Pancreatic follicular dendritic cell sarcoma (FDCS) is an exceptionally rare and low-to-moderate malignancy, with only seven reported cases to date. Clinical diagnosis of FDCS is challenging due to the lack of distinct biological and radiographic features.A 67-year-old woman presented to the hospital with a 4-day history of severe abdominal pain. Imaging studies (CT and MRI) revealed a large cystic mass located at the tail of the pancreas, which was suspected to be myeloid sarcoma (MS) based on EUS and CT-guided pancreatic puncture. Postoperative pathology and immunohistochemistry confirmed the diagnosis of pancreatic FDCS. After the diagnosis was confirmed, the patient received postoperative chemotherapy with the CHOP regimen. At 11 months of follow-up, there was no evidence of recurrence. Seven published cases have been reviewed to comprehensively summarize the clinical characteristics, diagnosis, and treatment options of FDCS.While imaging can be useful in detecting pancreatic FDCS, it should be interpreted with caution as it can be challenging to differentiate from other pancreatic tumors. Pathology and immunohistochemistry are considered the gold standard for diagnosis, with CD21, CD23, and CD35 being specific tumor cell markers. However, preoperative diagnosis of pancreatic FDCS remains difficult, and the pancreatic puncture may further increase the risk of misdiagnosis. The disease is highly prone to recurrence and metastasis, and surgery is the preferred method for both diagnosis and treatment of localized disease. Follicular dendritic cell sarcoma (FDCS) was first discovered and named by Monda et al. in 1986 . While tA 67-year-old woman presented to our hospital with a 4-day history of abdominal pain. On physical examination, marked tenderness was noted in the left upper quadrant without rebound tenderness or muscle tension, and there was no palpable superficial lymphadenopathy. Laboratory tests revealed a hemoglobin level of 90 g/L, while the liver and kidney function, amylase, alpha-fetoprotein, CA199, CA125, and CEA were within normal ranges. Computed tomography (CT) and magnetic resonance imaging (MRI) showed solid mass lesions in the caudal pancreatic region and cystic on the large curvature of the stomach, with poorly demarcated margins from surrounding tissues and liquefied necrosis in the center of the lesion. Based on these findings, the preliminary diagnosis was gastrointestinal stromal tumors (GIST) or pancreatic neuroendocrine tumors (pNENs). To confirm the nature of the mass, an EUS-guided pancreatic puncture was performed, but the obtained material was insufficient for a definitive diagnosis Fig. d. ImmunoFDCS in the abdomen is typically identified on imaging as a heterogeneous soft-tissue mass with complex internal structures, local necrosis, and lymphadenopathy. Tissue calcification may also be present, and contrast-enhanced scanning often reveals a “rapid wash-in and slow wash-out” or “progressive enhancement” –13. HoweHistological findings and specific immunohistochemical staining are the gold standards for diagnosing FDCS. Tumor cells with spindle-shaped or oval morphology arranged in swirls and bundles, and binuclear or multinuclear morphology, are typical pathological features of FDCS . In termThe lack of prospective studies related to treatment has resulted in the absence of a definitive uniform standard for FDCS treatment. The CHOP regimen is currently the most widely used for patients who cannot undergo surgery or have widespread disease, but for most patients, radical surgery remains the preferred option. Surgical excision is both helpful in disease diagnosis and the most direct means of symptom relief. Fonseca et al.  conducteThis article comprehensively explores the imaging features, treatment, and prognosis of pancreatic FDCS, with a focus on analyzing the causes of misdiagnosis during puncture, providing a new understanding of the disease. However, there are also shortcomings, due to the rarity of this tumor; long-term follow-up and systematic observation have not been conducted, so further exploration is needed for standardized treatment of this disease.Diagnosing pancreatic FDCS can be challenging due to the lack of specificity in laboratory and imaging tests. The external puncture may lead to misdiagnosis; thus, surgery is the preferred method for both diagnosis and treatment, although the development of standardized treatment protocols requires further investigation. Moreover, predicting the behavior of FDCS tumors can be difficult, and the abdomen is a common location for FDCS, which is a poor prognostic factor. Especially when it occurs in rare retroperitoneal organs such as the pancreas, it is often misdiagnosed due to unrecognizability, so high vigilance should be exercised.


Internal hernias are relatively rare and difficult to diagnose. Diagnostic delays lead to the progression of strangulation. In particular, pararectal fossa hernias are extremely rare. We encountered a case in which internal hernia occurred in the pararectal fossa.An 87-year-old woman was referred to our hospital because of persistent lower abdominal pain and vomiting. Contrast-enhanced computed tomography revealed findings of intestinal ischemia, such as closed loop formation with reduced contrast effect on the left side of the rectum in the pelvis. Strangulation small bowel obstruction was diagnosed, and emergency laparotomy was performed. The small intestine was found to invade the peritoneal reflection on the left side of the rectum. The patient was finally diagnosed with pararectal fossa hernia. The incarcerated small intestine was released with no bowel resection. The 4-cm hernia phylum was observed and closed by simple suture. The patient had a good postoperative course without recurrence.We encountered a very rare case of internal hernia in the left pararectal fossa. Preoperative diagnosis of this disease is difficult, but it should nevertheless be considered in cases in which the cause of the intestinal obstruction is unknown. Internal hernia is a rare condition that causes intestinal obstruction. It is defined as the herniation of an organ into an unusually large fossa, fovea, or foramen in the abdominal cavity . InternaAn 87-year-old woman presented with the chief complaint of lower abdominal pain and nausea. She was referred to our hospital with persistent lower abdominal pain and vomiting. On physical examination, mild tenderness was present in the lower abdomen. Blood test revealed elevated white blood cell count 12,200 /μl) and C-reactive protein levels (2.2 g/dl), with no other pertinent findings. The patient had diabetes and high blood pressure and had previously undergone total hysterectomy for uterine fibroids. Contrast-enhanced computed tomography showed beak-like changes in the small intestine, closed loop formation with reduced contrast effect, and localized ascites on the left side of the rectum in the pelvis (Fig. 2,200 /μlInternal hernia is defined as the herniation of an organ into an unusually large fossa, fovea, or foramen in the abdominal cavity . InternaA search on the PubMed and MEDLINE databases for literature published regarding “pararectal fossa hernia” or “perirectal fossa hernia” yielded a total of three studies , 7, 8 T. All preA differential disease of pararectal fossa hernia includes pararectal hernia, a type of perineal hernia. Perineal hernia is defined as a pathological condition in which intra-abdominal organs protrude beyond the pelvic floor into the subcutaneous area of the perineum. A defect is found in the pelvic floor, which is composed of pelvic floor muscles, and it is considered to be a condition in which a hernia orifice is formed . InternaIn this case, we performed laparotomy to release the strangulation and closed the hernia phylum with suture. In recent years, there have been cases in which laparoscopic surgery was performed for strangulated small bowel obstruction , and botClosure of the hernia phylum involves the use of ligation sutures and a repair method using a mesh , 8. If tWe present a case of pararectal fossa hernia, which is a very rare disease among the different types of internal hernias. Preoperative diagnosis is difficult, but a differential diagnosis of pelvic internal hernia should nevertheless be considered. In such cases, early surgery is desirable.


Ewing’s sarcoma (ES) is a malignant tumor that arises mainly from bone tissue. Primary extraosseous Ewing sarcoma (EES) is a rare form of the Ewing’s sarcoma family of tumor, and pelvic localization is even more unusual, considered to be one of the rarest localizations . We presTeaching point: Extraosseous Ewing’s sarcoma is a rare pediatric tumoral entity that requires clinician and radiological vigilance and detection. Ewing’s sarcoma (ES) is a highly malignant tumor, considered the second most common primary bone malignancy in the pediatric population . It can EES represents 10–20% of cases of the ES family , and pelA seven-year-old boy presented to the emergency department with worsening pain in the lower abdomen for one week and frequent voiding with small amounts of urine. On abdominal examination, a hard mass measuring approximately 7 cm in the right iliac fossa was palpated.Ultrasound (US) revealed a heterogeneous mass of low echogenicity  as well Contrast-enhanced computed tomography (CECT) scan confirmed the presence of this large mass with heterogeneous enhancement in the portal phase measuring 10 cm in maximum diameter and occupying almost the entire pelvis and extending up to L4–L5 . CECT coMRI showed a large, well-defined, lobulated and heterogeneous pelvic mass hypointense on T1 with persisting focal hyperintensies when fat is saturated, suggesting hemorrhagic areas . On T2-wThis lesion had a slight contact with the rectum and the lumbar spine, but without any signs of invasion. The bladder was compressed, which explains the urinary symptoms . MRI alsThe patient underwent an incisional biopsy and the pathological findings microscopically revealed the presence of a small round blue cell tumor and the immunohistochemistry was compatible with ES.There was no ESWRI (ES) or FKHR (RMS) rearrangement.The patient was referred to the surgical oncology department for chemotherapy and surgery.Pelvic ESS is considered to be one of the rarest locations of EES in children. Symptoms depend on the local mass effect of the tumor, such as oliguria following compression of the bladder or constipation following compression of the digestive tract.Pelvic EES has no specific radiological characteristics; the final diagnosis of pelvic EES is confirmed by characteristic features on histologic analysis, histochemistry, and Immunohistochemistry 5.Treatment of EES is multimodal with chemotherapy, surgery, and radiotherapy.Pelvic EES has a poorer prognosis when compared to other sites, with 30%–40% of metastases at the time of diagnosis .Pelvic EES is a rapidly growing tumor with no specific radiological features and a poor prognosis compared with ES of the bone. It is therefore important for clinicians to recognize this entity, as early diagnosis and treatment help to improve prognosis.


