar to gross total resection or incomplete resection with radiotherapy, with a >90% overall survival at 10 years. These data do not properly meet with functional results as important brain structures as hypothalamic–pituitary axis, optic nerves, chiasm, and cerebral vascular axis are comprised in the radiotherapy field and can be damaged during the surgery. No standard chemotherapy exists, and when the tumor returns after surgery and radiation, there are no successful therapies to use  is an essential signaling pathway in several malignancies: alterations in various components of the MAPK pathway, especially in the BRAF gene, have been in fact described in many solid tumors.BRAF is part of the Rapidly Accelerated Fibrosarcoma (RAF) family (cellular RAF (CRAF), BRAF, and ARAF) of serine/threonine protein kinases and appears as the second tier of the MAP kinase pathway (rat sarcoma (RAS)-RAF- MAPK/ERK kinase (MEK)- extracellular signal-regulated kinases (ERK) cascade), involved in responses to growth signals . BRAF itTargeting MEK is an attractive therapeutic strategy also in non-selected tumors for combination with immunotherapy , thus suActivating mutations of BRAF lead to consecutive downstream activation of the RAS–MEK–MAPK signaling cascade, promoting cell proliferation, and survival while inhibiting apoptosis, and driving tumor growth .There are fundamentally three classes of BRAF mutation :- Class I mutations are independent of both upstream RAS activation and the need for dimerization .- Class II mutations include several point mutations and fusions that activate MEK through RAS independent dimerization .- Class III mutations are kinase impaired and enhance MAPK signaling through RAS and subsequent CRAF activation .Among these, the V600E mutation, that is, valine substituted by glutamic acid at amino acid 600, a class I mutation, is the most frequent in solid tumors, but other uncommon mutations have been discovered , 20.Following its discovery, several therapeutic approaches targeting BRAF have been studied, first in melanoma –25 and tIn NSCLC, BRAF mutation is rare and associated with the lack of chemo-sensitivity and a worse prognosis in a patient treated with platinum. The European Society of Medical Oncology (ESMO) and the National Comprehensive Cancer Network (NCCN) guidelines recommend the use of BRAF/MEK inhibitors in first or subsequent lines therapy for NSCLC that harbors a V600E mutation, based on phase 2 trials .Similarly, BRAF mutant CRC related respond poorly to standard chemotherapy and show poor prognosis, that may benefit from targeted therapy in combination with Cetuximab, as suggested from recent trials , 28.BRAF mutations, particularly the V600E mutation and KIAA1549-BRAF fusions, are also present in a significant subset of primary brain tumors . PositivAt the last ASCO congress 2021, positive data from a phase II trial of the combination of BRAF and MEK inhibitors (Vemurafenib/cobimetinib) in PCP  have been presented: the objective response was obtained in all but one patient who received at least one cycle of therapy (15/16 patients); median tumor reduction was around 83%. Median PFS was not reached and grade 3 toxicity, which was occurred in 12 patients, was acceptable according to the previous data in other cancer types (6).These preliminary data further support the potentiality of this type of treatment and the clinical case presented is to be considered as a real-world case for this clinical setting.Thus, in conclusion, craniophayngioma is considered a tumor with high mortality and frequent recurrence, and its treatments are associated with several complications. Recent evaluations have also shown that craniopharyngiomas have a low frequency of somatic mutations other than BRAF V600E mutation and are not genomically complex. Nevertheless, further experience is needed to define frequency, durability, and extent of BRAF treatment response in PCP .In this present case report, the presence of BRAF mutation was investigated at the time of recurrence, which occurred some years later after the first diagnosis, while the patient was developing significant clinical complications, due to both the locoregional pressure from the relapsing growing mass and after surgical and RT treatments, thus representing a peculiar case of “targeted salvage therapy.”We believe that the presence of V600E mutation should be investigated as earlier as possible in CP patients, thus giving patients the opportunity to benefit from BRAF and MEK inhibitors treatment. Also, we believe that testing in the neoadjuvant setting of this targeted therapy could be a promising option: shrinkage before definitive treatment with surgery or/and radiation could improve the safety and efficacy of these treatments, potentially reducing the disabling morbidities that often follow current strategies.We hope that the next year's neo-adjuvant treatment approach may be considered in selected craniopharyngioma patients who underwent biopsy and BRAF mutational status analysis and that results from molecular studies could be available from a greater number of patients to extend knowledge of this rare tumor type.MF and CD wrote the manuscript and supervised the data collection. MC, MP, RR, TT, MF, GM, and SN collected clinical and radiological data and bibliography. FC supervised the full article processing. All authors contributed to the article and approved the submitted version.