In multiple myeloma and its precursor stages, precise quantification of tumor load is of high importance for diagnosis, risk assessment, and therapy response evaluation. Both whole-body MRI, which allows to investigate the complete bone marrow of a patient, and bone marrow biopsy, which is commonly used to assess the histologic and genetic status, are relevant methods for tumor load assessment in multiple myeloma. We report on a series of striking mismatches between the plasma cell infiltration estimating the tumor load from unguided biopsies of the bone marrow at the posterior iliac crest and the tumor load assessment from whole-body MRI. Sixty percent or higher PCI, Light-chain ratio ≥100 of the involved serum light chain vs. the uninvolved serum light-chain, >1 focal lesion ≥5 mm in MRI, elevation of Calcium concentration in serum by ≥0.25 mmol/l above the upper limit or ≥2.75 mmol/l, respectively, Renal insufficiency (creatine clearance <40 ml/min or serum Creatinine >173 mmol/l), Anemia with hemoglobin concentration reduced by >2 g/dl below the lower limit or <10 g/dl and ≥1 osteolytic Bone lesion in CT, PET-CT or skeletal radiography). The guideline by the IMWG for diagnosis of smoldering multiple myeloma (SMM) requires the absence of any myeloma-defining events or amyloidosis and the presence of elevated levels of serum monoclonal protein ≥30 g/L or urinary monoclonal protein ≥500 mg/24 h and/or a PCI of the bone marrow of 10–60% [The current recommendation by the International Myeloma Working Group (IMWG) for the diagnosis of multiple myeloma (MM) requires at least 10% plasma cell infiltration (PCI) in the bone marrow or a biopsy-proven plasmacytoma and at least one myeloma-defining event. These myeloma-defining events determined by the IMWG can be summoned by the SLiM-CRAB acronym , which is the most commonly used staging system today and classifies MM patients into three prognostic groups by survival probability (ISS I-III) depending on the serum levels of ß2-microglobulin and serum albumin . The ISSFor SMM, as defined by the IMWG in 2014, the IMWG undertook a risk stratification that categorized patients into three or, with the addition of prognostically unfavorable cytogenetic aberrations, four subgroups depending on the 2-year risk of progression primarily based on the fulfillment of three criteria: monoclonal protein >2 g/dl, PCI >20%, and the ratio of involved versus noninvolved serum free light chains (ratio >20). Cytogenetic risk factors , t14;16), +1q, and/or del13q/monosomy 13) have been recognized as an additional fourth criterion [, +1q, anEvaluating tumor load in monoclonal plasma cell disorders is therefore not only essential for diagnosis , but alsWb-MRI allows to assess the complete bone marrow of the patient including focal lesions  and diffIn this report, we present four cases, which have been diagnosed as referenced, with striking mismatches between tumor load estimated by PCI from bone marrow biopsy and the appearance of the bone marrow in wb-MRI as well as one case with coincidental biopsy of a small focal lesion showing a great difference in PCI results when comparing BMT versus BMA analysis.A 62-year-old male presented with one criterion met for the diagnosis of MM: bone marrow biopsy performed 16 days after wb-MRI resulted in a 90% PCI in both BMT and BMA. The wb-MRI showed a low to intermediate diffuse infiltration, which was not expected in a patient with a 90% PCI. Additionally, the wb-MRI revealed a 3.0 cm by 1.9 cm large focal lesion at the left posterior iliac crest, which has been hit coincidentally in the biopsy  of 152 with an absolute level of lambda light-chain of 442 mg/l and a PCI of 60% in BMT and 56% in the cytological analysis of BMA from an unguided biopsy of the right posterior iliac crest. In contrast to the bone marrow biopsy results, the wb-MRI performed 7 days after biopsy showed homogenous T1-hyperintense and STIR-hypointense bone marrow in all parts of the examined skeleton with only one 1.2 cm by 0.8 cm focal lesion in the right posterior iliac crest that had been coincidentally hit by the unguided biopsy (Fig. A 60-year-old female presented with two criteria met for the diagnosis of MM: wb-MRI showed multiple focal lesions, among them a 6.8 cm by 3.0 cm focal lesion at the right posterior iliac crest (Fig. A 51-year-old male presented with three fulfilled criteria for the diagnosis of MM: with wb-MRI showing multiple focal lesions and wb-CT revealing osteolytic lesions both imaging modalities reported positive findings for the diagnosis of MM. Furthermore, bone marrow biopsy results differed greatly with 60% PCI from BMT, which also met the third criterion for diagnosis, and 13% PCI in the cytological assessment from BMA Fig. . When reAccording to the current guidelines, biopsy results contribute to the diagnosis of plasma cell diseases and influence the decision to start systemic therapy, as ≥60% PCI is now a myeloma-defining event . MoreoveImaging prior to biopsy allows to identify locations for sampling representative tissue for PCI calculation, or to identify locations with specific MRI-phenotypes as for example large focal lesions, which were associated with adverse outcome , correspWe conclude that tumor load assessment should not be performed by biopsies alone but accompanied by MRI, preferably of the whole body –24, in o


Small bowel obstruction (SBO) refers to the inability of contents to pass through the lumen of the small intestine. This is a common surgical emergency in the United States. Although intra-abdominal adhesions are the predominant cause, SBO can occur secondarily to various etiologies, be it one cause or several. Management of SBO secondary to adhesions and metastasized rectal adenocarcinoma, complicated by pulmonary, hepatic, and ureteral disease, highlights the criticality of a multidisciplinary approach.We present a case of a 59-year-old male with SBO secondary to rectal adenocarcinoma. Treatment included surgical resection, acute stabilization, referral for outpatient surgical follow-up, and oncologic management. Small bowel obstruction (SBO) entails a disruption in the physiological movement of the gastrointestinal system. The most common cause of SBO in the United States is intraperitoneal adhesions, followed by mechanical obstructions and incarcerated herniations . Tumors Signs of acute SBO include nausea, vomiting, abdominal pain, and obstipation . The freAlthough patients with risk factors such as cancer or herniation can be suspected of SBO, physical exams and history are not enough to make a definitive diagnosis. Plain radiographs are used as a preliminary study to diagnose SBO and rule out the need for emergent surgical intervention . ClinicaClinicians can look for multiple signs indicative of bowel ischemia on CT scan; these include thickening and enhancement of the bowel wall, prominent mesenteric venous supply, and peritoneal edema . Free aiAfter resolution, the rate of recurrence of SBO is closely linked to the management. Patients who undergo surgical intervention tend to have a lower recurrence rate (13%) in comparison to those managed conservatively (21.3%) . ConservA 59-year-old male presented to the emergency department with a complaint of sudden-onset abdominal and suprapubic pain. The pain started twelve hours prior to the patient's arrival at the hospital and was progressively getting worse. Despite taking 1000 mg of acetaminophen, the pain did not improve. The patient also reported experiencing associated symptoms of nausea, vomiting, and diarrhea with streaks of blood. He denied dizziness, fever, chest pain, and shortness of breath.The patient's past medical history was significant for advanced adenocarcinoma of the rectum with metastasis to the liver, which was surgically removed through an end colostomy in June 2022. A significant residual tumor attached to the sacrum, urinary bladder, and prostate area was also noted. Thereafter, the patient underwent palliative chemotherapy. Moreover, the patient has a history of diabetes mellitus, hypertension, and hyperlipidemia.On examination, the patient was hemodynamically stable. The vital signs were within normal limits. The abdomen was mildly distended, with tenderness to palpation. No guarding, masses, or organomegaly was present. A CT scan of the abdomen showed bowel obstruction and metastatic colorectal cancer Figure .The general surgical team was consulted and recommended the patient undergo exploratory laparotomy. During surgery, the abdomen was open through a midline incision. Upon entering the peritoneal cavity, a large amount of ascites was observed along with a massively dilated small bowel descending into the pelvis. Tumor burden was noted in the pelvis, where the small bowel was adhered to the tumor mass. The distal ileum was completely collapsed, causing a closed-loop obstruction in the pelvis. The affected small bowel was resected Figure  and a nePathology report displayed small bowel tissue with serosal surface malignant glands Figure . ImmunohThe patient’s postoperative course was uneventful. On postoperative day three, the diet was advanced to clear liquids, which was well tolerated. He denied any nausea, emesis, fever, or abdominal pain.Small bowel obstruction (SBO) occurs when a blockage in the small intestine leads to the interruption of the intestine's normal flow. Symptoms of small bowel obstruction may include abdominal pain, distension, vomiting, constipation, and inability to pass gas . The etiAnnually, one in every 1,000 adults complains of acute abdomen during hospital admissions, which results in a diagnosis of small bowel obstruction. Elderly populations tend to be more susceptible to SBO due to the increased likelihood of past abdominal surgeries and inflammatory bowel diseases creating adhesions, inflammation, scarring, and strictures . Other cSBO is a potentially serious medical condition that can lead to various complications if not promptly diagnosed and treated. Complications can range from mild discomfort to life-threatening conditions. Some of the complications associated with small bowel obstruction include bowel ischemia, bowel gangrene, sepsis, electrolyte imbalance and dehydration due to vomiting, inability to eat or drink, fluid shifts, organ dysfunction, septic shock, scarring and adhesions, and delayed recovery ,13. The presented case illustrates a complex and challenging clinical scenario involving a patient with multiple coexisting medical conditions due to the progression and metastasis of adenocarcinoma. The patient’s medical conditions included, pulmonary nodules, cholelithiasis, hepatic lesions, rectosigmoid colonic mass with invasion into the urinary bladder and surrounding perirectal soft tissues, and hydroureteronephrosis. This presentation implied aggressive tumor behavior and raises concerns about the feasibility of surgical resection . The invTimely diagnosis and appropriate management are crucial for reducing complications and improving outcomes. Diagnostic management typically involves a combination of clinical assessment, imaging studies, and laboratory tests. The goal is to confirm the presence of obstruction, identify its location, determine the underlying cause, and assess any complications Figure . Common protocols in conjugation with physical, history, and laboratory tests include abdominal X-ray and CT scan ,11. TreaIn recent years, small bowel follow-through (SBFT), which involves the administration of contrast material followed by X-ray imaging, has become valuable as it can predict which patients can be managed non-operatively and which will require surgery. This fluoroscope technology has been found effective in predicting surgery, reducing the need for surgery, and limiting inpatient stay duration in patients with adhesive SBO . The conWhen dealing with patients diagnosed with rectal adenocarcinoma and metastasis, the choice between exploratory laparotomy and laparoscopy is a critical decision that requires careful consideration. These two surgical approaches offer distinct advantages, each tailored to specific clinical scenarios. Exploratory laparotomy, characterized by a larger incision and comprehensive access, is an optimal choice when an exhaustive abdominal cavity exploration is imperative . This apMortality rates associated with SBO have improved over the years due to advancements in surgical techniques, imaging, and critical care management . The morThis case highlights the multifactorial nature of SBO. Although adhesions are the leading cause of SBO, it is important to follow an interdisciplinary approach. In cases such as this, with the involvement of adhesiolysis, tumor burden, and ostomy placement, especially given the extent of tissue invasion, short- and long-term goals of medical intervention warrant deliberation. Patient disposition at discharge following acute SBO resolution should be considered when deciding disease management. However, just as inpatient care is managed, a careful and attentive multidisciplinary follow-up is critical to optimize long-term healthcare priorities.