Urethral hemangioma is an extremely rare occurrence and is not typically considered a common cause of hematuria. Since 2000, only 22 male cases have been reported.A 45‐year‐old man presented with recurrent painless gross hematuria and the passage of blood clots after ejaculation. The patient underwent a transurethral resection of a 6‐mm hemangioma. This isolated sessile lesion was situated between the distal end of the verumontanum and the external sphincter, following an induced erection. The patient remained asymptomatic during the 1‐month follow‐up visit.This study included the assessment of patient symptoms, diagnoses, and treatments and the literature review of 22 patients. We propose that relaxation of the external urethral sphincter muscle under general anesthesia and artificially inducing an erection can aid in the identification of urethral hemangiomas near the verumontanum during cystourethroscopy. Intermittent hematuria is the most common symptom of urethral hemangioma, usually devoid of pain, but can occasionally increase in intensity. Hematospermia may also manifest as a symptom on rare occasions. During cystourethroscopy, artificially inducing an erection through the relaxation of the external urethral sphincter muscle under general anesthesia is useful for identifying hemangiomas near the verumontanum.Urethral hemangioma is an extremely rare benign vascular tumor and is typically not the cause of hematuria. The most common symptoms associated with urethral hemangioma include indolence in the majority of cases, while intermittent hematuria, albeit infrequent, can be significant in quantity. Regarding the treatment of urethral hemangioma, approaches such as thermo‐ or laser coagulations have been proposed for smaller lesions, while surgical interventions are recommended for larger lesions.In this study, we demonstrate our experience with an endoscopic resection surgery for urethral hemangioma, particularly in cases involving urinary retention due to post‐penile erectile hematuria.A 45‐year‐old man with an unremarkable medical history and no prior surgeries has been consistently experiencing painless gross hematuria and discharge of blood clots following ejaculation since June 2020. He did not complain of hematospermia. Three months following the symptom onset, he received a medical examination at a urology clinic due to the presence of gross hematuria and urinary retention. Magnetic resonance imaging and cystourethroscopy could not confirm the cause of hematuria. Subsequently, for approximately 9 months, he remained unaware of hematuria cases.In June 2021, the patient was referred to our clinic due to a recurrence of gross hematuria and urinary retention after ejaculation. His physical vital signs were within normal ranges, and both his abdominal and external genital areas were normal. The diameter of the urethral opening was also within the normal range. Laboratory analyses of midstream urine, as well as biochemical, hematological, and coagulation parameters, yielded normal results. Abdominal and pelvic ultrasound and computed tomography scans showed no abnormalities. We performed a procedure wherein we artificially induced an erection by injecting alprostadil alfadex into the corpus cavernosum after securing the base of the penis. Subsequently, cystourethroscopy was performed. The examination revealed the presence of a 6‐mm isolated sessile lesion with varicose veins between the distal end of the verumontanum and the 6 o'clock position of the external sphincter Fig. . No lesiIn January 2023, the patient was re‐examined due to the recurrence of gross hematuria after erections. Cystourethroscopy conducted after inducing an artificial erection yielded results consistent with the previous observation. However, a transrectal power Doppler sonography revealed a strong blood flow near the apex of the prostate gland. In March 2023, we excised the lesion while the patient was under general anesthesia. Artificially inducing an erection enabled us to clearly identify an enlarged vascular lesion with varicose veins between the verumontanum and external sphincter Fig. . Transuret al. have indicated that performing cystourethroscopy in a non‐erectile state may fail to detect urethral hemangioma lesions, which tend to enlarge and become more evident during the erectile state.Urethral hemangioma is an extremely rare benign vascular tumor and is not a common cause of hematuria. Since 2000, only 23 male cases, inclusive of our patients, have been documented Table .1, 2et al. have proposed that conducting cystoscopy immediately after ejaculation with weakened perfusion enhances the likelihood of detecting hemangioma lesions.Saito et al., who reported the detection of bleeding around the hemangioma via this method.Asymptomatic lesions typically do not require treatment, but a wide range of lesions often require incision, resection, and urethral reconstruction.et al. reported that patients with urethral hemangioma in local hospital settings have received misdiagnoses such as seminal cystitis, urethritis, or prostatitis.Fang Kimiyasu Ishikawa: Project administration; writing – original draft; writing – review and editing. Haruaki Sasaki: Data curation; funding acquisition; methodology; project administration; resources; validation. Hideaki Shimoyama: Investigation; validation. Motoki Yamagishi: Investigation; resources; validation. Hiroo Sugishita: Investigation. Yu Hashimoto: Investigation. Yuki Ichimura: Investigation. Ayana Doguchi: Investigation. Kazuhiko Oshinomi: Investigation; validation. Jun Morita: Investigation; validation.The authors declare no conflict of interest.Not applicable.Written informed consent to participate in this study and for the publication of this report was obtained from the patient for ethics approval.Not applicable.


Spinal intradural abscesses are extremely rare. To our knowledge, only a few cases have been described in the literature. We report 2 cases of spinal intradural abscesses in patients presenting to our institution with different symptomatology. Both cases involved the lumbar spine, with different etiologies: Case 1 was presumptively related to spondylitis phenomena, with surgery confirming the intradural localization of the abscess; case 2 was of probable iatrogenic nature (secondary to lumbar drain positioning). The aim of this report is to briefly discuss the clinical significance and pathogenesis of these cases. Spinal intradural abscesses are an extremely rare condition. To our knowledge, only a few cases have been described in the literature with long-term sequelae  and poor prognosis if untreated We would like to report 2 cases of patients presenting to our institution with different symptomatology and a common diagnosis of intradural spinal collections.A 71-year-old female patient presented to our attention with a 1-month history of progressively disabling and intense back pain with bilateral cruralgia, not responsive to medical therapy. Neurological examination revealed chronic lateral hypoesthesia of the right thigh. Blood tests showed elevated values of C-reactive protein, normal white blood cell count with mild relative neutrophilia, and relative lymphopenia.MRI of the spine demonstrated the presence of suspected intradural collections in the lumbosacral region at the L2-S2 level with low values of ADC, intense peripheral enhancement after gadolinium injection, and internal colliquative appearance. There was a pathological dural enhancement extended cranially up to T9-T10. In addition, there was a focal inflammatory/infective spondylitis involving the left facet joint at the level L2-L3 .Fig. 1MaDuring surgery, a durotomy was therefore necessary, confirming the radiological suspicion of the intradural location of the collections.Postoperatively, the patient received empirical intravenous antibiotic treatment with vancomycin, ceftriaxone, and metronidazole, awaiting cultures. Follow-up MRI performed 2 days after the surgery showed markedly decreased abscess size.The biological examination of the purulent sample did not show any micro-organism growth neither blood cultures were positive.The last follow-up MRI (2 months later) showed the disappearance of lumbar intradural collections, with the presence of arachnoiditis as a sequela.Pseudomonas aeruginosa.A 70-year-old male patient with a history of a kidney tumor and brain meningiomatosis was treated with surgeries and stereotactic radiation therapy. During recovery due to a cerebro-spinal leak from the surgical site, a lumbar drain was positioned. Sudden onset of fever and increased inflammatory markers  appeared and follow-up MR images of the spine demonstrated the presence of an intradural collection in the lumbo-sacral region (L3-L4 level), with marked restricted diffusion on DWI and mild postcontrastographic enhancement; in addition, there were signs of arachnoiditis affecting the roots of the cauda which were particularly glued together, in an antigravity position . The patHe has not undergone new surgical procedures in both the cranial and spinal areas due to his poor clinical conditions.Intradural spinal abscesses are extremely rare in comparison to epidural ones In both our cases, MRI images led us to suspect that the localization of the infectious collections was inside the intradural space. Indeed, the dural profile appeared as a continuous ipo-intense line (0.5-1 mm) delimiting the epidural fat, on the external side, from the abscess collections, on the internal side. A pial involvement was present due to the residual adhesiveness of caudal roots with antigravity position appreciable at MRI images in both cases.Staphylococcus aureus is the most predominant pathogen isolated In case 1, the intradural localization of the abscesses, confirmed by surgery, was probably related to spondylitis phenomena and a secondary dural breach at the left L2-L3 interfacetal joint. As described in the literature it is possible that transient bacteremia from a self-limited infection site may have been the underlying cause of interfacetal inflammation In case 2 due to the unstable clinical condition of the patient, who was bedridden, surgery was not recommended and antibiotic treatment alone was initiated. Follow-up MRI images showed a slight reduction in the size of the lumbar intradural collection.Intradural abscess is a rare and potentially dangerous condition. Despite its rarity, a high level of suspicion must alert the radiologist when dealing with a patient with intense back pain, neurological symptoms, and increased inflammatory markers. Generally, if promptly diagnosed, surgical drainage, combined with antibiotic treatment, results in a favorable outcome.I hereby confirm that I have obtained written informed consent from the patients for the publication of their cases.