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.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Appendix’ anatomical variations are a rare occurrence which can mislead diagnosis and delay appropriate treatment.We present a 9-year-old female patient that came with a clinical picture compatible with acute appendicitis. However, a cecal mass was identified instead of an inflamed appendix during surgery. Therapeutic decisions were extremely challenging due to clinical deterioration and an uncertain etiology. Only the histopathology report revealed the presence of a complete subserosal appendix which was responsible for the entire symptomatology. Here, we review all case reports regarding intramural, intracecal or subserosal appendixes. A discussion of the general approach to this specific case and the importance of consensual diagnostic criteria for these specimens are also presented. At last, an incidental finding is exposed and final treatment options are discussed given the overall presentation.Considering these variants would guide physicians towards a more accurate approach to similar clinical pictures and hence an improved long-term prognosis. Acute appendicitis is one of the most common surgical emergencies worldwide . It is aAmong gastrointestinal neoplasms, appendiceal tumors are rare, presenting in only 1 percent of appendectomies’ specimens , 3, 5, 9The vermiform appendix is usually found on the right inferior abdominal quadrant, emerging from the posteromedial region of the cecum, and settling inferior to the ileocecal union. However, its location varies widely among surgical specimens. The orientation of the tip of the appendix defines its anatomic position , 16 eithOut of these uncommon anatomic variations, intracecal and intramural appendix are even rarer. To the best of our knowledge, there are only three cases reported worldwide , 18, 19.A 9-year-old female patient arrived at the emergency room because of a 36-h history of intermittent right lower abdominal pain, anorexia, vomit, and quantified high-grade fever. She had no pathological personal or family history of interest. On examination, the right iliac fossa was tender to palpation and no frank peritoneal signs were observed. Initial laboratory evaluation showed leukocytosis, neutrophilia, and an elevated C-reactive protein. Ultrasonography of the abdomen was inconclusive. A heterogeneous lesion of 40 × 37 mm within the colon, no appendix and some swollen mesenteric nodes of at least 10 mm were reported. A complementary abdominal CT scan revealed findings suggestive of ileocolic intussusception with an invagination area of approximately 6.6 × 4.9 cm. After surgical consult, the patient underwent an exploratory laparoscopy that required laparotomy conversion. A well-defined, 5 cm mass at ileo cecal valve and multiple hard pericecal lymph nodes were observed. Preserved permeability between the ileum and colon, complete integrity of the cecum wall and lack of vermiform appendix were also reported. The possibility of an auto-digested appendix and a cecal tumor were discussed. At this time, surgeons decided to resect retrocecal and pericecal lymph nodes and send these samples to pathology before any further intervention. The patient was admitted to the inpatient floor where antibiotic therapy based on ampicillin sulbactam, and metronidazole was initiated. The oncologist department was consulted and complementary laboratory exams including liver and renal function tests, uric acid, electrolytes, lactic dehydrogenase, and quantiferon-TB tests were ordered. Only lactic dehydrogenase was altered. A chest x-ray ruled out mediastinal masses. No alarming findings were reported. However, the patient presented gastric distension, abdominal pain and fever by the second hospitalization day. The content inside the suprapubic JP drain changed from a serohematic aspect to a dense cloudy fluid. A culture and cytochemical analysis of peritoneal fluid was performed without significant results. CBC showed mild leukocytosis and neutrophilia. Reactive C-protein remained elevated. Two blood cultures and an urinalysis were negative. Due to the uncertainty of the etiology of her clinical picture, infectology decided to change antibiotic therapy to piperacillin/tazobactam and amikacin. An abdominal x-ray showed air fluid levels in the small bowel and a colonic distention projected at mesogastrium. Gastroenterology suggested initiating bowel rest and placing a central line for parenteral nutrition.After five more days, elevated inflammatory markers, abdominal distension and pain, and the unusual JP drain aspect persisted. A new ultrasound confirmed that the mass and surrounding area had the same aspect as days before. The histopathological description of paracecal-retrocecal lymph nodes and the sample of mesenteric omentum obtained during the first intervention failed to detect neoplastic cells. Macroscopically, three encapsulated lymph nodes from 0.8 to 2 cm were received. Their physiological architecture was preserved; secondary lymphoid follicles with hyperplastic germinal centers containing macrophages with cellular debris were reported. The interfollicular population was polymorphic and contained frequent large cells with prominent immunoblast-like nucleoli. Other areas showed sinusoidal histiocytosis with eosinophils and neutrophils. There was fibrosis with a predominantly neutrophilic mixed inflammatory infiltrate that spread to