Glucagonomas, neuroendocrine tumors originating from the pancreas marked by excessive glucagon secretion, present a diagnostic challenge due to their rarity and diverse symptomatology. In this report, we present a 47-year-old female with a history of bariatric surgery, diabetes mellitus, and deep vein thrombosis who exhibited weight loss, anemia, migratory necrolytic erythema on the lower limbs and groin, and fecal incontinence. Imaging revealed liver secondary lesions without an identifiable primary tumor. After undergoing surgery, a pathologic examination of the excised tissue confirmed that the lesions were a glucagonoma. This case underscores the imperative of how common side effects of bariatric surgery could mask symptoms, delaying the diagnosis of glucagonomas. Glucagonomas are rare glucagon-secreting neuroendocrine tumors (NETs) originating from the alpha cells of the pancreas . They arGlucagonomas should be suspected in patients with NME, whether associated with other symptoms or not. Elevated serum glucagon levels, over 500 pg/mL, along with serum concentrations of amino acids and zinc, should be assessed. A complete blood count and blood smear should be obtained to check for concurrent normocytic anemia. It is also essential to get serum levels of parathyroid hormone, gastrin, insulin, pancreatic polypeptide, serotonin, vasoactive intestinal polypeptide, prolactin, and adrenocorticotropic hormone, as glucagonomas can rarely be associated with multiple endocrine neoplasia type 1 (MEN1) syndrome. A helical multiphasic contrast-enhanced CT scan is recommended, while MRI should be performed in cases of indeterminate lesions and may have better sensitivity in detecting liver metastases ,4. The oIn February 2023, a 47-year-old female patient went to a general surgeon's office, with complaints of general discomfort, anemia, erythema on the lower body, and diarrhea. Given her fragility and concerning symptoms, she was admitted to a hospital in Rio de Janeiro, Brazil.She had a surgical history of a gastric bypass in August 2022 and also a five-year progression of diabetes mellitus, hypothyroidism, and a history of deep vein thrombosis (DVT) in October 2022.During her pre-operative checkup for the bariatric surgery, a CT scan showed some hepatic nodules, which were presumed to be hemangiomas. Nevertheless, after the bariatric surgery, she had a worsening of her general status and showed refractory anemia, along with sudden weight loss and erythema on her legs and groin, with fecal and urinary incontinence. As months passed, the symptoms worsened, prompting her to get a medical assessment.Upon admission to the hospital, a CT was performed, revealing secondary neoplastic lesions on her liver, with no identifiable primary tissue source Figure . The maiAt admission, the physical examination revealed a subdued mood and a flaccid abdomen, painful upon deep palpation, and both inferior limbs had erythematous lesions extending up to the groin region, with no signs of DVT Figure .Laboratory measurements upon admission are shown in Table Based on the patient's medical history, imaging findings, and physical examination, a diagnosis of a NET associated with a glucagonoma was considered. As part of the treatment plan, the patient received a transfusion of three packs (500 ml each) of red blood cell concentrate, and octreotide was started. Additionally, the administration of Clexane (enoxaparin) was suspended, and fasting measurements of glucagon, insulin, and glucose were solicited. Surgery was scheduled for the following day as the next step in the patient's management.Following the initial surgical plan, the patient underwent surgery the following day. Unfortunately, due to a lack of hospital resources, measurements of glucagon and insulin were not obtained as initially intended. During the surgical procedure, which involved video-laparoscopic hepatic biopsy, a pathologist was present to collect a tissue sample from the surgical site Figures , 4. ThisSubsequently, a post-surgical computed tomography was conducted, revealing several notable findings. These included the insertion of a drain on the right flank with the endpoint on the left flank, evidence of partial hepatectomy of the lateral segments of the left lobe, and indications of partial resection of the largest suspected liver lesion, previously measured at 7.5 x 5.5 cm but now measuring 5.2 x 4.7 cm in segment IV-a. Furthermore, a new heterogeneous elongated lesion with contrast medium impregnation was identified in segment IV-b, measuring 3.0 x 2.3 cm, adjacent to the medial hepatic border and the surgical clips Figure .The remainder of the liver displayed a regular contour with multiple hypodense nodular lesions, the largest of which exhibited heterogeneity, areas of cystic/necrotic degeneration, and irregular contrast enhancement, raising suspicions of secondary lesions. The patient remained in the hospital for 10 days following her surgery, during which she received two additional blood transfusions. Subsequently, she was discharged and placed under ongoing supervision to monitor her recovery and progress. As of now, eight months after surgery, the patient is in a stable clinical condition and being treated for malnourishment.Glucagonomas are excessively rare tumors that are not easily diagnosed. According to an epidemiological study, the average time between the occurrence of symptoms and the actual diagnosis was two and a half years, and of the diagnosed patients, half of them already had tumor metastases, and about 82% of them presented with NME, demonstrating that this clinical sign can be determinant on glucagonoma diagnosis . In our With an estimated incidence of one in two million people and unclear pathogenesis, some glucagonomas are associated with mutations in the MEN1 gene. At the same time, malnutrition, particularly deficiencies in zinc, amino acids, and fatty acids and liver dysfunction have been hypothesized as potential causes of this rare tumor. Differential diagnosis is further complicated by the existence of an even rarer syndrome known as pseudo glucagonoma syndrome. Additionally, cutaneous manifestations of other disorders can introduce diagnostic challenges due to their similarity to NME, a characteristic sign of glucagonoma. Examples of such disorders include pellagra, eczema, acrodermatitis enteropathica, pemphigus, and candida .Interestingly, there is no clear correlation between the NME and the presence of hyperglucagonemia, as this sign can also be present in other diseases such as cirrhosis, malabsorption disorders, inflammatory bowel disease, and chronic pancreatitis. However, it is important to note that in some cases, this distinctive skin eruption may serve as the initial manifestation of the disease. Patients may initially receive topical treatments with antibiotics or antifungals, but these treatments mostly yield refractory results . An examSeveral case reports have highlighted the diagnostic challenges and delays associated with glucagonoma, often leading to diagnostic confusion. One case report highlighted the incidental nature of diagnosis in a patient whose serum glucagon levels were nearly within the normal range (225 pg/mL). Typically, a glucagonoma diagnosis is considered with levels of 500 pg/mL or higher. In this unique case, the diagnosis became possible due to the fortuitous measurement of serum glucagon levels coinciding with the incidental discovery of a pancreatic mass . LikewisWhen a glucagonoma is suspected but cannot be identified via abdominal CT scan, selective celiac, and superior mesenteric arteriographies are preferred due to the tumor's pronounced hypervascularity. Diagnosis confirmation involves histopathological techniques, including immunohistochemistry, electron microscope analysis, and in situ hybridization of glucagon messenger RNA . SurgicaIn cases of hepatic metastasis, resection has been recommended in patients without widespread liver involvement, diffuse extrahepatic metastases, and decreased liver function. Resection has led to a decrease in glucagon levels and significant improvement in NME . In thisOur case, to the best of our knowledge, is the only reported case report of a post-bariatric patient developing a glucagonoma. This case underscores the critical importance of considering all presenting symptoms, both typical and atypical when diagnosing a disease. Despite the patient's relatively young age compared to the typical age of presentation, all symptoms were evident. However, our study does have limitations, primarily the absence of certain laboratory measurements that could have further enriched the case. Our patient received nutritional support to mitigate the effects of the tumor, as complete excision, unfortunately, was not possible.In conclusion, this case report sheds light on the rare occurrence of hepatic glucagonoma in a post-bariatric female patient, a scenario previously unreported in medical literature. Glucagonomas are indeed challenging to diagnose due to their rarity and the diverse symptoms, which often lead to diagnostic delays. For this patient, some of these signs were masked by the effects of a bariatric surgery. Early recognition, multidisciplinary care, and thorough evaluation are the key to achieving optimal outcomes for patients. Further research and reporting of cases like this one will contribute to a better understanding of this rare NET and improve patient care and management strategies.


