Congenital giant megaureter (CGM) is uncommon in pediatic population, defined as congenital localized or total dilatation of the ureter to over 10 times the normal diameter with a normal bladder. Herein, we reported an entirely dilated CGM presented as neonatal bowel obstruction in a newborn baby.Our experience has suggested that CGM should be considered as a differential diagnosis of abdominal distension and occlusive syndrome. In addition, we reviewed the epidemiology, pathogenesis, diagnosis, and therapies of this rare condition by analyzing previously reported case.2A 1-day-old full-term girl (3.6 kg), with a prenatal ultrasonographic and fœtal magnetic resonance imaging (MRI) diagnosis of “intestinal dilatation” since the end of the fifth month of gestation, with bilateral kidney dilatation and right-sided renal parenchyma thinning , was traThe first passage of meconium had been regular, and then she developed a bowel obstruction. The renal biochemistry and urinalysis findings were normal. A plain abdominal x-ray  showed aAn ultrasound scan showed an important small bowel dilatation; with bilateral renal dilatation. Because of abdominal distension and unresponsive bowel obstruction to nursing, it was decided to explore the child with a laparotomy. At laparotomy, we found a huge tortuous right megaureter that represses all the intestinal tracts backward. A cutaneous ureterostomy was performed. The postoperative period was uneventful. A MAG-III scan was performed and had shown a very low cortical caption in the right kidney with an estimated function of 8%. Due to the occurrence of two urinary tract infections, it was decided to perform a right ureteronephrectomy. The baby is now a 10-year-old healthy child, with a normal left renal function on DMSA scan and a normal bladder and urethra on voiding cystourethrography.3The first CGM was published by Chaterjee SK in 1964.The diagnosis of CGM was habitually based on history, physical examinations, and imaging examinations.Ultrasound is an effective method of assessing the fetal urinary tract and can deliver the correct diagnosis in the vast majority of cases.The current case, although misdiagnosed, was detected prenatally. At birth, abdominal distension and bowel obstruction suggested immediate surgical treatment, without prior complete urological examination which is moreover mandatory to exclude associated anomalies and decide the appropriate surgical procedure.The treatment of congenital megaureter is controversial. Some authors proposed early surgical intervention, whereas others suggested temporary conservative treatment for most patients.4Cases of CGM have been reported in newborn infants or there was a history of a mass since birth in other cases of CGM(4). Our experience has suggested that, in the differential diagnosis of abdominal distension and occlusive syndrome, CGM should also be considered.


Levetirazetam is an antiepileptic drug with psychiatric adverse reactions. It includes psychosis, paranoia or hallucinations. The frequency is less than 1%.To describe a case of Psychosis produced by LevetirazetamRetrospective review of clinical records and complementary test, including psychiatry, electrophysiology and neurology. Diagnosis schales such as Salamanca Questionnaire were used as suport.A 42-year-old woman diagnosed with tuberous sclerosis and undergoing treatment with levetirazetam acudes to the emergency department for behavioral disorders. She has presented an episode of aggression against a relative threatening him with a kitchen knife. The family reports that since the change in antiepilepticus 1 month ago, the patient has presented strange behaviors. Te Patient is conscious, uncooperative. Barely Approachable. Suspicious of her surroundings, with psychomotor restlessness, self-reference ideas and sparse speech. Auditory hallucinations seem to be present, as well as depressed and irritable mood. Psychic and somatic anxiety is found.Levetirazetam is discontinued, being replaced by valproic acid. Risperidone is started at a 3 mg dose. Treatment is well tolerated, and clinical stability is achived. Cluster A personality traits are found. Complementary test Blood and Urine simples, Imaging tests (CT and MRI), electroencephalogram and Electrocardiogram show no alterationsLevetirazetam can cause psychiatric adverse effects. it is important to make a proper diagnosis before a first psychotic outbreak in later life. Drugs that can produce psychiatric side effects should be identified and patients should be inform.No significant relationships.


Myositis ossificans circumscripta is a self-limiting, benign, ossifying lesion that can affect any type of soft tissue. It is most commonly found in muscles as a solitary lesion. A history of recent trauma has been reported in approximately 50% of cases. Clinically, MOC presents as a painful swelling, which rapidly increases in size. The pain and inflammatory symptoms spontaneously disappear after approximately 2–6 weeks, and the mass stabilizes or decreases. Radiologically, myositis ossificans circumscripta can be divided into two phases. The first is the acute phase, which is followed by the mature phase 2–6 weeks later. During the acute phase, the radiological aspect does not show any specific abnormality. In the mature phase, plain radiographs and computed tomography show blurred calcifications around a hypodense center. We describe here the first case of myositis ossificans circumscripta, with appropriate follow-up, occurring during sunitinib exposure.We report a case of myositis ossificans circumscripta in a 34-year-old man (ethnicity unknown) receiving sunitinib for metastatic alveolar soft part sarcoma of the left thigh after surgery and radiotherapy. Four months after the first dose of sunitinib, the patient experienced painful swelling in the surgical scar area. Magnetic resonance imaging showed diffuse and marked edema of the anterior compartment of the thigh, without nodular lesions circumscribing a central core, and without bone signal abnormality. The increased visibility of the intermuscular fascia and convergence of normal muscle fibers (black hole effect), without the displacement seen in tumors, were suggestive of myositis. Therefore, antiangiogenic treatment was discontinued, and the symptoms rapidly resolved within a few days. Three weeks after the discontinuation of sunitinib, the inflammatory findings completely disappeared. Two months after the diagnosis of myositis ossificans circumscripta, plain radiographs and computed tomography showed an extensive calcified mass measuring > 12 cm. The continuation of favorable clinical outcomes was confirmed.To the best of our knowledge, this is the first case of myositis ossificans circumscripta with appropriate follow-up occurring during sunitinib exposure. Owing to multimodal treatment of sarcoma, we cannot rule out the radiotherapy and surgery causality. Myositis ossificans circumscripta (MOC) is a self-limiting, benign ossifying lesion that can affect any type of soft tissue, including subcutaneous fat, tendons, and nerves. However, it is most commonly found in muscles as a solitary lesion , 2.Herein, we report the case of a patient who developed MOC during treatment with sunitinib after surgery and radiotherapy for metastatic alveolar soft part sarcoma (ASPS).A 34-year-old man (ethnicity unknown) with a history of childhood eczema and asthma presented with an ASPS of the left thigh 10 cm) in June 2015 , and grade 1 diarrhea.In May 2016, 4 months after the first dose of sunitinib, the patient experienced painful swelling in the surgical scar area. Magnetic resonance imaging (MRI) showed diffuse and marked edema of the anterior compartment of the thigh characterized by high signal intensity on T2-weighted images, without nodular lesions, circumscribing a central core, and without any bone signal abnormality. The increased visibility of the intermuscular fascia and the convergence of normal muscular fibers (black-hole effect), in contrast with the displacement seen in tumors, were other characteristics suggestive of myositis  showed an extensive calcified mass > 12 cm in height in the anterior muscle compartment, which was continuous with the cortical bone through periosteal ossification in some places. Finally, on MRI, the mass was hypointense without enhancement with gadolinium on T2-weighted images, corresponding to a calcified mass Fig. .Fig. 4ReThe continuation of favorable clinical outcomes was confirmed. Five months after the diagnosis of MOC, the patient remained asymptomatic. A plain radiograph showed global ossification of the mass corresponding to a typical MOC fully incorporated into the femoral cortical bone Fig. .Fig. 5PlThe pathophysiology of MOC is poorly understood, but it likely results from the inappropriate differentiation of fibroblasts into osteogenic cells . This maThe epidemiology of MOC is also poorly understood. It occurs most often in adults aged < 30 years. However, the risk factors are poorly identified. A history of recent trauma has been reported in approximately 50% of cases.Clinically, MOC presents as a painful swelling, which rapidly increases in size. Additionally, patients may present with associated paresthesia, weakness, lymphedema, and venous thromboembolic disease when the lesion compresses nearby neurovascular structures . Some loAfter approximately 2–6 weeks, the painful and inflammatory symptoms spontaneously disappear, and the mass stabilizes or decreases. This spontaneous evolution distinguishes MOC from other lesions, particularly malignant tumors. This short clinical course helps to avoid unnecessary biopsies or surgery.Radiologically, there are two phases. The first is the acute phase, which is followed by the mature phase 2–6 weeks later.In the acute phase, plain radiographs are normal, and ultrasound may show a non-specific hypoechoic area. Magnetic resonance imaging (MRI) shows a T2 hypersignal and clear focal contrast . A charaIn the mature phase, the most useful tests are plain radiographs and computed tomography, which show blurred calcifications around a hypodense center. Ultrasonography shows hyperechoic arciform bands located at the periphery of the initial hypoechoic focus. This radiological evolution at 2–6 weeks after presentation is pathognomonic for MOC.Management is often non-surgical, based on the “RICE” protocol  or cryotherapy. In a case report of a football player with post-traumatic MOC in the quadriceps, two doses of pamidronate were associated with improvements in both clinical and imaging features . SurgicaTo the best of our knowledge, we cannot find prior descriptions of MOC occurring during sunitinib exposure. We found a letter describing “myositis” in patient treated with sunitinib, but there is no appropriate follow-up that could confirm the diagnosis of MOC .Trauma is the most frequently described etiology of MOC. This may be a single trauma or repetitive minor trauma. Here, we considered cancer surgery to be a major trauma. The symptoms of MOC appeared 7 months after surgery.To the best of our knowledge, no association between radiotherapy and MOC occurrence has been reported in literature.Sunitinib is a tyrosine-kinase inhibitor with several targets  that are involved in tumor growth, pathological neoangiogenesis, and metastatic cancer progression. In preclinical models, platelet-derived growth factor receptor, but above all c-KIT, two well-known targets of sunitinib, play a key role in bone formation, homeostasis of mesenchymal stem cell, and differentiation into osteogenesis progenitors. We hypothesize that in healing scars, sunitinib exposure modified the mesenchymal stem cell differentiation regulation into osteoblast-like cells –14. AnotTherefore, the role of sunitinib in the pathogenesis of MOC cannot be excluded. However, MOC development is not a known adverse event with sunitinib in the summary of product characteristics, literature, or French pharmacovigilance database.Although the pathophysiology of myositis ossificans remains poorly understood, drugs are rarely mentioned among its etiologies. However, according to the international database of the World Health Organization (Vigilyze), among 50 cases of MOC, biphosphonates were involved in 31. In contrast, tyrosine-kinase inhibitors have not been implicated. A previous study mentions drug abuse as a cause of myositis ossificans, but without further precise details .Considering these factors and the timing of events, sunitinib responsiveness cannot be formally excluded.Herein, we report the case of a patient who developed MOC 7 months after neoadjuvant radiotherapy and surgery for metastatic ASPS. The natural course of MOC does not allow for a clear assessment of sunitinib liability, particularly in the context of a recent history of extensive surgery and radiotherapy.