neighboring adipose tissue in the periphery of the nodes. The immunohistochemical study confirmed the presence of follicular dendritic cells and B lymphocytes in the germinal centers (CD23 + + +/+ + + and CD20 + + +/+ + + respectively), T lymphocytes in the mantle zone (CD3 + + +/+ ++), macrophages in germinal centers and sinusoidal area (CD68 +/+ ++). Frequent CD30 + + +/+ + + immunoblasts and actin + + +/+ + + myofibroblasts within areas of fibrosis were also observed. EBV study using EBER in situ hybridization was negative. Ziehl Neelsen and PAS did not show any pathogen. The 22 × 0.6 cm omentum sample showed fibrous thickening of the septa and the presence of a mainly lymphocytic infiltrate. Fibrino-leukocytic material was also seen in the serosa. Pathologists concluded the possibility of an unspecified acute versus chronic epiploitis, lymphadenitis and serositis. Nevertheless, due to her unfavorable clinical evolution and the elevated inflammatory markers, a second surgical intervention was decided. The patient underwent an omentectomy and resection of approximately 40 cm of terminal ileum, cecum and ascending colon. Pericolonic lymph nodes were resected as well. A sample of a collection observed near the cecum was taken for culture and cytochemical studies before aspiration and drainage. After surgery, the patient remained hemodynamically stable, without abdominal pain or distention. A nasogastric tube was placed and parenteral nutrition continued. The peritoneal fluid analysis was negative. Improvement in inflammatory markers lead to amikacin discontinuation. And by the fifth postoperative day, JP drain, and nasogastric tube were removed. Later, a regular diet was successfully initiated, and the patient was finally discharged.The histopathological final report described an 8 cm ileal segment, and a 14 cm ascending colon including the cecum with a diameter that ranged from 1 to 3 cm. The external surface was covered by a pinkish-gray serosa with fibrinopurulent material over the ileocecal area. A completely subserous dilated appendix was identified within the cecum wall. It contained a white-yellowish purulent material at the tip Fig. . The mucDuring our patient’s first surgical procedure, physicians found a 5 cm mass at the ileo-cecal valve instead of an inflamed appendix which was primarily expected by the clinical picture. The presence of the adjacent congested lymph nodes raised the suspicion of a neoplastic process. And given the lack of visible appendix, the probability of an inflamed appendix or any related complication to acute appendicitis were dismissed. The likelihood of the mass being responsible for her current symptomatology was superior. During the second intervention, they decided to resect the portion of the involved bowel because of her notorious clinical deterioration. After the procedure, the patient significantly improved giving the impression that the complicated neoplastic mass was a plausible explanation for her clinical picture. Nevertheless, the histopathological results revealed that, in fact, the mass in the cecum wall corresponded to a completely subserous inflamed appendix which was the origin of the patient’s worsening. Given the circumstances, taking into consideration the possibility of a neoplastic process and assuming the absence of the vermiform appendix was a reasonable approach. Appendix agenesia constitutes a rare anatomic variation that has been reported in 1 for every 100,000 laparotomies . It is aHere, we present a rare congenital anatomic variation that does not entirely meet the criteria exposed above: a complete subserosal appendix. The lack of a depression or pit in the cecum wall, which usually provides evidence of the original anatomic position, ruled out intussusception . The lacAt last, although the mass located at the ileocecal valve actually corresponded to an inflamed subserosal vermiform appendix, an asymptomatic neuroendocrine tumor was incidentally found during microscopic analysis. An analysis of 170 incidental appendectomies performed during living donor hepatectomies between 2005 and 2018 revealed normal histological characteristics in 80.6%, acute appendicitis in 2.9%, low-grade appendiceal mucinous neoplasm in 1.2%, and grade I neuroendocrine tumor in 0.6% of them . AkbulutNeuroendocrine neoplasms [NETs] correspond to up to 75 percent of appendix neoplasm . These aA review of the SEER and NCI database reported controversial results regarding the presence of lymph node metastasis and small tumors. It seems that there is a higher-than-expected probability of nodal involvement in tumors from 1.1 to 2 cm, raising the question of whether right hemicolectomy would be appropriate for this group . DespiteThe cecal appendix tends to present at a fairly constant location among patients. However, anatomical variations exist. And given the lack of enough evidence to accurately identify these presentations, unnecessary invasive interventions may occur. The correct management of cecal masses under hemodynamic instability is extremely challenging. Considering these variants would guide physicians towards a more accurate approach to similar clinical pictures and hence an improved long-term prognosis. Furthermore, consensus regarding intramural, intracecal and subserosal appendixes is required in order to correctly characterize these anatomic variations that seem to have common backgrounds and slightly differ in small aspects.