The most prevalent type of primary retroperitoneal tumors is soft tissue sarcoma (STS). Liposarcoma accounts for 40 % of retroperitoneal tumors . Retroperitoneal liposarcoma accounts for 12 % to 40 % of all liposarcomas . They typically present with advanced disease and often carry a poor prognosis. Because of their rarity and anatomic location, these malignant tumors can cause a diagnostic dilemma and present several therapeutic challenges .A 48-year-old male patient presented to our gastrointestinal oncology clinic with a 2-year history of abdominal discomfort, weight loss and steadily growing abdominal swelling. A soft, rubbery lobulated mass with a positive “slippage sign” was palpable over all the quadrants of the abdomen. CT scan conclusion was retroperitoneal lipoma with internal enhancing nodular components. During laparotomy, a fatty mass measuring 55*60*22 cm and weighing 14 kg was excised. Histopathologic report showed a well differentiated liposarcoma (WDLS).Giant retroperitoneal liposarcoma (RPL) is exceedingly rare. Liposarcomas have diverse MRI and CT appearances due to the various subtypes. WDLS are difficult to identify from lipomas before surgery. Histopathology is the only way to provide a reliable diagnosis; therefore en block resection is the recommended approach when malignancy cannot be ruled out.Although imaging with ultrasound, CT, and MRI can aid in the diagnosis, differentiating WDLS from lipoma pre-operatively can be difficult, and en block resection is the recommended approach when malignancy cannot be ruled out. •Giant retroperitoneal liposarcoma (RPL) (>30 cm in diameter/ >20 kg) is exceedingly rare with only a couple of reports in the literature.•In this article we describe a case of a 48- year- old male patient who had a massive retroperitoneal well differentiated liposarcoma measuring 55*60*22 cm.•Well differentiated retroperitoneal liposarcomas are difficult to identify from lipomas before surgery.•Histopathology is the only way to provide a reliable diagnosis; therefore en block resection is recommended when malignancy cannot be ruled out.•Following surgery, regular follow-up is advised, as recurrence is a possibility. Most A malignant tumor that arises from adipose tissue is called a liposarcoma. Anywhere that has fat tissue is where it can happen . AccordiWell-differentiated retroperitoneal liposarcomas are slow-growing, low-grade tumors that frequently reach large size before exhibiting symptoms and being found, raising the risk of surgery and the possibility of dedifferentiation . It can 2A 48-year-old male patient presented to our gastrointestinal oncology clinic with a 2-year history of abdominal discomfort and steadily growing abdominal swelling. He had experienced significant weight loss since the onset of the illness. He complained of occasional early satiety but no nausea or vomiting. He was constipated seldom but he experienced no significant changes in his bowel habits or rectal bleeding. He has no known chronic medical or surgical disease. He does not consume alcohol or smoke cigarettes. He does not use any illicit or prescribed drugs.2.1No noteworthy personal or family history was reported by the patient.2.2The patient appeared to be chronically ill (emaciated). His vital signs were all within normal limits. His conjunctivae were pink. The abdomen was severely distended, with bulging flanks . A soft,2.33/μL, HGB = 16.5 g/dl, PLT = 176 × 103/μL.He was investigated with complete blood count (CBC), WBC = 4.4 × 10Serum albumin level: 4.2 g/dl and serum electrolytes and organ function tests, which were all within normal limits.2.42.4.1Huge echoreflective intraabdominal mass with homogenous surface filling the whole abdomen; there is focal hypoechoic lesion within the mass measuring 5 cm*7.2 cm. No free fluid seen.Index: Mesenteric lipoma.2.4.2Huge fat attenuated intraabdominal mass measuring 30 cm*36 cm*39 cm, internal enhancing two solid components seen within the mass having different tissue attenuation, the larger one measures 7.8 cm*8 cm. Bowel loops are pushed posterior to the mass.Index: Intra-abdominal fat attenuated mass likely retroperitoneal lipoma with internal enhancing nodular components , Fig. 3Fig. 3.Fi3We diagnosed the tumor as a huge retroperitoneal lipoma based on its clinical symptoms and imaging evidence indicating an adipose origin. However, due to its large size and enhancing solid components, the possibility of malignancy could not be ignored. In addition, an MRI is typically needed to adequately define soft tissue masses, but one was not available to us.4After completing pre- operative assessment and optimization, three units of whole blood were prepared. Informed consent was obtained from the patient and he was taken to the OR. After administering prophylactic antibiotics, Ceftriaxone 1 g stat 30 min prior to skin incision, Patient was put under general anesthesia, positioned supine and exploratory laparatomy was performed.Intra-operative finding: During laparotomy, a bulky yellow to orange mass arising from the left retroperitoneum within a translucent capsule and almost filling the entire abdominopelvic cavities was discovered. Sigmoid, descending, and transverse colon were pushed to the right side of the abdomen, the left ureter and gonadal vessels were adherent on the right side of the mass, but it did not involve the Gerota's fascia, so the kidney was preserved. The bulk went through the mesocolon and was mildly adhered to the mesocolic vessels.  The smalDone: We did not do en bloc resection with the bowel because the tumor was grossly benign. The mesocolic vasculature was carefully isolated from the mass. We dissected away the left ureter, the gonadal vessels and the retroperitoneal vessels  and The mass was completely excised, measuring 55*60*22 cm and weighing 14 kg, with all other structures preserved except for some part of the dome of the bladder which was excised with the tumor. The bladder was repaired in two layers. Hemostasis was secured, and the abdomen closed after a drain was left in place , Fig. 6Fig. 6.FiPost-op course: He had an uneventful post operative recovery. Because he began ambulating soon after surgery, he was not given chemical DVT prophylaxis. On post-op day 2, enteral feeding was initiated and tolerated. The abdominal drain was removed after 6 days, when the output had greatly decreased, and the patient was discharged on the eighth postoperative day.4.1Section showed mature fat with low cellularity and variable sized adipocytes, bands of fibrotic stroma containing spindle cells having enlarged, hyperchromatic nuclei. There are focuses of lipoblasts with markedly atypical, few mitotic features. , Fig. 8Fig. 8 AlHe was observed at the outpatient clinic for a year after the operation. His imaging tests and clinical examinations were both clean. He'll keep up the constant monitoring.5Liposarcoma makes up 45 % of retroperitoneal soft-tissue sarcomas in adults, making it the most common kind . AlthougAccording to the World Health Organization's (WHO) 2002 classification, liposarcoma is a complex tumor, and categorizing it into three subtypes helps to best describe its pathogenesis. These include pleomorphic liposarcomas (PLS), myxoid and round cell liposarcomas (MLS and RCL), and well- and dedifferentiated liposarcomas (WDLPS/DDLPS). A diagnostic large marker and a ring chromosome which impacts unchecked cell proliferation will be used to identify WDLPS/DDLPS. In MCL and RCL, reciprocal translocation between chromosomes 12 and 16 occurs, leading to the activation of a number of downstream pathways, including PPARgamma2 and C/EBPalpha, which promotes cell proliferation . PleomorThe average diameter and weight of RPL during diagnosis is 20–25 cm and 15–20 kg. Although there is no consensus regarding the precise definition of giant RPL, it is defined by several literatures as an RPL of >30 cm in diameter or with weight of >20 kg. Giant retroperitoneal liposarcomas are exceedingly rare . Only 17Retroperitoneal tumors are typically asymptomatic in the early stages because of the enormous amount of space in the retroperitoneum. Once the tumors have reached massive sizes, local tissue and organ compression may take place, leading to obstructive urinary/bowel symptoms such lower extremities edema, early satiety, and abdominal discomfort. Like in the case of our patient, who had been asymptomatic throughout the illness except from a minor, nebulous abdominal ache. The clinical symptoms are often ambiguous . ImagingThe differential diagnosis of a retroperitoneal lesion might be narrowed down by the presence of fat on imaging. When investigating abdominal masses, ultrasound is frequently used as the first imaging method. An additional diagnostic method for retroperitoneal tumors is the use of CT and MRI. On computed tomography, fat typically attenuates minimally (10 to 100 HU). On CT and MRI, they show up as uniform fat-containing masses with thin septa. Because it has a higher sensitivity for displaying microscopic fat using chemical shift imaging and macroscopic fat using fat-suppression techniques, magnetic resonance imaging is a better imaging modality .Retroperitoneal liposarcomas are difficult to identify from lipomas before surgery. Both lipomas and WDLPS have a large amount of fat and minimal soft tissue and have similar appearances on CT and MRI, making it difficult to differentiate lipomas from well-differentiated liposarcomas preoperatively  On CT, cTherefore, the final diagnosis rests on histopathological evaluation to assess mitotic activity, cellular atypia, necrosis and invasion . DespiteThe main treatment for non-metastatic retroperitoneal liposarcoma is currently surgery. If local invasion is confirmed, the ultimate surgical strategy requires the removal of adjacent organs like the kidney and intestines as well as the complete surgical excision of the tumor with negative margins . In 1988This case has taught us that, even when a benign lipoma is clinically suspected, intraoperative tumor characteristics and subsequent judgment calls regarding the scope of resection are crucial. A wide excision should be performed if infiltrative growth is suspected because resection with negative margins (R0) is essential to the patient's prognosis in cases of liposarcoma ,39. OnlyOne of the most crucial prognostic indicators is histopathology, which shows the characteristics of the tumor and the degree of differentiation. The five-year survival rate for well-differentiated liposarcoma in the retroperitoneum is around 90 %. Dedifferentiated, myxoid, and pleomorphic variant rates, on the other hand, range from 30 % to 75 %, with pleomorphic variant having the worst prognosis . All lip6Giant retroperitoneal liposarcomas are very uncommon. Because of the peculiar nature of the retroperitoneum, it can grow to attain a massive size with no clinical signs, as demonstrated in the above instance. Although imaging with ultrasound, CT, and MRI can aid in the diagnosis, differentiating WDLS from lipoma pre-operatively can be difficult, and en block resection is the recommended approach when malignancy cannot be ruled out. Histopathology is the only way to provide a reliable diagnosis. Following surgery, regular follow-up is advised, as recurrence is a possibility.7The important take away from this case report is that clinically and on CT/MRI, it is difficult to distinguish a benign retroperitoneal lipoma from a WDLS. As a result, even for benign disease, wide local excision using oncologic principles remains the mainstay of treatment.Not applicable.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Tilahun Habte Nureta and Wongel Tena Shale are the guarantors of this study.Case reports are exempted from ethical approval in Jimma University. As long as identifying patient information is removed and written consent is taken from the patient.There are no sources of funding to declare for this study.Tilahun Habte Nureta contributed to writing of the manuscript, was the lead surgeon in performing the surgery, data collection and final follow up of the patient. First and Final draft of the article was written by Wongel Tena Shale, who also participated in the surgical treatment of the patient. Tewodros Belete Deneke did the histopathology examination and report. Critical revisions and final approval were made by all authors.None to declare.


