Appendicitis in adults is thought to occur because of luminal obstruction from a fecalith. We present a unique case of a patient who had her entire appendiceal lumen occupied by a fecalith (5.0 cm long) but had no appendicitis. We reviewed the records of 257 veterans who underwent surgical intervention at our institution for the management of acute appendicitis. Fecaliths occurred in 15.6% of patients. At laparotomy, 20.6% had a perforated appendix; pathology showed fecaliths in 20.8% of specimens. A review of the literature inclusive of 25 series showed fecaliths in 33.3% of patients with a normal appendix, 23.5% of patients with acute appendicitis and 24.9% with perforated appendicitis. These data show that appendicitis is not a common cause of fecalith obstruction in adults. Conventional teaching in the pathophysiology of acute appendicitis in adults dictates luminal obstruction from a fecalith , which leads to stasis, bacterial overgrowth, inflammation, ischemia and perforation . HoweverFurther, intraoperative management of appendiceal pressure in patient with appendicitis revealed that the inflammatory process might be the culprit of luminal obstruction rather than obstruction leading to inflammation . Thus, tThe classical teaching of a fecalith leading to appendicitis has been challenged by several investigators , but it In the present report, we present a unique patient with a very large fecalith occupying the entirety of the appendiceal lumen; we reviewed all appendectomies in our institution for the past 12 years to assess the role of fecaliths in appendicitis. We then reviewed the literature.A 50-year-old veteran woman presented to the emergency department (ED) with a 1-day history of abdominal pain, nausea and multiple episodes of vomiting. Her pain was intermittent, severe and poorly localized to the right lower quadrant. She had had similar episodes necessitating ED visits in the past several months, and pain medication had been prescribed, which improved her symptoms. Her past medical history is significant for inflammatory bowel disease, gastroesophageal reflux disease, post-traumatic stress disorder and fibromyalgia. She is a current smoker (4–5 cigarettes a day) but denies alcohol consumption or intravenous drug use. Her medications include ondansetron 8 mg by mouth three times a day, sertraline 50 mg by mouth twice a day and tramadol 50 mg per mouth as needed for pain.2. She was alert and oriented, but in clear distress from abdominal discomfort, rocking back and forth in bed and moaning from this pain. Her heart and lungs were clear. Her abdominal examination demonstrated no abdominal distension and no rigidity. She was tender to palpation on the right lower quadrant.On physical examination, her temperature was 97.9°F, her heart rate was 70 beats per minute, blood pressure 148/88 mmHg, respiration rate of 20 breaths per minute, her body mass index was 28.6 kg/mSerology analysis showed a white blood cell count of 7.7 K/μL , Hemoglobin of 12.7 g/dL , hematocrit of 38.5%  and platelets of 334 K/μL . Electrolytes were all normal, and creatinine was 0.91 mg/dL . Lactic acid levels were 2.1 mmol/L . Her modified Alvarado score was four.Computed tomography (CT) imaging showed an appendix with an appendicolith with mild dilation (9 mm) and no peri-appendiceal inflammatory changes . CompareGiven these findings, she was taken to the operating room for a laparoscopic appendectomy. Exploratory laparoscopy demonstrated no evidence of acute or chronic appendicitis. Pathological examination of the appendix demonstrated an appendix entirely occupied by a fecalith and no evidence of appendicitis .Of 257 patients who were operated on for acute appendicitis at the VA North Texas Health Care System from 2005 to 2017 only 40 had fecaliths (15.6%) . Of 257 patients who underwent an appendectomy initially for acute appendicitis, 38 (14.8%) had gangrenous appendicitis. Of patients with gangrenous appendicitis seven (18.4%) had an appendicolith. Fifty-three (20.6%) had a perforated appendix despite carrying an initial diagnosis of acute appendicitis. Of patients with perforated appendicitis, 11 (20.8%) had a fecalith.Between 1939 and 2019 a total of 25 series, inclusive of 94,592 patients, were reviewed. The average age of patients was reported in 11 studies (38.2 years old ±13.3 standard deviation [SD]). The overall fecalith in appendiceal specimens was 20.7% (range 1.4–67.0%). Fecaliths occurred with a frequency of 20.3% (range 5.3–100%) in 11 reports documenting a normal appendix. Fifteen studies reported an average of 23.5% (range 1.5–65.1%) fecaliths in patients with acute appendicitis. In patients with perforated appendicitis, fecaliths were reported in 24.9% (range 10.2–39.1%) of 10 reported series .A fecalith  is a fecal concretion or pellet occupying the appendiceal lumen and is thought to be the result of a low fiber diet that accompanies a Western society lifestyle . PopularThe present analysis was stimulated by an unusual case in which a patient presented with a history of abdominal pain and had a large fecalith within the entire lumen of the appendix but did not cause appendicitis. A review of 257 veteran patients who had undergone an appendectomy for acute appendicitis at our institution, only 15.6% had fecaliths on the pathological specimen. A review of the literature showed that less than a quarter of patients presenting with appendicitis demonstrated a fecalith either radiographically, in the operating room, or on final pathology examination .et al. that divides this era prior to the 1970s and after that to current studies [The present case negates the role of the fecalith in the infectious process of appendicitis, even if it is very large. Our case series consists of veteran patients presenting with acute appendicitis and shows that fecaliths are found rarely in this patient population. The literature review demonstrated the following findings: (i) There is a wide range in the incidence of fecaliths leading to appendicitis; (ii) more cases of a normal appendix have been documented that have incidental fecaliths within the lumen compared to the frequency of a fecalith causing appendicitis; (iii) A temporal variation in the incidence of fecaliths as a cause of acute appendicitis has been proposed by Sigh  studies  and (iv)A fecalith is, therefore, an infrequent cause of appendicitis as suggested by textbooks. Other causes of appendicitis have been proposed and reviewed by Norman J Carr  and inclThus, the current literature argues against a fecalith leading to acute appendicitis, but when present it is more likely to lead to perforation. This should be considered when assessing a patient for NOM of early appendicitis.


Epithelioid angiomyolipomas (EAMLs) are mesenchymal tumors that are part of the family of the perivascular epithelioid cell neoplasms (PEComas). These tumors portray a potential aggressive behavior with metastatic lesions found in around 30% of reported cases. EAMLs might present sporadically or in association with the tuberous sclerosis complex (TSC). They typically involve the kidneys, liver, and lungs. It is extremely rare for these tumors to arise from other organs. The present report describes an unusual case of an adult patient with a history of TSC who developed EAML of the adrenal gland. Moreover, he presented with metastatic disease to the liver, a feature rarely described. The diagnosis of EAMLs can be challenging as they are hard to distinguish from other adrenal or renal tumors without a thorough histopathologic and immunohistochemical evaluation. Due to the potential aggressive behavior of these malignancies, timely diagnosis is extremely important and has significant therapeutic and prognostic implications. Epithelioid angiomyolipomas (EAMLs) are rare, mesenchymal tumors that belong to the perivascular epithelioid cell neoplasms (PEComas). They also share some histologic features of angiomyolipomas (AMLs), but they are mainly composed of epithelioid cells and lack the typical fat tissue component. While AMLs are usually benign, EAMLs tend to be larger in size and can be malignant. They usually involve the kidneys, liver, and lungs. Therefore, involvement of other organs poses a diagnostic challenge –4. WhileA 32-year-old gentleman presented to the emergency department (ED) with a 1-week history of right-sided lower back pain. His medical history was significant for TSC. He endorsed fatigue, unintentional weight loss of around 50 pounds for the last 3 months, and night sweats for the past weeks prior to admission. He denied any preceding trauma, fever, urinary symptoms, hematuria, abdominal pain, or changes in bowel movements. Past surgical history was unremarkable. He is a lifetime non-smoker and denied any alcohol or recreational drug use.Physical examination revealed multiple facial angiolipomas over the nose and cheeks. No enlarged cervical or supraclavicular lymph nodes were found. Respiratory and cardiovascular exams were unremarkable. The abdomen was soft and nondistended, but the right flank was tender to palpation without rebound or guarding. A palpable mass was noted in the right hemiabdomen. Costovertebral tenderness was absent; however, right paraspinal lumbar tenderness was elicited by body movements.Laboratory testing was only remarkable for normocytic anemia with hemoglobin 7.8 g/dL (14–18 g/dl). Urinalysis was normal without blood or red blood cells. Computed tomography (CT) scan of the abdomen without contrast revealed a right suprarenal vs. renal mass measuring 16 × 17 × 20 cm . Areas oBiochemical workup was performed to evaluate whether the mass was of adrenal origin and hormonally active as part of the preoperatory evaluation. Evaluation for metanephrines, normetanephrines, aldosterone, and cortisol overproduction was unremarkable. Subsequently, the patient underwent total right adrenalectomy with en bloc right nephrectomy and resection of regional lymph nodes . ExcisioPathology evaluation showed involvement of the adrenal gland and perinephric soft tissue by malignant, large epithelioid cells with abundant pale to eosinophilic cytoplasm, enlarged and irregular nuclei, and conspicuous nucleoli. These cells are arranged in variably sized nodules with fibrous septae, prominent alveolar growth pattern, with admixed chronic inflammation and extensive necrosis .Immunohistochemistry showed expression of Melan-A/MART-1 , synaptoEpithelioid angiomyolipomas (EAMLs) are mesenchymal neoplasms that constitute a variant type of the angiomyolipomas (AMLs), benign tumors composed of adipose tissue, smooth muscle cells, and abnormal thick-walled blood vessels –4. EAMLsThe diagnosis of EAMLs can be challenging. Clinically, these tumors are usually asymptomatic. As they grow, patients may experience nonspecific symptoms such as weight loss, fatigue, and abdominal discomfort. Although imaging studies can effectively detect the presence of these masses, they have limited utility in distinguishing EAMLs from other neoplasms. Therefore, extensive histopathological and immunohistochemical analyses are necessary to establish the diagnosis.EAMLs belong to the perivascular epithelioid cell tumors (PEComas) family due to their reaction to immunohistological markers such as SMA and HMB-45 , Melan-A (more than 50%), and desmin (around 25%). Histologically, they present as cells arranged in nests, sheets, or alveoli surrounded by septae. These cells can be spindled or epithelioid with clear or eosinophilic cytoplasm, but EAMLs can also present as pleomorphic multinucleated cells –3, 7, 13Another important clinical feature described in our patient is his history of tuberous sclerosis complex (TSC). EAMLs and AMLs are frequently associated with TSC, with EAMLs presenting more often than AMLs. The incidence of AMLs in general in patients with TSC is 50–90% . A studyThe differentiation between renal and adrenal involvement was challenging in our patient due to the large burden of the mass and was ultimately determined postoperatively. Imaging studies might fail to establish the origin of the tumor, as in our case. EAMLs might share similar features with other malignancies in the imaging studies. Therefore, it is important to perform a thorough evaluation for alternative diagnoses such as hormonally active adrenal tumors prior to proceeding with surgical resection, as these can have implications on perioperative morbidity and mortality. The differential diagnosis considered in our patient included renal cell carcinoma, adrenal cortical carcinoma, hormone-producing adrenal malignancies such as pheochromocytoma and metastatic malignancies such as metastatic melanoma.Unfortunately, the best treatment approach for malignant EAMLs is not well-established given their rarity, especially extrarenal tumors. There are no clinical trials examining the efficacy of surgical and medical therapies. Management of patients with malignant EAMLs is often guided by findings from cases reported in the literature. Treatment should be individualized based on tumor size, signs of metastasis and progression, and operative risk. While asymptomatic small tumors (<5 cm) can be monitored initially, surgical resection is recommended for most tumors given risk of metastasis, growth, and rupture. However, due to the aggressive behavior of EAMLs, local recurrence, and distant metastasis has been reported after surgery , 4.Recently, focus has been placed on the mTOR pathway with promising results, especially in patients with TSC. TSC is characterized by germ-line mutations in the TSC1 or TSC2 genes which encode the proteins hamartin and tuberin. Tuberin is part of the cell signaling pathway involved in RNA translation (mTOR pathway). Therefore, drugs such as sirolimus or everolimus that suppress mTOR signaling might be potential therapeutic options. Prolonged responses can be achieved with these medications, but costs and side effects are limiting factors , 14–17. Primary adrenal epithelioid angiomyolipomas are extremely rare and aggressive malignancies with fewer than 10 cases of primary adrenal EAMLs currently reported in the literature. Histopathology and immunohistochemistry analyses are of utmost importance in establishing the diagnosis of EAMLs. The presence of TSC should also heighten the suspicion of mesenchymal tumors, including malignant EAMLs. Optimal therapeutic strategies, treatment responses, and prognosis are yet to be determined due to the low prevalence of this disease. Further research including clinical trials and prospective studies are necessary to identify and determine potential treatment options that could benefit these patients. Until then, surgical resection remains a mainstay of therapy with mTOR inhibitors as viable options for treatment of recurrence.