Ruptured hepatocellular carcinoma (HCC) can lead to peritoneal dissemination. However, gastric wall seeding from HCC is exceedingly rare, and little is known about its clinical course. Herein, we report a case of an 88-year-old man who presented with a four-hour history of nausea, vomiting, and upper abdominal pain. He has a history of ruptured HCC during surgery. The patient underwent an emergency laparotomy on account of haemorrhagic shock, which confirmed the diagnosis of ruptured HCC with gastric wall seeding. The findings from this study showed that the ruptured HCC can seed into the stomach wall, and the implanted lesions may rupture and lead to life-threatening haemorrhagic shock. Surgery is an effective treatment for bleeding from the implanted lesions. Although great progress has been made in the treatment of hepatocellular carcinoma (HCC) in recent years, recurrence rate is still very high even after curative therapy . ExtraheAn 88-year-old man with chronic kidney disease presented to our hospital with a four-hour history of nausea, vomiting, and upper abdominal pain. He exhibited no symptoms of hematemesis, melena, or anaemia. Fifteen months prior to presentation, he underwent surgical dissection with lateral hepatectomy due to HCC. The liver tumour ruptured during the surgical procedure. We washed the peritoneal cavity with 5 litres of normal saline to remove the tumour cells and reduce the risk of dissemination. Nine months earlier, abdominal computed tomography (CT) had revealed a nodule (10 mm) on the gastric antrum's serous surface. Three months later, the nodule size was not remarkably different, as confirmed by abdominal ultrasonography and CT. Due to the patient's advanced age and history of renal dysfunction, adjuvant chemotherapy was not administered.9/L), while other findings were unremarkable. Contrast-enhanced abdominal CT showed contrast leakage into the peritoneal cavity from a 30 mm mass on the gastric antrum's anterior wall , pulse rate (102 bpm), and temperature (34.9°C). He had notable tenderness in the epigastrium. Laboratory investigations showed elevated white cell count  [% [6]. InThe most common type of primary liver cancer is HCC. It usually develops in the setting of underlying chronic liver disease, especially in patients with cirrhosis due to chronic hepatitis B and/or C virus infection, excessive alcohol intake, and nonalcoholic fatty liver disease . DespiteIn many cases of postoperative recurrence after resection of HCC, tumour recurrence is seen in the remaining liver tissue . While eThe present case shows that gastric metastatic implantation can occur and the lesions can rupture. Gastric metastasis from HCC is rare , 10, andFor the treatment of extrahepatic metastases of HCC, effective molecular targeted drugs and surgery have been reported when the patient has a good Eastern Cooperative Oncology Group Performance Status and a class A Child–Pugh score . The treIn conclusion, we have reported a case of rupture of an HCC lesion seeded in the gastric wall causing haemorrhagic shock in a patient with a history of tumour rupture during HCC surgery. Emergency laparotomy saved the patient's life. HCC can cause peritoneal dissemination but rarely causes dissemination into the gastric wall. Clinicians should consider the possibility of rupture of implanted lesions. Surgery is an effective therapeutic option in cases with haemorrhagic shock due to rupture of the implanted lesions.


Diffuse neurofibroma is a benign tumor of peripheral nerves. Ten percent of neurofibromatosis type 1 (NF-1) patients can develop diffuse neurofibroma. Here, we report a case of diffuse neurofibroma involving the base of the skull in a 50-years-old patient with NF-1. The patient presented with diffuse involvement of the scalp with soft and mobile masses. Radiological investigations revealed skull bone lesions. Aggressive osteolytic lesions involving the base of the skull were present. Surgical excision with the repairing of the defects was suggested but the patient refused the treatment. The diagnosis of calvarial defects in diffuse neurofibroma is challenging. Early diagnosis can help in better management of the patients. Neurofibroma is a benign peripheral nerve sheath tumor with different types; localized, plexiform, and diffuse. Diffuse neurofibroma is a unique and uncommon class of neurofibroma . About 1Over 50% of patients with NF-1 have osseous defects, and among those, the skull bone defects are of rare occurrence. Sphenoid wing dysplasia and orbital defects are the most common calvarial anomalies in patients with NF-1 . So far,Here, we report a case of a 50-years-old female who presented in a medical emergency with complaints of altered level of consciousness from six hours and ataxia for three days. The patient was a known case of NF-1 for 35 years. The patient was unmarried and had no pregnancy. There was no history of any other disorder. On general physical examination, we found marked pallor and emaciation. Her scalp examination revealed various slow-growing, mobile, and soft masses present all over the scalp. There were no changes in the bone around the margins of the swollen mass. Diffuse café au lait spots were present all over the body. Relevant neurological and ophthalmological examination was normal. We did a computed tomography (CT) scan of the skull, which showed multiple neurofibromas in the scalp. It also revealed destructive lesions of bones of the skull base and a lytic lesion involving the left frontal bone  scan of the brain with intravenous gadolinium was done later. It revealed aggressive destructive lesions involving bones of the base of the skull causing compression effect on pons and medulla oblongata Figure .The case was discussed at the monthly conference of neurologists and neurosurgeons. Surgical removal of neurofibroma along with repairing the base of the skull was suggested. The patient and her attendants refused to undergo surgical management therapy. The patient was managed conservatively and discharged after 14 days. We followed up with the patient after six months. There was no change in the size of the lesion and the severity of symptoms.Neurofibromas are well-known benign tumors of the peripheral nerve sheath. According to their growth patterns, they may be categorized as local, plexiform, and diffuse types. Diffuse neurofibromas occur most commonly among children and young adults . DiffuseVery few cases of diffuse neurofibroma involving the skull are present in the literature. The underlying mechanism causing these lytic bone defects is still unknown. The skull bone defects may be attributed to resorption of bone due to overlying plexiform neurofibroma .Bony lesions can be observed on plain radiographs or CT scans. MRI is done preoperatively to assess the extension of the lesion as it may be close to the dura or can invade the sinuses ,5.The management of large neurofibromas involves partial or complete surgical excision. But there no guidelines available for the management of skull bone defects in diffuse neurofibroma. Multiple surgical techniques have been tried but the success rate was under target because of the progressive nature of the disease . A regulDiffuse neurofibroma is a benign tumor and lacks the distinctive clinical feature. The diagnosis is very challenging and therefore diffuse neurofibroma should be included in the differential diagnosis of any indurated mass on the scalp. Early diagnosis can help in better management of the patients.