Ankyloglossia is a congenital alteration in the development of the tongue characterized by the presence of a short or thick lingual frenulum, which leads to a limitation in its movements. There is an associative inconsistency between ankyloglossia and complications with breastfeeding, speech, swallowing, breathing, and the development of orofacial structures, and it is urgent to make more scientific research in this area. In the presence of polydactyly and syndactyly may be also present ankyloglossia. The purpose of this paper is to present two ankyloglossia cases with finger alterations, without a syndromic disease, and lead the medical team to research this topic and make an improved treatment plan for these cases. In recent years, interest in the topics of lingual frenulum and ankyloglossia in the scientific community has increased, due to its impact on the motor function and development of the oral cavity. The American Academy of Pediatric Dentistry (AAPD) defines ankyloglossia as a congenital alteration in the development of the tongue characterized by the presence of a short or thick lingual frenulum, which leads to a limitation in its movements ,2. MillsCurrently, there is an associative inconsistency between ankyloglossia and complications with breastfeeding, speech, swallowing, breathing, or the development of orofacial structures, with many published articles indicating the need for more studies of the high scientific impact of this topic ,2,4.As an oral surgery that involves incision and/or excision, hemostasis, and correct healing of the surgical wound, the AAPD recommends a soft diet, regular oral hygiene, and, if necessary, analgesic usage. Postoperative exercises are recommended to prevent reattachment of the wound. The use of electrosurgical or laser technology has demonstrated a shorter operative time, better bleeding management, reduced intra- and postoperative pain and discomfort, no need for sutures, and greater acceptance .Despite being a relatively common disorder in newborns (0.19% in live births) , polydacThe purpose of this paper is to present two cases that associate all these alterations, without a syndromic relationship: the first case presents ankyloglossia, syndactyly and polydactyly; the second case presents ankyloglossia and syndactyly. This could lead to a more detailed assessment of newborns who present these alterations and create a better treatment plan, with a multidisciplinary medical team.Case 1A four-month-old Caucasian boy presented to a private dental hospital in Oporto , for an evaluation of the lingual frenulum, referred by his pediatrician. His medical history was positive for syndactyly in his second and third, fifth and sixth left foot fingers. The patient had also a type A ulnar polydactyly in the left foot, and a type B ulnar polydactyly in both hands Figures , 2. His  On the appointment day, the baby was already on bottle-exclusive formula milk, and, during the lactation session, some alterations were observed in suckling and swallowing, snapback, loss of vacuum, loss of milk by the labial commissure, gagging during deglutition, exaggerated pharyngeal reflex (GAG) and gastroesophageal reflux.The oral cavity was observed and two protocols for evaluation of the lingual frenulum were applied: (1) Hazelbacker Protocol  (ATLFF)  and (2) After discussing treatment options with the family and obtaining informed consent, it was decided to perform polydactyly removal and lingual frenotomy at the same anesthetic moment, with the objective of simplifying and offering less stress to the baby. Regarding the syndactyly correction, it was postponed until after three years of age in accordance with the current guidelines for pediatric anesthesia for all non-emergent routine procedures .In both corrections, surgery was completed in one hour under general anesthesia and proceed without complications. The lingual frenotomy was performed by a diode laser in a simple frenotomy technique : the lin Case 2A three-month-old Caucasian boy presented to a private dental hospital in Oporto , for an evaluation of the lingual frenulum, referred by his family doctor. His medical history was positive for syndactyly in his first and second left foot fingers Figure . His priBreastfeeding ran into some difficulties from the beginning. The mother felt pain when breastfeeding and the baby had episodes of gagging and clicking during suckling and swallowing. In the appointment, the baby's latch and breastfeeding posture was corrected by an International Board Certified Lactation Consultant (IBCLC), which led to an efficient breastfeeding with no pain felt by the mother. The baby was then referred to speech therapy and to the pediatric orthopedics appointment.In the literature , some asDespite the increasing number of articles related to ankyloglossia or “tongue-tie,” it remains uncertain how effective the classification assessment tools are in diagnosing symptomatic tongue-tie and fulfilling lingual frenectomy criteria . For exaOn the other hand, a systematic review  was receIn the first case report, in addition to the two protocols applied (ATLFF and Coryllos), both anatomy and lingual function were evaluated, as well as signs and symptoms associated with ankyloglossia. After the lingual frenotomy, the patient performed speech therapy exercises to ensure optimal healing and lingual mobility. A few weeks after surgery, in the control appointment, with 5 months old, several improvements were observed in terms of oral functionality and motor skills. The patient began to improve free movement (rolling), lifting and holding of the head, no longer losing vacuum in the bottle teat, nor losing milk through the labial commissure. There were no more episodes of choking, the GAG normalized, and the gastroesophageal reflux improved. The rehabilitation work of the physiotherapy, osteopathy, and speech therapy team may have contributed to the outcome.Regarding the second case, despite the ATLFF protocol indicating surgical intervention, it was postponed, as the difficulties with breastfeeding were overcome and a single anesthetic moment is preferred. However, a reassessment is scheduled before the introduction of complementary food, as well as a follow-up by an experienced speech therapist. If syndactyly correction is to be performed at a later time and if tongue functional alterations are, meanwhile, detected, lingual frenotomy will be anticipated and performed under local anesthesia.In conclusion, in the presence of polydactyly and/or syndactyly, ankyloglossia may also be present, without being associated with any type of syndromic disease. This finding may be useful, as polydactyly and syndactyly are more easily diagnosed, and their presence may lead health professionals to search for the presence of ankyloglossia, which is usually associated with some signs and symptoms, mainly related to breastfeeding, at earlier ages, or speech difficulties. Early diagnosis and understanding of ankyloglossia are important, as it allows to prevent early weaning in breastfeeding infants and other possible complications in the future.


Choroidal osteoma is a rare benign bone tumor. It is unilateral in two-thirds of cases. It occurs unilaterally and more often in young women. The tumor is most often located near the papilla. The complications classically described are progressive retinal atrophy and choroidal neovascularization. We report the case of a 39-year-old female patient, with no previous history, who consulted us for a sudden unilateral decrease in visual acuity, associated with metamorphopsias and a para-central scotoma. Fundus examination of the right eye revealed a large, yellowish-white choroidal tumor with fairly well-delineated margins in a juxta-papillary arrangement. Fluorescein angiography showed an early hyperfluorescence becoming intense at late times, associated with papillary edema. In front of this picture, we evoke the hypothesis of a choroidal neovascular membrane. The optical coherence tomography (OCT) showed the presence of a hyper-reflective pre-epithelial lesion in the inter-papillomacular region, associated with a retinal serous detachment, indicating a choroidal neovascular complication. B-mode ultrasonography confirmed the diagnosis by showing a hyperechoic thickening of the choroid with a posterior shadow cone. In our case, it was a choroidal osteoma complicated by optic neuropathy and choroidal neovascularization. The patient received several intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF), followed by long-term monitoring. Choroidal osteoma is a rare benign tumor. It often progresses slowly, but may have complications, in particular choroidal neovascularization, the treatment of which is not well codified. The long-term visual acuity is often very poor.


Pilar cysts/trichilemmal cysts are benign lesions that arise from the hair follicle epithelium. The commonest area of occurrence is the scalp but it can also occur in the head and neck region. The pilar cyst grows at a prolonged rate. They are relatively rare and occur in about 10% of the population. They appear in the region of high concentrations of hair follicles. A 75-year-old male patient came to the Department of Oral and Maxillofacial Surgery with a chief complaint of swelling on the left side of the face for the past one year. Cytological examination revealed an infected cystic lesion. Computed tomography (CT) showed a well-defined lesion in the left temporal region. After surgical excision of the lesion, it was sent for histopathological examination. Excisional biopsy revealed a pilar cyst. We report a rare case of pilar cyst in the left temporal region in a patient who was previously operated on for osteomyelitis of the left side of the mandible up to the coronoid process. These cysts may mimic temporal space infection and lead to an incorrect treatment plan. Pilar cysts, otherwise known as trichilemmal cysts, are benign growths that arise from the hair follicle epithelium. They are most commonly seen on the skin of the scalp but can also occur in other areas of the head and neck regions . Pilar cTrichilemmal cysts could be passed down via the autosomal dominant gene. Patients with familial pilar cysts are typically younger and have multiple lesions at the same time. They develop from the epithelium between the sebaceous gland and the arrector pili muscle. They are most common on the head, especially the scalp. The pilar cyst grows at a very slow rate; it takes several years to grow to a large size . In this case report, we present a rare case of a pilar cyst in the left temporal region of a 75-year-old male with a history of surgery for osteomyelitis of the left mandible extending up to the coronoid process. This cyst was mimicking temporal space infection in the left temporal region.A 75-year-old male presented to the Department of Oral and Maxillofacial Surgery with a history of swelling in the left temporal region that had been slowly growing for the past one year. The patient complained of mild pain and discomfort at the site of the swelling. The patient also complained of restricted mouth opening. The patient also had a history of chronic suppurative osteomyelitis of the left mandible, which was operated on a year ago. The patient had no significant medical history and was not on any medications.On examination, a soft, non-tender, fluctuant swelling measuring 4.5 × 3.0 cm was noted in the left temporal region Figures , 2. The Fine needle aspiration cytology was performed, which yielded dirty brown pus-like material Figure . CytologThe patient was counselled about the benign nature of the lesion and the possibility of surgical excision. Informed consent was obtained from the patient, and the surgery was scheduled. Under general anaesthesia, nasoendotracheal intubation was done, and standard scrubbing and draping were done. Two percent Lignocaine with adrenaline 1:100000 was given as local infiltration. Popowich incision given Figure , layer dThe excised specimen given for histopathological examination revealed multiple sections of tissue which appear cystic in architecture and are lined by hyperparakeratinized stratified squamous epithelium with areas of confluent rete pegs formation. In some areas, the epithelial lining appears stretched. The basal cells are cuboidal to low columnar. The superficial spinous layer abruptly transitions to a dense compact layer of parakeratin filling the lumen without intervening granular cell layer. The supporting dense connective tissue wall shows sub-epithelial dense inflammatory infiltrate along with few satellite cysts and foreign body type giant cell reaction. There is also the presence of moderate vascularity, nerve bundle, skeletal muscle, and adipose tissue along with areas of haemorrhage. At sites, focal inflammatory infiltrate is seen extending into the skeletal muscle. The central portion of the cyst contained keratinous material, consistent with the diagnosis of a pilar cyst Figure . The hisOn follow-up after three months, the patient had no recurrence of the swelling or any major complications. The patient was advised to regularly follow up to monitor the area for any signs of recurrence.A pilar cyst, also known as an isthmus-catagen cyst, develops from the follicular isthmus' exterior root sheath. Around 20% of epithelial cysts are pilar cysts; the remainder are epidermal cysts . Pilar cOur patient presented with a benign, non-tender mass in the left temporal region, which was confirmed via needle aspiration and a CT scan of the head and neck. The patient underwent a successful surgical excision with no postoperative complications. A histopathological examination of the cystic mass confirmed it to be a pilar cyst. At first, this lesion mimicked a temporal space infection, which was misleading. But after performing the fine needle aspiration cytology, it was found to be an infected cystic lesion. Thus the present case emphasizes the important role of fine needle aspiration cytology in any swelling. However, histopathology is necessary to confirm the diagnosis of a pilar cyst especially to rule out its closest differential of epidermal inclusion cyst .Surgical excision is considered the standard treatment for pilar cysts. The procedure is cosmetic and can be done under local anaesthesia or general anaesthesia. In our case, the surgery was done under general anaesthesia. Tissue dissection should be done carefully to prevent spillage of the cystic contents, which could lead to an acute inflammatory response, a foreign body reaction or recurrence. The most important point to be noted here is the crater-like defect that was present on the temporal bone after the excision of the lesion. This defect shows that the lesion was present for a long time.Our patient had a rare coincidence of a pilar cyst in the left temporal region, which is an unusual site of occurrence, along with a history of osteomyelitis of the mandible. Although the presence of a pilar cyst is not related to osteomyelitis, taking a thorough medical history and performing regular follow-ups in such cases is essential for prompt diagnosis and management.Pilar cysts are benign lesions that may occur in various regions of the head and neck area. Surgical excision remains the standard treatment for these cysts, with minimal risk of recurrence. In this article, we would like to conclude that the occurrence of a pilar cyst in a patient with a history of osteomyelitis of the mandible is rare, but taking a thorough medical history in such cases is crucial for prompt diagnosis and management. Regular follow-ups are essential for monitoring the area for any signs of recurrence or complications. Even though such cases occur in rare instances, we, as oral and maxillofacial surgeons, need awareness regarding such lesions for proper diagnosis and to provide the appropriate treatment to the patients.