A chronic expanding hematoma (CEH) is a rare clinicopathologic entity that may simulate the clinical and radiologic presentation of soft tissue sarcomas. Etiology has been attributed to repeated exudation and bleeding from capillaries in granulation tissue, resulting in a gradually enlarging mass. A 51-year-old male presented with a large thigh mass following a gunshot wound one year prior. Diagnostic imaging revealed a large complex mass with cystic areas overlying cortical erosions in the femoral diaphysis suggestive of osteomyelitis versus a primary aggressive new growth. Biopsy confirmed CEH and the absence of malignant cells. Hip disarticulation was performed after noting massive necrosis of the thigh compartments and neurovascular compromise. CEH is an important differential diagnosis to be considered in a patient with a slow-growing soft tissue mass and history of significant trauma. Its similar clinical presentation with a soft tissue sarcoma necessitates a high index of suspicion, diagnostic imaging, and biopsy prior to performing definitive surgery. Subsequent accounts of CEH are case reports and small case series, and the authors surmise its rarity may be due to underreporting of such cases. CEH are commonly found in the abdomen and pelvis, while those of the lower extremity are less common2 possibly owing to less potential space for expansion.Chronic expanding hematomas (CEH) were first described as a clinicopathologic entity in 1980 by Reid 2. Clinically, both present as a slow-growing mass2. On magnetic resonance imaging (MRI), CEH mimic neoplasms by presenting as a central mass of blood surrounded by a wall of dense fibrous tissue1. A biopsy is necessary to establish a diagnosis followed by appropriate surgical management2.This poses a diagnostic dilemma due to its similarities to soft tissue sarcomas (STS)Here, we present a rare case of a CEH occurring in the thigh of an adult male, initially considered to be a soft tissue sarcoma.A 51-year-old male sought consult due to a left thigh mass. The patient sustained a gunshot wound to the thigh one year prior, for which he was treated with debridement. Two months after surgery, patient remained non-ambulatory with progressive enlargement of the left thigh which prompted consult.Examination revealed a tense, warm, and tender mass measuring 73.5cm in circumference . Left hiUltrasound revealed a non-compressible mass with areas suggestive of necrosis. Computerised tomography (CT) scans  showed aA sono-guided core needle biopsy revealed necrotic tissues admixed with blood and fibrin. A subsequent open biopsy was consistent with fibroblastic proliferation, chronic osteomyelitis, focal foreign-body giant cell reaction, and no overt evidence of malignancy . PatientSpecimen cultures revealed growth of Escherichia coli, treated with culture-guided antibiotics. Duplex scans showed 50-99% stenosis of the external iliac arteries, as well as acute deep vein thrombosis of the external iliac and common femoral veins. An electrophysiological study of the affected extremity revealed severe axonal lumbosacral (L2-S1) plexopathy.Due to massive necrosis of the anterior and medial thigh compartment, destruction of the femur, chronic infection, and neurovascular involvement, ablative surgery was recommended. The patient consented and underwent hip disarticulation. Two years post-operatively, the patient is well and without further complaints.et al in 1980, five presented with a slowly expanding truncal masses, while only one patient was described as having a mass on the lower extremity1. Subsequent reports5 describing lower extremity CEH consisted of similar patients presenting with soft tissue masses that resembled an STS including synovial sarcoma, epithelioid sarcoma, extraskeletal Ewing sarcoma, and undifferentiated pleomorphic sarcoma. Similarly, this case presented with a gradual enlarging thigh mass which appeared as a soft tissue sarcoma, prompting comprehensive diagnostic examination.In a series of six patients by Reid 1. Histologically, CEH presents with a peripheral zone of collagenous tissue containing hemosiderin-laden macrophages, and multinucleated, foreign body-type, giant cells4. A midzone has also been observed, containing loose connective tissue fibrin and predominantly amorphous debris, often with capillary ingrowth4.Despite variable locations, gross appearance from all cases consisted of an outer wall of fibrous tissue and a central collection of blood1. Bleeding from injury causes hematoma formation1. The breakdown of blood within the hematoma activates vasoactive substances that induce capsule formation, while propagating inflammatory cascade resulting in increased vascular permeability and expansion of the hematoma5. Another theory is the presence of vascular endothelial growth factor (VEGF). Activation of VEGF results in angiogenesis, leading to repeated exudation of blood from newly formed capillaries5. Chronic increased pressure within a closed compartment was theorised to lead to compression of adjacent muscles, impeding blood flow and causing rhabdomyolysis followed by subsequent necrosis1.An inflammatory cascade is considered in the aetiology of CEH5. MRI remains the modality of choice and may show a “mosaic sign” which are multiple signal intensities within the lesion corresponding to blood products of different ages5. Musculoskeletal ultrasound will show a multilocular cystic appearance5.Evaluation of CEH with CT scans appear multicystic with varied attenuation within the lesion, with or without a partially calcified rim5.Biopsy with adequate specimens should be done to establish a diagnosis prior to any surgical management. Histologic sections of CEH consist of a mixture of necrotic debris, fibrin, blood clots, cholesterin crystals, and histiocytes, without evidence of malignant cells5. Incomplete excision has been reported to cause further bleeding from a hypervascular subcapsular lesion5. Aspiration is not enough to remove the contents of the hematoma and will not remove the fibrous capsule5. In this reported case however, the chronicity of disease lead to massive muscle necrosis of the thigh compartments and bony destruction of the femur compounded with osteomyelitis, hence ablative surgery was recommended. This reiterates individualised plan of management for all patients following completion of thorough and complete investigation.While treatment guidelines for CEH are not established, complete surgical excision including the pseudocapsule is the current recommendation of choiceIn the presence of a patient with a chronically enlarging extremity mass and a history of trauma, CEH should be considered as a differential diagnosis. With a high index of suspicion, proper imaging modalities can help detect these pseudotumours. Tissue biopsy is necessary to confirm histopathologic diagnosis prior to management.


Uterine leiomyoma is the most prevalent benign type of gynecological tumor. It affects more than 80% of women worldwide and, within this group, more than 50% may be asymptomatic. However, large fibroid volumes may be associated with symptoms of extrinsic compression, and most of the cases do not present atypical cells. We present the case of a 49-year-old woman who underwent a total abdominal hysterectomy of a 13.5-kg uterine leiomyoma with no malignancies at histopathology and review the literature about giant uterine leiomyomas and their clinical repercussion. We concluded that large volumes do not always pose a threat regarding malignancy; however, future molecular studies are needed to investigate giant uterine fibroids. Uterine leiomyoma is a benign gynecological tumor originated from smooth muscle cells that affects up to 80% of women worldwide. From these, only 20 to 30% resent symptoms.rd, 2020 in the PubMed database with 577 results. Manuscript language was not considered a barrier for inclusion in this review. References were also consulted for analysis, as well as similar studies that were suggested in the PubMed website. Of these studies, 19 case reports that described giant leiomyomas were selected for analysis, andLeiomyomas are classified as giant when their weight exceeds 11.4 kg.A 49-year-old nulliparous, obese woman with chronic hypertension sought medical care due to an increase in the size of her abdomen  for oneAs there were no previous radiological exams evaluating the uterine growth rate and we could not rule out leiomyosarcoma for this patient preoperatively  without sufficient material, a total abdominal hysterectomy with bilateral salpingo-oophorectomy was scheduled. One week before surgery, the patient underwent a magnetic resonance imaging (MRI), and the size of the tumor did not present any alterations. No hypothesis for leiomyosarcoma was performed. One day before the surgery, the patient was complaining of progressive abdominal pain, constipation, and urinary retention. Low-molecular weight heparin (enoxaparin) was administered prophylactically prior to surgery. A median incision was performed, and surgery  occurred with no abnormalities, with an estimated blood loss of 600 ml, measured by compress weighing. An enormous uterine mass, weighing 13.5 kg, was removed . UreterIntraoperative histological analysis of the mass revealed no atypia, and the specimen was sent to pathology. No complications occurred in the immediate postoperative period, and the patient was discharged two days after the surgery. Gross macroscopy did not find any abnormalities, except for a giant uterine fibroid, with abundant vasculature in the periphery of the lesion. No areas of degeneration were seen. Histopathological examination evidenced leiomyoma with no atypia or degeneration and unaltered adnexa. The patient was discharged with no complications.Giant leiomyomata are rare, and not frequently reported in the literature. They are of special interest when presenting with a fast growth rate, which suggests malignancy, as uterine cancers usually present with signs and symptoms similar to benign tumors of the uterus and there are no specific criteria for the suspicion of leiomyosarcoma.n = 2) published by Oelsner et al.A case series (Surgery can be challenging in these cases. It is quite difficult to consider a minimally invasive approach in a large abdomen, as most of the incisions were xiphopubic. Within the selected cases, only one case was performed myomectomy—it was a nulliparous woman.The present case report does not show an association between tumor size and malignancy. We need to understand the biological behavior of significantly enlarged leiomyomas with molecular approach. A study comparing large leiomyomas versus simple uterine leiomyomas has shown that there are large growth rates are present in fibroids from adolescent women.


Plasmablastic lymphoma is a rare type of non-Hodgkin lymphoma that generally presents an aggressive clinical course. It is strongly associated with human immunodeficency virus (HIV) infection, and the most common site of involvement is the oral cavity. Although extraoral PBL has been reported in several places, small intestine involvement is extremely rare.Here, we describe an exceptionally rare case of a 24-year-old immunocompetent Asian Male patient with newly diagnosed plasmablastic lymphoma of the duodenum. The patient was admitted to our oncology facility due to the patient's clinical course, which included persistent vomiting, hematemesis, weight loss, and generalized weakness. Computed tomography of the abdomen (triphasic) of the patient showed thickness at the 2nd part of the duodenum measuring 2.6 cm in width and 16 cm in length blocking the pancreatic and common bile ducts by entering the second section of the duodenum. The biopsy specimen's pathological investigation indicated abnormal cells with plasmacytoid characteristics and a high proliferation index. The diagnosis of PBL was confirmed by immunohistochemical profiling. Supportive therapies like blood transfusions, antacids, and antiemetics were started to manage the patient's symptoms. Palliative radiation was also anticipated for the lesion site.Duodenal involvement to the extent seen in our patient is exceptionally rare and, to the best of our knowledge, has hardly been described. The main goal of the article is to review the literature and report a case. Plasmablastic lymphoma (PBL) is a rare variant of diffuse large B-cell lymphoma (DLBCL) that is aggressive in nature and pathologically similar to plasma cell myeloma . PBL wasA 24-year-old Asian Male patient was admitted to the clinical oncology ward of Jinnah Postgraduate Medical Center—JPMC,  on 23rd August 2021. The patient had a history of intractable vomiting, hematemesis, generalized weakness, weight loss, epigastric pain and melena that had lasted more than a month.Upon physical examination, the patient seemed underweight and anaemic, a palpable mass in the epigastric region was noted with hepatosplenomegaly felt during abdominal palpation and profound jaundice.Notably, the patient's medical history did not reveal any recognized risk factors or underlying health issues that would have contributed to the plasmablastic lymphoma's duodenal presentation. There was no prior history of HIV infection or any other impaired conditions.The initial laboratory workup Table  revealedEsophagogastroduodenoscopy identified irregular circumferential ulcerated growth in the first part of the duodenum (D1) extending up to the junction of the first and second parts of the duodenum (D2). A large clean-based ulcer with an overlying clot was also noted, consistent with Class II-B of the Forrest Classification for peptic ulcers. Biopsy of the ulcerated mass was taken during esophagogastroduodenoscopy for histopathology.Computed tomography of the abdomen (triphasic) showed thickness at the 2nd part of the duodenum measuring 2.6 cm in width and 16 cm in length. Thickening infiltrating into the medial head of the pancreas resulting in obstruction and dilatation of the pancreatic and common bile duct was also significant. Ill-defined soft tissue density with necrosis at the pelvis and the upper lobe of the right kidney with enlarged para-aortic lymph nodes was noted, which was suggestive of aggressive primary neoplastic lesions.The pathological analysis was congruent with haematolymphoid neoplasm with plasmacytoid features. Microscopic description of the specimen revealed sheets of atypical cells of medium to large size that exhibit plasmacytoid features with a high proliferation index (Ki-67 raised) and brisk mitotic activity. Tumour cells were positive for LCA, CD79a, Pax-5, Mum-1, and CD138 and negative for CD20, Pan T (CD3) and CD30. The diagnosis of PBL was made by the physicians at JPMC, Karachi. Supportive treatment was started, including 3 pint packed red blood cell (PRBC) blood transfusions to correct anaemia, injection of tranexamic acid 500 mg TDS, omeprazole (40 mg IV OD) and octreotide continuous IV infusion of 25 mcg/hr to control GI bleeding and ondansetron (8 mg IV BD) for vomiting. In addition, palliative radiotherapy (RTP) at the site of the lesion was planned.The patient was lost to follow-up despite these efforts, which made it difficult to further assess the therapeutic response and long-term results.et al. as a rapidly proliferating B-cell lymphoma occurring in the oral cavity in the context of HIV infection [Plasmablastic lymphoma remains a diagnostic challenge, as the biological characteristics of the disease are enigmatic to date. The diagnosis of such cancers might be more difficult in the setting of extraoral locations and in immunocompetent patients. As a rare entity, PBL was originally described as a separate clinicopathologic entity by Delecluse nfection . Althougnfection . Furthernfection .Extraoral sites of PBL, including skin, subcutaneous tissue, stomach, anal mucosa or perianal area, lung and lymph nodes, have been reported in several cases; however, involvement of the small intestines is rare, with only a single case reported of duodenal involvement in the last ten years (January 2010–January 2020) , 6. The In 2014, a case series reported the manifestation of gastrointestinal plasmablastic lymphoma (GI-PBL) in four patients who presented to the Moffit Cancer Center for management. The age at presentation for all four cases was between 40 and 65 years with underlying immunosuppressive statuses such as HIV and EBV positivity . This isFor oncologists, the diagnosis of PBL in immunocompetent and extraoral site localization is very demanding because the clinical, morphological, phenotypic, and molecular features are ambiguous.In addition, due to the lack of a distinguishing phenotype, differential diagnosis with the activated B-cell-like (ABC-like) subgroup of DLBCL and plasma cell malignancy (PCM) with plasmablastic morphology is still considered a common dilemma. Although there are wide-ranging differential diagnoses including plasmablastic multiple myeloma, Burkitt lymphoma (BL), Hodgkin lymphoma, primary effusion lymphoma and extramedullary plasmablastic myeloma –12.In practice, PBL can be differentiated from BL and immunoblastic DLBCL based on immunohistochemistry (CD20 and LCA immunoreactivity present in BL and absent in PBL) , 14. PlaNarrowing down the working diagnosis might help in ruling out life-threatening or time-critical conditions, thus increasing the overall survival rate of GI PBL patients. Ruling out other differential diagnoses will also guide oncologists in better medical evaluations and treatment plans.et al. also favoured the CHOP regimen in GI-PBL with HIV-negative and CD 20-negative IHC patients, as complete remission was achieved after 6 cycles [The range of treatment provided to patients with PBL has been substantial and vast, from medical to radiation, depending upon the extent and severity of the disease . A liter6 cycles . Less da6 cycles . As remi6 cycles  and hema6 cycles , oncolog6 cycles . ControlIn conclusion, plasmablastic lymphoma is an extremely rare form of HIV-related lymphoma commonly involving the oral cavity; thus, duodenal involvement in an HIV-negative patient makes this possibly the first properly documented case. Plasmablastic lymphoma is an aggressive non-Hodgkin lymphoma; hence, its diagnosis is challenging, particularly when it arises in extraoral locations and in immunocompetent patients. A delayed diagnosis might negatively impact both treatment and survival.


