BRAF mutation and infantile fibrosarcoma-like morphology. Though rare, malignancy should be considered in the differential diagnosis for bowel perforation in an otherwise healthy, term infant as complete surgical excision can be curative.Spontaneous intestinal perforations in the neonatal population are mostly associated with low birth weight, prematurity, and necrotizing enterocolitis. Spontaneous intestinal perforation in the absence of these risk factors is extremely rare and should raise clinical concern for an underlying bowel pathology. Here we present a unique case of a normal-weight, full-term girl with spontaneous intestinal perforation due to a spindle cell neoplasm with a novel  BRAF-altered spindle cell neoplasm similar in histologic appearance to infantile fibrosarcoma (IFS); however, it lacked the characteristic ETV6:NTRK3 tyrosine kinase mutation seen in IFS  in a normal-weight, term baby girl. Pathology from the bowel segment containing the perforation revealed a n in IFS . This ran in IFS –4. Our p disease , 6.A 3-day-old, full-term baby girl presented at her first pediatrician appointment with decreased appetite, urine output, stool output, and a 14% weight loss since birth. She passed meconium on the first day of life and was breastfed. Her abdomen was noted to be distended and taut with prominent abdominal veins .She had no significant prenatal history. The family history was significant only for breast cancer in a female paternal first cousin in her 4th decade of life and was otherwise unremarkable for malignancy or any intestinal diseases.The patient’s pediatrician sent the patient to the emergency department where an abdominal X-ray revealed a large volume of pneumoperitoneum concerning for bowel perforation . Vital sAn exploratory laparotomy revealed a large perforation in the mid to distal jejunum. Purulence and friable tissue consistent with a subacute presentation of bowel perforation was noted in the abdominal cavity, but there was no evidence of necrotizing enterocolitis or any other intra-abdominal lesions. The segment of the bowel containing the perforation was resected and an enterostomy and mucus fistula were created. Given the degree of gross intra-abdominal contamination due to perforation, a primary anastomosis was not attempted.Pathology of the resected bowel revealed a perforation measuring 1.4 cm × 0.8 cm in maximum diameter. There was reactive bowel wall thickening around the perforation. A microscopic tumor was identified at the site of the perforation . The neoETV6:NTRK3 fusion transcript associated with IFS. A solid tumor comprehensive panel confirmed this result and additionally revealed two distinct mutations in BRAF: the first, a missense mutation of lysine to arginine in exon 15; and the second, a novel exon 1-11 variant splicing consistent with the intragenic deletion of exons 2-10. With this genetic profile, the tumor was classified as a BRAF-altered spindle cell neoplasm with IFS-like morphology and non-specific immunoprofile containing a novel BRAF mutation.The histopathology of the neoplasm resembled IFS; however, a single fusion gene panel did not detect the characteristic Following the diagnosis of neoplasm, a CT scan of the chest, abdomen, and pelvis was obtained to look for other sites of disease. This revealed sub-centimeter soft tissue nodules on the anterior chest and abdominal wall, but no evidence of distant disease. The patient had an ostomy reversal about 2 months after her initial laparotomy. A soft tissue nodule adjacent to the surgical incision was biopsied and showed signs of benign fibromuscular tissue with reactive changes but no malignancy. One of the radiographically identified anterior chest soft tissue nodules was biopsied at this time and pathology also revealed no evidence of malignancy or pathologic abnormality.Based on the available literature, a primary resection with negative margins was determined to be the only necessary treatment and no adjuvant treatment was planned. The patient was followed with routine imaging and physical exams. The genetic evaluation showed no germline BRAF mutation and no BRAF-associated genetic syndromes. The patient was discharged home at 3 months of age and is currently thriving with no signs of disease recurrence.ETV6:NTRK3 translocation that affects a tyrosine kinase , which makes up only 7.5–11% of non-V600 BRAF-associated tumors. In two prior studies, this mutation has been shown to confer gain-of-function to the kinase portion of the BRAF gene and increases cell proliferation  and is associated with high mortality and morbidity ’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.LTC and LSC were the primary authors of the manuscript. AL and NQ contributed to the