This study depicts the case of a young female presenting with intestinal obstruction. Surgery uncovered a small bowel hamartoma that has caused a transmesenteric internal hernia and volvulus. As far as we know, this is unheard of before, as all three mentioned entities are very rare. The study also covers a literature review of cases of internal hernia with volvulus and stresses over the need for urgent diagnosis and management. Our case revolves around a previously healthy 28-year-old female who presented to the emergency department for acute onset of severe abdominal pain. She described the pain as being knife-life and diffuse, radiating to the back bilaterally. The patient also complained of nausea and several episodes of vomiting. She noted no changes in bowel habits. Finally, the patient denied any consumption of suspicious foods or drinks in the past few days.Her vitals were normal upon presentation. The review of system did not add any other information. Physical examination was significant for a mildly distended abdomen and palpation revealed it to be soft but diffusely tender.Withdrawn laboratories included complete blood count with differential, chem6, and liver and pancreatic enzymes. They were significant for leukocytosis at 19,000 cells/μL  with 97% (40–74%) neutrophils and microcytic anemia with a hemoglobin of 10.1 g/dL (12–14 g/dL) and mean corpuscular volume of 78 fL (80–100 fL). Due to the severity of the pain and high white blood count, a computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast was ordered.The CT showed  a fluidAfter discovering these findings, the patient was scheduled for an urgent surgical exploration. Aggressive hydration was given preoperatively, written consent was taken and the patient was transferred to the operating room. A midline laparotomy was done. Upon examination of the bowels, an ischemic and distend segment of the terminal ileum was found herniated through the mesentery and rotated at 360 degrees . HerniaThe patient's postoperative period was uneventful. Her Foley and nasogastric tube were removed on days 2 and 3, following which per os feeding was progressively started. She was discharged on day 6 postoperatively. On pathology, the lesion was found to be a hamartomatous polyp with associated enteritis cystica profunda. Immunostaining was positive for CDX2 but negative for CK7, suggesting the possibility of Peutz–Jeghers syndrome. Sadly, no additional tests could be done as the patient refused for financial reasons.Internal hernia of the small bowel is a very rare entity. It can be challenging to diagnose and can rapidly lead to serious complications like volvulus, obstruction, and even bowel ischemia and gangrene. Rapid detection and treatment are crucial as they can save the patient's life.Internal hernia is a unique entity with an incidence of 0.2 to 0.9%. The male-to-female ratio is 3:2.Our case is of the transmesenteric type and is relatively uncommon, with an incidence rate of 8% of all internal hernias.Causes of internal hernia in adults are various but most commonly encompass previous gastrointestinal surgery, abdominal trauma, intraperitoneal inflammation, or neoplasm.The clinical presentation can be deceiving. Internal hernia can be asymptomatic for years or present with nonspecific abdominal pain of distention. Note that, internal hernia accounts for 4% of acute intestinal obscuration.Imaging is the tool to accurately determine the diagnosis. Simple X-ray can show signs of intestinal obstruction or stacking of bowels in a specific quadrant of the abdomen.Diagnosis is not always reached via imaging. Following the acute and severe presentation, most patients are rushed for surgical exploration. Urgent surgical management is crucial for early accurate diagnosis and to prevent bowel ischemia and necrosis. Reduction of hernia/volvulus is the first step to be done. After that, lavage of ischemic bowel and inspection for nonviable segments should be done, and necrotic segments should be resected. Intestinal anastomosis cannot be always achieved and carries a high risk of leak and fistula, especially in severely distended bowel, but is the preferred option to stoma creation.Open versus laparoscopic surgery is a topic of debate for every surgical case. Nevertheless, Jeong et alThe diagnosis of internal hernia is not always easy and so it should be kept in mind in front of every acute abdomen. Rapid diagnosis and management can prevent bowel necrosis and patient morbidity and even mortality.