A well-marginated fat-containing renal lesion gives strong suspicion of renal angiomyolipoma (RAML) that usually requires no surgical intervention. Radiologically, renal masses with a large amount of fat can rule out renal cell carcinoma (RCC). Calcifications are very infrequent in RAML. However, the presence of calcifications in a fat-containing renal lesion is highly suggestive of RCC. These lesions should undergo surgical resection and histopathological assessment to reach the right diagnosis and avoid poor outcomes if diagnosed late. We present a case of bilateral renal tumors, in which one of them radiologically contained abundant fat with calcifications on CT scan, which was confirmed to be an RCC on histopathological examination. Abdominal imaging has been used extensively over the past decade in patients presenting with gastrointestinal and urological symptoms. With the increasing use of radiological modalities, there is a rising trend of diagnosis of solid renal tumors incidentally . In a weClinical historyA 69-year-old male with a history of hypertension presented to the emergency department with complaints of urinary frequency, urgency, and hesitation to pass urine. Physical examination was unremarkable. Laboratory tests showed high leukocytes in urine (19 WBC/hpf) and elevated prostate-specific antigen (PSA) of 14 ng/mL.Radiological featuresUltrasound examination of the urinary tract showed an enlarged prostate with an estimated volume of 67 mL. In addition to this, two solid mass lesions were found incidentally in the right kidney. The first lesion was a well-defined hypoechoic mass lesion in the middle pole region, measuring 25 x 22 mm. A second hypoechoic lesion was identified in the lower pole measuring 10 cm and had calcifications as well. The left kidney also showed a small hypoechoic lesion in the lower pole  exophytic heterogeneous lesion with foci of macrocalcifications. It showed heterogeneous contrast enhancement and multiple areas of fatty components. In the lower pole of the left kidney, there was a small lesion measuring 14 mm in diameter, showing heterogeneous contrast enhancement with a central non-enhancing area , multifocal clear cell RCC (eosinophilic variant), multifocal chromophobe RCC, and oncocytoma. Another possibility was RCC infiltrating angiomyolipoma, but the morphology and immunohistochemical features were against RAML. Based on morphological and immunohistochemical features, the final diagnosis in both specimens was RCC.Follow-upDue to the malignant features of the tumors on histopathology, the patient underwent a CT scan of the thorax, abdomen, and pelvis six months after the surgery to look for recurrence. Fortunately, the CT scan showed no recurrence or residual tumor on the right side. Although the left-sided tumor was of the same size in the repeated CT scan, the uro-oncology team offered the patient biopsy or partial nephrectomy. But, the patient refused to take any further management.RAML, fat-containing RCC, lipoma, liposarcoma, and oncocytoma are the renal tumors that contain fat . Our priIncidental diagnosis of renal tumors is becoming more common due to recent advancements in radiological investigations and increasing trend in the use of the diagnostic techniques for common diseases. Incidentally diagnosed cases have a better prognosis due to the early stage of detection and low grade . A well-One of the following mechanisms can explain the reason behind the presence of fat in RCC. Lipid producing necrosis, osseous metaplasia, and entrapment of sinus fat by an extensive irregular RCC can explain the mechanism of the existence of fat within RCC . RAML wiThe confirmation of a hyperechoic (fatty) renal mass with a CT scan is a crucial step in further evaluation as it can prevent surgery. The presence of calcifications in fat-containing renal mass is highly suggestive of RCC. The management of these lesions is by resection and histopathological confirmation. Also, fat-containing tumors involving perirenal or renal sinus fat, large irregular mass containing areas of small fat necrosis, lymph node involvement, or venous invasion raise the suspicion of RCC.


DAXX and ATRX has been added to the criteria for well-differentiated NETs.Gallbladder neuroendocrine tumors (GB-NETs) are extremely rare, representing only 0.5% of all NETs because no neuroectodermal cells are present in the gallbladder. In 2019, the World Health Organization updated the classification of NETs based on their molecular differences. The mutation status of A 50-year-old man presented to our hospital for further examination of a gallbladder polyp. He had no right quadrant pain, fever, jaundice, weight loss, or carcinoid syndrome-related symptoms. The patient hoped to avoid cholecystectomy. During the 3-year observation period, the polyp gradually increased in size from 8.3 to 9.9 mm. He decided to undergo surgery, and whole cholecystectomy was successfully performed. Immunohistochemical staining revealed positivity for chromogranin A, synaptophysin, and CD56. The Ki-67 index was < 3%. Taken together, these results led to a diagnosis of a grade 1 GB-NET. We also performed immunohistochemical staining of DAXX and ATRX, which revealed that DAXX protein expression was negative. The patient’s postoperative course was uneventful, and he developed no recurrence for 8 years after surgery.We experienced a very rare case of GB-NET. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. A GB-NET should be considered as a differential diagnosis of gallbladder tumors. A neuroendocrine tumor (NET) is a rare type of tumor with an incidence of about 5.25 per 100,000 people . Most GBA 50-year-old man with a history of an atrial septal defect and hypertension presented to our hospital for further examination of a previously diagnosed gallbladder polyp. He had no right quadrant pain, fever, jaundice, weight loss, or carcinoid syndrome-related symptoms such as diarrhea, flushing, edema, or wheezing. The patient hoped to avoid cholecystectomy. Abdominal ultrasonography revealed an 8.3-mm elevated polyp in the region around the gallbladder neck Fig. a. DuringThe patient had smoked 20 cigarettes per day for 20 years. He also had a history of drinking alcohol (one bottle of beer per day). Physical examination showed no abnormalities. Blood tests also showed no abnormalities, including elevations of tumor markers such as carcinoembryonic antigen 1.5 ng/ml) and carbohydrate antigen 19–9 9.3 U/ml). Taken together, these results suggested that the polyp included a malignant component. Therefore, we performed laparoscopic cholecystectomy. The whole gallbladder was successfully removed. Macroscopic examination of the resected specimen revealed a tumor of approximately 10 mm in diameter in the gallbladder neck region  was defined as a benign tumor by Oberndorfer , but a pn = 518, 69%) and white  and presented with stage IV disease  and high-grade lesions . Sixty-four percent of the patients underwent surgery, mainly simple cholecystectomy , and 145 patients (21%) underwent multimodal therapy. The median overall survival of the patients with GB-NETs was 25 months. The authors reported that older age, large cell histology, and positive margins were independently associated with worse overall survival. Cen et al. [NETs are reportedly rare; they arise mainly in the gastrointestinal tract, where they account for < 2% of all primary gastrointestinal tumors , 5. Thisn et al.  analyzedn et al. –17. ImmuFurthermore, we were able to stain serial tumor specimens with antibodies to DAXX and ATRX, the mutation status of which has been added to the criteria of well-differentiated NETs . In our MEN1, DAXX, and ATRX could be an entirely new strategy to diagnose gastrointestinal well-differentiated NETs.The serum chromogranin A and urinary 5-HIAA concentrations have been the gold standard biomarkers for the identification and follow-up of carcinoids . AlthougWe have herein reported a rare case of a GB-NET. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation of such patients. As indicated by our case, a GB-NET should be considered as a differential diagnosis of gallbladder tumors.