Mature teratoma is a benign neoplasm, mostly composed of well-differentiated derivations of almost two or three germ cell layers, while immature teratoma is a malignant neoplasm composed of immature neural and embryonic tissue. Immature teratoma in the context of ovarian endometrioma has not been reported yet.A 34-year-old woman with primary infertility is reported in this study who suffered from immature teratoma associated with ovarian endometrioma. After several rounds of fertility treatment, the patient was referred for severe abdominal pain and underwent emergency surgery for the rupture of ovarian endometrioma. To preserve the ovary, the cyst was not resected in areas attached to the ovary. Some months later, the patient noticed a progressive abdominal enlargement. The sonographic evaluation revealed multiple solid-cystic lobulated masses on the abdominal wall and throughout the pelvic cavity. The histologic diagnosis was consistent with immature teratoma. The patient was treated with high-dose neoadjuvant chemotherapy and fertility-sparing surgery (FSS). The histologic evaluation of the extracted masses revealed teratoma maturation.This study reveals the importance of complete removal of endometrioma and highlights the role of neoadjuvant chemotherapy in fertility-sparing surgery and potentiating teratoma maturation. Endometriomas are benign and common ovarian cysts that are found in 15–42% of females suffering from endometriosis , LH (11.1 mIU/ml), HE4 (63.2 ng/ml), and AMH (5.13 ng ml), and elevated CA-125 (371.1 U/ml) and ROMA-index (14.6%). The patient underwent hysterosalpingography to detect the cause of infertility, which was normal with no blockage in the fallopian tube and uterus. The patient received several courses of letrozole that were not effective in the treatment of infertility.This case was presented in March 2022 in Firouzgar Hospital of Iran University of Medical Sciences. A 34-year-old female nurse, with a history of dyspareunia and dysmenorrhea and a diagnosis of primary infertility, was referred to a gynecologist for the treatment of her symptoms. The patient had normal FSH (6.3 mm) with volume of 400 ml in the midline position of the pelvic, raising the probability of ovarian torsion. The amount of free fluid around the cyst was about 85 ml. The patient underwent an emergent operation for the rupture of ovarian endometrioma which spread throughout the abdominal cavity. The surgery was done by a general gynecologist. In order to preserve the ovary for future ovulation and pregnancy, the surgeon did not resect the cyst in areas firmly attached to the ovary. After the left ovarian cystectomy, an abdominal drain was placed and the patient was discharged afterward. The histologic examination of the tissue specimen from the cyst wall was consistent with the diagnosis of endometrioma.Almost five months later, the patient was referred to the emergency department of a teaching hospital with severe abdominal pain. The sonographic evaluation revealed a very large cystic lesion (74*95*108 mm) with an echogenic center of 25 mm, without internal blood flow. Furthermore, moderate ovarian attachment to the uterus and bilateral uterosacral ligament thickening were noticed. The patient received high-dose of medication for ovulation induction  for 12 days. From the first days of medication, the patient felt the presence of a progressively growing palpable cyst in the exact site of the previous abdominal drain. However, the doctor assured her that they are endometriosis nodules in the abdominal wall and are nothing to be concerned about. She only performed follow-up vaginal sonographic evaluations which showed the adequate size and number of follicles in both ovaries. Therefore, the ovarian puncture was performed as planned. However, no abdominal sonographic evaluation was performed to investigate the abdominal wall nodules.Almost two months later, the patient was referred to an obstetrician and gynecologist for an infertility problem. Before ovulation induction, a sonographic evaluation was done, which showed an endometrioma in the left ovary . The patient underwent a biopsy. Histologic evaluation of the biopsy sample showed immature neuroglial tissue and neuroepithelial rosettes in the context of cellular stroma, consistent with the diagnosis of immature teratoma  regimen. The chemotherapy of the patient was associated with numerous complications including chemotherapy-induced neuropathies and several infections leading to hospitalization. Three months after chemotherapy, the patient was elected for surgical debulking of tumors of the abdominal wall and pelvic cavity by a gynecologist and a surgical oncologist. Due to the wide adhesion of the tumor to the abdominal wall, a substantial part of the abdominal wall was resected. The debulking of multiple huge abdominopelvic tumors of about 10*20 teratoma . Two dayAfter the operation, the patient had some difficulties doing her routine activities such as walking, which required several occupational therapy sessions. During the three months of follow-up, the patient required two further surgeries for the repair of the implanted meshes in the abdominal wall. No sign of recurrence was observed until the last follow-up.Malignant germ cell tumors are rare and mostly involve females in their reproductive years. Surgery is the first and most important option in the treatment of malignant germ cell tumors. Fertility-sparing surgery that allows preserving one ovary and the uterus has shown long-term safety for young women seeking future pregnancy and is also effective in preventing early menopause. However, recent evidence suggests avoiding FSS in cases with an increased risk of peritoneal adhesions .Immature ovarian teratoma is a rare germ cell tumor with challenging diagnosis and treatment . Its conWhile the role of extensive surgery is acknowledged for the treatment of ovarian germ cell tumors, there is no consensus on the timing of chemotherapy . AdjuvanAltogether, the present report reveals that complete removal of endometrioma overweighs preserving fertility because mismanagement of endometrioma could be associated with subsequent devastating outcomes. In addition, the present case highlights the role of neoadjuvant chemotherapy in the management of ovarian immature teratoma, by increasing the feasibility of fertility-sparing surgery and the possibility of teratoma maturation.


Echinococcosis, also known as hydatid disease, is a zoonotic parasitic disease prevalent in pastoral areas, mainly involving the liver and lungs, and less frequently the bones and surrounding soft tissues. Diagnosis and treatment of bone hydatid disease is a challenge, and because of the insidious course of the disease, the lesions are often widely disseminated by the time patients seek medical attention.A 29-year-old woman presented with a painless mass that was gradually increasing in size in the cervical thorax. Imaging revealed an enlarged clavicle with multiple bone cortical defects and the existence of cysts in the soft tissues surrounding the clavicle, for which complete excision of the clavicle and the attached cysts was performed. There was no recurrence of the cyst within one year after the operation, and the patient felt well and had normal shoulder joint movement.Bone hydatid may appear in bones throughout the body, and cysts that leak from the bone into the surrounding soft tissues may spread at a relatively rapid rate. Prompt surgical removal of the affected bone and surrounding cysts is necessary for treatment. Echinococcosis is a zoonosis caused by parasitic infections of the genus Echinococcus, which may affect tissues and organs in various parts of the body. The infection rate of bone tissue is low, accounting for only 0.5% ~ 4% of the total number of cases, but it is very destructive . Spine, The patient was a 29-year-old female, a herder by occupation. The place of residence was a traditional husbandry area. About 3 months before admission, the patient developed a mass on the left neck with no obvious incentive, with a diameter of 2 cm and no obvious tenderness. The mass gradually increased within 3 months, and by the time of admission, the mass had grown to a size of 6 × 5 cm. One month after the appearance of the mass in the neck, the patient found that a mass with similar characteristics also appeared in front of the left shoulder joint, and gradually increased to an area of 5 × 4 cm on admission Fig. a. The paEchinococcosis is widely distributed in all continents except Antarctica. Western China has a wide distribution of traditional grazing areas and is a high prevalence area for the disease . Accordi