The optic nerve(s) may be compressed by a number of intracranial and intraorbital masses. Compression may be isolated to the optic nerve or may involve other intracranial or intraorbital structures with variable presentation. A 26-year-old man presented with complaints of progressive painless visual loss in the right eye for eight months. Examination revealed isolated unilateral optic atrophy consistent with a diagnosis of compressive optic neuropathy. Magnetic resonance imaging of the brain showed compression of the anterior visual pathways due to a lesion radiologically compatible with clival chordoma. He underwent subtotal neurosurgical resection and pathology was consistent with chordoma. Although rare, isolated unilateral visual loss may be the only presenting manifestation of clival chordoma. Clival chordoma (CC) is a rare neoplasm that arises from remnants of the embryonic notochord. Common sites of chordoma development are the sacrococcygeal area and the skull base. Intracranial chordomas usually arise from the region of the clivus. Diplopia is a common complaint but isolated optic nerve compression is rare; among intracranial neoplasms, chordomas were found in less than 0.7% of cases. We describe a case of visual loss due to unilateral compressive optic neuropathy as the presenting manifestation of this uncommon intracranial tumor. A 26-year-old man presented complaining of progressive visual loss in the right eye (OD) for the past eight months. He denied headache, diplopia, or any other neurologic symptoms and felt otherwise well. His past medical history was non-contributory and he was taking no medications. Best-corrected visual acuity (BCVA) was 20/200 OD and 20/20 in the left eye (OS). Extraocular motility was full. External exam was normal and there was no proptosis or ptosis. Pupils were isocoric with a right relative afferent pupillary defect (RAPD). Intraocular pressure measurements and anterior segment examination were within normal limits in both eyes (OU). Fundus examination Figure  revealedThe standard computerized visual field Figure  showed aCC is a rare neoplasm with an incidence rate of less than 0.1 per 100.000 per year  and showHistologically chordomas show a typical pattern of lobules separated by fibrous bands. Each lobule displays vacuolated, atypical neoplastic cells within a myxoid stroma . Age at In several large reviews of patients with intracranial neoplasms, chordomas were found in 0.1-0.7% of patients . IntracrVolpe and coworkers reviewed the neuro-ophthalmologic findings in chordomas and chondrosarcomas of the skull base . In the Although isolated and progressive unilateral visual loss from compression of the anterior visual pathway is an uncommon finding in CC, clinicians should be aware of this presentation. Neuroimaging typically demonstrates the compressive lesion and the origin at the clivus consistent with CC. Gross total resection is the best treatment.


Hepatic hemangioma (HH) is a congenital vasal malformation that seemed like the most probable benign liver neoplasm, composed of masses of blood vessels, which are anomalous in arrangement and size. In most cases, HH is asymptomatic, and patients have an excellent prognosis. According to research, the location and size of the mass are correlated with the symptoms and complications. Reports of spontaneous rupture of HH have been less reported in the literature. In this emergency condition, dynamic contrast-enhanced CT scanning, especially triple-phase computed tomography (CT) with delayed imaging, is preferred.Here, we presented two middle-aged female patients with spontaneous rupture of HH in our hospital. Following an accurate diagnosis of enhanced CT and emergency surgery, patients recovered well and were discharged from the hospital.Appropriate imaging studies, especially enhanced CT, and emergency surgery are indispensable for patients with spontaneous rupture of HH. As a surgeon, we need to pay attention to the asymptomatic patient with HH. Hepatic hemangioma (HH) is the most probable benign tumor of the liver, and often asymptomatic, can be found accidentally during the imagological examination, has a prevalence of approximately 20%, and is more frequent in women. Clinically, the most common type is a cavernous hemangioma, and most patients have an excellent prognosis because of the benign nature of hemangioma . So, mosA 33-years-old woman was admitted to the emergency department with persistent hurt in the upper quadrant of the abdomen, which had occurred suddenly, and the pain was not relieved after resting or changing position. She denied a history of any medical condition (including hepatitis), recent trauma, or oral contraceptive use, and a family history of hepatic disease. On admission physical examination (PE), the following results are observed: temperature (T) is 36.5°C, heart rate (HR) 75 beats/min, respiratory rate (RR) is 20 times/min, and blood pressure (BP) is 110/75 mmHg. Tenderness in the right upper quadrant of the abdomen was noted. Cardiopulmonary function examination showed no obvious abnormalities. Laboratory examination results showed a hemoglobin (Hb) level of 98 g/L . The rest of the laboratory tests, including liver function and coagulation function, are normal. The abdominal enhanced-CT result  showed aA 36-year-old woman was admitted to the clinic with a chief complaint of “sudden right the upper quadrant pain for 14 h.” PE indicated abdominal pain, especially in the upper right abdomen. There was no obvious muscle tension and rebound pain. The results of the laboratory were normal. The enhanced abdominal CT said a mass in the right liver lobe, which was a patchy progressive enhancement shadow, a fusiform shape was visible in the adjacent liver capsule, and there was a small amount of fluid around the liver . The patHepatic hemangioma is the most probable benign neoplasm and is often found in women. The majority of HH is a cavernous hemangioma, with little chance of malignant transformation . After iReports of HH with spontaneous hemorrhage are less, causing a misunderstanding and ignorance of its serious complications . The incClinical manifestation of spontaneous rupture of HH consists of sudden abdominal pain, anemia that is secondary to a hemoperitoneum, and disseminated intravascular coagulopathy (DIC). Spontaneous rupture of HH is considered a life-threatening condition , as its At present, due to a poor understanding of the natural history of asymptomatic hemangiomas , patientThe raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Written informed consent was obtained from the patient for the publication of this case report. Written informed consent was obtained from the patient for the publication of any potentially identifiable images or data included in this article.BP and S-CL contributed to the planning and organization, collected clinical data, supervised the findings of this work, analyzed the results, and prepared the manuscript. QH aided in the data collection and supervision. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Case Presentation. A 73-year-old woman with no pathological history came to our emergency room with a one-month history of umbilicus enlargement. A 9-centimeter uncomfortable umbilical swelling with hard consistency was discovered during a clinical examination. An ovarian tumor with several local expansions was seen on an abdominal CT scan. It was linked to peritoneal metastases, one of which extends via a supraumbilical hernial orifice and into intestinal tissues in the same hernia sac. The umbilical tumor was removed from the patient. A moderately differentiated serous carcinoma with ovarian origin was identified in a periumbilical site on histological testing.  Sister Mary Joseph's nodule (SMJN) is a rare illness characterized by an umbilical mass caused by tumor metastases in the abdomen or pelvis. The most common main site of SMJN in women is ovarian cancer.  The presence of an SMJN is a rare but significant issue that clinicians must examine because it is associated with a bad prognosis. Early detection and diagnosis of the original lesion can lead to more effective treatment and a higher rate of survival. Sister Mary Joseph's nodule (SMJN) is a rare disorder characterized by an umbilical mass caused by tumor metastases in the abdomen and/or pelvis . AccordiThe most common main site of SMJN in women is ovarian cancer . SMJN miThe purpose of this case is to discuss a case of Sister Mary Joseph's nodule as a misdiagnosed ovarian cancer metastasis.A 73-year-old woman appeared with a firm umbilical mass that had been increasing for a month and had no history of an umbilical hernia. The patient was initially unconcerned about the lump because it was painless. The umbilical tumor had become uncomfortable two days before the presentation, causing the patient to seek medical help. She does not have any medical history.A healthy-looking woman was discovered during the physical test. An inflammatory and uncomfortable umbilical mass of 9 cm with hard firmness was discovered during the abdominal exam . It alsoA 12 cm suspicious tumor in the left ovary was discovered by pelvic transvaginal ultrasonography.We opted to finish with a pelvic scanner because of the importance of the pelvic mass, which exceeds the limits of ultrasound.The patient received an abdominal-pelvic computed tomography (CT) scan, which revealed a 12 × 11 cm left ovarian tumor with several local expansions. It was linked to intraperitoneal metastases, one of which is 9 × 8 cm in size and extends through a supraumbilical orifice with digestive processes in the same hernial sac .The results of the biochemical tests revealed a normal total blood count and electrolytes as well as a high CA-125 level (200 UI/ml).The patient was admitted to the hospital, and the diagnostic and treatment options were addressed with the patient and a multidisciplinary team. We first performed a resection of the umbilical mass with omentum reintroduction into the abdominal cavity and the removal of any remnant malignancies.The resection margins were devoid of tumor, and histological evaluation revealed a periumbilical site of a moderately differentiated serous carcinoma compatible with ovarian origin .We made the decision to perform an exploratory laparotomy. During surgery, a solid mass measuring 12 cm was discovered on the left ovary, along with a tiny amount of ascites. There were also intra-abdominal and omentum metastatic lesions found. Total hysterectomy, bilateral salpingo-oophorectomy, bilateral pelvic lymphadenectomy, and omentectomy were the procedures performed.Histopathology revealed an ovarian serous carcinoma, which was also found in the umbilical tumor. The lymph nodes in the inguinal and iliac arteries were positive, and the omentum had metastatic disease. As a result, the definitive diagnosis was ovarian cancer stage IV B (FIGO ovarian cancer staging).2) was started one month after hospital discharge and continued every three weeks for six cycles.The recovery went smoothly, and the patient was discharged a week following surgery. Chemotherapy with carboplatin (starting dosage AUC 5–6) and paclitaxel  and the genitourinary tract (12 to 35%) were the most common sites of primary neoplasia [5% and thThe ovaries (14 to 41%), endometrial (1 to 10%), and pancreatobiliary tree (12%) were the most commonly described main sites in women in the literature .SMJN's mechanism is unknown, but some theories have been offered, such as a direct expansion of the tumor, lymphatic dissemination, or hematogenous spread .An SMJN can take many forms, ranging from a soft, harmless nodule to an uneven, painful mass .An umbilical mass, in fact, can be the earliest indicator of an intra-abdominal or pelvic tumor with peritoneal metastases, and it is easy to miss during a physical exam. As a result, early detection and appropriate treatment are critical.In the presence of an umbilical mass, other diagnoses such as umbilicitis, umbilical abcess, umbilical hernia, umbilical endometriosis, omphalith, keloid, pyoderma gangrenosum, or foreign body can be made.To confirm the diagnosis of SMJN, a biopsy and histological testing are required. Adenocarcinoma is the most common histological type in about 75% of SMJN cases with intra-abdominal malignancy .An ovarian cancer with intraperitoneal metastases, one of which extends via a hernial supraumbilical orifice, was the final pathological diagnosis in our case.Although the therapeutic strategy has not yet been standardized, it must be multidisciplinary. Treatment for ovarian cancer is mainly palliative care; however, occasional cases of aggressive treatment with surgery and chemotherapy have been recorded with higher long-term survival .The primary surgical treatment in our case was the removal of the umbilical tumor with the reintroduction of the omentum into the abdominal cavity. It is possible that adjuvant chemotherapy will be required.Umbilical metastasis is usually associated with a dismal prognosis, with just 15% of patients surviving for two years , 11.An SMJN is a rare yet significant illness that is frequently misdiagnosed.Early detection and diagnosis of the original lesion can lead to more effective treatment and a higher rate of survival.