Epidural or spinal anesthesia is commonly administered in births in the US, and the potential risks for epidermoid tumors are not well-characterized. We present the case of a 29-year-old female patient who developed an intradural epidermoid tumor in the lumbar spine, discovered seven years after spinal anesthesia for childbirth. MRI revealed a 4 cm tumor filling the entire spinal canal. Pathology confirmed the mass to be an epidermoid. Complete surgical resection of the intradural lesion was accomplished with full symptomatic relief. This case supports the relationship between delayed development of epidermoid tumors and spinal puncture in adult populations. Epidermoid tumors comprise less than 1% of spinal tumors. Previous case reports have established some epidermoid tumors as a known rare complication of lumbar puncture with most cases being described in children . EpiduraA 29-year-old female presented with increasing back, groin, and bilateral leg pain over a six-month period. She had no bowel or bladder involvement and no weakness. The patient had no relevant past medical history but had received spinal anesthesia administered with a styleted spinal needle during childbirth seven years prior to clinical presentation. Contrasted lumbar spine MRI revealed a 4 cm long space occupying lesion without enhancement filling the spinal canal Figures -2. A lumWe report a rare case of an epidermoid tumor of the lumbar spine associated with lumbar puncture presenting in a delayed fashion in an adult, which was treated with complete surgical resection of the intradural lesion and full symptomatic relief was achieved. This case supports the relationship between delayed development of epidermoid tumors and spinal puncture in adult populations.Manno et al. reviewed 90 cases of intraspinal epidermoid tumors and found that the vast majority occur in the intradural space of the lumbar spine (83/90 [92%]) . A recenApproximately 40% of iatrogenic epidermoid spinal tumors are late complications due to lumbar puncture . HoweverIn the present case, the origin of the epidermoid tumor was likely initiated by the lumbar puncture intervention, which was done for anesthesia during childbirth. The patient presented with symptoms of back, groin, and bilateral pain. The MRI findings revealed a 4 cm lesion in the spinal canal, which was successfully resected by lumbar laminectomy. The cytopathological diagnosis confirmed a mass as a benign tumor and post-operatively the patient experienced no complications at eight-week follow-up with complete resolution of back, groin, and bilateral leg pain. Therefore, the total resection of the spinal epidermoid cyst seems to have been an effective treatment option and can result in complete resolution of all the related symptoms. Furthermore, women who undergo similar interventions during childbirth or for any other surgical procedure should be monitored for the various symptoms, which could result from the development of similar types of cysts. Importantly, proactive measures should be taken to confirm the diagnosis and surgically remove these cysts.Here, we report a rare case that suggests that lumbar puncture for spinal anesthesia during childbirth carries the risk for the development of intradural epidermoid tumors that can be diagnosed several years post-procedure, highlighting the importance of careful administration of anesthesia during childbirth by adhering to best practice methods of lumbar puncture with an atraumatic needle.


Vulvar varicosities in nonpregnant females, either isolated or as a part of the pelvic congestion syndrome, are rare. We present a case of an adolescent girl with morbid obesity with bilateral bluish protrusions on the labia minora, as an incidental finding, that coincided with her excessive weight gain. The adolescent underwent thorough clinical examination, doppler ultrasound, contrast venography and varicography, and magnetic resonance angiography to rule out alternative diagnoses. Imaging results confirmed the presence of large venous lakes. Venous drainage to the internal iliac vein and connections with the long saphenous vein were delineated. Incompetence, dilatation, or reflux of ovarian or internal iliac veins, or their main tributaries, were not noted. Since the adolescent was asymptomatic and other pathologies, such as vascular malformations or hemangiomas were excluded, she was managed conservatively with counseling about lifestyle modification and weight reduction. This is only the third reported case of vulvar venous varicosities in adolescents. Female sex, along with obesity, are known risk factors for varicose vein formation; however, the pathogenesis is not fully understood. Additional research is needed to elucidate the role of excess adipose tissue in the pathophysiology of vulvar varicose veins and to optimize diagnostic workup and management in adolescence.  Vascular varicosities, i.e., dilated venous channels in the vulvar area, are rare and almost exclusively affect women during pregnancy. Almost 4–22% of pregnant women present with vulvar varicosities. The majority of cases disappear either immediately after parturition or during puerperium, and only 4–8% persist or worsen with time ,2,3. TheMost females with visible vulvar varices do not report any symptoms, especially during pregnancy. Palpable mass on the external genitalia, vaginal discomfort, swelling, heaviness, and pain are rare manifestations. These symptoms usually deteriorate at the end of the day after standing up, exercise, or coitus. Due to the paucity of symptoms and the embarrassment to report changes in the external genitalia, most cases of vulvar varices in pregnant women are diagnosed at term or even during labor.A 16-year-old Caucasian girl was referred to the Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care of the First Department of Pediatrics, School of Medicine, National and Kapodistrian University of Athens, at the Aghia Sophia Children’s Hospital. Her main concerns were oligomenorrhea and a mass over her external genitalia observed by the referring gynecologist.The adolescent presented with a history of excessive weight gain (over 30 kg during the past 2 years), recently diagnosed Hashimoto’s thyroiditis, and oligomenorrhea. She had a family history of type 2 diabetes mellitus. She had menarche at 12 years of age, and she reported normal menstrual cycles during the first two gynecological years. In the last two years, menses were irregular with secondary amenorrhea for 4 months. She denied recent trauma at the vulvar or perineal area, and her mother reported that no vulvar mass had been observed in her past pediatric health visits. The adolescent did not complain of heaviness, discomfort, or regional pain at any position or following exercise or prolonged standing. She did not report sexarche; however, a urine pregnancy test was performed, and pregnancy was ruled out. Initially, the girl was examined in the lithotomy position and then in the upright position. In the lithotomy position, bilateral bluish protrusions were observed on labia minora . On the 2 (WHO Class III obesity); however, her arterial blood pressure (124/74 mmHg) was below the hypertensive range for her age and height [The adolescent had morbid obesity, with a body mass index of 41.9 Kg/mUltrasound evaluation of the vulvar masses revealed a helical, retractable, tubular structure with dense fluid content of low flow, which varied in size with pressure and had a morphology of nervous, varicose dysplasia. Varicography and ascending venography were performed by femoral approach to assess communications between varicosities and deep pelvic veins. No areas of arteriovenous dysplasia or reflux were observed on the tributaries of either the ovarian or the internal iliac veins. The examination was completed by catheterization of the vulvar mass on the right side. The vascular lake was delineated in the area of the right labium minus, which drained through estuarine tributaries into the iliac vein. The aforementioned findings were confirmed by magnetic resonance angiography (MRA), as shown in Due to the absence of chronic pelvic pain suggestive of Pelvic Congestion Syndrome (PCS), no intervention was decided for the adolescent, apart from lifestyle modification to ameliorate obesity health hazards. During 2-year follow-up visits, she remained asymptomatic and did not report any embarrassment or other negative feelings about the appearance of her vulvar varicosities, which neither enlarged nor reduced. She still had morbid obesity along with menstrual irregularities, and she was diagnosed with polycystic ovary syndrome.The first report of vulvar varices and their management was in 1967, though its affined Pelvic Congestion Syndrome (PCS) was known since the 19th century ,11. ThorVascular drainage of the vulvar area is achieved through external pudendal veins , which drain into the long saphenous vein at the saphenous opening. Ιnternal pudendal veins are the second pathway of drainage of the labia and clitoris, venae comitantes of the internal pudendal artery, and tributaries of the internal iliac vein . The thiVulvar varices pathogenesis is poorly understood, because of the complexity and variation of the pelvic circulation. Sometimes the problem is isolated in the vulvar veins, whereas, when it is part of PCS, anastomoses and drainage in both great saphenous and internal iliac impede the discovery of a single affected proximal vessel. Adipose tissue participates in the pathogenesis of pelvic venous syndromes. Lower extremity varicosities are more frequent at higher BMI, whereas PCS is less frequent ,13. A poThe differential diagnosis of a vulvar mass in the nonpregnant state can be confusing. Bartholin gland disorders (cysts or abscesses) are the most probable diagnosis when a vulvar mass appears suddenly. Other type of cysts, dermoid or epidermoid, can also appear as nodules with variable characteristics on external genitalia. Inguinal or femoral hernias should also be excluded, especially when the mass increases in size along with intrabdominal pressure. Traumatic event can cause a hematoma, with transient appearance as nodule before creating a flat pigmented lesion. Vascular malformations, venous or arteriovenous, and hemangiomas usually present as bluish masses and make difficult the differential diagnosis without any imaging studies.In most studies, work-up begins with doppler ultrasonography of pelvic veins to identify the affected vessels and valvular incompetence and measure the diameter of the dilated vessel and blood flow . AccordiThe choice of therapeutic approach in nonpregnant women is equivocal. Since there are not any randomized controlled studies, the investigation and treatment plan are usually individualized based on small-scale case series and case reports. The two main factors that guide the decision for intervention are symptomatology and association with PCS or leg varicosities. It is important with any venous circulation disorders that have been identified with imaging to design the treatment plan. Advanced imaging of the surrounding venous anatomy of the pelvis and legs should be obtained to guide management, which includes compression, sclerotherapy, embolization, or surgical ligation . When vuSince our adolescent did not report any symptoms and was not concerned about the aesthetics of her external genitalia, she was managed conservatively. The lack of cases of vulvar varicosities in the literature in adolescent nulliparous girls and the fact that adolescence is a period of body development guided our decision. Moreover, weight gain, in our case, seemed to coincide with the appearance of the varicosities, and, since this was a modifiable factor, lifestyle modification and weight reduction were advised before any therapeutic approach. In the current literature, there are only two other case reports of nulliparous young women with vulvar venous varicosities ,5. LeungRelatively recent commendations from a multidisciplinary research consensus panel recognized the multiple evidence gaps on the spectrum of symptoms and signs related to the pelvic venous circulation and suggested that the term “syndrome” should be abandoned, and the term “pelvic venous disorders” should be used instead to avoid confusion. The panel acknowledged the need for multidisciplinary research to avoid over- or undertreatment of such cases . Despite extensive imaging to exclude other pathologies, especially vascular malformations or hemangiomas, a conservative follow-up approach was considered appropriate in this asymptomatic adolescent case. Female sex, along with obesity, are known risk factors for varicose vein formation; however, the pathogenesis is not fully understood . The rol