Appendicular mucinous neoplasms are a collection of rare tumors with diverse clinical presentations and pathologic potential, which can cause diagnostic and therapeutic challenges. Traditionally, they are diagnosed by radiologic imaging or identified intraoperatively; however, rarely, they may be diagnosed during an endoscopic procedure. In this unusual case, we present the case of a 62-year-old Caucasian male undergoing routine surveillance colonoscopy due to a history of colonic neoplasia. During the colonoscopy, a submucosal, non-bleeding 1cm mass of benign appearance was found in the appendix. Further workup determined the mass was likely a mucocele, and surgical consultation was recommended. The patient denied any symptoms suggestive of a mucinous neoplasm prior to and during evaluation. A laparoscopic appendectomy was subsequently performed, and the histopathology report confirmed the diagnosis of a low-grade appendiceal mucinous neoplasm. The patient recovered without complications and continued to deny any symptoms during his postoperative course and follow-up care. Given their rare incidence and unpredictable nature, appendiceal mucinous neoplasms remain difficult to identify. Discovering a low-grade mucinous neoplasm in an asymptomatic patient via colonoscopy illustrates the spectrum of unique presentations and modalities for diagnosis. Appendiceal mucinous neoplasms are a group of tumors that are relatively rare and can present in a variety of clinical and pathological presentations. These neoplasms can have either benign or malignant activity, and classification can be controversial . AppendiA 62-year-old Caucasian male with a past medical history of colonic neoplasia was undergoing surveillance colonoscopy when an appendiceal mass was identified. A submucosal non-hemorrhaging 1cm mass of benign appearance was found in the appendix. No other abnormalities were identified during the surveillance colonoscopy. Follow-up imaging was ordered, and an abdominal CT scan with contrast demonstrated a focal hypodense mass at the mid appendix, which measured 1cm x 1cm. The more distal portions of the appendix were unremarkable, and there was no evidence of periappendiceal fat stranding  is even rarer by comparison. LAMNs are three times more likely to affect females than males and are typically found in patients between the ages of 50-60 years ,4. The cThere is debate over the origin and classification of LAMNs; however, one popular theory is that these neoplasms arise from mucoceles in the appendix. A mucocele is a cyst-like space that harbors mucin-producing cells, which progress by filling the appendix with mucus . In respMultiple modalities can be used to initially identify appendiceal mucinous neoplasms, including ultrasound, CT, and MRI. Some favor using ultrasound in order to differentiate between acute appendicitis and LAMNs, as it allows for the visualization of mucinous effusion and the characteristic appearance of an “onion skin” mucocele ,9. HowevSurgical resection is the standard treatment of low-grade appendiceal mucinous neoplasms as it is the only management that is potentially curative. Appropriate surgical management is critical because rupture of the neoplasm in the appendix allows for mucinous neoplastic cells to spread throughout the peritoneum . LAMNs tThe rate of metastatic disease from appendiceal adenocarcinoma to regional lymph nodes ranges from 20% to 67%, which means nonmetastatic adenocarcinoma confined to the appendix should be treated with a right hemicolectomy . FurtherThere are no formal surveillance guidelines for appendiceal neoplasms after appendectomy. It is suggested to obtain MRI with tumor markers, carcinoembryonic antigen (CEA)/carbohydrate antigen 19-9 (CA 19-9)/cancer antigen 125 (CA 125), every six months for two years because most early recurrences occur within that time frame. Patients with HAMNs or who have had a right hemicolectomy should undergo CT or MRI every four-six months for the first two years and yearly thereafter for greater than five years .A low-grade appendiceal mucinous neoplasm is treated with surgical resection through an appendectomy for unruptured tumors localized to the appendix and not involving the appendiceal base or right hemicolectomy, which has the added advantage of allowing the histopathologist to ascertain the status of lymph node metastasis. In cases of peritoneal metastasis or a ruptured appendix, a cytoreductive surgery combined with intraperitoneal heated chemotherapy is the recommended approach nowadays.


Acute abdominal pain during pregnancy is challenging, both from a diagnostic and management perspective. A non-localized, persistent pain out of proportion to physical examination is a sign that advanced imaging may be necessary. Mesenteric venous thrombosis in a pregnant patient is extremely rare, but if diagnosis is delayed, can be potentially fatal to both the mother and the fetus. We present here a pregnant patient in the tenth week of gestation with classic clinical manifestations of mesenteric vein thrombosis and the corresponding findings on magnetic resonance imaging (MRI) and computed tomography (CT). Mild abdominal pain during pregnancy is common, but intense pain can be a sign of a serious problem . Some etA 33-year-old gravida 2, para 1, female presented to our emergency department in her tenth week of gestation complaining of epigastric pain for 2 days that did not improve, despite medical treatment at an outside hospital. She denied significant medical history. She also reported nausea, vomiting, poor appetite, and abdominal distension. Upon arrival, she was tachycardic (105 bpm), but she had no fever, hypotension, or tachypnea. Physical examination revealed epigastric and suprapubic tenderness though no rebound pain. Pertinent laboratory examinations included leukocytosis , elevation of C-reactive protein (8.7 m/dL), and elevation of Glutamic-Pyruvate Transaminase (138 U/I).Abdominal ultrasonography revealed a living 10-week-old fetus, mild maternal ascites, and no sonographic evidence of acute cholecystitis or appendicitis. Magnetic resonance imaging (MRI) was performed and demonstrated a T2 hyperintense filling defect within the superior mesenteric vein (SMV), small bowel wall thickening, and ascites . With a The patient then underwent emergent diagnostic laparoscopy which, following the observation of ischemic changes to the proximal small bowel and dark red ascites, was converted to laparotomy. Segmental resection of the ischemic bowel with side-to-side anastomosis was performed . The resHer condition improved, and she was able to resume an oral diet on postoperative day 7. During her admission, a hematologist was consulted to assess for hypercoagulability. The patient’s coagulation profile revealed low levels of antithrombin III (64%) and free protein