Minimally invasive treatment of uterine fibroids usually requires a power morcellation, which could be associated with several complications. A rare sequela is disseminated peritoneal leiomyomatosis. Indeed, recurrence or metastasis in these cases could be attributed to iatrogenic or under-evaluation of primary tumors, although a subset of cases is a sporadic sample of biological progression. We present an extremely rare case of a patient who underwent laparoscopic morcellation and after 12 years developed a pelvic leiomyosarcoma with two omental metastases, disseminated peritoneal leiomyomatosis with a parasite leiomyoma with bizarre nuclei and a parasite cellular leiomyoma simultaneously. The diagnosis was predicted preoperatively by an expert sonographer who recognized the ultrasound characteristics of uterine sarcoma and the localization of some of the masses, so the patient was referred to the gynaecological oncologists who could appropriately treat her. We present here a case report and a systematic review that could be a useful tool for further discussion and future clinical practice guidelines. Uterine leiomyoma is the most common benign tumor in women. Its prevalence is approximately 70–80% in women by the age of 50 . SurgicaA 47-year-old woman (para 2/0/0/2) presented to our hospital in March 2021 with lower abdominal pain, not responsive to medical therapy. She had regular menses and she had previously taken oral contraceptives (gestodene + etinyl estradiol) for 12 years until the age of 30. Her cousin had died from a uterine leiomyosarcoma at 49 years old. In 2008 she underwent a laparoscopic myomectomy for a rapidly growing uterine intramural-subserosal myoma, and power morcellation was performed. Histological examination was positive for uterine leiomyoma. At admission, the physical examination showed abdominal distension caused by a voluminous hard palpable mass, which ended four fingers above the transverse umbilical line. The computed tomography scan of the abdomen and of the pelvis, performed in the emergency department, revealed a voluminous inhomogeneous solid mass of 24 × 13 × 23 cm in size, referable to a right adnexal mass; the appearances of the uterus and the left adnexa at CT scan were regular. The tumor markers  were negative and the LDH levels were 575 U/L. The transvaginal and transabdominal ultrasound examination showed a normal anteverted uterus, of 73 mm in size, with irregular margins and an inhomogeneous echo pattern. The endometrial echo pattern was secretive, with an endometrial thickness of 7 mm. The ovaries were both regular in size, shape and echotexture. A voluminous mass of 253 × 151 × 235 mm in size occupied the pelvis and the abdomen. The mass showed irregular margins and an inhomogeneous echotexture for the presence of some cystic areas with anechoic content. The lesion showed stripes and it appeared richly vascularized at color Doppler examination A. The laNo pelvic free fluid and no ascites were seen. The patient underwent a laparotomic surgery. The exploration of the abdomen and of the pelvis confirmed the presence of a large lobulated mass of about 30 cm, which presented necrotic, colliquate and hemorrhagic areas .It was tenaciously adherent to the prevesical peritoneum, to the small intestine, to the left colon and to the sigma. The uterus appeared irregular in shape. The adnexa were both regular in size and shape. Further nodular lesions on the abdominal and pelvic peritoneum were detected. Two omental lesions were also seen. The patient underwent a total hysterectomy, a bilateral salpingo-oophorectomy, and an omentectomy, and all the peritoneal nodules were carefully removed. The final pathology of the large abdominal–pelvic mass was positive for leiomyosarcoma of high grade, with extensive necrosis, widespread severe cytokaryological atypia, and a high mitotic index (>20/10 HPF), ki-67 = 70%. A small peritoneal lesion was a focus of endometriosis. The mass of the right pelvic peritoneum had cellulate aspects with ki-67 = 10%, and it was defined as a cellulate leiomyoma. A lesion of the posterior wall of 6 cm was a leiomyoma with bizarre nuclei. All the other four isolated masses were myomas. The omental lesions were both positive for leiomyosarcoma metastases. The post-operative course was uneventful and free from surgical complications, and the patient was discharged on the ninth post-operative day, with a total regression of the initial symptoms.A systematic review of DPL with malignant transformation reports was performed through a literature search in the following electronic databases: PubMed, the Cochrane Library, and Web of Science. The article research was performed in agreement with preferred reporting items for systematic reviews and meta-analyses (PRISMA) The following search terms were used: “disseminated peritoneal leiomyomatosis, malignant transformation, pelvic leiomyosarcoma, sarcoma, omental metastasis and laparoscopic morcellation”. We considered particular articles, case series and case reports published in English . No restMost mesenchymal cancers that occur in the uterus are smooth muscle cancers. The majority are classified as leiomyomas, and subtypes of leiomyoma have been classified according to the World Health Organization (WHO) classification (4th edition). Kurman RJ, Carcangiu ML, Herrington C, Young RH, editors. WHO Classification of Tumours of Female Reproductive Organs. Lyon: IARC; 2014. Conservative follow-up in the absence of symptoms and procedures of myomectomy or hysterectomy can be completely curative in cases where surgical treatment is selected. However, although such tumors are histologically considered benign after treatment, some cases still show local recurrences or distant metastases. Only a few cases of DPL with malignant transformations are described in the literature . The eleWe collected these previous cases and summarized their clinical features  and the pre-operative examinations performed. Moreover, we reported if the diagnosis of malignancy was made or suspected preoperatively or only after surgery. The patients’ age ranged from 20 , with a There are several criteria through which the pathologist makes the diagnosis of leiomyoma or leiomyosarcomas. Macroscopic evaluation is undoubtedly essential. For this reason, where possible, hysterectomy or myomectomy is always preferable to morcellation, since in the first two cases, a complete and oriented evaluation of the anatomical piece is always possible. Moreover, as demonstrated in the literature, transvaginal extraction in the bag can also be considered a safe option for the recovery of surgical samples after laparoscopic myomectomy, for which rare and transient complications (0.6%) such as vaginal bleeding, and 0.3% vaginal pain are found ,29. IntrIn fact, tumor cells do not present an important pleomorphism, usually being all fusocellular/spindle, monomorphic, with an eosinophilic cytoplasm and elongated cigar-shaped nucleus almost always devoid of nucleoli; mitotitis figures are practically absent or very rare, as are tumor necrosis or other types of cellular degeneration ,32. AlthIn cases of leiomyosarcoma, there have also been several genetic mutations among which we most frequently find TP53, ATRX and MED12 , in addiPatients with uterine leiomyosarcomas have a poor prognosis, considering they tend to be very aggressive and often recur. The literature reports a percentage that can even touch 70% with a maximum survival of 70% at 5 years in stages I and II, while patients in more advanced stages see this percentage reduced up to just 10 months from diagnosis ,57. The Our work confirms the correlation between laparoscopic morcellation of uterine myomas and the onset after several years of some complications such as DPL and endometriosis. Therefore, morcellation with a pouch would be mandatory to avoid iatrogenic recurrence. On the other hand, although a subset of cases has shown rare examples of biological progression, recurrence or metastasis, these can sometimes be related to iatrogenic or undervalued tumors of primary tumors. Therefore, more sophisticated diagnostic criteria for uterine smooth muscle tumors should be applied in a multidisciplinary manner so that diagnostic and clinical information between gynecologists and pathologists can be correlated for optimal patient management, in order to avoid recurrences due to incorrect primary diagnosis. In addition, the present case shows the simultaneous presence of pelvic endometriosis, a large leiomyosarcoma with omental metastases and six multiple myomas with different histotypic characteristics, and was the first case to report malignant DPL degeneration with omental metastases. In cases of uterine stromal tumors, after having carefully radiologically investigated this type of tumor, en bloc resection of the mass would always be preferable. Furthermore, in case of suspected sarcoma, the patient must be managed from a diagnostic and surgical point of view in centers with specialized experience and a large number of cases. This would allow patients to be referred to gynecological oncologists who can treat them appropriately.