Lymphangioma is a rare and understudied pathology that is usually detected in the first decade of life, and its appearance in adults is rare. This report details a 51-year-old man with morbid obesity who presented, for the last eight months, multiple asymmetric tumor lesions with extension to the scrotal region. The diagnosis of circumscribed lymphangioma with associated infection was confirmed. This case report demonstrates an unusual presentation of the characteristics of the lymphangioma that are seldom described in the literature. Circumscribed lymphangioma (LC) is infrequent and usually develops congenitally, being detected up to 90% of the cases in the first decade of life; however, it can also develop in adulthood.LC in the adult generally appears in areas affected by trauma, infection, or radiation, but it is also reported to appear spontaneously.Reports of large-volume lymphangioma are very seldom described in the literature.A 51-year-old male from Peru, with a history of liver failure and morbid obesity, was transferred to the emergency department with a time of illness of eight months due to the presence of small tumor lesions at the pubic level, which evolved to become large.Upon physical examination, multiple lesions with a cystic appearance, conglomerated, with raised and umbilicated borders were evidenced in the pubis; with a diameter of up to13 cm × 7 cm in which, through their orifices, they presented a purulent and foul exudate. The lesions extended to the scrotal region where they were associated with grouped vesicles that bled during mobilization .Figure 1The lower limbs showed scaly and cracked plaques with hard-consistency hyperkeratosis due to chronic lymphangitis.Histopathological studies of the biopsy concluded with the diagnosis of circumscribed lymphangioma . The patLymphangioma is an infrequent and benign malformation of the lymphatic system, which can be classified according to the size or depth of the lesion. Although the authors report this entity as having a large volume, it lacks malignant potential, explaining the increase in size due to its ability to branch out and grow in a disorderly manner.The location of this entity is more frequent in the extremities, neck, and trunk, while the appearance in the scrotal region is rare.The examiner should consider the characteristics of the lesions, which may be confused with condyloma acuminatum, molluscum, contagiosum, or herpes. A thorough inspection and an adequate clinical history can exclude these differential diagnoses.2 laser, or sclerotherapy; however there is the possibility of a spontaneous regression after three months that happens only in the macrocystic lymphangioma, while in the rest, the risk of recurrence appears between six months to six years.In lymphangiomas, the basic treatment without risk of infection involves the use of surgical resection, COThe biopsy sample was considered to be a limitation because it was not representative, since some histopathological characteristics were not similar to those reported by other studies.In summary, lymphangioma is an infrequent pathology in adults and the authors infer that the time of evolution, infection, and morbid obesity of the patient could have contributed to the atypical clinical manifestations described.None declared.Cristian Morán-Mariños: Statistical analysis; approval of final version of the manuscript; drafting and editing of the manuscript; collection, analysis, and interpretation of data; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.Wendy Nieto-Gutierrez: Approval of final version of the manuscript; drafting and editing of the manuscript; critical review of the literature; critical review of the manuscript.Josmel Pacheco-Mendoza: Approval of final version of the manuscript; drafting and editing of the manuscript; critical review of the literature; critical review of the manuscript.None declared.


The celiacomesenteric trunk (CMT) is one of the most striking among the different variations of the normal vascularisation of the gastro-intestinal tract. It is often accidentally discovered during autoptical dissections, angiography or abdominal computed tomography (CT).A 27-year-old man was admitted to emergency for an acute abdominal pain. For his critical condition, the patient was immediately brought to the operating room. A extensive intestinal necrosis was found. Post-operator CT discovered a common CMT that is complicated by extended thrombosis. Despite all resuscitation measures, the patient died of septic shock two days later. An autopsy was performed showed that the main cause of intestinal ischemia was related to dissection of a common CMT.A CMT is a highly unusual variation in humans. It is usually asymptomatic and may be discovered incidentally during vascular surgery, radiologic imaging, or cadaver's dissection. Lesion of this entity can lead to serious gastrointestinal complications including necrosis. Different classifications are proposed in the literature.As it is associated with the risk of mesenteric ischaemia, CMT should be kept in mind as a differential diagnosis for cases of recurrent non-specific abdominal pain. It is an extremely rare occurrence, accounts for less than 1% of all celiomesenteric anomalies [Injury of CMT can lead to serious gastrointestinal complications including bowel necrosis. In this document, we describe a case of CMT dissection leading to mesenteric ischaemia. To the best of our knowledge, this is the first case to be reported in the literature.The purpose of this article is to review the appearance of this extremely rare entity, to evaluate the diagnostic utility of CT in the detection of this disease and to discuss the management methods. This work has been reported in line with the SCARE criteria .2A 27-year-old man was admitted to emergency of a small peripheral hospital for an acute abdominal pain. Previously, he was healthy and had no remarkable antecedents, other than history of frequently smoking. According to his family, this abdominal pain had occurred several days before admission, and became so severe that he consulted for further examination and treatment.In the emergency room, the patient was in a state of shock. His blood pressure was 80/60 mmHg and the pulse rate was 120 beats per minute. The physical examination was remarkable for abdominal distention with peritoneal signs. The results of laboratory data indicated that everything was within reference limits. The abdominal radiogram showed a nonspecific gas pattern.With this critical condition patient, transfer to specialized surgical hospital was impossible and surgeons decided to operate. In operating room, a necrotic bowel extending from the upper ileum to distal ileum was found. A prolonged resection with an ileostomy was performed. However, no obvious etiology has been found to explain this ischemia.Later, a CT scan was realized. So, a very rare anatomic variant was revealed. The coeliac trunk and the superior mesenteric artery arose as common trunk from the ventral surface of the abdominal aorta.Furthermore, an extended thrombosis of the common trunk to the superior mesenteric artery was diagnosed. This thrombosis was associated with a non-specific periarterial fat infiltration around a, b. How3D volume rendered images showed that this common trunk is at the level of the L1 vertebrae. It has a length of 10 mm before his bifurcation to a superior mesenteric artery and coeliac trunk which gives three classical branches; hepatic, splenic and left gastric arteries a, b. TheAn urgent administration of an anticoagulant was started and the patient was immediately transferred to another hospital to see the possibility of vascular management. Later, the patient was evolved with a septic state requiring his hospitalization in the reanimation unit. Despite all resuscitation measures, the patient died of septic shock two days later. For the goal of scientific interest, an autopsy was performed. Results showed that the main cause of extensive intestinal ischemia was related to thrombosed dissection of a common CMT. Unfortunately, the cause of dissection remained unknown, as no histological examination was realized. However, the interrogatory to his family revealed the history of Wegner granulomatosis in his sister.3A CMT is a highly unusual variation in humans. It is usually asymptomatic and may be discovered incidentally during vascular surgery, radiologic imaging, or cadaver’s dissection . HoweverThis vascular variant resulting from anomalies of embryonic development that is the failure of disruption, at an early stage of fetal development of the ventral longitudinal anastomosis between the third and fourth roots is arising from the primitive dorsal abdominal aorta . DiffereDisease involving this rarely vascular anomaly is extremely uncommon. Only nine cases of aneurysmal dilatation and one case of thrombosis have been reported in the literature . To our Multi slice tomography with arterial and portal phase acquisitions plays a major role to detect these diseases. It allows noninvasive assessment of normal anatomy and anatomic variant of celiac trunk. Three-dimensional images in combination with multiplanar reformation (MPR) images are used to study his anatomical origins and the major branches. In fact, in this present case, dissection was confirmed by autopsy and it was be not possible to view the intimal flap in CT. Based on the classification of superior mesenteric artery dissection established by Zerbib et al. , the disTheoretically, if interruption of circulation in superior mesenteric artery, the residual lumen, the collateral circulation from celiac trunk and the inferior mesenteric artery should provide proper blood flow to avoid ischemia. Thus, the thrombosis of the origin of CMT had a lethal effect because it caused the full stoppage of the splanchnic arterial supply and can lead to the necrosis of the gastro-intestinal tract . So, CMTGiven that the dissection of common CMT is extremely rare, optimal management has not yet been established and in the both cases of CMT thrombosis previously reported ,8, surgiIn literature, the choice of the repair method of splanchnic arteries dissection has been applied depending on the severity of the dissection and the patient’s hemodynamic status. In the absence of visceral mal perfusion and as long as the patency of the vessel is well compensated by the collateral circulation, most authors recommend initial conservative treatment . But, inNowadays, open surgery is only recommended when bowel necrosis or peritoneal irritation is found and when endovascular treatment fail. So, for emergency, it may be necessary that physicians performing endovascular treatment cooperate with ones doing gastrointestinal surgery.4The CMT is an exceptionally rare anatomical variation and when it is observed, it is desirable; that a surgical treatment be planifed to avoid any possible abdominal complications. This can be easily achieved by multidetector computed angiography.We have no conflicts of interest to disclose.We have no source of funding for this reported case.The case is exempt from ethical or ethnical approval, according to Tunisian Medical Ethics Committee.“Written informed consent was obtained from the patient’s guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request”.Study conception: Mabrouka Boukoucha.Data collection: Alifa Daghfous, Abdelwahed Yahmadi, Hakim Znaidi.Writing: Mabrouka Boukoucha.Critical review and revision: all authors.Final approval of the article: all authors.This case is not interested to be registered in a publicly accessible database.Mabrouka Boukoucha.Not commissioned, externally peer-reviewed.