In this case report we present a very rare case of intramuscular cavernous hemangioma in the temporalis muscle which was successfully managed with surgical excision with no evidence of recurrence in follow‐up. Intra‐operative image showing the lesion in the caudal aspect of the temporalis muscle. The most common types of vascular malformations are capillary telangiectasias, cavernous malformations, venous malformations, and arteriovenous malformations.2A 51‐year‐old female was admitted to the oral and maxillofacial surgery department of a tertiary university hospital in August 2019, complaining about the presence of a mass in her left temporal area  scan of the lesion showed a well‐defined soft tissue mass in the temporal region with no bony erosion or invasion Figure . SubsequAfter acquiring informed consent, the patient underwent full surgical excision of the lesion under general anesthesia. Al‐Kayat Bramley incisionGross pathologic examination showed one piece of lobular encapsulated grayish‐brown soft to elastic tissue measuring 3 × 2.5 × 1.5 cm with variable‐sized cystic spaces filled by fragile brown material. Histologic examination of the lesion revealed a vascular lesion consisting of numerous large endothelium‐lined vessels with intraluminal papillary projections, sinusoidal, and anastomosing vessels in a fibrotic stroma. Intravascular thrombosis, adipose tissue, and patchy inflammatory cell infiltration were also seen Figure . There wThe patient was followed up periodically for up to 2 years with no sign of recurrence and no limitation in mandibular movements.3There are very few reported cases of intramuscular hemangioma in the temporalis muscle with the first cases dating back to the 1950s.Unlike the common subcutaneous types of hemangioma, intramuscular hemangioma is not present at birth, is usually detected in the second or third decades of life, tends to grow slowly and progressively, and does not show spontaneous regression. Although the masseter muscle is the most common site of intramuscular hemangioma of the head region,Hemangiomas are divided into five common categories based on their predominant histologic vasculature: capillary , cavernous (large vessel), arteriovenous, venous, and mixed.In a literature review by Alqahtani et al.These lesions can easily be diagnosed by MRI with typical hypersignal images on T2 and hypo‐to‐iso signal images on T1 sequences.Although many consider surgery as the best treatment option for intramuscular hemangiomas of the temporalis muscle,Following the experience with the present case and a few others reported in the literature, it is recommended to consider intramuscular hemangioma in the differential diagnosis of isolated pathologic lesions of facial muscles when radiologic and clinical symptoms are in accord with a benign neoplasm of vascular origin. Surgical treatment is generally successful with very low rates of recurrence.4In this article, we reported a rare case of intramuscular cavernous malformation in the temporalis muscle; an unusual site with very few cases reported in the literature. The patient was successfully treated with complete surgical excision of the lesion with preservation of the facial nerve branches. There is no evidence of disease recurrence or treatment‐related adverse events with 2 years of followMohammad Jafarian: Conceptualization; data curation; project administration; supervision; validation; writing – original draft; writing – review and editing. Mohammad Amir Alizadeh Tabrizi: Conceptualization; data curation; project administration; resources; writing – original draft; writing – review and editing. Fatemeh Mashhadi Abbas: Data curation; resources; supervision; writing – original draft; writing – review and editing. Zahra Sadat Torabi: Conceptualization; data curation; investigation; project administration; resources; writing – original draft; writing – review and editing.None.None.Our study was performed in line with the principles of the Declaration of Helsinki. The subject signed informed consent and patient anonymity was preserved.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


The odontogenic keratocyst (OKC) is known for its high recurrence rate and disputed treatment modalities. In this report, we review the literature elucidating the efficacy of 5-fluorouracil (5-FU) topical application for recurrent OKC, and discuss the management of an OKC with 5-FU after enucleation and a 12-month follow-up.A 38-year-old female patient with an aggressive OKC in the right mandible underwent surgical curettage followed by topical application of 5-FU. Regular follow-up examinations for 12 months  showed no signs of recurrence, with complete resolution of the cystic lesion and gradual bone regeneration. New bone formation was identified in the radiographic follow-up. This case demonstrates the potential efficacy of topical 5-FU as a promising treatment modality for OKC, warranting further research and validation. A novel and successful therapy for OKC is the topical application of 5-FU. After enucleation, topical application of 5-FU efficiently treats OKC, leading to normal bone healing and regeneration without any adverse local or systemic effects. The odontogenic keratocyst (OKC) is a fascinating and clinically challenging entity due to its aggressive nature, high recurrence rate, and controversial treatment modalities. It was first described as a separate clinical entity by Phillipsen and Reichart in 1956 . The mosTo establish an accurate diagnosis, a comprehensive evaluation, including a clinical examination, radiographic analysis, and histopathological examination, is essential. The optimal treatment strategy for OKCs remains a subject of debate, with recurrence rates varying widely from less than 10% to over 60% .Recurrence theories for OKCs include incomplete removal of the cyst lining, growth from satellite cysts or residual odontogenic epithelial rests, or the development of unrelated OKCs in adjacent regions of the jaws . The recRecently, a novel approach using topical application of 5-fluorouracil (5-FU) has gained attention for OKC management. It is an antimetabolite that targets proliferating cells and is used to treat basal cell carcinoma (BCC) and other malignancies. It is a recognized treatment for actinic keratosis . Based oThe 5-FU stops the enzyme thymidylate synthetase (TS), which is necessary for the synthesis of DNA and results in cell death. Although the exact mechanism of action is unknown, 5-FU has been linked to a decrease in both the production of arachnoid acid metabolites and inhibition of apoptosis, as well as an increase in immune surveillance and anti-angiogenesis, thus impacting the cancer cells' capacity for invasion . It actsThe results of this study confirm our theory that satellite cysts and remnants of keratocyst epithelium, which are assumed to be the root of recurrence, can be efficiently eliminated by topical application of 5-FU to the wound cavity right after curettage and peripheral ostectomy. Topical 5-FU acts differently from MCS or liquid nitrogen, which both produce nonselective tissue necrosis. Three different enzymes, i.e., TS, thymidine phosphorylase (TP), and dihydropyrimidine dehydrogenase (DPD), have an impact on the pharmacologic response of 5-FU . DihydroThe availability and technical simplicity of the 5-FU application may make it preferable to other supplementary therapies like MCS or liquid nitrogen, in addition to offering focused antiproliferative therapy for OKC treatment. Simply coated onto ribbon gauze, the 5% 5-FU is placed into the bone cavity in such a way that it may be easily removed 24 hours after surgery. On the other hand, the administration of Carnoy's solution significantly lengthens the duration of the operation due to the measures needed to avoid damaging nearby tissues. The 5-FU-coated gauze should be used carefully to make contact with all surfaces of the wound cavity. The OKC might reoccur in areas that lack exposure to the 5-FU gauze. Also, 5-FU is not known to be neurotoxic, and the size and location of the OKC may be the reason for paresthesia rather than interaction with 5-FU. We follow a protocol that involves applying 5-FU to gauze for 24 hours following peripheral ostectomy and enucleation. To cease tissue exposure to 5-FU, the gauze is removed intact and the bone cavity is irrigated.The pathogenesis of OKC involves genes such as PTCH1, PTCH2, and SUFU, which are associated with the SHH pathway . The simA 38-year-old patient reported to the department with the chief complaint of pain and ulceration in the lower right back region of the jaw for two weeks. The patient was asymptomatic for one to two weeks before she felt pain in the right lower back region of her jaw. The pain was described as sharp, continuous, and radiating to the preauricular region and temporal region. The pain was aggravated by mastication and relieved upon taking medication. The patient also gave a history of ulceration in the same region for three to four days.The patient's dental history revealed that she underwent surgery in the left lower posterior region of the jaw five years ago at our hospital (a biopsy-proven case of OKC in the left mandible). Also, the patient underwent the extraction of teeth no. 45 to 48 (premolar to the third molar) and no. 35 to 37 (premolar to the second molar) six to seven years ago.Extraoral examination showed no remarkable facial asymmetry or swelling in the region. Tenderness on palpation in the right-angle region was evident. No signs of paresthesia were present. Intraoral examination showed alveolar ridge tenderness on palpation. No expansion of the buccal and lingual cortical plates was felt on palpation. Ulceration was seen in the 48th region (third molar region), and paresthesia was absent. The patient was partially edentulous in that region.A preoperative orthopantomagram (OPG) revealed an extension of the lesion from the right ascending ramus region to the subcondylar region Figure . A CT reEnucleation was done under general anesthesia. Exposure was made through an intraoral vestibular incision in the molar region that extended to the ascending ramus region. Access to the cyst was made through a perforation on the ramus, which was gradually extended by using a round bur. Enucleation and debridement of the cystic cavity were done. A sterile ribbon gauze soaked in topical 5-FU was inserted into the cavity after enucleation of the lesion. The wound was closed, leaving only a small opening for the removal of the gauze soaked in 5-FU that was inserted into the cystic cavity after enucleation. The gauze's distal end was left exposed for about 1 cm to facilitate its removal 24 hours after surgery. This was followed by weekly iodoform gauze dressings for six months until the cavity decreased in size with new bone formation. New bone regeneration sites were found in the radiographic follow-up after three months.The biopsy specimen revealed para-keratinized, stratified, and corrugated squamous epithelium with a thick five- to six-cell layer lining the cystic lumen. The basal cell layer was arranged in a palisading pattern resembling a tombstone. The epithelium was detached from connective tissue. The connective tissue was moderately collagenous, with a few areas of focus showing blood vessels lined by endothelial cells and extravasated RBCs. Histopathological impressions indicated it was an OKC.Without showing any signs of recurrence, the patient was consistently followed up on the third, sixth, and 12th months Figures -8. RadioThe keratocystic lesion, among various odontogenic cysts, is unique due to its aggressive nature and high recurrence rate, similar to tumors. The main reasons for OKC recurrence include a higher level of cell proliferative activity in the epithelium, budding in the basal layer of the epithelium, parakeratinization of the surface layer, supraepithelial split, subepithelial split, the presence of epithelial remnants or cell rests, and daughter cysts.Various treatment modalities have been proposed for OKC. Enucleation with different techniques , marsupialization alone or followed by enucleation, and resection are among the options mentioned in the literature . The choThe use of marsupialization as a treatment option for OKC has shown high success rates and is considered a conservative approach with low morbidity . This teControversies surrounding OKC treatment arise due to its diverse nature and high recurrence rates. Simple enucleation without additional measures has been associated with recurrence rates ranging from 17% to 56% ,14. HoweDashow et al. , in theiThe study conducted by Ledderhof et al. provided valuable insights into the management of OKC using Carnoy's solution and 5-FU . They reIt is important to note that the use of Carnoy's solution in OKC treatment was associated with delayed wound healing. Although Carnoy's solution showed some efficacy in reducing the recurrence rate, its potential drawbacks should be considered, especially in terms of postoperative healing and patient comfort. In comparison to MCS therapy, 5-FU is also recommended to effectively reduce postoperative inferior alveolar nerve paresthesia. Furthermore, Balamurgun's report highlighted 5-FU as a trendsetter in the treatment of OKC . This inOverall, these studies and histological classifications contribute to the ongoing understanding of OKCs and provide insights into the selection of treatment modalities that may offer improved outcomes and reduced recurrence rates for patients with this unique and challenging odontogenic cyst.It is important to establish protocols for the early diagnosis of OKCs to prevent the need for radical treatments that may result in substantial tissue loss. Long-term follow-up with periodic radiographic examinations is crucial to monitoring the condition and detecting any potential recurrence. With the emergence of newer treatment modalities, such as the application of 5-FU, the recurrence rate of OKC has decreased compared to traditional methods like Carnoy's solution.From the results of our study and based on various reviews of the literature, 5-FU may be considered ideal due to its availability, technical simplicity, low cost, decreased operating time, reduced morbidity, and inhibition of recurrence. Moreover, the literature also states that the application of 5-FU caused minimal nerve injuries, infection, and swelling, with no recurrence and no compromise in aesthetics or function. Our case too, showed no complications postoperatively. In summary, early identification, utilization of conservative approaches, and long-term monitoring are crucial in the management of OKC. Though the application of 5-FU shows promising results in reducing recurrence rates, continued research and follow-up studies are necessary to further understand and optimize the management of OKC.