Acute testicular torsion is a surgical emergency due to acute testicular ischemia. Manual testicular detorsion is a testis-saving, bedside therapeutic when performed correctly and in a timely fashion. This procedure is most commonly performed blindly with pain relief as the endpoint for detorsion. However, up to one-third of patients continued to show signs of residual torsion in the operating room even using pain relief as the stopping point for the procedure.We present a case demonstrating the utility of color Doppler ultrasound to confirm complete manual detorsion in a 14-year-old male with acute testicular torsion. The patient underwent 360-degree detorsion and had relief of pain; however, color Doppler demonstrated incomplete return of flow to the testis. After an additional 180-degree turn was made, color Doppler demonstrated complete return of normal vascular flow to the torsed testis.When it comes to testicular viability, timely restoration of blood flow to the testicle is of utmost importance. Manual detorsion is a non-invasive intervention that can be quickly and effectively performed at the bedside. Moreover, using color Doppler ultrasound guidance can ensure that physicians detorse in the proper direction and to completion, by providing instant visualization of restorative flow and ensuring reperfusion of the testis while awaiting definitive surgical management. The estimated yearly incidence of testicular torsion in males under the age of 18 presenting to the emergency department (ED) is 3.8 per 100,000.Manual testicular detorsion is a bedside procedure taught to emergency care practitioners and performed blindly. Conventional emergency medicine teaching dictates that torsions occur in the lateral-to-medial direction and, therefore, detorsion should be performed in the medial-to-lateral or “open-book” fashion until pain relief.6With as little as 180 degrees of torsion causing testicular infarct,A 14-year-old male presented to the ED with vomiting and sudden onset of left testicular pain, waking him up from sleep approximately two hours prior. The patient denied experiencing trauma, fever, chills, diarrhea, or difficulty urinating. Physical examination revealed a high-riding left testicle and absent left-sided cremasteric reflex without erythema or swelling. Scrotal ultrasound was performed in the radiology ultrasound suite using both color Doppler and spectral Doppler. Both testes had normal echotexture and appeared to be of the same size. Arterial and venous waveforms were identified in the right testis but were absent on the left .In the ultrasound suite, with the aid of the ultrasound technician, the emergency physician manually detorsed the left testicle by turning it from medial to lateral and re-evaluated for return of flow with CDU after each 180-degree turn. After the first 180-degree rotation, the patient’s pain did not begin to decrease, bringing into question torsion directionality. Color Doppler ultrasound demonstrated a small increase in vascular flow to the left testis, suggesting the correct detorsion direction but the need for further rotations. An additional 180-degree rotation was performed, resulting in a marked decrease in pain; however, repeat CDU showed no improvement in flow. Given the continued paucity of vascular flow to the left testis when compared to the right  there waWhat do we already know about this clinical entity?Testicular torsion is a surgical emergency and manual testicular detorsion, when performed correctly, is a testis-saving bedside therapeutic.What makes this presentation of disease reportable?Color Doppler ultrasound detorsion guidance was performed with the aid of an ultrasound technician to confirm complete testicular detorsion with reperfusion.What is the major learning point?Color Doppler ultrasound guidance can be used to guide manual testicular detorsion for correct direction and confirm complete detorsion.How might this improve emergency medicine practice?Color Doppler ultrasound guided testicular detorsion can be performed both as a point-of-care ultrasound procedure or performed with an ultrasound technician.The on-call urologist then brought the patient to the OR for bilateral testicular orchiopexy. During surgery, the left testicle was delivered from the left hemiscrotum and was noted to be completely detorsed, pink, and viable. There were no surgical complications, and the patient was discharged that day.Testicular torsion accounts for up to 25% of pediatric cases of acute scrotal pain.Conventional teaching dictates that to detorse a testis, it should be manually rotated in a medial-to-lateral direction.When it comes to testicular viability, timely restoration of blood flow to the testicle is of utmost importance. Manual detorsion is a non-invasive intervention that can be quickly and effectively performed at the bedside. Moreover, using CDU guidance can ensure that physicians detorse in the proper direction and to completion, by providing instant visualization of restorative flow and ensuring reperfusion of the testis while awaiting definitive surgical management. Prior case reports demonstrate CDU guidance as a viable point-of-care US (POCUS) procedure.


The process included a critical review of the literature and multidisciplinary expert consensus. LMs are congenital but are not always discovered at birth. Nearly 75% of them are located in the head and neck because of the highly dense lymphatic system in this region. Physical examination  and color Doppler ultrasonography, usually allow for diagnosis. MRI  is the tool of choice for evaluating anatomical extension, characterizing lesions (microcystic and macrocystic), and before considering therapeutic management. A biopsy, coupled to a blood sample, can also be used for molecular biology analyses, to search for activating mutations of the PIK3CA gene, particularly with LM integrating in a syndromic form (CLOVES or Klippel-Trenaunay syndrome) but also in certain isolated (or common) LMs. The spontaneous evolution of LMs, in particular microcystic forms, is often toward progressive aggravation, with an increase in the number of vesicles, thickening, increased oozing and bleeding, while pure macrocystic LMs may regress due to “natural sclerosis”, i.e. fibrosis secondary to an inflammatory reorganization after common infantile infections. In case of voluminous LMs or syndromic forms, functional and psychological repercussions can be major, deteriorating the patient’s quality of life. LMs must be treated by physicians integrated in multidisciplinary teams, and be personalized. Management is a life-long process that involves one or several of these therapies: conservative management, physical therapy (compression), sclerotherapy, surgery, drugs such as mTOR inhibitors (sirolimus), that has shown efficacy in decreasing the volume of LMs, and, more recently, PI3K-inhibitors in syndromic forms. Psychological and social support is necessary, taking into account the patient and his family.Cystic lymphatic malformations (LMs) are rare chronic conditions which management differs according to the type . Studies are lacking due to rarity of the pathology. We aimed to establish a French National Diagnosis and Care Protocol , to provide health professionals with free open access synthesis on optimal management and care of patients with LMs ( Cystic lymphatic malformations (LMs) are rare chronic conditions with extremely heterogenous clinical features. Their management is also very variable depending on the characteristics, location, and natural history of the lesions. Treatment often includes a combination of conservative management, sclerotherapy, surgery, specific drugs, and should always be decided after multidisciplinary discussion. The purpose of this article based on a multidisciplinary French expert consensus is to present a state-of-art overview of currently available options for the management of LM.https://fimarad.org/ for FIMARAD (rare dermatologic diseases and coordinator of the present study); https://favamulti.fr/ for FAVAMULTI (rare vascular diseases with multisystemic components); https://maladiesrares-necker.aphp.fr/magec/ for MAGEC (rare skin and mucosa diseases with a genetic origin) and https://maladiesrares-necker.aphp.fr/malo/ for MALO . The authors, members of the different networks involved in the management of lymphatic malformations, participated in the writing of this document. These recommendations have been established after an extensive review of the literature.In France, the management of rare diseases is ensured by several reference networks throughout the country. These networks provide care and orientation for patients as well as coordination of research and development of educational programs for health professionals. Different reference networks can cooperate and pool their expertise, as illustrated by the present recommendations on LM. The websites of the different networks involved in the writing of this document can be consulted here: The objective of the present expert recommendations is to explain to the professionals concerned, and especially to the general practitioners, the current optimal diagnostic and therapeutic management and the care pathway for a patient affected with simple (cystic) LM. Their aim is to optimize and harmonize the management and follow-up of the rare disease throughout the country. They also make possible to identify pharmaceutical specialties used in an indication not provided for in the marketing authorization as well as specialties, products or services necessary for patient care but not usually covered or reimbursed by social security and/or private insurances. This PNDS is designed to be used as a reference by the physician at each step of the patient’s management. It aims to provide a clear and understandable state-of-the-art synthesis of the current knowledges and existing protocols on lymphatic malformations. Taking into consideration most of the possible clinical presentations, this document does not aim to define a single possible decision tree, but to present all current management possibilities: it cannot consider all the specific cases and therapeutic particularities. It describes the possible options in the reference management of a patient with a lymphatic malformation in 2022.https://www.has-sante.fr/jcms/c_1340879/fr/protocoles-nationaux-de-diagnostic-et-de-soins-pnds (available in French) and is briefly summarized below and in the Fig. The methodology followed can be found here The coordinating network chose the group of experts, all belonging to the reference networks involved in the management of LMs and from different medical specialties. These experts performed an extensive literature review in their fields and wrote the synthesis document. The pooling, analysis and correction of the data was done during several meetings with the experts and the coordinating team. The final document has been corrected, reviewed and approved by all the co-authors and ultimately sent to the health authorities for online publication.Cystic lymphatic malformations (LMs) are rare benign congenital slow-flow anomalies that feature abnormal cystic dilatations of lymphatic vessels –3. The tLMs, present at birth as subcutaneous or deep soft masses that develop in proportion to the child's growth , are cysDepending on their size estimated clinically or on imaging, LMs are said to be macrocystic (diameter > 1 cm), microcystic (< 1 cm and often much less) or mixed, combining the two forms. For example, LMs of the oral cavity are often microcystic, but those in the armpit or groin are macrocystic or mixed. One hypothesis explaining these differences in size is the density of the connective tissue underlying the cyst during its formation: loose connective tissue would be associated with larger cysts.LMs can be isolated or syndromic Table . They caSuperficial LMs are more frequent than visceral ones but we lack epidemiological data. The incidence of cervico-facial LMs is estimated at 1.2‰–2.8‰ .CCBE1, ADAMTS3, FOXC2, NRP2, and GATA2. Dysregulation could lead to increased lymphangiogenesis and LM formation. Indeed, somatic-activating mutations of PIK3CA, coding for the catalytic subunit of protein kinase PI3K, have been identified in some LMs [PIK3CA mutations have also been identified in various PIK3CA-related overgrowth spectrum (PROS) hypertrophic syndromes, sometimes including LMs.The origin of LMs is still not fully elucidated. The embryonic development of lymphatic vessels involves the differentiation of venous endothelial cells into lymphatic endothelial cells under the influence of transcription factors . These cells then migrate and multiply in the surrounding mesenchyme to form the primary lymphatic sacs, which, by expanding, form the primary arborescent lymphatic vasculature under the action of the VEGF-C/VEGFR-3 signaling pathway and angiopoietin-1. Other genes involved in the formation of lymphatic channels and valves include some LMs . This exsome LMs . Mutatiosome LMs , 8. SomaThe International Society for the Study of Vascular Anomalies separated vascular anomalies into tumors and malformations . LMs belFetal ultrasonography and MRI are the imaging tools for the detailed prenatal diagnosis and anatomical evaluation of LMs , 12. LMsThe demonstration of LMs justifies management in a Multidisciplinary Center for Prenatal Diagnosis (CPDPN). These structures are the only ones authorized in France to care for fetal disorders since 1994.The exploration of a fetal LM will generally include a reference ultrasonography, an MRI (after 28 weeks of amenorrhea), then a multidisciplinary discussion with the pre- and perinatal stakeholders  and the postnatal stakeholders . In the absence of a particular association between the LM and chromosomal abnormalities, amniocentesis for karyotype or comparative genomic hybridization is not systematic and is left to the reasoned assessment of the CPDPN physicians .Prenatal imaging aims to characterize the lesion . Despite the very good resolution of fetal MRI, especially T2-weighted sequences, accurately assessing the presence and/or extension of the microcystic infiltrating component, particularly in the ENT sphere or on the limbs is difficult, which leads to remaining cautious in the anatomical evaluation of the microcystic components. The main differential diagnosis is teratoma, but segmental tissue hypertrophy and abnormalities of the extremities  must also be sought, which may lead to evoking LM in the context of a PROS syndrome.Superficial or bulky LMs are most often visible at birth. However, they can become apparent later, often during the first 2 years of life, following a viral infections or intracystic hemorrhage . MacrocyIn all cases, the diagnosis of LMs requires clinical examination and characterization by imaging . DependiThe evolution of LMs is marked by inflammatory or infectious episodes, or intracystic