Dysgerminoma is an extraordinarily rare neoplasm arising from the malignant germ cells of the ovary. Early antenatal diagnosis and proper management of the neoplasm to improve maternal-neonatal results are the considerable challenges facing the gyne-oncologist. We summarize the clinical features and discuss treatment strategies of the ovary dysgerminoma (OD). Besides, we also review the literature on OD in PubMed, Web of Science Core Collection, Library of Congress, and LISTA from 1939 to 2019 to evaluate its clinical characteristics, feto-maternal compromise, management, and fertility outcome.A 25-year-old pregnant woman reported lower abdominal pain and vomiting.The patient was diagnosed as right OD.She received a cesarean section due to severe abdominal pain, delivered a healthy girl at 38 C 4 weeks of gestation, and accepted fertility-preserving surgery. However, the patient refused chemotherapy postoperatively.The patient was followed up 42 days, 3 months, and 6 months after surgery, and no tumor recurrence was observed.OD has non-specificity characteristics, including age, symptoms, image date, and tumor marks. However, these abnormal indicators may provide some evidence for accurate antenatal diagnosis. The management strategies should be considered comprehensively on an individual basis, and fertility-preserving surgery should be carried out in the second trimester if further pregnancy is desired. Adjuvant chemotherapy needs to be applied to the treatment of OD patients with The International Federation of Gynecology and Obstetrics (FIGO) stages II, III, and IV and timely chemotherapy is suggested if there are several weeks before the expected date of delivery. The overall prognosis of OD patients is excellent. OD is the most common subtype of MGCT and often occurs in adolescence and early adulthood.,7–10 In pregnant women, OD patients only account for about 0.0002% to 0.001%, and OD usually has a unilateral onset and is diagnosed at an early stage. It is difficult to achieve a large sample of OD due to its relatively low incidence. Thus, more studies are needed to summarize the clinical features and determine the optimal management strategies of OD. Furthermore, OD associated with mental retardation in pregnant women is even rarer. Therefore, the purpose of this study is to report our seldom case, as well as to review the literature on OD features, differential diagnosis, management strategies, and prognosis of pregnant patient with OD.Malignant germ cell tumor (MGCT) is an extraordinary rare ovarian cancer, which occupies no >5% of all ovarian cancers2This case report was approved by the institutional review board of the second hospital of Jilin University. Informed written consent was obtained from the patient for publication of this case report and accompanying images.3We report a case of OD with mental retardation and review relevant literature in PubMed, Web of Science Core Collection, Library of Congress, and LISTA from 1939 to 2019  was 6.9 cm, the head circumference (HC) was 26.6 cm, the abdominal circumference (AC) was 24.7 cm, and the femur length (FL) was 5.6 cm. The posterior wall of the placenta was grade I and the lower margin was 1.6 cm from the inner cervix. The amniotic fluid index (AFI) was 16.1. The ultrasound also revealed a hypoechoic mass in the lower part of the posterior wall of the uterus with a size of 14.8 cm × 8.5 cm. At 38 C 4 weeks of gestation, the ultrasound before admission of the patient revealed cephalic presentation. The BPD was 7.8 cm, HC was 31.5 cm, AC was 32.9 cm, and FL was 6.8 cm. The right wall of the placenta was late grade II and AFI was 12.2 cm. A U-shaped impression was found on the neck of the fetus. The ultrasound also revealed a hypoechoic mass located at the right rear of the uterus with a size of 23.0 cm × 12.5 cm  and a 10-minute Apgar score of 10 by CS.Intraoperatively, we found a large solid mass of 25 cm × 19 cm × 24 cm, which originated from the right ovary, with a moderate amount of pale-yellow ascites. The tumor was substantially lobulated, the texture was soft, the surface was intact, and the tissue was crunchy. Large blood vessels were visible, and the boundary between the tumor and adjacent organs  was not clear. No abnormalities in the appearance of the ovaries and fallopian tubes were found. At sectioning , followed by abdominal distention (19.6%), a growing mass (19.6%), multiple symptoms (18.6%), and non-symptoms (21.6%). In our study, abdominal pain was the main complaint of the patient and led to a cesarean section.OD can occur in women aged from 7 months to 70 years,,31 At sectioning, the tissue is lobulated, soft, fleshy, and gray-white or light tan. Occasionally, areas of hemorrhage and coagulative necrosis, which are typically related to cystic changes, can be observed. OD is most commonly unilateral in pregnancy, accounting for approximately 95%, while only 5% to 20% is bilateral.,34–36 In our study, the tumor was unilateral and showed substantially lobulated soft texture and the entire surface. This finding was consistent with the previous literature.,25,31–33Considering the gross pathologic features of OD, it usually presents well encapsulated and characteristically solid, with a diameter range from 8 to 15 cm.Regarding the microscopic pathologic feature of OD, it is like that of testicular seminomas. OD is composed of round cells with a uniform population, which is usually infiltrated by T lymphocytes and separated by fibrous strands. A large round or flattened nucleus that contains one or a few prominent nucleoli and clear eosinophilic cytoplasm can be observed in the center of cells. In addition, mitoses are always in large quantities.–39 In our study, ultrasound results show unclear boundaries, component lobules, with heterogeneous echogenicity. This feature suggests that the mass may be malignant. At CT, the lobular pattern may also be observed with a predominantly solid tumor accompanied by enhancing septa and areas of cystic change.,40 Kim and Kang claimed that calcification might be shown as a speckle. In magnetic resonance imaging (MRI), the most characteristic appearance is a solid mass, which is divided into lobules by fibrovascular septa. On T2-weighted images, the signal intensity is isointense or slightly hyperintense. On T1-weighted images, the signal intensity of OD is lower than that of muscles. Kitajima et al described that the MRI features of epithelial ovarian neoplasms were similar to those of multilocular cystic masses with irregular septations. Unfortunately, this patient did not undergo CT and MRI examinations during the hospitalization.Regarding the imaging features of OD, it is characterized by pure solids. In ultrasonography, they show well-defined borders, smooth lobulated contours, and component lobules, with heterogeneous echogenicity. At Doppler ultrasonography, they are abundantly vascularized at power and color. Literature reported that high levels of serum CA125 rapidly fell after chemotherapy. Previous studies described that partly OD patients exhibited increased NSE content and positive NSE of IHC,44 The serum levels and IHC expression of NSE in pediatric patients with OD may be of value in patient monitoring. In this study, CA125 and NSE increased significantly preoperatively. Some other indicators are abnormal, including HCG, AFP, CA-199, and CA-50. We hope that these positive indicators can provide some help for other scholars to diagnose OD accurately. Besides, LDH is another reliable indicator for predicting the effect of chemotherapeutic intervention.,45Considering the tumor marks of OD, CA125, and NSE may provide reliable evidence in OD.5.2OD has nonspecific features, which lead to the difficulty in making an accurate diagnosis. However, the age of patients, the imaging features of the neoplasm, and the abnormal tumor markers may help to determine a correct differential diagnosis. In general, OD should be distinguished from other purely solid masses of ovarian, including fibrosarcomas, granulosa cell tumors, Brenner tumors, epithelial ovarian, and metastatic carcinomas.5.3 OD staged IA-C could achieve acceptable surveillance by fertility-sparing unilateral salpingo-oophorectom. Bilateral salpingo-oophorectomy and hysterectomy are recommended for stage II and III diseases. In addition, if tumors do not invade the contralateral reproduction organs, unilateral salpingo-oophorectomy can be considered. The management strategies of stage IV patients mainly include fertility-sparing surgery, cytoreduction, and adjuvant chemotherapy.,49 Regarding second-look surgery of OD, if the tumor contains teratomatous elements or has residual disease, patients may benefit from second-look surgery after initial cytoreductive surgery and chemotherapy.,50 However, if the tumor does not have a teratomatous element, <5 cm of residual disease, or normal tumor marker levels after chemotherapy, second-look surgery is not recommended.With regard to surgery treatment of OD, accurate surgical staging is relatively critical to determination of the reasonable and accurate risk-based management. Currently, the FIGO classification is the most accepted method.In this study, the patient's mental retardation and lack of awareness of contraception may lead to repregnancy and increase the family burden. Thus, the patient's guardian strongly requests hysterectomy and bilateral appendectomy for the patient. However, the patient does not meet the indications for hysterectomy and bilateral appendectomy according to the FIGO stage, age, and grade of malignancy. Also, in China, especially in rural areas, women who have lost fertility function are not competitive in the remarriage population. What is worse, she has mental retardation, which means that it is difficult for the patient to reconstitute a family once she divorces after hysterectomy and appendectomy. Therefore, after careful consideration, we performed fertility-preserving surgery for the patient. The patient showed a satisfactory treatment effect during the follow-up visit. Chemotherapy is recommended based on pathological evidence, especially in cases with advanced-stage tumors, mixed epithelial and germ cell tumors, large tumor size, and rapidly increasing ascites. To date, platinum-based chemotherapy is the main strategy, including paclitaxel-carboplatin (TC) and bleomycin-etoposide-cisplatin (BEP).,51–56 In 2004, Hubalek et al claimed that TC could elicit an excellent response and posed no adverse impacts on the fetus. BEP is usually applied to the treatment of nonepithelial ovarian tumors of nonpregnant patients. However, the incidence of adverse advents  of etoposide is high.–60 Therefore, in pregnancy, paclitaxel-carboplatin chemotherapy instead of BEP is an optimized scheme for the treatment of nonepithelial ovarian cancer. The influence of chemotherapy during pregnancy on maternal survival must be considered. Literature,63 reported that chemotherapy during the first trimester could increase the incidence of fetal death, abortion, and malformations. Furthermore, the study also showed that the central nervous system, hemopoietic system, the eyes, and genitals were still vulnerable to sustained exposure to antineoplastic agents after organogenesis. However, increasing evidence suggests that chemotherapy for the second and third trimesters is relatively safe.With regard to chemotherapy of OD, OD with FIGO stages II, III, and IV are indicated for chemotherapy.In our study, the patient was diagnosed as OD staged II B, and chemotherapy was recommended by gynecologists postoperatively. However, she refused. Optimistically, there was no recurrence during the follow-up period of 6 months. We attribute this positive outcome partly to the low malignancy of the tumor and the standard and thorough operation carried out by a gynecologist with >30 years of experience, and partly to the short follow-up period.5.4 Besides, age over 45 years is also a significant predictor of recurrence. In most cases, tumors are detected early, which contribute to accurate prognosis. The prognosis of early-stage OD patients is excellent,,49,67 and the overall 5-year survival rate is approximately 100%.Residual disease, tumor markers, the FIGO stage, and the volume of the residual tumor are all the critical factors of prognosis.6In conclusion, features of OD, including age, symptoms, images date, and tumor marks, have non-specificity. However, these abnormal indicators may provide some evidence for accurate antenatal diagnosis. The management strategies should be considered comprehensively on an individual basis, and fertility-preserving surgery should be carried out in the second trimester if further pregnancy is desired. Adjuvant chemotherapy needs to be applied to the treatment of OD with FIGO stages II, III, and IV. If there are several weeks before the expected date of delivery, timely chemotherapy is indicated. The overall prognosis of OD patients is excellent.Conceptualization: Tong Yu, Shu-Yan Liu, Man-Hua Cui.Data curation: Xi-Wen Zhang, Li-Rong Zhai, Dong-Wei Huang, Zhen-De Jiang.Formal analysis: Xi-Wen Zhang, Dong-Wei Huang, Zhen-De Jiang.Methodology: Li-Rong Zhai, Zhen-De Jiang, Shu-Yan Liu.Resources: Dong-Wei Huang.Supervision: Tong Yu, Man-Hua Cui.Writing – original draft: Xi-Wen Zhang, Li-Rong Zhai.Writing – review & editing: Tong Yu, Shu-Yan Liu, Man-Hua Cui.