Multiple hereditary exostosis syndrome is a rare diagnosis with approximately 1:50000 incidence prevailing in males. The exostoses or osteochondromas are benign but have the potential for malignant transformation in 1-5%. There is a strong genetic component, with exostosis (EXT) signaling pathways being an underlying cause. They can be symptomatic, with pain and functional deficit as the main complaints. We present a case of a 17-year-old male who presented with pain and anatomical deformity in his left lower femur. Magnetic resonance imaging revealed multiple osteochondromas compressing the popliteal neurovascular bundle. Excision of the osteochondromas was performed to decompress the neurovascular bundle in a multidisciplinary approach. Histological examination demonstrated no evidence of malignancy. Currently, there is no consensus for patients diagnosed with multiple osteochondromas regarding further investigation and/or screening for malignant transformation. Osteochondromas are defined as bony outgrowths with a cartilage cap . They teMutations affecting the EXT (exostosis) gene or the repeated presence of a family history of EXT gene abnormality have been reported in the majority of patients . Both EXExostosin-1 and 2 are the gene products - they are endoplasmic reticulum localized type II transmembrane glycoproteins crucial in forming a complex that catalyzes a reaction that is critical for heparan sulfate polymerization, an essential step in bone remodeling ,8. In caA 17-year-old male presented with a one-year history of a painful lump on the distal left thigh, located medial and just above the knee joint, most noticeable on walking. It was becoming progressively worse and more recently limiting the ability to walk and participate in sports. Plain X-ray and MRI were obtained Figures . ImagingUnder general anesthesia, with both orthopedic and plastic surgeons in the team, a combined approach was utilized to excise both osteochondromas Figure  using a Histology revealed three bony fragments macroscopically together measuring 25 x 15 x 5 mm. Microscopically sections of cortical bone were identified, with one fragment containing a surface cartilaginous tumor. The base of the tumor was sealed by endochondral ossification, with active bone remodeling. The lesion was continuous with the medullary cavity of the underlying bone. The cartilage cap showed no significant cytologic atypia and measured up to 1.5 mm in thickness. There was no evidence of malignancy.Having multiple osteochondromas is a rare presentation that requires careful understanding to ensure the best outcomes for the patient. Such cases are best managed by a multidisciplinary team that consists of specialists within the field of bone tumors. Given the germline mutations that are most likely the culprits, these patients should be screened for EXT1 and EXT2 mutations. Where a positive family history is present then this screening is not necessary ,10.Malignant transformation should be investigated for growth occurring after growth plate fusion using T2-weighted magnetic resonance imaging . This caIt is important to consider differential diagnoses such as dysplasia epiphysealis hemimelica and metachondromatosis when dealing with solitary and hereditary osteochondromas; however, these have been shown not to impact the EXT pathway ,7. Both Osteochondromas are benign lesions where the risk of malignant transformation is 1-5% The decision to excise them is typically dictated by the symptoms the patient may be experiencing, which are usually pain and functional complaints of nerve/vessel compression ,15. CompIn case of malignancy or proximity to important structures, careful dissection is advised as well as en-bloc resection of the osteochondromas and its pseudocapsule to ensure tumor-free margins .Having multiple osteochondromas is a rare and complex diagnosis that is still yet to be properly understood. Mainstay management includes history, examination, and thorough radiological assessment. The main presenting complaints include pain and a functional deficit. They are associated commonly with a mutation of the EXT gene and should be managed by a specialist multidisciplinary team to achieve a favorable outcome by surgical intervention to reduce the burden of symptoms. Signs and symptoms suggestive of malignant transformation include relapsing and/or worsening pain, regrowth of the tumor, as well as histological features such as internal lytic areas, irregular margins or calcifications, and a cartilage cap thickness >2 cm in adults or >3 cm in children. A screening protocol following primary surgery may offer benefits in the long term in identifying at-risk patients.


We report the case of a retroperitoneal paraganglioma in a 35-year-old man discovered by abdominal pain. The abdomino-pelvic computed tomography (CT) showed a retro-peritoneal latero-aortic mass compatible with a paraganglioma, confirmed by the 24-hour urinary metanephrines and normetanephrines test. After an adequate pharmacological preparation, we decide to perform a laparoscopic resection of the paraganglioma. The surgery went without any complication. Blood pressure and urinary catecholamines were normal after the surgery. Our observation presents the particularity of the mode of discovery which is atypical, as well as the particularity of the therapeutic management, which is the laparoscopic resection of the mass. Paraganglioma, also called extra-adrenal pheochromocytoma, is a neuroendocrine tumor that arises from ectodermal cells of the autonomic nervous system, or from chromaffin tissues remains. The adrenal site is usual (90%), extra-adrenal localization is rare, representing only 10% of paragangliomas with an estimated incidence of 2 to 8 per million [Patient information: a 35-year-old male with no medical history was seen at the emergency department for acute abdominal pain with moderate headache.Clinical findings: on physical examination, we found a performance status of 0, examination revealed high blood pressure (180/90 mmHg) with tachycardia (heart rate: 120 beats per minute).Timeline of current episode: the symptoms had been evolving for 8 months with a gradual exacerbation.Diagnostic assessment: ultrasonography showed a 34 x 31 mm retro-peritoneal mass compatible with retro-peritoneal lymphadenopathy. Abdominal CT scan with contrast was performed and showed a 41 mm oval left latero-aortic mass with heterogeneous enhancement . The mass was in intimate contact with the aorta. This description was compatible with paraganglioma or metastatic lymphadenopathy (enopathy . A 24-hoDiagnosis: considering the data from clinical, radiological and biological examinations, the diagnosis retained is a retroperitoneal latero-aortic paraganglioma secreting.Prognostic characteristics: if the surgical resection is complete, the prognosis remains good with a rapid improvement in clinical signs, in particular arterial hypertension.Therapeutic intervention and follow-up: after anesthetic and cardiac assessment, our patient underwent a laparoscopic resection of the mass which was in intimate contact with the aorta inside, the renal pedicle above and the left ureter outside ( outside .Follow-up and outcome of interventions: after surgery, the patient returned immediately to normal blood pressure and no longer needed to take antihypertensive therapy. Also, urinary normetanephrine levels were back to normal 1 month after surgery. At the anatomopathological level, the excision was complete; macroscopically it is a nodule of 3.3 X 0.5 cm of white-grayish aspect, well encapsulated with some bleeding reshuffle (eshuffle . Histoloeshuffle .Patient perspective: satisfied with the minimally invasive treatment without a large incision, clear improvement in clinical symptoms with normalization of biological assessment.Informed consent: an informed, dated and signed consent was obtained from the patient allowing the said exploration to be carried out.Primary retroperitoneal tumors in adults represent a highly heterogeneous group; more than 80% of these tumors are malignant . ParaganThe preoperative diagnosis is based on biological tests. The most sensitive and specific of these are the 24-hour urinary metanephrines and normetanephrines test with the free plasma metanephrines test. The plasma test offers a superior sensitivity with, however, less specificity than urinary tests. The chromogranin A test lacks specificity and sensitivity but may be useful to diagnose a non-functional paraganglioma and for follow-up . The preRetro-peritoneal paragangliomas are rare, the presentation of these tumors is not always specific. Their management must be multidisciplinary, surgical resection represents the only potentially curative treatment, it can be carried out by open or laparoscopic surgery in a specialized center; in case of incomplete resection the prognosis is reserved and the treatment is completed by radio or chemotherapy.