hemorrhages, which can be life-threatening on compression  and be the cause of pain, hemorrhagic oozing, functional impairment  and major deformities. The quality of life of individuals and their families can be strongly affected , 9, 14.Interpretation of imaging data should take into account clinical data. Ultrasonography is the imaging modality for the initial assessment of LMs, especially small superficial lesions . In macrMRI is the tool of choice for evaluating anatomical extension; characterizing lesions (microcystic and macrocystic), in particular in the event of deep extension; and before considering therapeutic management. It must involve T2 sequences with fat saturation in at least two spatial planes. It can be supplemented with a T1 sequence to identify hemorrhagic changes and visualize infiltration within fatty structures , 15. TheMacrocystic malformations are characterized by a juxtaposition of hyperintense cystic structures on T2 septa with possible internal fluid levels, characteristic of intracystic hemorrhage. On T1 sequences, macrocystic malformations are hypo-intense or iso-intense to the muscle, but the septa are more difficult to identify. After administration of the contrast product, the septa within the macrocystic malformation can be enhanced, unlike the cystic components, which allows for distinguishing the macrocystic LM from venous malformations.LMs with a microcystic component appear as a hyper-intense infiltration on T2-weighted sequences and hypo-intense or iso-intense T1 sequences. Hypodermic infiltrations or within deep fatty structures are much better identified on T2 sequences because of the fat saturation. As on ultrasonography, on MRI, microcystic LMs can appear as a solid lesion, most often infiltrative, and careful examination will sometimes identify small cystic components leading to the diagnosis.In case of periorbital LM, a cerebral MRI is also recommended to detect associated cerebral venous abnormalities.Arteriography, venography, lymphography and isotopic lymphoscintigraphy have no value in common LMs. CT-scanning may have some interest in LM involving bones, notably by showing the invasion and deformation caused by microcysts.PIK3CA gene, particularly with LM integrating in a syndromic form (CLOVES or Klippel-Trenaunay syndrome) but also in certain isolated LMs. Fine-needle aspiration biopsy is sometimes performed for diagnostic purposes. It would show a citrine liquid rich in lymphocytes.Biopsy is rarely necessary to establish the diagnosis of LMs. However, in case of doubt, the examination shows thin-walled vessels and cysts with empty lumen (hematoxylin–eosin-saffron staining). Lymphatic vessels express the lymphatic markers podoplanin, CD34, LYVE-1 and VEGFR-3 . A biopsThere is no specific biological test for isolated LM. In syndromic or combined forms, consumption coagulopathy must be sought . In case of chronic bleeding, one should look for iron deficiency anemia. Finally, a specific assessment can be requested pre-therapeutically.The differential diagnoses are mainly congenital cysts ; superficial venous malformations; deep infantile hemangiomas; and other malformations or tumors (mainly teratomas). The microcystic forms can be confused with angiokeratomas, botryomycomas, molluscum contagiosum or condyloma when they are on the genital or perianal areas.The evolution of LMs is marked by asymptomatic periods, sometimes several years, potentially interspersed with episodes of painful inflammatory flare-ups, superinfections or intracystic hemorrhages of variable frequency. Not all patients have complications or flare-ups and some have a stable form of LM for very long periods.When occurring, these outbreaks are often painful, resulting in large and sudden increases in volume. They last from 48 h to 1 month, about 10 days on average. They can appear after trauma or with infectious episodes, in particular viral ones.During these attacks, compression of neighboring organs is possible, which can be life-threatening or induce functional complications. Effusions or hemorrhage are possible , and intra-abdominal mesenteric locations can also cause intestinal torsion around the cyst. Sex and LM location do not seem to affect the progression and complications. Puberty might be marked by a peak in progression, although the role of hormones has not been demonstrated.Distinguishing inflammatory outbreaks and infection of macrocysts is difficult because in both cases, the lesions increase in volume, become painful, and can be associated with signs of cutaneous inflammation and fever. Fibrosis secondary to an inflammatory reorganization can explain certain spontaneous regressions . This spontaneous regression would be more frequent for cervical localizations of LMs and would occur in just over 10% of cases and 2 to 24 months after the episode , 17. SpoIntracystic hemorrhage is also possible: the swelling then becomes suddenly tense, painful, and potentially bluish but without fever or inflammatory signs.Microcystic LMs may present as swellings under normal skin or as translucent or hemorrhagic vesicles millimeters in size, scattered or grouped in plaques, called lymphangiectasia. They may be covered with verrucous hyperkeratosis and may be complicated by lymph oozing and chronic bleeding.The spontaneous evolution generally is toward progressive aggravation, with an increase in the number of vesicles, thickening, increased oozing and bleeding. Oral locations may be malodorous due to bacterial colonization.Orbital microcystic LMs pose specific problems  and require the advice of an ophthalmologist specialized in orbito-palpebral pathology. The main complication is compressive optic neuropathy, which can occur acutely (bleeding or more rarely infection) or chronically: decreased visual acuity, relative afferent pupillary deficit, papilledema, visual field alterations and dyschromatopsia.Some LMs are barely symptomatic or not at all, in which case no therapy, with a follow-up proposal, is the most appropriate management. If treatment is necessary, the different options can be successive or concomitant, and decisions must be made in multidisciplinary consultation.Apart from very small LMs that can be completely resected, the goal of treatment is generally not curative but to maintain functionality, control associated symptoms, preserve aesthetic integrity and quality of life, and prevent aggravation.Sclerotherapy is the standard first-line treatment for most LMs, generally performed under general anesthesia, although it is possible under local anesthesia for certain products . It is sSeveral sclerosants can be used: sodium tetradecyl sulphate, sodium morrhuate, OK-432, polidocanol or lauromacrogol 400 (3% Aetoxisclerol®), absolute ethanol, doxycycline and bleomycin. Each sclerosant has its advantages and disadvantages, and their use is also linked to the center’s experience. For example, absolute ethanol is potent but generates a risk of cardiac arrest if it enters systemic circulation . BleomycThe side effects of the most commonly used sclerosing agents are similar and include swelling, pain, skin ulcerations and nerve paralysis. The risk of a serious complication  is in the order of 0 to 3% .Sclerotherapy is the first-line treatment for macrocytic LMs, and the sessions can be repeated several times for a complete result, generally spaced out by at least 3–6 months.In most cases, complete surgical removal of LMs is difficult or even impossible because of infiltrating lesions that are difficult to identify . Therefo2 laser treatment is safe and transitory effective but painful. Adverse effects are usually minor and infrequent and include dyspigmentation and mild scarring [Physical treatment  is useful for superficially destroying lesions, stopping bleeding, and reducing redness. It must be repeated and is only suspensive , 28. Linscarring . TopicalPhysiotherapy includes compression and lymphatic drainage. Compression has not been found effective in LMs . SeveralSirolimus (or rapamycin) inhibits lymphangiogenesis by interfering with the PI3K/AKT/mTOR signaling pathway. It has been used in complicated LMs for 10 years –39. ManySildenafil is a potent and selective inhibitor of phosphodiesterase type 5. A few small series have suggested some efficacy but without further confirmation , 40. ThePropranolol is a non-selective beta-blocker that induces peripheral vasoconstriction and endothelial cell apoptosis and is effective for infantile hemangioma. It has not been found effective in LMs , 42.Bevacizumab is a monoclonal antibody active against VEGF-A, with an effect on lymphangiogenesis. Bevacizumab has been the subject of few clinical cases, systemically and as a sclerosing agent . Hence, PIK3CA gene have been identified in LMs integrated in syndromes with hypertrophy of the underlying soft tissues (CLOVES and Klippel-Trenaunay) as well as in certain isolated LMs. A pilot study reported promising results, and this molecule is undergoing clinical evaluation (efficacy and safety profile) for syndromic forms [Alpelisib is a targeted therapy that directly inhibits the PIK3. However, somatic activating mutations of the ic forms .LMs must be treated by physicians/surgeons integrated in multidisciplinary teams . Several LM cases can be distinguished.These very large, macrocystic or mixed LMs are often detected in utero. They can generate dystocia and respiratory distress in the newborn in the event of cervico-facial involvement. An EXIT procedure is indicated in case of foreseeable obstruction during childbirth , 45. HowSclerotherapy is the first-line treatment in case of discomfort. It must be programmed, outside the context of the emergency. Surgery is a second-line treatment and requires prolonged hospitalization for post-operative care. Therapeutic abstention is possible in the case of small and non-troublesome macrocystic LMs, which can regress spontaneously because of a spontaneous inflammatory phenomenon.These are more difficult to treat because of the infiltration of tissues by innumerable small cysts and require a therapeutic strategy including a combination of repeated treatments that rarely lead to sufficient regression to be satisfactory. Therapeutic abstention is possible for small lesions that are not at all troublesome.Sirolimus is the disease-modifying treatment of choice. It should be started early in life (early childhood) to prevent the increase in volume of the LM. If started after age 6 years, it has shown efficacy for pain, bleeding, lymphorrhea and partial efficacy for malformation volume , 46–50. Surgery is possible but rarely complete, with high risk of recurrence of LM on underlying scars. However, a partial reduction might be useful to reduce a deforming LM, even though delayed healing with oozing is often observed.2 laser or radiofrequency for the mucous membranes, with CO2 lasers or pulsed dye laser, or with topical sirolimus = or > 0.1% for cutaneous lymphangiectasis.Superficial microcystic components can be treated with COThe treatment of orbital LMs depends above all on their visual impact. In the event of acute proptosis (intracystic hemorrhage), surgery may be necessary with major visual impact . In some cases, bony orbital decompression may be necessary. In the absence of acute complications, the decision is according to the ocular impact, pain, induced aesthetic discomfort and, more generally, the impact of the LM on quality of life. These LMs may be treated with sclerotherapy, sirolimus, or their combination. Surgery, which is complex, is rarely complete When LMs are part of a complex syndrome , the management is complex and combines surgery, sclerotherapy, anticoagulants for venous abnormalities with associated thrombosis, physiotherapy etc. Sirolimus and targeted anti-PI3K therapies are increasingly used –58.No specific study has compared the different therapies. Analgesic treatment is always indicated (paracetamol then tramadol/paracetamol + codeine depending on the age). In case of fever and suspected infection, probabilistic antibiotic therapy with a fairly broad spectrum is proposed (amoxicillin + clavulanic acid or josamycin for 6–10 days). A high-dose corticosteroid  is usually prescribed in case of inflammation for 3–6 days. Antibiotic prophylaxis has sometimes been proposed . DrainagIn addition to conventional medicine, “alternative and complementary” medicines are possible and often useful for LMs. They should in no way replace conventional medicine or the follow-up of patients by multidisciplinary medical teams.With lack of any study validating the superiority of such alternative medicines compared to placebo, they seem to constitute an added value in improving well-being, alleviating pain, acting on inflammatory flares, regulating emotions and sleep disorders, preventing depression and stress, or helping with school integration, accepting one's differences and better living with stares from the public. They allow for better mobilizing the psychosomatic resources of patients and take part in a global approach. Such alternative therapies include body and mind therapies , manual therapies , comprehensive approaches based on theoretical bases that are specific to them  and biological therapies, which are based on natural products . These products, if taken orally, can interact with medications, which needs to be rigorously checked.The complex forms of LMs can be difficult to live with for the patient and the family and justify at least a proposal for a psychological interview with, if necessary, prolonged follow-up .Depending on local or national legislation, various aids may be possible . The patient or family must be referred to the social service department of the hospital or patient associations.Patients must be informed of the chronic nature of the pathology, the need for prolonged follow-up, and the generally incompletely curable nature of the lesions . The frePregnancy does not seem to significantly affect the evolution of LMs. However, cases of inflammatory attacks and an increase in volume of the LM have been mentioned after delivery. Basic treatments should be avoided as much as possible during pregnancy. Sirolimus, for example, should be suspended a few weeks before planning conception and not be administrated during pregnancy and breastfeeding.PIK3CA gene. This situation considerably reduces the risk of recurrence in the offspring of an affected individual or any siblings, and thus parents can be reassured.In isolated LMs and syndromic forms, somatic (mosaic) mutations have sometimes been identified, affecting the This work summarizes knowledge, the state of the art and the main indications for assessment and management of LM in 2022. It confirms the decreasing importance of surgery in favor of sclerotherapy and other drug therapies. More than ever, decisions regarding LM patients must be made during multidisciplinary meetings and by teams accustomed to their care.