S antigen (49%). Follow up abdominal CT on postoperative day 8 showed that the thrombus had decreased in size. After discussion with the patient, the decision was made to terminate the pregnancy, and she underwent dilation and curettage on postoperative day 10. She was discharged uneventfully on postoperative day 13, continuing anticoagulation therapy with daily rivaroxaban (15 mg). The patient regularly followed up at both our obstetrics and cardiovascular clinics, with complete resolution of the thrombus noted on follow-up abdominal CT one year later. Our patient presented with intractable epigastric pain for 2 days, nausea, and vomiting. Physical examination revealed tachycardia and tenderness over the upper abdomen though without rebound pain. This pain, out of proportion to physical examination, is a warning sign of potentially serious pathology, and an indication for the use of advanced imaging. MRI is therefore an appropriate subsequent imaging tool in pregnant patients who present in this manner. In this case, the definitive diagnosis of mesenteric venous thrombosis was ultimately obtained on CT. Mesenteric venous thrombosis in pregnant patients is extremely rare. To the best of our knowledge, less than 20 cases have been reported in the literature . While iClinical signs and symptoms of mesenteric venous thrombosis include abdominal pain, abdominal distention, nausea, and vomiting, which are notoriously nonspecific ,10,11. LImaging can offer definitive diagnosis of mesenteric venous thrombosis . In non-Due to its lack of ionizing radiation, MRI has always been considered one of the most important diagnostic tools for abdominal pain in the pregnant patient. Previous reports have shown that MRI is accurate for the diagnosis of mesenteric thrombosis, with some studies reporting nearly 100% sensitivity and specificity for MR angiography (MRA) . One of Alternatively, the application of Doppler ultrasound can be a decisive examination in diagnosing SMV thrombosis. Diagnosis can be confidently established if the obstruction of the portal or mesenteric vein by an intraluminal echogenic lesion shown on ultrasound. Color flow Doppler ultrasound has an excellent sensitivity (100%) and specificity (93%) in detecting portal and mesenteric vein thrombosis . Other aTreatment of mesenteric venous thrombosis depends on the severity of the patient’s condition. If there are no signs of bowel infarction, the patient can be managed conservatively with fluid resuscitation, broad-spectrum antibiotics, bowel rest and, most importantly, systemic anticoagulation . HoweverIn summary, we present the rare case of a pregnant 33-year-old female who experienced mesenteric vein thrombosis and ischemic bowel in her tenth week of gestation. Timely and accurate diagnosis in such a scenario requires a high-index of clinical suspicion, and through this case we highlight the importance of early imaging, especially conventional MRI.


Hirschsprung’s disease is caused by the failure of migration of neural crest cells to the hindgut, causing a lack of development of ganglion cells in the submucosal and myenteric plexuses of the colonic wall. Hirschsprung’s disease most often presents in infants with failure to pass meconium in the first two days of life. We present the case of an eight-year-old male with chronic constipation since birth who presented to the emergency department with signs concerning spinal cord compression. To our knowledge, this is the first of such a case presenting with urinary incontinence. Hirschsprung’s disease occurs in 1/5,000 live births with a male to female ratio of 3:1-4:1 . This faThe patient is an eight-year-old male with a history of chronic constipation since birth who presented to the emergency department after an episode of urinary incontinence that he did not feel. He was in his usual state of health until five days ago when he started to complain of pain in the posterior left thigh. The pain progressed over several days and became excruciating, radiating from the buttock to the back of his knee. The intensity of the pain awoke him from sleep. He also endorsed numbness of the left big toe. Trials of Motrin and naproxen at home did not improve his pain.Upon further questioning, pregnancy was uneventful, and the patient passed meconium soon after birth. His parents had been managing his constipation with over-the-counter suppositories at least once a month. A trial of polyethylene glycol did not help. At baseline, he has a bowel movement once or twice every two weeks. His last bowel movement was six days ago, and it was hard and small. His mother also reports that he has daily encopresis.On physical exam, he was in excruciating pain. His abdomen was distended, with diffuse, mild tenderness to palpation. He walked with an antalgic gait. Tenderness to palpation over the left buttock was noted. Patellar reflex was 2+, and gastrocnemius reflex was 1+. Pulses were 2+ through the left lower extremity, and the sensation was notable for being slightly decreased in the left big toe.At this time, the differential diagnosis was broad but suspected spinal cord compression and neuromuscular disorder were the most concerning, and thus pediatric neurology was consulted. They recommended an MRI of the spine to rule out spinal cord compression. Results showed marked distension of the rectosigmoid colon with intrinsic mass, likely fecal impaction, partially compressing the urinary bladder Figure . The masFinally, the patient underwent rectal suction biopsy. Unfortunately, the biopsy was limited to the level of the muscularis mucosa, and no submucosal tissue was seen. Thus, the biopsy was unable to evaluate for aganglionosis. The patient's symptoms had improved after fecal decompaction, and his leg pain had resolved. As of now, there are no plans for repeat biopsy as the parents would like to pursue alternative therapies.Hirschsprung’s disease has been reported to be diagnosed as late as 67 years of age . Some auOf note, our patient presented with signs concerning spinal cord compression. The radiating buttock pain and urinary incontinence raised concern for cauda equina syndrome, prompting immediate spinal imaging. Interestingly, sciatica has been reported as a consequence of large fecal impaction, with pain relief after decompression . The EMGHirschsprung’s disease most often presents in children with delay or failure to pass meconium. In some cases, fecal impaction can be so severe that it compresses intra-abdominal structures. In this case, the impaction caused sciatica and urinary incontinence, concerning spinal cord compression. To our knowledge, this is the first case in the literature to present both symptoms. Pediatricians should be aware that this disease may go undiagnosed during infancy and consider possible workup for patients with chronic constipation.