First branchial cleft cysts (FBCCs) arise due to an incomplete fusion of the cleft between the first and second branchial arches. Classically, they are found inferior to the pinna or along the external auditory canal. This report presents a unique case of a nine-month-old male with a first branchial cleft cyst completely within the pinna. The patient presented with a left auricular pit and pinna mass. Ultrasound revealed a homogeneous hypoechoic mass isolated to the pinna. Surgical resection revealed the cyst to be anterior to the inferior pinna cartilage, with the tract projecting anteriorly and inferiorly. Final pathology revealed a benign cyst lined by squamous epithelium and a rim of cartilage, confirming an FBCC. To our knowledge, FBCCs isolated to the pinna have not been previously reported. Awareness of the various presentations of this rare anomaly is essential for a prompt and accurate diagnosis. First branchial cleft cysts (FBCCs) arise due to a developmental defect of the first branchial cleft and arch. They are rare, representing only 1% of all branchial anomalies . The firA nine-month-old infant presented with a history of a left pre-auricular pit since birth Figure  and the FBCCs are rare with an estimated incidence of one in 100,000 live births . They maBranchial  structures begin to develop early in the fourth week of gestation to become components of various head, neck, and thorax structures. They start as four clefts that form five mesodermal ridges, which later become six branchial arches. The arches then externally form grooves or clefts and internally form pouches. Each arch ultimately transforms into a component of an anatomic structure. Branchial cleft cysts arise following incomplete fusion of branchial arch structures. The first branchial arch becomes the mandible, a portion of the maxillary process of the upper jaw, and a portion of the inner ear. The first branchial cleft and pouch become the external auditory canal (EAC), eustachian tube, middle ear cavity, and mastoid air cells. FBCCs are commonly found adjacent to the EAC. This contrasts the more common second branchial cleft cysts which comprise up to 95% of BCC cases . These aThe pinna develops along with the EAC from the first two branchial arches. Each arch forms three hillocks, which ultimately form the tragus, helix, concha, and antihelix. It first develops in the lower cervical region and then migrates posterolaterally to its final position. It is along this course that the FBCC becomes intertwined with the facial nerve and parotid gland . Given tIn 1972, Work proposed two types of first branchial cleft cysts: type I and type II lesions . Type I Clinically, FBCCs typically present with swelling and sometimes purulent drainage through an open tract. Often, a pit or sinus tract can be visualized, as seen in this patient. Although a benign pathology was presumed for this patient, clinical suspicion was low for a branchial cleft cyst given the unusual location. Imaging studies can help determine the best surgical approach, though there is no clear imaging technique that is universally recommended. US is generally the first-line modality given its ease of use and avoidance of radiation in young children. CT and fistulography introduce radiation exposure, while MRI requires sedation of young patients, making US the most practical option . CT and Complete surgical resection is regarded as the definitive treatment, as infection and the need for incision and drainage procedures can lead to scar tissue, fistula formation, and ultimately recurrence of the cyst. Additionally, surgical pathology ultimately still serves the important role to allow us to confirm our diagnosis of a branchial cleft cyst. Resection is ideally performed when the patient is free of infection and inflammation, sometimes requiring an antibiotic course prior to operation. FBCCs can often involve the facial nerve, making preservation of the nerve a vital part of the surgery. In this case, given the isolated location to the pinna, a simple resection was possible without the risk of injuring the facial nerve and parotid gland. Final surgical pathology ultimately still serves as the gold standard in making the diagnosis of branchial cleft cysts.We present a unique case of a first branchial cleft cyst completely within the pinna. Although mostly benign and asymptomatic, prompt diagnosis of FBCCs allows for resection of any possible cases that may become symptomatic. This case highlights the importance of considering branchial cleft cysts in the differential diagnosis of pinna masses, as the anatomical location of first branchial cleft cysts may be variable.


Pelvic venous insufficiency (PVI) is frequently associated with symptoms of abdominal pain or discomfort that is overlooked or under-diagnosed in women. Despite the fact that pelvic venous insufficiency in men is very well documented, its occurrence in women needs to be further studied. Patients with pelvic varicose veins undergo a long and inconclusive diagnostic work-up before the exact cause of the symptoms is identified. Gonadal venous insufficiency (GVI) is a condition that can present acutely, leading to diagnostic challenges. We present a case report of a 47-year-old female with acute abdominal pain and GVI, where endovascular embolization was used for successful treatment. The patient was diagnosed with GVI based on imaging findings of an enlarged left ovarian vein with retrograde flow and dilated pelvic veins seen on magnetic resonance imaging (MRI) with contrast material. Due to the severity of her symptoms and imaging findings, endovascular embolization was chosen as the treatment modality. The embolization was successful, and the patient’s symptoms resolved completely. This case highlights the challenge of diagnosing GVI with acute clinical expression and the potential benefits of endovascular embolization as a treatment option. Further studies are needed to determine the optimal management strategies for acute GVI, but endovascular embolization should be considered a safe and effective option. At the same time, we present a short review of the recent literature data related to this topic. Varicocele, a varicosity of the gonadal venous plexus, is a well-recognized disorder, occurring in up to 10% of men. In women, an analogous varicosity of the salpingo-ovarian plexus is rare. This may be explained, in part, by the lack of clinical findings on objective examination in women compared with men and the need, until recently, to use invasive venographic methods to confirm the diagnosis . When feVenous disorders of the pelvis are associated with a spectrum of symptoms arising from reflux, most commonly involving the gonadal and internal iliac veins, and obstruction, usually of the left renal and iliac veins. These hemodynamic patterns are associated with at least four broad clinical presentations, including left flank or abdominal pain and hematuria, chronic pelvic pain, venous claudication, and symptomatic lower extremity varicosities in either atypical or typical saphenous distributions, the latter frequently recurring after initial treatment. The relationship between pelvic symptoms and venous pathology is far more complex than in the lower extremity. Multiple symptoms may be present concurrently, and several potential pathophysiologic mechanisms, such as left renal and iliac venous compression, may be simultaneously present. Additionally, similar symptoms may arise from disparate underlying causes , and similar anatomic derangements may lead to different symptoms . This can lead to diagnostic errors and may be responsible for the suboptimal results of many interventions .Transvaginal duplex ultrasound may be a feasible investigation, and may be useful in terms of diagnosis. The sonographic appearances of the ovarian and pelvic varicocele are multiple dilated veins around the ovary and uterus with a venous Doppler signal of varying amplitude. The presence of circular or linear venous structures with a diameter >5 mm is indicative of pelvic varicosities .2), mobile with respiratory movements, spontaneously painful in the lower site, with tenderness and slight muscular defense during palpation, especially in the left iliac region. From the patient’s medical history, we noted 3 pregnancies with 3 natural births and high blood pressure treated with Perindopril 5 mg/Indapamide 1.25 mg, one tablet/day. Blood tests did not reveal significant pathological modifications. The patient was admitted to the Surgical Department for further investigation and treatment.Detailed Case Description. A 47-year-old woman presented herself to the emergency department complaining of diffuse lower abdomen pain, the slowing of intestinal transit, and fatigue. The painful symptomatology affirmatively started about two weeks prior with mild intermittent episodes, which had worsened in the last few hours. The local clinical examination revealed an abdomen with normal adipose panniculus , and it indicated that both kidneys are of normal size, shape, and echo pattern with normal cortical thickness. No calculus, growth, or hydronephrotic changes were seen in either kidney. Corticomedullary junctions of both kidneys were sharply defined. The bladder was moderately distended and did not show any intraluminal pathology. Upon the physical examination of the patient, the bladder dropped only a short way into the vagina, so a mild cystocele (type 1) was seen. The microscopic exam of the urine was normal. A uroflowmetry was performed with a voided urine volume of 280 mL and a maximum flow rate of 16 mL/s; therefore, it excluded a urodynamic pathology. Post-voiding residual volume showed that the bladder was emptied completely.A computed tomography (CT) scan was performed, and a few images of the cystic characters on the left ovary were observed without signs of local complication . The parRegarding local GYN examination, as well as standard transvaginal ultrasound (TVUS), no clinical pathological signs were detected. .The MRI exam using contrast material revealed a thin blade of free intrapelvic fluid (infracentimetric) at the level of the posterior recess, which, in a physiological context, is as follows: a few bilateral, millimetric, functional-looking ovarian cysts ≤5 mm (follicular cysts); parauterine vasculature with dilated (about 7 mm), tortuous, arcuate, T2 hyperintense and T1 flow voids appearance; and the appearance of simple Naboth cyst with dimensions of 4/2 mm . The dia®, GEM, Milan, Italy), ratio 1:1, was injected, with the synchronous withdrawal of the microcatheter until the vein was closed . On the microcatheter placed in the distal portion of the ovarian vein, a mixture of lipiodol and acrylic glue . At six months, a control MRI was additionally performed, with no complications being encountered. In order to evaluate the superficial venous network of the lower limbs, a duplex ultrasound was performed. No signs of venous reflux were identified.When pain appears due to pelvic vein insufficiency, it is most often chronic, and it can be associated with other non-specific manifestations, including dysmenorrhea, dysuria, nausea, bloating, and abdominal and rectal pain . PreciseDue to the fact that our patient was hospitalized in the surgery department with the suspicion of an acute abdominal pathology, even if this was later excluded through the use of an MRI examination that established diagnostic certainty, it is necessary to follow several elements of differential diagnosis, which includes acute surgical diseases encountered in the lower abdomen, such as appendicitis, Meckel diverticulum, bowel obstruction, or ovary torsion, as well as the differential diagnosis of pelvic congestion syndrome. The list of differential diagnoses for pelvic congestion syndrome is vast, including diseases of the urinary tract, gastrointestinal tract, musculoskeletal disorders, disorders of neurological origin, gynecological problems, and mental health disorders. Painful bladder syndrome, pelvic inflammatory disease, interstitial cystitis, endometriosis, pelvic neuralgia, irritable bowel syndrome, myofascial pain, and pelvic floor myalgia are the common causes of chronic pelvic pain. In some cases, formulating an accurate diagnosis of the underlying causes of chronic pelvic pain is difficult, even with the use of laparoscopic and diagnostic radiological tests ,13,14. DGenerally, this condition is mainly caused by pregnancy, and in rare cases, ovarian and internal iliac venous insufficiency occurs due to valvular incompetence or the compression of the left renal or left common iliac vein. The hormonal impregnation of estrogens and progesterone causes significant dilation, a loss of elasticity in the venous wall, and the separation of the valve walls, which are responsible for venous reflux and a failure to return to normal in the months following childbirth . The etiSeveral options have proven to be effective in pain resolution, including medical treatment, surgery, and percutaneous embolization, which have become the standard treatment for pelvic venous insufficiency and results in symptomatic improvements in 80–94% of patients ,18,19,20With the development of medical technologies, many treatment methods for pelvic venous insufficiency have been developed. Currently, the treatment of pelvic engorgement syndrome and pelvic varicose veins with coil embolization, plugs, or transcatheter sclerotherapy, used alone or together, are recommended by the Society for Vascular Surgery (SVS)/American Venous Forum (AVF) guidelines (GRAD 2B) ,22. The On the other hand, other therapeutic alternatives are currently available for patients suffering from severe dilatation and reflux in the gonadal veins, such as the use of progesterone ,27. It cPVI, although a clinical entity, is not properly evaluated and, in most cases, is underdiagnosed in women. In contrast, in men, PVI is represented by varicocele, which is well-documented and diagnosed. PVI in women is not fully understood, but it is thought to be related to hormonal changes that occur during pregnancy, which can lead to increased blood flow and pressure in the pelvic veins . The risIn this study, we observed that unilateral left ovarian vein embolization in the patient required low pain management and enabled effective post-interventional recovery. We established that the patient’s multiple pregnancies had no effect on pain management, but there is evidence that parity and increased pelvic blood flow during pregnancy are involved in the etiology of pelvic pain during and after pregnancy. At present, no study has demonstrated the effect of parity on endovascular embolization treatment success ,21. In sFemale gonadal venous insufficiency is a rare pathology, often manifesting with pain in the lower abdomen, a clinical appearance that, in rare cases, can suggest an acute abdomen. Since the objective clinical signs are poor, misdiagnosis can often be encountered. A thorough anamnesis correlated with the clinical examination and imaging investigations are essential for a positive diagnosis. Once the diagnosis is established, the minimally invasive therapeutic approach is preferred, with interventional radiology procedures being the method of choice. An overall evaluation of the venous system to detect venous insufficiency with other topography and a periodic follow-up are essential for the favorable evolution of the case.