The patient was admitted to the otolaryngology clinic, where empirical antibiotic therapy was initiated, comprising 1 g each of vancomycin and meropenem administered twice and thrice daily, respectively. A previously healthy 21-year-old man presented with a one-week history of generalized malaise, fever, and erythematous neck swelling with central black discoloration . He appeNeck ultrasonography showed an abscess, and computed tomography showed a large right-sided fluid-density lesion. This lesion, interspersed with air patches, extended to the supraclavicular region . DespiteNecrotizing fasciitis, a rare and life-threatening bacterial infection, necessitates immediate identification and treatment to prevent fatality


We report 2 cases of pheochromocytoma that showed nondiagnostic 68Ga-DOTATATE uptake but were subsequently visualized using alternative functional imaging modalities. Additionally, we provide a review of the literature to highlight the underappreciated limitations of functional adrenal imaging with somatostatin-based compounds.Pheochromocytomas are intra-adrenal sympathetic neuroendocrine tumors that arise from chromaffin cells. Paragangliomas similarly arise from chromaffin cells, although at extra-adrenal sites such as sympathetic paraganglia in the abdomen/thorax, or parasympathetic paraganglia in the head/neck. Collectively, pheochromocytomas and paragangliomas are important to diagnose and resect because they may secrete harmful levels of catecholamines, have mass effects, hemorrhage, and/or metastasize. Anatomic imaging of pheochromocytomas is usually completed with computed tomography or magnetic resonance imaging; however, functional imaging may be used to provide additional localization, staging, and/or biologic information. Accordingly, selection of the proper functional imaging modality can be critical to developing the optimal therapeutic strategy.  Pheochromocytoma and paraganglioma (PPGL) represent a subset of neuroendocrine tumors with distinct clinical, biochemical, and anatomic features. PPGL of the adrenal medulla usually secrete excess catecholamines and catecholamine metabolites. Paragangliomas that arise from sympathetic paraganglia in the abdomen or thorax may also overproduce catecholamines, whereas those originating in parasympathetic paraganglia in the head/neck rarely secrete catecholamines. PPGLs are frequently hereditary with an identifiable causative germline mutation, in 1 of more than a dozen genes, evident in approximately one-third of cases in adults.68Gallium-1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid (DOTA)-octreotate (68Ga-DOTATATE) uptake. Although the sensitivity and specificity of 68Ga-DOTATATE for PPGL is widely regarded to be exceptional, most studies focus on metastatic lesion identification rather than primary tumor diagnosis and do not intentionally assess PPGL diagnostic performance  is used to diagnose, localize, and characterize pheochromocytomas. Nuclear medicine studies provide additional diagnostic, localization, staging, and biologic information. Notably, the biologic heterogeneity of pheochromocytomas is reflected in the variable sensitivity/specificity of the distinct imaging modalities. Consequently, selecting the appropriate modality for individual patients is essential to providing appropriate diagnostic, staging, and therapeutic guidance. We report 2 cases of pheochromocytoma with nondiagnostic SDHC gene (c.397C > T (p.Arg133*)). Genetic testing was obtained because her sister was diagnosed with breast cancer and her father was diagnosed with prostate cancer. The patient had no personal or family history of SDHC-related disease, including PPGL, gastrointestinal stromal tumor, or renal cell carcinoma. She was normotensive and physical examination was unremarkable.An asymptomatic 60-year-old woman was incidentally found to have a pathogenic variant of the RET gene variant (c.1901G > T (p.Cys634Phe)). Her father died at age 38 years from unspecified complications of pheochromocytoma. Medullary thyroid carcinoma was diagnosed in her paternal grandmother and 3 paternal cousins. The patient's initial evaluation revealed mild diastolic hypertension. Physical examination was negative for palpable thyroid nodules.An asymptomatic 35-year-old woman was found to harbor a familial (high-risk) pathogenic SDHC gene mutation, 68Ga-DOTATATE PET/CT was performed to confirm a suspected pheochromocytoma and assess for paragangliomas. The 68Ga-DOTATATE positron emission tomography (PET)/CT demonstrated bilateral adrenal positivity (18F-fluorodeoxyglucose (18F-FDG) PET/CT, obtained to clarify the diagnosis, demonstrated intense uptake in the right adrenal nodule , whereas 24-hour urine metanephrines were normal. An adrenal CT scan revealed an enhancing 1.2-cm right adrenal nodule with an unenhanced CT attenuation of 31 Hounsfield units (HU) . Fifteensitivity  and E, il nodule  and G, H68Ga-DOTATATE PET/CT, performed to assess possible bilateral pheochromocytomas and metastasis, showed physiologic uptake bilaterally (123I-metaiodobenzylguanidine scintigraphy (123I-MIBG) was obtained to resolve whether bilateral disease was present; increased signal was observed in the right adrenal mass only  and PTH of 23 pg/mL . Screening for medullary thyroid cancer showed elevated serum calcitonin of 42 pg/mL . Plasma metanephrines were also abnormal, with elevated normetanephrine  and metanephrine . Thyroid ultrasound showed two .2-cm solid hypoechoic nodules (both TI-RADS 4). MRI of the abdomen revealed an enhancing 4.1-cm right adrenal mass without signal loss on out-of-phase imaging . Two nodaterally . 123I-meass only  and H.Preoperative blockade was deferred in favor of close intraoperative hemodynamic monitoring at the anesthesiologist's request; the patient was asymptomatic, home blood pressure monitoring was normal, only mild catecholamine excess was present, and the tumor was of small size. She underwent an uneventful laparoscopic total right adrenalectomy.The patient was started on α-adrenergic blockade with doxazosin 1 mg daily, which was up-titrated to 2 mg daily. She underwent laparoscopic cortical-sparing right adrenalectomy followed by total thyroidectomy. She tolerated surgery well and doxazosin was discontinued with resolution of diastolic hypertension.The patient had complete recovery. Pathology revealed a 1.4-cm pheochromocytoma with loss of SDHB immunostaining. There was no invasion through the adrenal capsule. At less than 1 year since surgery, continued biochemical and radiologic tumor surveillance remain negative.Adrenal pathology showed a 3.8-cm pheochromocytoma with focal capsular invasion, without involvement of periadrenal fat. Thyroid pathology showed .2-cm and .1-cm medullary thyroid microcarcinomas. Postoperatively, the patient initiated thyroid replacement and has maintained normal plasma metanephrines and calcitonin levels (5 years). Surveillance MRI scans demonstrate stable left adrenal thickening without evidence for residual/recurrent right adrenal disease.68Ga-DOTATATE PET imaging. The first case describes a patient with hereditary pheochromocytoma paraganglioma syndrome, caused by a pathogenic loss-of-function mutation of SDHC, which demonstrates autosomal dominant inheritance with incomplete penetrance. The mitochondrial SDH enzyme catalyzes the conversion of succinate to fumarate in the tricarboxylic acid cycle and functions as complex II of the electron transport chain. SDH-deficient tumors accumulate succinate, which acts as an oncometabolite that contributes to tumorigenesis .We report 2 cases of hereditary pheochromocytoma with false-negative igenesis . Case 2 68Ga-DOTATATE is a radiolabeled somatostatin analog that has demonstrated superiority for detection of PPGL at extra-adrenal sites. 64Cu-DOTATATE (expected to have similar performance to 68Ga-DOTATATE) is increasingly used by imaging centers because its longer half-life makes it easier to use in the clinical setting. In contrast,123 I-MIBG, which relies on the structural similarity of iobenguane to norepinephrine, may be used to identify neuroendocrine tumors with enhanced catecholamine storage but has lower detection rates than 68Ga-DOTATATE for paragangliomas and metastatic PPGL. 18F-fluorodihydroxyphenylalanine (18F-FDOPA) is a radiolabeled amino acid that takes advantage of upregulated amino acid transport by neuroendocrine tumors and has relatively high sensitivity for PPGL but is not widely available. 18F-FDG is useful for patients with primary and metastatic PPGL, although it lacks specificity for neuroendocrine tumors.The diagnosis and appropriate treatment of pheochromocytomas is essential because of the risks of acute and chronic exposure to catecholamine excess, the presence of malignant potential, and the potential to identify causative germline mutations that allow early diagnosis in at-risk family members. Proper management requires tumor localization, medical management of functional tumors, and surgical resection when practical. Because selection of the optimal surgical approach depends on reliable assessment of the extent of disease, it is important for clinicians to recognize the limitations of each imaging modality. Imaging with radiolabeled somatostatin analogs has demonstrated utility for characterizing neuroendocrine tumors, including pheochromocytomas, which exhibit increased somatostatin receptor expression. 68Ga-DOTATATE PET/CT was used to determine whether bilateral pheochromocytomas were present and did not demonstrate increased right adrenal uptake. Although failure to visualize the MEN2A-associated right pheochromocytoma with 68Ga-DOTATATE was unexpected, previous work has shown a higher detection rate in MEN2A-related pheochromocytoma patients using anatomic imaging  (100%) but lower for pheochromocytoma (73%) (68Ga-DOTA-conjugated somatostatin receptor-targeting peptides (68Ga-DOTA-SST)  was not significantly different between confirmed pheochromocytomas and normal adrenal glands  expression. 68Ga-DOTA-SST uptake depends on the tumor expression profile of SSTR subtypes. Because 68Ga-DOTATATE is most specific for SSTR2 and SSTR5, lower sensitivity for pheochromocytoma may be caused by absence of these SSTR subtypes on some tumors. Studies of SSTR expression in pheochromocytoma have yielded variable results. In 1 study of immunostaining for SSTR subtypes in 52 pheochromocytomas, SSTR3 was positive in 90% of tumors, whereas only 25% and 15% were positive for SSTR2 and SSTR5, respectively   , 7, 10. 4 , 7, 10A personalized approach is appropriate when choosing functional imaging studies for pheochromocytomas and paragangliomas.68Gallium-tetraazacyclododecane-1,4,7,10-tetraacetic acid-octreotate (68Ga-DOTATATE) positron emission tomography/computed tomography is a powerful tool for neuroendocrine tumor localization but more studies assessing its sensitivity for pheochromocytoma are needed.68Ga-DOTATATE may be better for metastatic disease and parasympathetic paraganglia in the head/neck.Pheochromocytoma and paraganglioma detection with 68Ga-DOTATATE positron emission tomography/computed tomography for pheochromocytoma may be explained by low SSTR2 expression, tumor necrosis, or high uptake by healthy adrenal glands.False-negative results with 68Ga-DOTATATE imaging and high suspicion of having pheochromocytoma.Additional functional imaging modalities may be necessary for patients with negative J.P.A. was involved in the diagnosis and patient management, writing of the manuscript, and manuscript submission. L.N. wrote the manuscript. S.E. provided images used for the figures. All authors reviewed and approved the final draft.