Chordomas are rare low malignant neoplasm arising from remnants of the notochord with predilection site of the clivus or the os sacrum. Standard therapy is radical excision and adjuvant radiation. Due to invasive growth and adjacent to vital structures resection is often incomplete, and therefore, local recurrence is frequent. First, to the best knowledge of our authors, we present a 70-year-old man with a recurrent chordoma infiltrating the corpus cavernosum. Asymptomatic recurrence was diagnosed by magnet resonance imaging according to the standard follow-up. Our interdisciplinary tumor board recommended surgical resection. We performed a total penectomy and perineal urethrostomy to achieve negative resection margins and preserve best quality of life for the patient. Chordoma is a rare malignancy with an incidence reported up to 0.08/100,000 person per year with a predominance in male patients according to the Surveillance, Epidemiology, and End Results (SEER) . IndepenHere, we report about a rare case of local recurrence of a sacral chordoma after multiple resections. The patient was referred to our urological department for surgical treatment of a clinically inapparent recurrence located on the basis of the corpora cavernosa. We performed a complete resection of the recurrent chordoma by total penectomy and perineal urethrostomy.A 70-year-old patient was referred to our Department of Urology, Faculty of Medicine, Munich Germany, for a surgical treatment of a recurrent chordoma located at the radix penis. The primary chordoma of the os sacrum was diagnosed in 1996 and primarily resected. In the following eight years, the patient underwent three resections (R2) of local recurrences before starting medical therapy with imatinib 400 mg per day in 2009. In 2014, a dorsal complete compartment resection was performed for a fifth local recurrence (R0). Imatinib was paused until in the same year, a single pulmonary metastasis was diagnosed and treated by surgery (R0). Consequently, the medical therapy with imatinib was restarted. In the following year, a local recurrence of the proximal femur was diagnosed. Furthermore, the complete resection (R0) of the gluteal metastases was performed in 2017. In February 2019, another pulmonary metastasis was treated by atypical pulmonary resection (R0).The follow-up computer tomography (CT) and magnet resonance imaging (MRI) indicated a suspicious lesion of the radix penis . TherefoFor preoperative preparation, a standard blood sample was taken, an abdominal CT scan in order to plan surgical procedure was performed and, antibiotic perioperative prophylaxis was given. We performed a total penectomy and perineal urethrostomy Figures . The intThe macroscopic preparation showed an asymmetric indurated lesion with a maximal expansion of 4.1 × 2.5 × 2.6 cm. The resection margin to the corpus cavernosum was measured 0.3 cm and to the corpus spongiosum 1.9 cm. The tumor showed a suppressive, but not infiltrative growth of the corpus cavernosum.The microscopic evaluation revealed a microcystic and partly solid tumor embedded in a myxoid and chondroid matrix Figures . In the Local recurrence of chordoma is common even after aggressive surgical excision of primary tumor depending on surgical margins , 6. IncoMost penile tumors are squamous cell carcinoma . MetastaThere is one case that reported of a primary vaginal chordoma treated successfully by surgery; other than that, no chordomas of the genital region have been described so far .We describe the first case of a recurrent chordoma located at the corpus cavernosum treated with total penectomy and perineal urethrostomy. For best oncological outcome, wide resection margins were achieved and perineal urethrostomy was performed to preserve continence.The treatment of chordoma and its recurrence disease demands an aggressive surgical excision for best long-term survival in consideration of conserving vital structures and preserving best quality of life.


Aneurysm of the left brachiocephalic vein is a very rare clinical disease and only 40 cases have been reported so far.The patient was a 61-year-old woman with no related medical history. She underwent CT to investigate the cause of a cough and a mass was noted in the anterior mediastinum. Dynamic computed tomography with contrast medium injected into the left basilic vein demonstrated the venous aneurysm with blood flow to the left brachiocephalic vein. The patient had no symptoms, but because of the risk of pulmonary infarction and aneurysm rupture, the aneurysm was surgically resected. A median sternotomy was a reasonable approach because of the fragility of the venous aneurysm wall with little working space in the anterior mediastinum.We diagnosed an aneurysm of the left brachiocephalic vein on preoperative imaging and excised it through a median sternotomy. The venous wall was thin and fragile in some areas and so this approach was appropriate in view of the possibility of intraoperative injury. Aneurysms of the left brachiocephalic vein (LBCV) are extremely rare; only 40 cases have been reported so far. They are usually asymptomatic and most often discovered incidentally .Recent reports have indicated that surgery is the treatment in many cases because of the risk of thrombus formation and rupture of the aneurysm, but nearly half the patients are followed up and no treatment guidelines exist . We repoThe patient was a 61-year-old woman with no related medical history. She underwent CT to investigate the cause of a cough and a mass was noted in the anterior mediastinum. Blood biochemistry tests including tests for tumor markers yielded normal results. Dynamic CT of the chest was performed with contrast medium , which was injected via a 370 syringe into the left basilic vein. The mass measured 54 entclass1pt{minimaentclass1pt{minimaentclass1pt{minimaentclass1pt{minimaLBCV aneurysm is a rare condition for which no treatment guidelines exist. Congenital conditions, trauma, inflammation, vascular regression changes, excessive blood flow pressure, and malignancy have been noted as causes of LBCV aneurysm. Many cases of LBCV aneurysm are asymptomatic and diagnosed incidentally. In some cases, a close examination of pain, cough, hoarseness, and respiratory distress may lead to diagnosis .When the contrast agent is administered into the left median cubital vein, the distance to the cardiac cavity is normally long, which may delay the arrival time of the contrast agent, interfere with arterial delineation, and prevent sufficient contrast agent from reaching the target vessel. Therefore, the contrast agent is usually injected into the right basilic vein. However, delivery through the right basilic vein may not enable sufficient contrast agent to reach the LBCV, which may prevent visualization. Moreover, if extensive thrombus occupies the venous aneurysm, the flow of contrast agent may be blocked and the mass may not be confirmed by imaging . We injeSurgery is an option when compression of an organ by the mass causes symptoms, bleeding in the anterior mediastinum results from rupture of the mass, or thrombus formation increases the risk of pulmonary infarction. However, many asymptomatic and untreated cases have been reported and the decision to treat them is controversial. Shen et al. reported a case of thrombus in an aneurysm that was noted on preoperative imaging , and thrIn view of the risk of thrombus formation and dissemination within an aneurysm, it is necessary to initially block blood flow between the aneurysm and brachiocephalic veins. Because the space between the sternum and the aortic root is narrow, the aneurysm wall can become compressed and can rupture during thoracoscopic surgery. In our patient, the neck was 8 mm in diameter and the ligation process was considered easy, but median sternotomy was the approach chosen because of the difficulty in securing the working space. The decision to excise an aneurysm should take into account the condition of the neck and the size of the aneurysm. In many cases, the maximum diameter of the aneurysm hampers the safety of the working space and median sternotomy might be preferable.In conclusion, we diagnosed an LBCV aneurysm on preoperative imaging and excised it through a median sternotomy. The venous wall was thin and fragile in some areas and so this approach was appropriate in view of the possibility of intraoperative injury.


Immunohistochemistry of lung specimens was compatible with a diagnosis of malignant melanoma which was confirmed to be BRAF wild‐type. Furthermore, positron emission tomography (PET) and contrast‐enhanced magnetic resonance imaging (MRI) of the head revealed multiple metastatic lesions in the brain, liver, and bones. The patient was referred to another hospital for specific treatment. After that, the patient's melanoma was confirmed to have the BRAF wild‐type gene and PD‐L1 expression was 80%. Then, combined therapy of nivolumab plus ipilimumab was subsequently administered.A 47‐year‐old Japanese man was referred to our hospital with a one‐week history of chest discomfort. Chest computed tomography (CT) revealed a mass in the right upper lobe suspected to be primary lung cancer. A biopsy of the mass using endobronchial ultrasonography (EBUS) with guide sheath (GS) was performed, and a black‐colored mass was observed which occluded almost all of the right B Black pigmentation of the airways (black bronchoscopy) can be caused by many etiologies. We herein reported a case of black bronchoscopy for pulmonary malignant melanoma.