A sellar spine is a rare osseous projection from the dorsum sellae, resulting in variable compression of sellar and suprasellar structures and varied clinical presentations. CT is the diagnostic modality of choice, while variable signal intensity on MRI may mimic a pituitary microadenoma. A patient presented with hypoprolactinaemia and puerperal alactogenesis due to a sellar spine diagnosed on CT Brain. Neurosurgical and endocrine review and pituitary MRI were recommended with subsequent loss to follow-up. This reported case of a sellar spine is unique for two reasons. To our knowledge, this is the first reported sellar spine that arises from the mid-ventral aspect of the dorsum sellae, with most of the previously described cases in the literature arising from the inferior ventral aspect of the dorsum sellae and two arising from the superior aspect of the dorsum sellae.A 37-year-old woman with no known co-morbidities presented with a single episode of menorrhagia , severe constant headaches for one year, and a history of puerperal alactogenesis in all four of her previous pregnancies. Physical examination was unremarkable. Hormone levels: Thyroid-stimulating hormone (TSH) and prolactin were mildly decreased. Follicle-stimulating hormone (FSH) and Luteinizing hormone (LH) were elevated , thyroxine (free T4): 17.5 pmol/L , prolactin: 4.7 µg/L , FSH: 31 IU/L, LH: 7.7 IU/L, oestradiol: < 19 pmol/L, progesterone: < 0.2 nmol/L). Pelvic ultrasound was unremarkable. She was referred to the radiology department for CT Brain to exclude any pituitary pathology.CT Brain demonstrated a bony spur, consisting of a narrow stalk with a mildly distended tip, arising from the mid-anterior aspect of the dorsum sellae in the midline, projecting anterosuperiorly into the pituitary fossa. The spur measured 5.5 mm in length and compressed the posterior pituitary and the distal posterior aspect of the pituitary stalk  and 2. NNon-urgent neurosurgical review, repeat endocrine profile and MRI of the pituitary gland with dynamic contrast enhancement were recommended; however, the patient was lost to follow-up.1 In 1977, Lang et al. described the first case in an article describing atypical ossifications of the sella turcica.11 It is a rare entity with an estimated incidence of 1 in 5000–8000.12A sellar spine is a rare midline osseous spur, which arises from the dorsum sellae and projects into the pituitary fossa.1 Other proposed hypotheses by Dietemann et al. include ossification of a dural fold and an ossified vascular channel.1The leading hypothesis for its development is that it is due to the failure of regression of the most cephalic aspect of the notochord through the clivus into the vertebral column, resulting in a remnant of a notochordal rest in the fetal sella; this remnant then ossifies.16 The most consistent imaging morphology of the bony spur among the literature was that of a narrow cylindrical bony stalk with a mildly distended tip and a flattened triangular base in continuity with the dorsum sellae.13 In most cases, the tip had a smooth contour; however, in one of the cases, it was irregular.12 The maximum described length was 9.0 mm.2 For the remainder of the reported cases, the length of the sellar spine ranged between 3.8 mm and 5.0 mm, making this case the second longest reported case at 5.5 mm.3,4.5,11,12,14 In most of the cases, the sellar spine arose from the inferior aspect of the anterior dorsum sellae with only two cases reported to be arising from the superior aspect.10 To our knowledge, this is the first case, reported, where the sellar spine arises from the mid-anterior aspect giving a ‘figure of 3’ sign of the dorsum sellae in the sagittal plane (2In all the described cases, the sellar spine was located in the midline in keeping with the foremost theory that it is an ossified remnant of the notochord.al plane . The spi8 In the two cases where there was an anterosuperior origin from the dorsum sellae, compression of the pituitary stalk as well as the optic chiasm was observed.6 Our case compresses the junction of the stalk and posterior pituitary due to its mid-anterior origin.The most commonly compressed structure is the posterior pituitary, in keeping with the anteroinferior origin from the dorsum sellae.16 A possible explanation for this distribution is the slow progressive growth of the sellar spine over time, as well as normal progressive enlargement of the pituitary gland. This combination increases the probability of compression of the pituitary gland and thus the development of symptoms. This theory is supported by the findings of Hosokawa et al. who demonstrated interval growth of the sellar spine in a patient for whom serial images were obtained over time.14 On the contrary, Chivukula et al. demonstrated no significant growth on serial images obtained from their patient.8 Another possible explanation for the development of symptoms in our case is the normal growth of the pituitary gland in pregnancy, as postulated by Chivukula et al.8The age at presentation is varied and ranges from 8 to 53 years, with most of the cases reported between the ages of 13 and 29 years.7 The most common presenting complaint among the reviewed literature was headache.15 The other common neurological manifestation was bitemporal hemianopia due to optic chiasm involvement.15The spectrum of described clinical presentations is varied, with most cases being asymptomatic.15 Few of the cases demonstrated pituitary hyperfunction, including hyperprolactinaemia and Cushing’s syndrome; the pathophysiological mechanism for this is not understood.12 Our case describes the first presentation with puerperal alactogenesis and mild hypoprolactinaemia. Other endocrine disturbances in our case included elevated FSH and LH levels and decreased TSH levels; this combination of findings has not been previously described and may be attributed to the combination of pituitary and pituitary stalk compression or laboratory error. A repeat hormonal profile would have been of great benefit had the patient not been lost to follow-up.Of the endocrine manifestations, there were varying degrees of hypopituitarism, with diabetes insipidus being the most explicable of these findings.7 On MRI, sellar spines have variable signal intensity depending on the marrow content.16 In the absence of marrow, they are hypointense on T1- and T2-weighted images due to densely packed cortical bone and hyperintense on T1 and T2-weighted images if bone marrow is present; in addition, they are nonenhancing.16 A sellar spine is most commonly seen as a T1 hypointensity in the posterior pituitary and can thus be easily misdiagnosed as a pituitary microadenoma.7 The pituitary bright spot may be lost, and there may be deformation of the pituitary gland as adjunctive findings.15CT is the gold standard imaging modality to assess for a sellar spine as it appears as a well-ossified structure in continuity with the dorsum sellae.8 There was a reported complete resolution of symptoms and hormonal imbalance following the surgical resection of the sellar spine in their case.8Chivukula et al. described transsphenoidal resection of the sellar spine in their patient with panhypopituitarism.17An important differential for an intrasellar bony projection is a post-traumatic base of skull fracture with a projection of a bony fragment into the pituitary fossa such as in the case described by Parizel et al.Although a sellar spine is a rare entity, it should be considered in patients being referred for diagnostic imaging for potential pituitary pathology as they can present with variable symptoms and endocrine disturbances. CT is the imaging modality of choice because of its accurate visualisation of osseous pathology, while, caution should be practised when using MRI, as sellar spines can have variable signal intensity depending on the marrow content and can therefore mimic pituitary microadenomas.