Sclerosing encapsulating peritonitis is a rare condition with a typical macroscopic appearance, with fibrocollagenous membrane enclosing loops of the small intestine, causing intestinal obstruction. Unexplained recurrent abdominal pain, obstruction, and a large array of other possible clinical signs and symptoms make sclerosing encapsulating peritonitis a diagnostic challenge.A 48-year-old man of Persian ethnicity was admitted multiple times to the emergency surgery department due to recurrent sudden abdominal pain and chronic obstruction without significant findings in medical history or clinical evaluation. Computed tomography was positive for proximal jejunal dilatation and duodenojejunal flexure stenosis due to internal mesenteric hernia. Exploratory laparoscopy, followed by laparotomy, confirmed thick membrane-like fibrous tissue with complete small intestinal loop envelopment. Extensive membrane excision and adhesiolysis was performed, but no mesenteric herniation was found. Early postoperative paralytic ileus with introduction of low-dose steroid therapy, based on histopathological and immunological results, confirming type III sclerosing encapsulating peritonitis, was completely resolved.Sclerosing encapsulating peritonitis is a rare and difficult-to-diagnose condition, further divided into primary and secondary sclerosing encapsulating peritonitis, on the basis of underlying etiology, dictating treatment modality and prognosis. Intraoperative diagnosis and surgical treatment are mandatory, besides a wide variety of abdominal computed tomography scans, inconclusive results, and clinical presentations. There are so far no known specific markers for the diagnosis of sclerosing encapsulating peritonitis. Primary sclerosing peritonitis (PSP) is a rare clinical entity, with nonspecific radiological markers for which we currently have no noninvasive clinical markers. Early recognition and recurrence in mild form allow medical treatment. Extensive surgical resections in the absence of recognition of the clinical entity result in significant morbidity with consequent short bowel syndrome and dependence on total parenteral nutrition.Sclerosing encapsulating peritonitis (SEP) is a rare condition in which chronic inflammation causes fibrocollagenous membrane formation around the small intestine, leading to small intestine entrapment and therefore gastrointestinal obstruction at different levels with resulting clinical presentations –3. LargeCandidate factors associated with the development of primary PSP include infectious factors, the use of certain medications, and autoimmune factors or responses. Besides, the proposed etiopathogenesis of SEP includes also developmental disorders related to vascular anomalies and omental hypoplasia. Among factors related to higher incidence of the disease are the use of beta-blockers, abdominal trauma/surgery, ventriculoperitoneal shunt, peritoneal tuberculosis, sarcoidosis, systemic lupus erythematosus, gastrointestinal malignancy, and liver cirrhosis with different etiopathologic triggering factors and pathophysiological mechanisms.Up to now, the diagnosis is mostly made during surgical exploration; various radiological diagnostic methods are unreliable for diagnosis, including an abdominal computed tomography (CT) scan that, among others, showed better specificity and sensitivity and might play an important role preoperatively  in case We report the case of a 47-year-old man of Persian ethnicity who presented to the emergency department several times, complaining of abdominal pain and long-ongoing constipation. Radiologic imaging suggested an internal herniation at the level of the duodenojejunal flexure, but it was not present at the time of explorative laparotomy due to complete upper intestinal obstruction. The diagnosis was made intraoperatively, confirmed promptly by histopathology. Extensive adhesiolysis with careful membrane excision and a short course of postoperative steroid therapy was efficient in postoperative paresis resolution and functional recovery of the small intestine. Treatment was based on histopathological evaluation.A 48-year-old man of Persian ethnicity presented multiple times to an outpatient surgical emergency department (ED) with complaints of colicky abdominal pain in the left upper and lower quadrant, which became worse after a meal, nausea, vomiting, and a history of obstipation, which lasted 3 months before his first episode of abdominal pain. The patient had no previous history of abdominal pain or abdominal surgery. He also had no significant medical history of chronic diseases such as hypertension, obesity, or diabetes, only occasional problems with acid reflux disease. The patient denied using any medications, drugs, or alcohol, or having any allergies. The patient was examined at the emergency department (ED) four times with very similar complaints and diagnostic findings. There were no alarming findings on physical examination, only abdominal tenderness and pain in the left upper and lower quadrants. Bowel sounds were normal in all four quadrants.Laboratory blood findings were insignificant. An abdominal X-ray suggested obstipation, but there were no signs of intestinal obstruction. The patient was treated with an osmotic laxative, a proton pump inhibitor (PPI), an analgesic, and a spasmolytic. After that, he felt relief and was discharged home with instructions in case of further abdominal pain, fever, or vomiting. He was also referred for further diagnostic investigation.The patient underwent gastroscopy, colonoscopy, abdominal ultrasound, and abdominal computed tomography (CT). Gastroscopy showed erythematous gastric mucosa and fluid retention in small bowel loops. Colonoscopy revealed no pathological findings. Abdominal ultrasound suggested subileus of the small bowel, which is why the patient underwent further abdominal CT. An abdominal CT scan was suggestive of duodenojejunal flexure stenosis due to an internal mesenteric hernia Fig. a–d. ProxDuring exploratory laparoscopy, we found thick membrane-like fibrous tissue, enveloping loops of the small intestine and adhesions between the loops of the small intestine. Due to the nonrecognition and inaccessibility of structures in the abdominal cavity, covered with a thick fibrous membrane, we decided to proceed with the conversion to laparotomy and performed vast membrane excision and adhesiolysis. The fibrous membrane was removed, and adhesions between small bowel loops were released. We also took tissue samples and peritoneal swabs for histopathological and microbiological examination. Although the CT scan suggested internal mesenteric hernia, we did not find any during the operation despite careful and thorough observation. We also did not find any congenital small intestinal pathologies.Escherichia coli, Klebsiella oxytoca, and Enterococcus faecalis and negative for anaerobic culturing. In a short, early postoperative course, the patient complained of persistent nausea. Abdominal ultrasound indicated dilated small bowel loops and no peristalsis. An abdominal X-ray on day 9 postoperatively suggested paralytic ileus due to recurrent duodenojejunal flexure stenosis. Peripheral venous blood showed normal SIgA (serum immunoglobulin A) 2.95 g/l (range 0.61–3.56 g/l), elevated S-IgG4 3.1010 g/l (range 0.30–2.1010 g/l) and elevated Il-6 97.9 ng/l (range up to 7.0 ng/l). (ELISA), a serum iron level of 2.1 micromol/l (range 10.7–28.6 micromol/l) and a normal haematological profile . Taking into account the histopathological findings, the levels of S-IgA, SIgG-4 and Il-G (interleukin-6) in the peripheral venous blood and the clinical picture, steroid treatment of PSP was initiated at a dose of 1.5 mg per kg body weight (bw) with dose tapering depending on the resolution of the paralytic bowel obstruction 2 weeks after surgery. The patient gradually obtained regular defecation habits and oral nutrition and reported no nausea, so we discharged him on day 22 postoperatively. Peritoneal swaps were tested for acid-fast bacteria. The results were negative. Cultivation of mycobacteria from peritoneal fluid was also made and tested negative. Peritoneal tissue samples were stained with hematoxylin and eosin. Histologically, chronic fibrinous sclerosing peritonitis with an uneven and relatively mild lymphoplasmacytic inflammatory infiltrate was found (Fig. The patient was treated with complete parenteral nutrition for an early postoperative course of 10 days, followed by a combined parenteral and liquid oral diet until complete recovery. A short course of prolonged antibiotic surgical prophylaxis with cefazolin and metronidazole was followed by an amoxicillin course for 7 days, based on intraabdominal intraoperative swab culture results that were positive for aerobic Other differential diagnostic possibilities such as amyloidosis and other forms of immune-borne diseases, subclinical primary virus peritonitis, or complication from beta-blocker treatment were excluded.The patient received instructions on medication in case of recurrence of the same symptoms at the time of hospital discharge. At outpatient follow-ups, months 1, 3, and 6 postoperation, the patient reported no pain, normal bowel movements, and a return to normal weight before the onset of the disease. Laboratory results showed no abnormalities in haemogram, electrolytes, hepatogram. Exceptionally, he returned to the outpatient clinic again 10 months after hospital discharge for anal thrombosis unrelated to the underlying disease.SEP, also referred to as abdominal cocoon, abdominal cocoon syndrome, peritonitis chronica fibrosa incapsulata, sclerosing peritonitis, or encapsulating peritoneal sclerosis, is a chronic inflammatory condition, characterized by a fibrocollagenous membrane that forms around loops of the small and/or large intestine, causing abdominal pain and intestinal obstruction on different levels , 3, 5. SSome case reports also present cases of patients with SEP and only a few or none of the aforementioned symptoms and clinical signs, which confirms a wide array of different clinical presentations of SEP , 13. OurSEP is usually diagnosed only during exploratory laparoscopy , 14. Labet al.] and immunologically mediated [Manzano et al.]. Based on the literature search, there are newer descriptions on the possible role of immune-mediated SEP with the basic concept of autoimmune mechanisms involved; in our case presentation, we also support autoimmune concepts of disease as we confirmed histopathologically approximately 17 IgG4 plasma cells per HPF with an IgG4/IgG ratio of 39% [et al. [Currently, we do not have any specific markers that might be used for the diagnosis of SEP , 3. Accoo of 39% –25. We s [et al. . BesidesWe are limited in presentation as we did not analyze plasma IgG4 concentration, even though, according to the literature, it is a useful tool supporting SEP diagnosis after exclusion of other possible diseases .New diagnostic tools are mandatory to reduce long-term complications, both medical and surgical, and early medical treatment is mandatory . The curet al., as well as data and literature, including case reports and a literature review, support our conclusions from the described case presentation study that, in SEP, the implication of the investigative combination IL-6 and IgG4 might be used as an activity marker and predictor of the disease [Treatment and management of SEP are based on the underlying etiology, severity of the case, and the patient’s clinical signs and symptoms , 27. The disease , 31. Als disease , 32.Surgical intervention remains the gold standard for management of primary SEP , 28. MosAbdominal CT has high specificity and should be useful as a tool in preoperative diagnostic modalities. So far, no serum markers have been described to be used as diagnostic criteria; our case report study showed increased IL-6 and IgG4, and low serum iron combined with a normal CRP level, which might be an important diagnostic criterion and support other newer descriptions in the literature; IL-6 diseases should be excluded before the introduction of conservative treatment. To date, most SEP cases are still diagnosed during exploratory laparoscopy, and for advanced disease, surgical membrane excision and adhesiolysis remain the gold standard. The high burden of disease in women with proven endometriosis and complications of the disease, in addition to the clinical picture described above, requires consideration of evidence from the literature and the collection of candidate markers that could be used to infer the occurrence and prevalence of the disease, taking into account the triggering factors associated with fibrous tissue overgrowth in the abdominal cavity.SEP is a rare but very important differential diagnosis in patients presenting with unexplained recurrent abdominal pain, vomiting, and intestinal obstruction on different levels. The collection of candidate markers that could be used to infer the occurrence and prevalence of the disease is needed, taking into account the triggering factors associated with fibrous tissue overgrowth in the abdominal cavity.Finally, we encourage all experts to add primary SEP to their differential diagnostic pool in patients with unexplained abdominal pain and obstruction and use the optional diagnostic tool before starting early conservative treatment.