Extraovarian Brenner tumors (EOBTs) are extremely rare and can be observed incidentally in both female and male patients, raising concerns regarding the origin of Brenner tumors.A 53-year-old postmenopausal woman presented with a nodular lesion in the left side of the corpus uteri, which was found at a routine health check. Macroscopically, the lesion appeared as a solid nodule with a yellowish-gray cut surface, approximately 6 cm in greatest diameter. Microscopically, the lesion consisted of well-defined epithelial nests and spindled stromal cells. Parenchymal cells expressed CK7, GATA3, CK5/6, 34βE12, and p63. A single layer of cavity-lined cells with umbrella-like shape showed apical Uroplakin III positivity. Stromal cells were positive for SMA, ER, and PR. The final diagnosis was EOBT and the patient was followed for 2 months with no recurrence.We report here the third case of EOBTs in the uterus. The combination of morphologic and immunohistochemical results supported the involvement of urothelial metaplasia in the development of EOBTs. The similarities between EOBTs and Walthard nests made Müllerian epithelium an attractive candidate as the cellular origin. Changes of tissue structure or sex hormones imbalance may lead to the translocation of Müllerian remnants to distant organs, explaining the pathogenesis of EOBTs. Brenner tumors (BTs) are relatively rare and account for approximately 5% of benign ovarian epithelial tumors . It has Except for the ovary, it has been reported that BTs may also involve extraovarian tissues. Extraovarian BTs (EOBTs) are extremely rare and mainly occur around the female reproductive system such as the uterus , 3, vagiIn this study, we report the third case of the uterus BT found beneath the serosa of the left corpus uteri. Immunohistochemistry was used to explore the phenotype of its epithelium and stroma.A 53-year-old female patient, gravida 1, para 1, with menopause at age 50, presented to the Department of Gynecology and Obstetrics with a 2-year history of a nodule in the left corpus uteri. The asymptomatic lesion was incidentally detected by ultrasound 2 years ago at a routine health check and had slowly increased in size  was performed on serial sections to further confirm the nature of the tumor and explore its pathogenesis. BTs and urothelium-derived tumors commonly show highly overlapping phenotypic features. The primary antibody panel, consisting of cytokeratin (CK) 7, CK20, GATA3, CK5/6, 34βE12, p63, PAX8, p53, p16 and cyclinD1, was used to gain more insights into its nature and whether BT involves the transitional-cells metaplasia. Consistent with previous studies, epithelial cells expressed strong to diffuse reactivity for CK7 and negativity for CK20  and ER/PR positivity in stromal cells suggests the involvement of sex hormones imbalance. Overlapping features of WNs and EOBTs implies a common pathogenesis from Müllerian epithelium. Changes of environment such as inflammation and surgery may promote the translocation of Müllerian remnants to distant tissues. Further studies are needed to determine the underlying molecular mechanisms.


To illustrate the technical feasibility and diagnostic value of including the assessment of the bladder and the ureters during a standard transvaginal ultrasound examination.We present four cases illustrating ureter and bladder pathology diagnosed by transvaginal ultrasound.In a first case, transvaginal ultrasonography in a woman with lower abdominal pain showed a calculus in the left distal ureter. The small echogenic lesion was easily detectable within the ureter lumen. A second patient, presenting with suprapubic pain, urgency and back pain, was also diagnosed with a ureteral calculus and additionally a hydroureter. The presence of a hydroureter is part of the differential diagnosis of any cystic structure in the pelvis. In a third case, an elderly woman, referred with uterine prolapse, transvaginal ultrasound examination showed a tubular anechoic structure lateral in the pelvis due to a hydroureter, illustrating the differential diagnosis with a hydrosalpinx. A fourth patient, presenting with hematuria, showed an irregular and highly vascularized mass in the bladder and was diagnosed with a transitional cell carcinoma.The bladder and pelvic part of the ureters are easily identifiable on transvaginal scan. Important pathology of the lower urinary tract and the distal ureters can be readily diagnosed by transvaginal ultrasound examination. The evaluation of the bladder and the ureters should therefore be part of the standard gynecological ultrasound investigation. Gynecological ultrasound assessment performed at the gynecology outpatient clinic or at the emergency department is often limited to the evaluation of the uterus and the ovaries. However, in the last decade, the introduction of ultrasound examination in urogynecology  and in tGynecological ultrasound examination is often performed in case of abdominal pain or abnormal bleeding. Besides gynecological pathology, the differential diagnosis includes urological conditions such as urolithiasis, infection or malignancy. Transvaginal ultrasound is readily available, cheap and quickly performed. Because the vaginal probe can be placed in the immediate vicinity of the bladder, the urethra or the distal ureters, the image is not hampered by overlying tissue, contrary to transabdominal scanning. As opposed to CT-scan or MRI, applying gentle pressure with the transvaginal probe, site-specific tenderness can be assessed, often guiding the examiner to the area of interest.The aim of our paper is to illustrate the feasibility and diagnostic value of including the evaluation of the bladder and the ureters during routine transvaginal ultrasound examination.To emphasize the importance of including the evaluation of the anterior pelvic compartment in routine gynecological scanning,we present a case series illustrating ureter and bladder pathology diagnosed by transvaginal ultrasound. Chronic conditions such as deep endometriosis or uro- gynecological pathology, such as a cystocoele, are beyond the scope of this paper. We focus on imaging technique, while mentioning some tips and tricks and discussing some pitfalls.A 29-year-old nulliparous patient presented to the emergency department with continuous pain in the left iliac fossa, slightly irradiating to the left lumbar region. She had one episode of vomiting the day before. Transabdominal ultrasound examination did not show any significant abnormalities. The bladder was empty and could not be evaluated. She was referred to the gynecology department to rule out any gynecological emergency such as an ovarian torsion. Transvaginal ultrasound examination showed a small, 4mm diameter, hyperechogenic calculus within the left distal ureter lumen, at 18mm from the ureteral ostium .A 41-year-old patient presented at the gynecology outpatient clinic with urgency, suprapubic pain and occasional right backpain in the last two weeks. Vaginal ultrasound examination revealed a 4mm diameter hyperechogenic distal right ureteric calculus. Cranially from the lithiasis, the ureter showed a progressive dilatation up to 25mm diameter . AbdominA 79-year-old woman (P2G2) was referred to the gynecology outpatient clinic for uterine prolapse. On vaginal ultrasound examination, a tubular thick- walled cystic structure with anechoic content was seen left of the uterus . The difA 74-year-old nulliparous woman presented at the gynecology outpatient clinic with hematuria since one month. According to the patient, a urological examination one month before, including a transabdominal scan, could not evidence any lesion. Vaginal ultrasonography showed an irregular, strongly vascularized lesion of 10x8mm protruding into the bladder cavity positioned cranially to the urethra . CystoscThe presented cases illustrate the importance of the evaluation of the anterior compartment of the pelvis during ‘gynecological’ ultrasound assessment. We believe that gynaecological ultrasound training should include the teaching of the normal anatomy of the structures surrounding the uterus allowing to make the right differential diagnosis, cope with incidental findings, and choose the most appropriate management.The urethra, the bladder and the distal part of the ureters are easily accessible by transvaginal ultrasound. As soon as the bladder shows minimal filling, the anechogenic bladder content is visible on ultrasound. To differentiate a distended bladder from a large anechogenic (ovarian) cyst, the urethra is visualized at the most caudal part in the midsagittal plane, ventral of the lower anterior vaginal wall.The urethra is also a reference point in urogynecology to assess urethral hypermobility during Valsalva manoeuvre . Gentle The trigonum is delineated by the urethra and both ureteric orifices; it is localized ventral to the anterior fornix of the vagina. Site-specific tenderness may be present in case of cystitis or deep vesicouterine endometriosis . The blaThe bladder walls are generally regular, especially if the bladder is full. Irregular, highly vascularized protrusions into the bladder lumen are highly suspicious for transitional cell carcinoma (TCC)  Figure , while dEspecially in elderly women, the differentiation between vaginal/uterine bleeding and hematuria may be difficult to deduce from the patient’s history. In case of urinary incontinence, reported bloodstained underclothing may as well be from urological as from gynecological origin.The ureter can be seen at vaginal ultrasonography as a thin relative hypoechogenic stripe tangential to the posterolateral bladder wall , typicalAlternatively, to find the orifices of the ureters, we scan in the transverse plane between the urethra and the lower posterior bladder wall till both the ostia become visible as two small, round hypoechogenic spots . RotatinMost of the time the lumen of the ureter is empty. Using a high-frequency probe with appropriated image-zooming, the passage of urine through the ureter can easily be evidenced as transient dilatation of the ureter lumen by anechogenic urine. The ureter wall thickness can be measured, and peristalsis is intermittently visible.Using color- or power-Doppler, a brief urine jet can often be seen from the orifice of the ureter into the bladder. Although this might be of some help in finding the ureter for the less experienced sonographer, color imaging is not a requisite for proper visualization of the terminal ureters.A feasibility study by Pateman et al.  showed tThe distance between the urethra and the ostia of the ureters depends on bladder filling. Typically, the ureteral orifice is about 2 to 3 cm from the urethra and both ureteral ostia are about 3cm from each other . AlthougA ureteral calculus is visible on ultrasound as a small bright hyperechogenic lesion within the lumen of the ureter . Often some dilatation of the ureter proximal to the calculus is seen, as well as ureteral peristalsis.In case 3, the dilatation of the proximal ureter was prominent and, in transverse section, could be mistaken for an adnexal cyst .If a rounded cystic structure is evidenced on ultrasound examination, the probe should always be turned over 90° to exclude a tubular structure. The differential diagnosis of an anechogenic or hypo- echogenic parauterine tubular structure includes a hydrosalpinx, a dilated vessel, a bowel loop and a hydroureter. A dilated ureter is well delineated, does not have persistent flow on color- or power Doppler examination and can be followed caudally till it enters the bladder wall and ends at the ostium of the ureter.Our cases illustrate the importance of the sonographic evaluation of the anterior compartment including the bladder and the distal ureters in lower abdominal pain or abnormal bleeding. Common urological causes of lower abdominal pain in women are cystitis, urethritis, lithiasis, and urinary retention . SelectiThe differential diagnosis of abnormal bleeding in elderly women includes, besides gynecological pathology, intestinal causes  and urological conditions, such as carunculae of the urethra, hemorrhagic cystitis, bladder or renal cancer, lithiasis, stricture of the ureter or kidney disease .Although endometriosis was beyond the scope of this paper, we acknowledge the importance of including the assessment of the ureters during each systematic sonographic expert examination in the preoperative endometriosis setting . In caseA woman presenting with lower abdominal pain or abnormal blood loss is usually referred to a gynecological unit for a transvaginal ultrasound scan. Ultrasonography is a cheap and dynamic tool, not only to assess the gynecological organs, but also to easily identify and evaluate the bladder and pelvic part of the ureters.Important pathology of the lower urinary tract causing symptoms mimicking gynecological pathology can be readily diagnosed by transvaginal ultrasound examination. The evaluation of the bladder and the ureters should therefore be part of the standard gynecological ultrasound investigation.


Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare ischemic bowel disease with venous occlusion resulting from the proliferation of smooth muscles in the venous intima. In most patients, the disease affects rectosigmoid colon and causes persistent abdominal pain and hematochezia, which is similar to inflammatory bowel disease (IBD). In addition, it is difficult to make a precise diagnosis of IMHMV without surgery.An 81-year-old woman was admitted to our hospital with mild abdominal pain, nausea and vomiting. Repeated adhesive ileus was suspected due to the previous open and laparoscopic surgeries. Surgery was planned to treat small bowel obstruction. Intraoperatively no adhesive lesions were observed. However, a mass lesion was seen at the terminal ileum, which was suspected to have caused her bowel obstruction. Partial resection of the small intestine was performed. Macroscopic and histopathological examinations of the excised specimen showed circumferential ulceration with scarring, a thickened venous wall with active inflammation, and fibrotic changes that consequently produced stenosis and obstruction of the venous lumen in the subserosa. Additionally, Elastica van Gieson staining demonstrated thickening of the venous intima. The final diagnosis was IMHMV. At two years and 8 months after the operation, the patient was well without any additional medication.IMHMV of the small intestine is rare. We described a case of IMHMV that was associated with ileus. Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare ischemic bowel disease without thrombus and with venous occlusion resulting from the proliferation of smooth muscle in the venous intima . In mostAn 81-year-old woman was admitted to our hospital with mild abdominal pain, nausea, and vomiting. She had a history of open appendectomy and laparoscopic cholecystectomy. She had received conservative therapy for small bowel obstruction twice in the previous year. Thus, adhesive intestinal obstruction was suspected at admission. A physical examination showed a slightly distended abdomen with diffuse and mild abdominal tenderness. A hematological examination showed elevated inflammatory markers . Abdominal X-ray showed air fluid level of the small intestine. Contrast-enhanced computed tomography (CECT) demonstrated stenosis and the wall thickness of the terminal ileum and intestinal distention of the proximal small intestine, which was the site that had been affected in the first bowel obstruction event one year previously Fig. . We planIMHMV is a rare disease that was first described by Genta and Haggitt in 1991 . It is cIn previous reports, no cases of small intestinal IMHMV were diagnosed without bowel resection. The preoperative diagnosis of IMHMV is still difficult because there are few specific image findings. However, a precise preoperative diagnosis of IMHMV is important, since IMHMV can be completely cured by resection of the affected area , which iEnterocolic lymphocytic phlebitis (ELP), known as mesenteric inflammatory veno-occlusive disease (MIVOD), is a venogenic intestinal disease similar to IMHMV in terms of its association with intimal hyperplasia of the mesenteric veins –18. HoweWe described a rare case of IMHMV of the ileum with intestinal obstruction.


Fibromuscular dysplasia (FMD) is a type of unexplained nonatherosclerotic vascular disease that usually involves the renal and internal carotid arteries and rarely involves the mesenteric artery. Mesenteric artery FMD is difficult to distinguish from Crohn’s disease (CD) and Behcet’s disease (BD) solely based on symptoms. Patients with mesenteric artery FMD can present with an acute abdomen, but case reports of patients who have a long medical history and undergo multiple bowel resections are extremely rare.The patient was a 45-year-old woman with an 11-year history of intermittent lower abdominal pain and fever. At the age of 34 years, she underwent right hemicolectomy and appendectomy due to an acute abdomen. She suffered from oral ulcers between 34 and 36 years old. A clinical diagnosis of presumed CD was made by the age of 41, and she was treated with mesalazine; however, the effect was poor. At the age of 42, she came to our centre, and based on her atypical symptoms and examination results, we thought she had CD. Hence, she was treated with glucocorticoids for 3 years. However, when she was 45, due to steroid dependence, thalidomide tablets were added. Unfortunately, she suffered from another episode of intestinal obstruction. Therefore, she underwent enterectomy. The postoperative histopathological diagnosis was mesenteric artery FMD. She no longer underwent pharmacotherapy after the surgery. Although she did not have any of her previous symptoms and postoperative colonoscopy showed no signs of recurrence, splenomegaly and abnormal routine blood results were still present.Patients with mesenteric artery FMD can present with an acute abdomen. In addition, the symptoms and endoscopic manifestations of mesenteric artery FMD may appear similar to CD and BD. Hence, it is difficult to make a clear clinical diagnosis and proceed with treatment. Mesenteric artery FMD often requires surgical pathology to confirm its diagnosis. For patients who suffer from this disorder, surgery may be the best choice to improve the patient’s quality of life.The online version contains supplementary material available at 10.1186/s12876-021-01702-y. FMD is a type of unexplained nonatherosclerotic vascular disease that usually involves the renal and internal carotid arteries but rarely involves the mesenteric artery. Clinical symptoms of mesenteric FMD, including postprandial abdominal pain, incomplete intestinal obstruction, multiple or focal ulcers of the intestinal mucosa, abdominal distension, and abdominal vascular murmur, with or without fever, are difficult to distinguish from those of CD and BD. Notably, FMD of the mesenteric arteries can lead to multiple bowel resections. However, case reports of patients who have a long medical history and undergo repeated enterectomies are extremely rare. To date, there are very few studies and only limited literature reporting a few cases with this condition in Asia, Europe, North America and other regions.The patient was a 45-year-old woman with an 11-year history of intermittent lower abdominal pain and fever. At the age of 34, she developed intermittent lower abdominal pain with irregular fever, which was easily induced after a meal. After undergoing computed tomography (CT), X-ray, ultrasound, and routine blood examinations, right hemicolectomy and appendectomy were performed due to an acute abdomen. In the postoperative specimen, a huge ulcer (7 × 5 cm) was found at the junction of the ileum and colon. The histopathological examination of the biopsy specimen showed chronic inflammatory changes in the intestinal tissue.The patient had a history of oral ulcers between 34 and 36 years old. At the age of 41, intermittent lower abdominal pain accompanied by fever recurred. Colonoscopy  was performed in a local hospital, revealing a large ulcer in the terminal ileum. The histopathological examination of the lesion showed the formation of inflammatory granulation tissue. Plain films of the abdomen revealed incomplete intestinal obstruction. Splenomegaly was found on an ultrasound examination. The clinicians at the local hospital considered that she was likely to suffer from CD, so she was treated with mesalazine (4 g daily). The effect, however, was poor. When she was 42 years old, she was referred to our centre for systemic diagnosis and treatment.2), and an enlarged spleen. She had a history of anaemia for more than 20 years and no history of hypertension.The positive signs on the physical examination at admission were anaemia, weight loss (body mass index: 15.63 kg/m9/L (nl within 4–10 × 109/L) and her thrombocytes were reduced at 70 × 109/L (nl within 100–300 × 109/L); her haemoglobin (Hb) was low at 77.0 g/L (nl within 110–150 g/L); anti-small intestinal goblet antibody was positive; C-reactive protein (CRP) was elevated at 57.5 mg/L (nl < 8 mg/h); and here erythrocyte sedimentation rate (ESR) was elevated at 28 mm/h (nl < 15 mm/h).Routine blood examination revealed that her leukocytes were low at 2.55 × 10The computed tomography enterography (CTE) examination revealed an unevenly thickened and enhanced wall of the sigmoid colon, splenomegaly, and portal hypertension  and proximal ileostomy . Her steroid medication was ceased after the surgery. In addition, antiplatelet therapy was not initiated. Although she did not have abdominal pain with fever again, and a re-examination by colonoscopy showed no signs of recurrence .FMD, or muscle fibre hyperplasia, is a non-inflammatory and non-atherosclerotic vascular disease that mainly involves the middle and small arteries . FMD is FMD can be clinically symptomatic or asymptomatic, and its haemodynamics can be variably affected, which mainly depends on the distribution of the involved vessels and the type and severity of the disease. FMD involving the mesenteric arteries is fairly rare , 8, and In this case, FMD of the mesenteric arteries was mainly distributed in the patient’s intestinal wall  around the bowel wall that lack other multivessel involvement. In addition, the prognoses of these diseases are different. For patients with CD, pharmacotherapy can be used to control the disease, while surgery may be a curative method for patients who suffer from MAVD/D. In terms of patients with mesenteric artery FMD, which usually involves multiple vessels in other organs, surgery may be the best choice to improve the patients’ quality of life.Additional file 1.Figure 1-6 The small-intestine stimulation enhanced computer tomography (CT) examination revealed an unevenly thickened and strengthened wall of the sigmoid colon, splenomegaly, and portal hypertension. Figure 7-9. Colonoscopy revealed two ulcers in the small intestinal mucosa, multiple ulcers and erosions throughout the colon, and normal mucosa between the ulcers. Figure 10-11. The specimen of the diseased ileum (approximately 10 cm) after the enterectomy.Figure 12. Postoperative histopathological examination showed inflammation of the arterioles of the intestinal wall, obvious stenosis or even occlusion of the arterial vascular lumen, proliferation of arterial smooth muscle and destruction of elastic fibres. Figure 13-14. Re-examination of the electronic colonoscopy after surgery showed no signs of recurrence. Figure 15-16. Re-examination of the CT after surgery showed splenomegaly still existed. Figure 17. The patients underwent abdomen CT revealed that her renal vessels were not involved.