The hemangioma is the most common vascular tumor, involving the head and neck in 60% of cases. It is rare in the larynx. In children hemangiomas are more frequent on the subglottis where it causes stridor and dyspneaPatients in this study were seen at the Clinical Hospital of the Botucatu Medical College (Unesp).This was a 29-year-old female patient reporting hoarseness since childhood. She had a rough, low, breathy and dyplophonic voice. Rigid telescopy revealed a 2.5 cm violet sessile tumor on the left aryepiglottic fold, atrophic vocal folds and a fusiform slit on phonation.  and b.FiThis was a 34-year-old male patient reporting hoarseness under conditions of vocal abuse for the past five years. In the past month, during upper digestive tract endoscopy, a “bluish spot on the larynx” was seen. Rigid telescopy revealed a bluish sessile tumor involving the left vestibular fold, the vocal fold and the base of the tongue. The left vocal fold was irregular and atrophic  and d.In both cases patients did not report respiratory symptoms. Clinical monitoring was the chosen option for these cases.When asymptomatic, laryngeal hemangiomas may be diagnosed by other specialists, such as during endoscopy, as in case 2. The patient had no significant vocal symptoms in his daily activities.Vocal folds were altered in both cases. They were atrophic in case 1, with a reduced glottic wave and a fusiform slit, which suggest a probable vocal cord sulcus. Pontes et al.Endoscopy is almost always sufficient for the diagnosis of a hemangioma. Other exams, such as magnetic resonance imaging with contrast and angiography, are reserved for large tumors and for surgical patients with respiratory symptoms. Biopsies are not indicated due to the risk of severe bleedingThe laryngeal hemangioma may or not cause symptoms. Endoscopy should be careful and detailed due to possible extension of the lesion to adjacent structures and associations with other laryngeal lesions.


Ameloblastoma is a benign jaw tumor that can grow to a very huge size with high rate of recurrence. The large tumors pose a challenge during resection and reconstructive surgery. We present a massive ameloblastoma of the mandible that was surgically treated with the aid of 3D printed model. Using 3D printed model to achieve better results and shorten intra‐operative time in maxillofacial reconstruction. It is an odontogenic tumor arising from residual epithelium of the tooth germ, though etiology remains uncertain.Ameloblastoma is the commonest jaw tumor and is characterized by various histological subtypes. There has been recent reclassification of this tumor into conventional, unicystic, extraosseous/peripheral, and metastasizing  ameloblastoma. The unicystic variant tends to occur in the younger age groups.The management of ameloblastoma is resection of the affected jaw with a safety margin of more than 1.5 cm and reconstruction to restore form and function of the resultant surgical defect. The affected patients among black Africans tend to present to the hospitals late with massive tumors, an observation that has been attributed to a range of factors. Some of these factors include poverty, lack of awareness, traditional beliefs, and possibly asymptomatic nature of the disease.We present a case of an extensive ameloblastoma in a middle‐aged male that was managed with the aid of 3D printed models.2A 31‐year‐old male reported to the University of Nairobi Dental Hospital in June, 2018, complaining of massive swelling on the left side of the face. He gave a history of having been well until 2012 when he noticed a swelling after a tooth (38) extraction. The painless swelling had been slowly increasing in size; the associated teeth progressively became mobile and fell off. He had lost about 6kgs over the last 1 year  scan of the lesion showed an extensive destruction of the mandible and compression of the left maxilla Figure .An incisional biopsy was done and showed features of tumor cells suggestive of mixed ameloblastoma Figure .The patient had challenges in accessing adequate funds to cater for the cost of surgery and appropriate implant and it took a while for the department to solicit for support for his treatment from corporate partners. He was recalled 10 months later when arrangement for his surgical treatment including sourcing for appropriate reconstruction implant had been finalized. During the waiting period, cutaneous ulceration occurred on the overlying skin thereby creating apprehension of possible malignant transformation. Figure .Preoperative 3‐D bio‐printing was done from the CT scan images and mirror image of the normal side used to generate a stereographic 3D model of a normal mandible Figure .Routine pre‐operative investigations were done, including full hemogram, urea electrolytes, and creatinine as well as blood grouping and cross matching. The hemoglobin level at admission was 5.7 g/dl, therefore, 3 units of blood were transfused preoperatively and additional 2 units given intra‐operatively.2.1The patient was prepared in the standard way for craniomaxillofacial surgery and the skin markings made for the planned surgical approach. Through a lip‐split incision that extended to the mental, submandibular, and retromandibular area, the lesion was exposed by a combination of sharp and blunt dissection and tumor successfully resected Figure .A titanium mandibular reconstruction plate with condylar head extension was bent into anatomical shape with the aid of 3‐D bio‐printed model of the simulated normal mandible. The plate was then secured to the normal side of the mandible with bicortical screws. The surgical defect was then closed in layers Figure .Ranferon 30 mg once a day and Freshubin 400 mls thrice daily due to the preoperative cachexia. The post‐operative recovery was uneventful and at 3 months follow‐up, the patient was found to weigh 67 kg up from preoperative of 57 kg. The occlusion on the non‐operated side was satisfactory enough for masticatory function, and he awaits a second surgery to bridge the continuity defect with a bone graft  but rarely exhibits malignant behavior. This most common odontogenic tumor was previously called adamantinoma and was first identified by Cusack in 1827. It can grow to a very massive size, exhibits high recurrence tendency, and has been reported to recur even more than 5 years after surgery, including recurrence in the bone graft used in the reconstruction.It occurs in individuals aged 20–40 years, but a unicystic type occurs more frequently among the adolescent age group. The posterior aspect of mandible is the most common location and the tumor shows no gender predilection, though some authors have reported a higher female incident.The management of ameloblastoma remains marginal or en bloc resection with a margin of safety of 1–2 cm, however, some histologic sub‐types have been shown to be less aggressive especially the unicystic variant seen in younger age category. This unicystic type has been treated conservatively in a number of cases often by enucleation with little recurrence.It has been noted that recurrent ameloblastomas can be diagnosed even 10 years after the first treatment, hence the need for long‐term follow‐up.The late presentation poses a serious challenge not only in terms of surgical resection of the extensive tumor but also reconstruction of the continuity defect as well as functional rehabilitation. The use of 3D printed helps in highlighting the extent of the tumor as well as appreciating the anticipated challenges that can be discussed with the patient prior to obtaining consent. But what proved useful in this case was the application of the generated 3D stereographic mirror image in creating a near normal mandible that aided in accurate bending of the reconstruction plate to achieve the pre‐pathology normal anatomy.3.13D bio‐printing is a useful aid in the treatment planning, surgical reconstruction of extensive jaw tumors and can reduce operating theater time as well as minimize intra‐operative morbidity with improved anatomical profile.Dr. Walter A. Odhiambo involved in concept and development of the manuscript as well as the management of the patient. Dr. James Mwangi Gatune contributed to manuscript writing and management of the patient. Prof. Symon W Guthua involved in concept of the 3D and head of surgical team. Chrirs Muraguri involved in development of the 3D printing of CT models.The authors declare no conflict of interest.Ethical clearance was obtained from the UoN Ethics and research committee.Patient gave written free un‐coerced consent and was assured of anonymity.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


The identification and evaluation of leaking areas in FA pictures are essential steps in the diagnostic process as well as the treatment and management of a variety of choroidal and retinal illnesses. We reported a case of rhegmatogenous retinal detachment in a seven-year-old boy with positive leakage on FA, which confused the presentation. A thorough contact lens examination and scleral indentation are mandatory to reach the diagnosis easily. The detection and assessment of areas of leakage in fluorescence angiography (FA) images are crucial for both the diagnosis and management of different choroidal and retinal diseases. Fluorescein leakage can occur due to attenuated blood vessels such as retinal neovascularization (NVE), choroidal neovascularization (CNV), or a detached retinal pigment epithelium (RPE) that no longer prevents fluorescein leakage from the choroid . FA leakA seven-year-old male presented to the ophthalmology outpatient clinic complaining of sudden decreased vision in the right eye for nine days. No past history of photopsia or ocular trauma was reported. An ophthalmic examination of the right eye showed that the best-corrected visual acuity (BCVA) is 0.1. Left eye BCVA is 1. On fundus examination, the presence of pigment cells in the anterior vitreous, white temporal lesions floating in the vitreous cavity, and shallow inferior RRD involving the macula were observed. The left eye exam was unremarkable except for temporal white lesions involving the vitreous. No retinal breaks or telangiectasia were detected. Optical coherence tomography (OCT) of the right eye showed macula-off RD (Figure Even though the border of detachment was convex, the gravity dependency of fluid and the presence of smoothness or corrugations of the retinal surface couldn’t be judged well due to the shallowness of the detachment. B scan - to rule out posterior scleritis - showed unremarkable findings. To rule out the exudative RD, FA was ordered and showed retinal vascular leakage corresponding to the inferior area of detachment Figure .Therefore, a vasculitis work-up was ordered and showed unremarkable findings. Contact lens fundus examination showed inferior dialysis from 5.00 to 7.00 clockwise, which was managed with buckling surgery Figure . The patOn follow-up examination, BCVA was improved to 0.4, the retina was flat and the leakage on repeating FA after eight months was absent (Figure This case report followed the CARE checklist for case reports guidelines . RRD affMissed retinal breaks in RRD are responsible for almost 65% of the cases of failed RD surgery .The detection and assessment of areas of leakage in FA images are crucial for the management of different choroidal and retinal diseases. There are many causes of leakage in FA. However, ophthalmologists should consider rhegmatogenous retinal detachment as one of the differentials of leakage in FA. Meticulous fundus examination with the appropriate lens should be done for all patients with visual loss complaints before running into extensive investigations.