Tolosa-Hunt syndrome (THS) is an idiopathic inflammatory condition involving the cavernous sinus and orbital apex with an incidence of 1 case per million per year. We report on a case of a 70-year-old male with atypical MRI findings, vision loss, and painless ophthalmoplegia.  Ophthalmic evaluation revealed his best-corrected visual acuity was 20/40 in the right eye and counting fingers at a 0.5-foot distance in the left eye. External examination of the left eye revealed limited ocular movement, proptosis, and a positive relative afferent pupillary defect. Complete blood count, inflammatory markers, and full biochemistry tests, including thyroid and liver function tests, were within the normal range. A magnetic resonance imaging of the orbits with and without contrast demonstrated a homogenously enhancing lesion at the posterior intraconal compartment of the left orbit, extending to the orbital apex with the involvement of the adjacent extraocular muscles. The patient was started on intravenous methylprednisolone 60 mg daily and later discharged on prednisone 5 mg daily with partial symptom improvement on follow-up. Resection and biopsy revealed a soft tissue lesion with mixed inflammatory infiltrate. The clinical, pathological, and imaging findings favored the diagnosis of THS.  Tolosa-Hunt syndrome (THS) is an idiopathic inflammatory condition involving the cavernous sinus and orbital apex -8. The iIn the time before the ready availability of imaging technologies, THS was largely a diagnosis of exclusion, based on clinical presentation and response to steroid therapy . DiagnosWe present herein the atypical MRI findings of a 70-year-old male experiencing vision loss and painful ophthalmoplegia 14 days after cataract surgery.A 70-year-old male presented to our emergency department with vision loss and limited eye movements in the right eye, persisting for five days. The patient's medical history was notable for hypertension and cataract surgery performed 14 days prior. His review of systems indicated left-sided headaches and facial numbness. His social and family history were otherwise unremarkable.Upon ophthalmic evaluation, his best-corrected visual acuity was 20/40 in the right eye and counting fingers at a 0.5-foot distance in the left eye. External examination showed that extraocular movements were within normal limits for the right eye. The left eye exhibited limited ocular movement, proptosis, and a positive relative afferent pupillary defect. Complete blood count, inflammatory markers, and comprehensive biochemistry tests, including thyroid and liver function tests, were all within normal limits.An MRI scan of the orbits, both with and without contrast, was performed using a Philips Achieva model scanner with a 1.5-tesla magnet. The MRI revealed a homogeneously enhancing lesion in the posterior intraconal compartment of the left orbit, extending to the orbital apex and involving the adjacent extraocular muscles (Figure During admission, the patient was treated with intravenous methylprednisolone 60 mg and then discharged on prednisone 5 mg daily per oral with partial symptom improvement on follow-up. Resection and biopsy of the orbital apex soft tissue was subsequently performed demonstrating a soft tissue lesion with mixed inflammatory infiltrate. These findings favored THS rather than a neoplastic lesion such as lymphoma.Reports of THS are limited and display a wide array of clinical presentations and associated findings -11. THS The existing literature on THS indicates that it is a granulomatous inflammatory condition affecting the cavernous sinus, which may extend to the superior orbital fissure, orbital apex, or optic nerve ,11. ThisThe International Headache Society diagnostic criteria classify THS under painful cranial neuropathies and other facial pains ,5. AccorSpecific clinical diagnostic criteria are delineated; however, literature on the radiological aspect of the diagnosis remains ambiguous ,3,6,9,10MRI changes pre- and post-steroid therapy may confirm the diagnosis of THS; however, radiological resolution takes months to appear . AuthorsMRI is a very useful tool as it may contribute valuable data about a patient’s disease in a noninvasive manner ,3,6,9,10Tolosa-Hunt syndrome is an extremely rare inflammatory condition with a variable clinical presentation and response to treatment. It is primarily a diagnosis of exclusion. Patients often present with painful ophthalmoplegia that improves with steroid therapy. While specific imaging criteria do not exist, MRI can serve as a valuable tool in supporting the diagnosis. The radiologist must recognize MRI findings suggestive of THS, as well as differentiate other causes of painful ophthalmoplegia, to establish appropriate management and follow-up. Although the potential link between cataract surgery and the triggering or exacerbation of inflammation is noteworthy, further research is needed to clarify this possible association.


MDM2 gene and a homozygous loss of CDKN2A on 9p21. A review of the literature regarding primary cardiac malignancies and intimal sarcoma in children is provided.Undifferentiated mesenchymal tumors from the intimal layer  are rare within the ventricles and exceptional in children. A rare case of an intimal sarcoma located in the right ventricle in a young child is presented with need for urgent surgical resection due to mechanical flow obstruction. Tumor cells showed amplification of  Cardiac intimal sarcomas are very rare. In contrast to vascular intimal sarcoma, cardiac involvement is an infrequent finding especially in children. A retrospective study of 100 cardiac sarcomas however, describes intimal sarcoma as the predominant primary sarcoma of the heart –4.We present a case of a 4-year old child presenting with an intimal sarcoma located in the right ventricle with need for urgent surgical resection. This is a one of the few reports of cardiac intimal sarcoma and the first report of a ventricular localization in a young child.A 4-year old child was admitted to the pediatric cardiology department with suspicion of right heart failure: ascites, edema and poor circulation. There was a history of progressive fatigue and exertional dyspnea for some days. Medical history was negative apart from food allergy.The patient presented with edema of the lower limbs with poor circulation, abdominal distention with hepatomegaly. There was no cardiac murmur, heart rate was 53 BPM, blood pressure of 101/73 mmHg and oxygen saturation of 100%.Abdominal ultrasound confirmed ascites and hepatomegaly. Chest x-ray showed cardiomegaly with presence of bilateral pleural effusion. The cardiac ultrasound revealed a large mass in the right ventricle bulging through tricuspid valve into the right atrium; delineation of the leaflets of the tricuspid valve was impossible due to the mass.  The righUrgent surgical resection was needed within 24 h after admission. The tumor was steeled on the anterior papillary muscle of the tricuspid valve ,B. A comMDM2 and EGFR was seen  and ifosfamide  was proposed, but parents refused postoperative chemotherapy. Cardiac magnetic resonance imaging did not show any macroscopic neoplasm 6 months after surgical resection.Adjuvant chemotherapy with doxorubicin  and no stenosis.Primary cardiac sarcomas are rare but represent the majority of primary malignant cardiac tumors. There are few series describing sarcomas of the heart, and the majority of publications are case reports , 5–10. RIntimal sarcoma is a malignant mesenchymal tumor which is usually a poorly differentiated sarcoma composed of atypical spindle cells and/or pleomorphic cells with the possibility of myxoid areas or epithelioid morphology. Tumor cells generally exhibit immunoreactivity for vimentin and variable expression for smooth muscle actin but are usually negative for desmin and endothelial markers . In the Primary cardiac sarcomas are highly aggressive tumors which rapidly infiltrate all the layers of the heart and metastasize rapidly, especially when right-sided . At the MDM2 (MDM2, as well as loss of CDKN2A, supported the diagnosis.The presented case is exceptionally rare for age of presentation as well as for tumor location for this type of tumor. According to the literature this is the first report of an intimal sarcoma within the ventricle in a young child. Previously a case of pulmonary artery intimal sarcoma has been reported in an infant and in the left atrium in another child , 22. SofMDM2 . In our In the case reported here complete resection of tumor could be obtained. Further tumor staging was negative apart from presence of multiple hypermetabolic lymph nodes on PET-CT scan. The lymphadenopathy could happen due to multiple reasons, as inflammation, a recent infection or more likely due to congestion caused by the sarcoma blocking the flow in the right ventricle and causing the effusions in the pericardium and the pleura, as well as causing the ascites. In the diagnostic work-up of intimal sarcomas CT and MRI are optimal for assessing tissue characteristics, infiltration and metastases . Given tMDM2 amplification. Although the prognosis of primary cardiac sarcomas is overall poor, the presented case did show a good survival up to 8 years after surgical resection of the tumor.Intimal sarcomas, undifferentiated mesenchymal tumors arising from the intimal layer of the ventricle are an extremely rare entity in children. The molecular diagnosis is confirmed by a