Ultrasound-guided ethanol ablation (EA) is a less invasive alternative to surgical resection for the management of thyroglossal duct cysts (TGDCs). However, to date, EA is rarely used in the United States to treat TGDCs. We present a case of TGDC successfully treated with EA in the United States. A 66-year-old man presented with a mobile anterior neck mass. Neck ultrasonography revealed a complex cystic mass in the midline directly anterior to the trachea, measuring 52 × 41 × 50 mm. Fine needle aspiration revealed no malignant cells, and pathology was consistent with TGDC. The patient had no contraindications to surgical resection. The patient's pretreatment symptom score was 7 and cosmetic score was 3. One month after EA, volume reduction ratio was 40%, symptom score was 1, and cosmetic score was 3. Four months after EA, the TGDC was resolved without need for an additional procedure. The volume reduction ratio was 96.8%, and symptom score and cosmetic score were both 1. In summary, EA is a viable alternative to surgical resection, even in patients who are surgical candidates. EA is attractive due to its simplicity, cost effectiveness, and tolerable side effect profile. Further studies are needed to evaluate long-term safety and efficacy, particularly in United States patients. Thyroglossal duct cysts (TGDCs) are the most common congenital cyst of the neck, consisting of epithelial remnants of the thyroglossal duct. TGDCs are benign and present clinically as a mobile midline anterior neck mass. Treatment is advised to exclude malignancy and prevent recurrent infection. Standard of care for management of TGDCs is the Sistrunk procedure, which entails surgical resection of the cyst along with the midportion of the hyoid bone and a core of tissue from the hyoid bone to the foramen cecum.Another, less invasive treatment option for TGDCs is ultrasound-guided ethanol ablation (EA). EA is currently rarely used for TGDC in the United States; however, it has been used successfully in Europe and Asia for the last decade, with increasing frequency , 10. In A 66-year-old man with a history of hypertension, hyperlipidemia, and psoriatic arthritis presented for evaluation with an enlarging neck mass following an upper respiratory infection. He denied any dysphagia, dyspnea, dysphonia, or any history of radiation. However, due to the large size of the mass, he noted increased neck pressure, and was significantly bothered by the cosmesis of the mass. He had no past thyroid history but a positive family history of Hashimoto thyroiditis in his son (33 years old). On examination (conducted via video visit) the patient had a visible midline mass inferior to the mandible that was mobile with deglutition. His thyroid stimulating hormone level was 2.68 µIU/mL (mIU/L) and serum calcium was 9.6 mg/dL (2.4 mmol/L).Neck ultrasonography performed in clinic by the patient's endocrinologist revealed a complex cystic mass in the midline directly anterior to the trachea and above the thyroid cartilage, measuring 52 × 41 × 50 mm . The patThe patient had no contraindications to surgical resection but had strong reservations about the Sistrunk procedure, which typically removes a piece of the hyoid bone, and researched nonsurgical interventions. Given the cystic nature of the TGDC, he wanted to pursue EA first and would be amenable to surgical resection if EA failed. He was given the option to undergo Sistrunk procedure vs ultrasound-guided EA, and he elected to undergo EA. His pretreatment cosmetic score was 3  His pretreatment symptom score was 7 .A 16-gauge needle was inserted into the cyst, and approximately 36 mL of dark, hemorrhagic fluid was aspirated. The cyst was thoroughly irrigated with cold sterile saline repeatedly until the fluid cleared and then irrigated continuously with 100% ethanol until the fluid became cloudy. Finally, 7 mL of 100% ethanol were infused. The patient tolerated the procedure without any voice change and with minimal pain.One month after undergoing EA, follow-up ultrasonography showed a mixed composition thyroglossal duct cyst measuring 40 × 28 × 37 mm with an anechoic lateral-superior aspect and isoechoic solid portion more medially . Volume Percutaneous ethanol instillation is a form of chemical ablation that has been well studied in the treatment of hepatocellular carcinoma for patients with cirrhosis, as well as more recently in the treatment of benign thyroid cysts. Diffusion of ethanol into cystic epithelial cells dehydrates the cytoplasm and denatures proteins, resulting in coagulative necrosis of the cyst wall. Subsequent diffusion of ethanol into local circulation causes vascular endothelial necrosis, platelet aggregation and thrombosis, and ischemic necrosis of surrounding tissues. Chemical ablation is an attractive option due to its simplicity, cost effectiveness, and tolerable side effect profile, which includes transient fever, pain, swelling, hoarseness, and dysphagia.Recent studies comprising fewer than 200 patients in Korea, Hong Kong, and Turkey have demonstrated mean VRRs ranging from 73% to 96.2% at final follow-up examination , 10. TheP = .055). No major complications occurred in the ethanol group (n = 82), and only one major complication of laryngeal edema occurred in the OK-432 group (n = 47) 2 days after the procedure and was successfully treated with steroids [A recent meta-analysis compared the efficacy of EA to OK-432 sclerotherapy in 129 patients with TGDC. The success rate, defined as VRR >50%, was found to be superior in the EA group compared to that of OK-432, although with equivocal statistical significance  but higher rates of treatment failure . In addition, EA was 3.4 times less expensive compared to surgery [In comparison to surgery, EA has better short-term morbidity but higher likelihood of treatment failure. A recent study from Korea including 345 patients  found that EA demonstrated fewer complications compared to Sistrunk operation (1.8% vs 10.0%, It should be noted that the vast majority of published cases of TGDC treated with EA occurred outside the United States. One report exists of 3 patients with TGDC treated with EA in Florida in 2006. In that report, 2 of the 3 patients experienced cyst recurrence . One othOur patient tolerated the procedure well with minimal pain or discomfort. At his 4-month follow-up evaluation, his VRR was 96.8%, with symptom and cosmetic scores of 1. Ultrasonography demonstrated almost complete resolution of the cyst, with only a small, solid, hypoechoic portion remaining. A VRR of 96.8% represents an excellent response to therapy, greater than the mean of all studies in EA may be a safe and viable alternative to surgery for the treatment of TGDC and may be considered as a first-line therapy in patients wishing to avoid surgery.EA has a greater likelihood of recurrence compared with surgery but has fewer complications.EA is simple, cost-effective, and has a tolerable side effect profile.M.D.S. and N.A.W. drafted the manuscript. J.H.K. performed diagnostic and surgical procedures, patient counseling, and editing of the manuscript. The authors read and approved the final manuscript.


Surgical intervention pursued progressive symptoms and new onset headaches. The patient underwent uncomplicated retrosigmoid craniectomy for gross total resection. Histopathological evaluation revealed a glioneural hamartoma. A MEDLINE database search used the terms' cerebellopontine angle' OR 'internal auditory canal' AND 'hamartoma' OR 'heterotopia'. Clinicopathological characteristics and outcomes of the present case were compared to those in the literature. The literature review yielded nine articles describing 11 cases  of intracanalicular glioneural hamartomas. Patients most commonly presented with hearing loss and were presumed to have a diagnosis of vestibular schwannoma before histologic diagnosis. Glioneural hamartomas are rare lesions that may be found in the IAC. Although benign, they may be safely resected for cranial nerve function preservation goals with a low risk of recurrence.Glioneural hamartomas are exceedingly rare lesions. When localized to the internal auditory canal (IAC), they can cause symptoms referrable to seventh and eighth cranial nerve compression. Here, the authors present a rare case of an IAC glioneural hamartoma. A 57-year-old male presented for evaluation of presumed  Glioneural hamartomas or heterotopia are exceedingly rare lesions that may arise in the internal auditory canal (IAC) and masquerade as a more common pathology . Most maClinical presentationA 57-year-old male presented to the otolaryngology and neurosurgery clinic after evaluation of dizziness and progressive right-sided hearing loss revealed an intracanalicular mass. The patient was found to have slight asymmetric sensorineural hearing loss on the audiogram Figure . His neuSurgical interventionThe patient underwent an elective right retrosigmoid craniectomy to resect his IAC mass. Intraoperative neuromonitoring with somatosensory evoked potentials was employed along with electromyography of CN VII. Auditory brainstem responses were monitored throughout the procedure. The CN VII/VIII complex was isolated before drilling the porus acusticus. Direct nerve stimulation demonstrated intact CN VII/VIII with evidence of the tumor located anterosuperiorly to the facial and cochlear nerve Figure . The tumHistologyHistopathological evaluation revealed a glioneural hamartoma (or heterotopia) Figure . HistoloPostoperative courseThe patient tolerated the procedure well despite mild postoperative nausea and persistent dizziness. He had normal facial function (House-Brackmann 1). He was discharged home on postoperative day four with recommendations for home therapy services. Postoperative imaging was consistent with gross total resection of mass without recurrence. Audiology evaluation demonstrated slight worsening in hearing loss on the right side, amenable to hearing aid placement. He was last seen in the clinic 2 years after surgery with normal facial nerve function and no evidence of tumor recurrence.A computerized search of the MEDLINE database (Pubmed) through January 2023 was conducted using the search terms' cerebellopontine angle' OR 'internal auditory canal' AND 'hamartoma OR 'heterotopia' Figure . ArticleOur literature review yielded nine articles from 1980 to 2018 describing 11 patients with glioneural hamartomas arising in the IAC. A summary of patient demographics and case characteristics is found in Table The most common preoperative diagnoses, based on imaging, were vestibular schwannomas n=6/8) ,6-9, and/8 ,6-9, The most common mass lesions in the IAC and cerebellopontine angle are neoplastic, with 80-90% being VS ,2. The nAlthough rare, they may present with symptoms similar to their more common counterparts of auditory and vestibular dysfunction. This was how the majority of patients in the review of the literature, as well as in the present case, had presented. Unlike the neoplasms in this location, hamartomas are generally isodense with brain parenchyma. Interestingly, in our review of the literature, a slight majority of cases n = 5/8) reported enhancement of the lesion ,7,8, and reportedManagement of these lesions should be tailored to each patient. Asymptomatic or incidentally identified small lesions may be monitored, given the benign nature of glioneural hamartomas. Conversely, atypical or symptomatic lesions may warrant surgical resection to diagnose and preserve CN function. The surgical approach should be up to the discretion and comfort level of the surgeons as well as dependent on the patient's anatomy. In all cases with reported post-surgical outcomes, including ours, facial nerve and auditory nerve function were preserved ,6-8, eveGlioneural hamartomas are rare non-neoplastic lesions found in the IAC, causing symptoms related to mass effect on the CN VII/VIII complex, but can be safely managed with preservation of CN function and low risk of recurrence. These cases are important to note as they are not common lesions but can cause significant CN deficits secondary to their location. Additionally, they may be easier to recognize once they are already symptomatic, given their slow-growing nature and the lack of consistent enhancement on imaging.