Panthera tiger) treated with a gonadotropin-releasing hormone (GnRH) agonist to control reproduction, detailing histomorphological and immunohistochemical characteristics. Reproductive cancer affects reproduction, and since maintenance of fertility in large felids is essential for captive breeding programs, it is important to regularly monitor animals for development of neoplasia, allowing for early diagnosis and effective management. Finally, the possible role of contraceptives in the pathogenesis of cancer in non-domestic felids is worthy of future investigation.Reproductive system cancers occur frequently in captive non-domestic felids, negatively impacting wildlife conservation by affecting reproduction. Ovarian tumours are very rare and occasionally mentioned in retrospective studies of large numbers of wild felids aimed at determining tumour types, prevalence rates, and species distribution. In this report, we describe the occurrence of an ovarian leiomyoma in a 22-year-old white tiger (Panthera tiger), treated with deslorelin implants, detailing pathological and immunohistochemical characteristics. Gross examination revealed a markedly enlarged, firm, whitish right ovary with a multinodular appearance. On a cut surface, multiple brown-fluid-filled cysts interspersed with solid grey-to-white areas were observed. On histopathological examination, the ovary was enlarged and replaced by a densely cellular neoplasm composed of spindle cells arranged in fascicles, or occasionally in a herringbone pattern, embedded in a large stroma of collagenous connective tissue. Neoplastic cells showed mild nuclear atypia and pleomorphism and low mitotic rate. Immunohistochemistry confirmed smooth muscle origin of the neoplasm, and cells were positive for vimentin, alpha-smooth muscle actin, desmin, and caldesmon. A low rate (<1%) of Ki-67-positive cells was observed. Although rare, ovarian leiomyoma should be considered when a mass is present in the ovary of a tiger with reproductive failure. Because cancer of the reproductive system impacts on species conservation by affecting reproduction, regular health monitoring is warranted to support wildlife conservation. Finally, the adverse effects associated with long-term treatment with synthetic GnRH analogues as contraceptives in non-domestic felids are worthy of future investigation.Ovarian leiomyomas are very rare in domestic cats and occasionally mentioned in studies reporting general pathological findings and neoplasm occurrence in non-domestic large felids. This report describes a case of ovarian leiomyoma in a 22-year-old white tiger ( Neoplastic disease causes high morbidity and mortality rates in wildlife , and theIn non-domestic felids, neoplasms are common and often malignant ,9. A grePanthera tigris), mammary tumours account for almost 60% of all reproductive neoplasms, followed by uterine neoplasms with a high prevalence of leiomyomas [Panthera tigris).Particularly, in tiger  kept in a circus in the province of Catania , was presented to the Department of Veterinary Sciences of the University of Messina for post-mortem investigations. The owner reported that the tiger suffered from severe recurrent respiratory symptoms in the last three years of its life, and showed nonspecific clinical signs in the previous two weeks, including anorexia, weight loss, and weakness with permanent lateral recumbency. The tiger was euthanized for poor health conditions. The tiger received regular vaccinations for Felid Herpesvirus 1, Feline calicivirus, and Feline panleukopenia until the age of 4, was periodically treated to control internal parasites, and was treated for up to 4 years with deslorelin implants  for reproduction control.A 22-year-old, intact female white tiger  due to prolonged lateral decubitus. All mucous membranes were pale pink/white on physical examination.Upon opening the abdominal cavity along the midline, the right ovary was 23 cm × 18 cm × 6 cm in size, partially firm, whitish, with a multinodular appearance, and characterized by extensive peritoneal adhesions with adjacent abdominal viscera . The cut surface had multiple cystic cavities, varying in size, and filled with yellow to brownish fluid interspersed between firm grey/white to mottled dark red solid areas. A diffuse and moderate dilation of the uterus was noted, with a thinner and paler uterine wall, and accumulation of viscid fluid inside, compatible with mucometra. No macroscopic metastases were found in other organs. Additional findings during gross pathological examination included: chronic, diffuse, and severe bronchopneumonia with multifocal areas of alveolar emphysema and oedema and severe cardiac dilation.Representative portions of the ovarian mass were sampled and fixed in 10% neutral buffered formalin, and after trimming, samples were routinely embedded in paraffin for histopathological examination. Paraffin sections 3 μm thick were stained with hematoxylin and eosin (HE) and Masson’s Trichrome stain using a commercially available kit  and following manufacturer’ instructions.2)  . ScatterWith the Masson Trichrome staining, the cytoplasm of smooth muscle cells was stained red, and thin, abundant, blue-stained collagen bundles intersected between individual neoplastic cells .Immunohistochemistry (IHC) was performed on paraffin-embedded 3 μm thick tissue sections, using the Ventana BenchMark ULTRA automated platform with cell conditioning 1 for 64 min and pre-peroxidase inhibition and primary antibody incubation for 16 min at 37 °C. The OptiView DAB IHC Detection kit  was used to detect protein expression of the following primary antibodies: vimentin ; alpha-smooth muscle actin ; desmin ; caldesmon ; Ki67 ; inhibin, alpha . Slides were counterstained with Haematoxylin II  and Bluing Reagent  for 4 min at room temperature. Negative controls were obtained by omitting the specific antisera and substituting PBS for the primary antibody. Suitable positive controls were used for the IHC reactions.Immunohistochemically, spindle neoplastic cells were positive for vimentin, alpha-smooth muscle actin, desmin, and caldesmon . ConversOvarian cancers have been described in all domestic species, although they are rare, and this most likely could reflect the high proportion of spayed animals examined by veterinarians rather than an inherent resistance of the ovaries to tumour development . The claPanthera tigris) as an accidental finding at necropsy. Previous retrospective studies reporting general pathological findings and neoplasms in a cohort of large felids have rarely and summarily reported ovarian leiomyoma in Panthera species, such as lion (Panthera leo), leopard (Panthera pardus), and tiger (Panthera tigris) [Panthera species, representing a common gross pathological finding at autopsy [Here, we detail pathological and immunohistochemical features of an ovarian leiomyoma in a white tiger ( tigris) ,10,11,12 autopsy ,9,10,11. autopsy ,9,10,11.Morphological features that support the diagnosis of ovarian leiomyoma include the arrangement of cells in intertwined bundles, occurring in both long and cross sections, with occasional herringbone pattern of growth. Neoplastic cells resembled smooth muscle with scant eosinophilic cytoplasm and “cigar-shaped” nuclei with blunt ends. The main differential diagnoses for the tumour described here included well-differentiated leiomyosarcoma, thecoma, and fibroma. Although it may be composed of spindle-shaped cells that maintain the morphology of normal smooth muscle cells with mild pleomorphism, well-differentiated leiomyosarcoma is generally more cellular, and mitoses can be conspicuous (1–2 in high power field) . In addiIn large felids, a causative role of hormonal treatments in cancer of the reproductive system has been suggested, although data in the literature are sometimes conflicting ,8,12,21.e.g., mucometra, pyometra) have been associated with long-term treatment with deslorelin in bitches [The tiger included in this study was treated with a GnRH agonist , which acts by suppressing ovarian follicular activity for 4–14 months . In gene bitches . TherefoPanthera species.Ovariohysterectomy is the method of choice for preventing the development of reproductive tract neoplasms in domestic cats, while the reproductive management of threatened or endangered zoo-maintained felids requires safe and reversible contraception . Indeed,Here, we have described a case of ovarian leiomyoma as an accidental finding during necropsy in a white tiger, detailing pathological and immunohistochemical findings and discussing the possible aetiology. Research into the role of widely used contraceptives to control reproduction in non-domestic felids is needed to shed light on risk factors for the development of reproductive system tumours in these animals, and health monitoring is warranted to support wildlife conservation. Investigations on wild and captive felids contribute to a better knowledge and understanding of the tumour types developed by these species, paving the way for early diagnosis and effective management.


Adenoid cystic carcinoma (ACC) is a rare tumor, with a yearly incidence of 1.4 cases per million inhabitants in northern Portugal.ACC has been described as a tumor that grows and invades local tissues, with a high propensity for infiltrating through perineural invasion, but regional lymph node metastization is regarded as rare.This biological behavior translates into the characteristic development of late distant metastases (DM), despite the optimal treatment with combined surgery and radiotherapy (RT), with a favorable 5 year survival rate but poor long term survivals.The frequent development of distant metastases determines the unfavorable outcomes. Even when locoregional control has been achieved, patients frequently develop DM several years after the initial diagnosis.In this paper, we report a case of a patient with ACC that develops pachymeningeal carcinomatosis. Few cases of hematogenous DM of the dura by ACC have been published, however, as far as we are aware, this is the first one of a submandibular gland primary ACC.We report the case of a 56-year-old female patient who presented with a painless swelling on the left submandibular gland. After an aspiration biopsy compatible with a pleomorphic adenoma, she underwent a left submandibulectomy with lymph node dissection of the adjacent compartments. The definitive histological report showed a high-grade ACC, with a solid pattern present in more than 30% ; nodal mThe patient was staged as pT2N2a (ENE+) R1 (AJCC 8th edition) and was proposed for RT and radiosensitising CTX. She received 50–60 Gy to the base of the skull and left nodal levels IA to IV, and 66 Gy to the surgical bed , with concomitant cisplatin and was kept under surveillance. After 8 months, she was diagnosed with vertebral metastasis and a surgical procedure for vertebral stabilization of L3 and D8 was done. At that time, the FDG-PET/CT showed extensive bone involvement, so the patient was proposed for palliative RT to D8 and L3  and started CTX with carboplatin and paclitaxel, and bisphosphonates. After completing the 6 cycles, the FDG-PET/CT revealed worsening of the bone metastasis and liver metastasis, and the patient started a 2nd line of CTX with gemcitabine. Three months later, the FDG-PET/CT questioned the existence of metastatic cerebellar involvement , and theThe neurological examination showed only a slight ataxia and hypoesthesia of the left thigh. The patient was proposed for whole brain RT and received 30 Gy in 10 fractions, and then started CTX with pegylated liposomal doxorubicin. In the following months, the patient remained under CTX and presented neurologically stable, with no evidence of clinical intracerebral condition worsening. The patient died of multiple systematic metastasis 4 months after finishing RT.The overall frequency of intracranial invasion of ACC has been reported to be between 4% and 22%.Direct extension and perineural spread have long been recognized as methods of spread to locations of the intracranial metastatic lesions. This explains why most intracranial metastasis are located at the base of the skull, having extended transcranially directly or perineurally along cranial nerves from the primary lesions in neighbor structures as nasopharynx, paranasal sinus, and the lacrimal gland. Even though hematogenous metastasis to liver, lung and bone are reasonably common in the late course of this disease, true hematogenous metastases to the intracranial compartment are rare.6We present a patient with a presumed hematogenous extensive metastases to the dura from a submandibular gland ACC. In this case, the hematogenous route must be invoked to explain the location of the lesion. The lesion in our case, while partially extra-axial, is in an anatomic position inconsistent with retrograde perineural spread from the primary site in the submandibular gland and, furthermore, MRI showed no evidence of contiguous spread of the tumor to support direct extension across the base of the skull.The few reports existent in the literature consistent with hematogenous intracranial spread are, in the majority, in a form of parenchymatous metastasis, from primaries tumors from the breast,Hematogenous intracranial metastization is a particularly unusual way of spread of this tumor, with a narrow variety of primary sites and intracranial locations reported. No cases of involvement of the dura from a submandibular gland primary are reported in the literature, making this the first report of dura involvement of ACC from the submandibular gland.The authors declare no conflicts of interest.