Magnetic resonance imaging revealed an intrasellar and suprasellar tumor with significant homogeneous contrast enchancement . Under microscopy showed the dilated cavernous spaces of irregular size and shape, which are embedded in the loose collagenous matrix and adipose tissue, lined by normal flattened endothelial cells. These spaces are mostly empty and some cavities are filled with proteinaceous material . This mass is immunohistoreactive for CD34  and D2‐40 . Preoperative basal values of thyroid‐stimulating hormone, growth hormone, luteinizing hormone, follicle‐stimulating hormone, cortisol, and adrenocorticotropic hormone were unremarkable except for a slightly increased prolactin. Computer tomography showed an enlarged sella turcica and isoattenuated sellar lesion. Magnetic resonance imaging (MRI) revealed an enlarged sellar turcica and sellar mass with suprasellar and parasellar extension. Coronal T1‐weighted MRI showed a predominantly isointense to cerebral cortex circumscribed sellar/suprasellar mass. Coronal T2‐weighted image showed a high signal of the tumor. A significant homogeneous increase in the signal intensity of the tumor was showed by contrast enhancement (Figure 2Photomicrograph of the operative tumor specimen showed the dilated cavernous spaces of irregular size and shape, which are embedded in the loose collagenous matrix and adipose tissue without lymphoid aggregates, lined by normal flattened endothelial cells showing no nuclear atypia. These spaces are mostly empty and some cavities are filled with proteinaceous material Figure . This ma3Intrasellar cavernous hemangioma.4Extra‐axial cavernous hemangiomas are most frequently seen in the fourth and fifth decades of life with a substantial female prevalence of about 7:1 and are more prevalent in the Asian population especially Japanese which have different clinical and imaging features compared with the more common intra‐axial cavernous hemangiomas. The term “intrasellar cavernous hemangiomas” was used to refer to cavernous hemangiomas mainly or soundly involving the sella. The authors believe it is a type of cavernous sinus hemangiomas growing into the sella which may originate from the components of the cavernous sinus itself or the surrounding tissues.Because of its rarity, little published information regarding the pathogenesis of this entity is available. Some studies , 2 suggePathologically, intra‐axial hemangioma often demonstrates hemosiderin‐laden macrophages, which usually are small and exhibit multiple vascular channels in various states of thrombosis, which likely reflects the low flow nature of these lesions. Extra‐axial counterparts, however, can be large and can contain vascular channels that lack histological evidence of thrombosis and calcification, which are consistent with their high‐flow state. Intra‐axial pathology is thought to represent true vascular malformations, which grow in response to intralesional microhemorrhage, whereas extra‐axial disease is believed to constitute benign vascular tumors, with intrinsic growth of endothelial cells. Based on both pathological and clinical differences, extra‐axial cavernous hemangiomas have been categorized into two subtypes. Subtype A is reported to be associated with severe intraoperative bleeding. Pathologically, these lesions exhibit thin‐walled, sinusoidal, adjacent vessels with scant intervening connective tissue. In contrast, subtype B lesions have more intervening connective tissue than subtype A. The vessels of subtype B are less sinusoidal than those of subtype A, and their size and shape are more variable and irregular.Interestingly, our case showed both vascular and lymphatic endothelial phenotypes based on their immunoreactivity with D2‐40 and CD34. Initially, we made a pathological diagnosis of lymphangioma based on a positive response to D2‐40, but this vascular lesion does not show the presence of lymphoid aggregates in the stroma typical of lymphangioma. One explanation might be that D2‐40, which was considered specific for lymphatic vessels can be expressed in other vascular lesions and is not sufficient to accurately differentiate a lymphangioma. Another potential explanation might be that the tumor cells derive from stem cells possessing the characteristics of both lymphatic and blood vessel endothelial lineages.Despite their benign nature, these lesions are sometimes difficult to treat because of their tendency to be poorly demarcated, to be infiltrative, bleeding prone, and to involve vital structures.The authors declare that they have no conflict of interest.Yanli Du: drafting the manuscript, acquisition of data. Taili Li: Interpretation of pathological results. Hui Qi: drafting and revising the manuscript. Zongli Han: study design, supervision of study.We declare that all human and animal studies have been approved by the Peking University Shenzhen Hospital ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.Informed consent was obtained from all individual participants included in the study.