Subependymal giant cell astrocytoma (SEGA) is the most common intracranial tumor in tuberous sclerosis (TS) patients. The tumor generally localizes in the proximity of Monro's foramen; as it grows, it subsequently causes hydrocephalus and increases intracranial pressure (ICP). However, acute symptoms of increased ICP due to intratumoral bleeding rarely manifest in SEGA patients.We present a 27-year-old male with TS who presented due to hemorrhagic complications of SEGA with intratumoral bleeding and vitreous orbital hemorrhage. We then conducted a systematic review with four databases  to identify similar cases using the following keywords: "Subependymal giant cell astrocytoma," "Hemorrhage," "Haemorrhage," and "Bleeding."Our review identified 12 articles reporting 14 cases of hemorrhagic complications of SEGA in addition to our case report. The median age of diagnosis was 21 (range 5-79) years with unequal gender distribution . Headache was the most presented symptom, followed by hemiparesis, seizure, altered mental status, visual deterioration, and headache accompanied by seizure. TS was seen in most of the cases (80%). Gross total resection (GTR) was achieved in 53.5% of the patients. Regarding the clinical outcome, 66.7% had a good outcome, 20% died, and 13.3% had no report of their outcomes. No tumor recurrence was seen in the cases with a reported duration of follow-up.Catastrophic presentation of SEGA apoplexy is a rare occurrence. We present a case report with a systematic review and discuss SEGA apoplexy's possible pathophysiology and outcome.  Tuberous sclerosis (TS) is an inherited autosomal-dominant neurocutaneous disorder characterized by tumors involving many organs, including the brain, heart, kidneys, and skin . A cliniThe updated World Health Organization (WHO) classification of central nervous system tumors considers SEGA a grade 1 tumor, and it is generally localized in proximity to the foramen of Monro . SEGA isWe describe a 27-year-old male previously diagnosed with TS who presented with rapid consciousness and visual acuity deterioration due to intratumoral hemorrhage in SEGA with simultaneous intraocular hemorrhage. Moreover, we conducted a systematic review of the literature regarding intratumoral hemorrhagic complications (apoplexy) of SEGA and summarized their management and outcome.A 27-year-old male patient presented to our emergency department with a history of progressive visual deterioration over two days associated with a mild headache. There is no history of seizures, and the patient has a positive family history of TS. He had butterfly distribution of facial freckling and small hypopigmented macules on examination. His Glasgow coma score (GCS) was 15/15, and there were no motor-focal neurological deficits in the extremities. Ophthalmologic examination revealed bilateral papilledema with vitreous and retinal hemorrhage suggesting Terson syndrome. Visual acuity was poor, with light perception in the right eye and 20/300 in the left eye.An emergency unenhanced computed tomography (CT) scan of the brain was performed. It showed a well-defined mass involving the subependymal surface and projecting into the frontal horn of the left lateral ventricle. It is heterogeneous but mainly hyperdense, with fluid density areas and blood-fluid levels indicating hemorrhages Figure . The masBrain magnetic resonance imaging (MRI) showed left intraventricular mass with solid and cystic components measuring 27 X 25 mm at maximum diameter. The solid component showed intermediate T1 and T2 signal intensity with different ages of subacute hemorrhage, and there is a significant blooming on the susceptibility-weighted imaging Figure -2C. The The patient underwent left frontal craniotomy, and complete resection of a hemorrhagic lateral ventricular tumor was achieved through a transcallosal approach. He had an uneventful postoperative recovery and was discharged home after a week with stable vision. His early postoperative MRI scan revealed complete resection of an intraventricular tumor and resolution of peritumoral ischemic changes Figure .Pathologic examination of the resected lesion revealed a well-circumscribed neoplasm composed of large, plump, gemistocytes-like cells admixed with smaller, elongated, and spindle cells, arranged into sweeping fascicles with variable fibrillary background Figure . A few gThe patient had a satisfactory neurological recovery and was discharged home in good condition. He maintained good neurological, cognitive, and motor function during follow-up; however, he had a slight visual acuity improvement. The follow-up MRI scan at one year showed no tumor recurrence.Systematic review and analysisStudy ObjectivesThe study's primary objective was to review all reported cases of hemorrhagic SEGA and compare the clinical outcome between patients harboring hemorrhagic SEGA with TS and those pertaining to hemorrhagic SEGA without TS.Search MethodsWe performed a systematic review searching PubMed, Web of Science, Google Scholar, and Cochrane databases using a combination of the following keywords with Boolean operators (OR/AND): “Subependymal giant cell astrocytoma,” “Hemorrhage,” “Bleeding.” It was conducted based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines . AdditioInclusion and Exclusion CriteriaIntratumoral hemorrhagic SEGA was first reported by Waga et al. in 1977 ; howeverSelection of StudiesThe eligibility of each article found through the database search was assessed and determined by two independent reviewers (AT and MF) by screening the title/abstract and then reviewing the full-text versions of the articles. All disagreements were resolved either by a consensus or by arbitration of a third reviewer (SB).Data CollectionExtracted data from each eligible article included: the number of hemorrhagic SEGA patients, demographic data, clinical presentation, the presence of TS, type of surgery, tumor recurrence, clinical outcome, and the duration of follow-up.Quality AssessmentThe quality and risk of bias assessment of included studies were appraised by two independent reviewers (AT and MF). In addition, the quality of case reports was appraised using the Joanna Briggs Institute Critical Appraisal Checklist for Case Reports, which consists of eight yes/no/unclear questions Table .The quality of case series was appraised by Joanna Briggs Institute Critical Appraisal Checklist for Case Series, which consists of 10 yes/no/unclear questions . A high Systematic Review ResultsA total of 827 studies were identified from the selected databases, and 364 were removed as duplicates. Among the remaining 463 studies, 431 were excluded after title/abstract screening, and 21 were excluded after reviewing the full text. Twelve articles that fulfilled the inclusion criteria were included in the study Figure . Demographic DataA total of 15 cases with hemorrhagic SEGA have been identified in the literature, including our patient, summarized in Table Clinical PresentationsMost of the cases presented with headache , followed by hemiparesis , seizure , altered mental status , visual deterioration , and headache accompanied by seizure .Imaging and Pathological CharacteristicsNon-contrast CT scan of the reported cases demonstrated hemorrhagic hyperdense lesions constantly arising adjacent to the foramen of Monro, mainly in the lateral ventricle, causing obstructive hydrocephalus. The reported measures of the mass ranged from 1.5 cm to 5.6 cm. MRI scans performed in nine cases typically show a well-defined cystic, solid mass with mixed intensities in T1 and T2 weighted images with heterogeneous post-contrast enhancement. Moreover, multiple subependymal nodules and subcortical calcification were observed in four cases associated with TS.Histopathologically, all cases showed the typical features of SEGA, namely, neoplastic astrocytes in glial fibrillary background, large cell gemistocytes-like with rounded nuclei intermingled with spindle cells, and multinucleated giant cells, prominent vascularity as well as areas of hemorrhages, some inflammatory cells such as mast cells, positive immunohistochemistry for GFAP, low Ki-67 labeling index and negative IDH-1. In addition, genetic analysis was performed recently in two cases both showed TSC1 germline mutation ,9.Operative Characteristics and OutcomeGross total tumor resection was achieved in eight 53.5%) cases, subtotal resection in four (26.7%) and three (20%) cases; the extent of the tumor resection was not explicitly mentioned. The clinical outcome was good after surgical treatment in 10 cases, while death occurred in three cases, all of which had TS. One patient was due to an intramural hemorrhage, and the other two were due to treatment complications. In two cases, the outcome was not reported. Of those with a good outcome, tumor recurrence after resection was not seen during the follow-period in seven patients, while for the rest, their duration of follow-up was not reported  inhibitors. Even though the sporadic cases limited the role of radiosurgery in SEGAs, these reported results suggest radiosurgery may be an additional option for SEGAs where complete resection has not been safely achieved ,35. In aSEGA apoplexy is a rare and catastrophic presentation that may occur in any age group. Our systematic review and case illustration recommend urgent surgical excision of the tumor and associated hematoma as the best treatment with a good outcome. The pathophysiological mechanism of intratumoral bleeding of SEGA is still unknown. Future genetic analysis is required to understand the behaviors of SEGA and the possible predisposing factors for hemorrhagic complications.