Hepatoid adenocarcinoma of the duodenum is a rare special type of adenocarcinoma, featured by hepatocyte components in primary adenocarcinoma of the duodenum. It has the characteristics of high malignancy, invasiveness, rapid progress, and poor prognosis. An abnormal elevation of serum alpha-fetoprotein (AFP) may occur in most cases. The diagnosis is mainly based on pathological morphology. Here, we reported a case of hepatic adenocarcinoma of the duodenum. The middle-aged female patient had an ampulla mass at diagnosis and received radical pancreaticoduodenectomy. The postoperative pathology was stage IIIA duodenal adenocarcinoma. At 1 month after surgery, she had multiple intrahepatic metastases and retroperitoneal lymph node metastasis; the AFP level was 300 ng/ml at that time. As she refused target therapy, two cycles of capecitabine-oxaliplatin (XELOX) chemotherapy were performed. However, the AFP elevated from 300 to 1,931.90 ng/ml, and the disease progressed rapidly. Immunohistochemistry (IHC) of tissue samples from presurgical endoscopic ultrasound guided fine needle aspiration (EUS-FNA), surgery, and liver biopsy showed positive AFP staining. Combining the abnormal elevation of serum AFP and microscopic pathological morphology, this case is diagnosed as hepatoid adenocarcinoma of the duodenum with liver metastasis. The physical condition of this patient was too poor to receive follow-up treatment. She died of the rapid disease progression with an overall survival time of 161 days. Considering that in most patients with hepatoid adenocarcinoma the abnormal elevation of serum AFP occurs preoperatively and returns to normal postoperatively rather than normal before surgery and increased after surgery, the primary lesion is located in the stomach rather than the intestine, and the patients are more often older men rather than middle-aged women; this case is rare particularly. Therefore, reporting this case with complete case data may be helpful to further study, so as to improve the understanding of this special type of malignant tumor. In 1970, Bourreille et al.  proposedThe clinical manifestations of HAC patients are usually non-specific and mostly depend on the specific location of the primary tumor. For example, patients with colorectal cancer (CRC) may have abdominal pain, fever, or hematochezia. When a tumor occurs in the ampulla and nipple area of the duodenum, clinical manifestations such as yellow skin, yellow eyes, and yellow urine (such as in this case) can be seen. In most cases, patients have abnormally elevated serum AFP expression, but normal serum AFP expression  can also be seen.Here, we introduce a middle-aged female patient diagnosed with hepatic adenocarcinoma of the duodenum with hepatic metastases; serum AFP was normal at the time of diagnosis but increased significantly with the progression of the disease. As the patient refused genetic testing and targeted therapy, she was treated with two cycles of XELOX chemotherapy and subsequently died due to rapid progression.Our patient is a 59-year-old woman with a history of hypertension and hyperglycemia for more than 15 years without previous history of alcohol consumption or smoking nor history of liver disease. She presented with intermittent fever without obvious inducement for 3 weeks  score result of 1. Physical examination revealed yellow skin and yellow sclera. The patient and family had no previous medical history of chronic viral hepatitis, bowel polyps, and inflammatory bowel disease (IBD). The patient underwent pancreaticoduodenectomy on 30 October 2020. Intraoperative exploration showed that there were no free ascites or metastatic nodules in the abdominal cavity, the liver had a normal size with cholestasis-like changes, and the gallbladder was swollen. A mass with a size of about 3 cm * 3 cm could be reached in the ampulla, and the texture was hard. Postoperative pathology (pancreaticoduodenectomy specimen)  circular or vacuolar, the nucleolus is large and obvious, mitotic images can be seen, and some of them have flake necrosis . Some scGastric HAC is relatively more common than the others, so the research on this special type of tumor mostly focuses on gastric HAC. Clinical treatment of gastric HAC is usually preceded by radical gastrectomy. Radical gastrectomy combined with resection of liver metastases is feasible when liver metastasis is clearly identified and patient conditions permit. If radical resection of gastric lesions is not possible, surgical treatment is still recommended, and the surgical method should be changed to palliative gastrectomy. For patients with hepatic metastasis, palliative gastrectomy can be combined with other treatment methods, such as hepatic artery intubation chemotherapy . At presMost of the previously reported cases of HAC had a primary lesion in the stomach, and most of the cases showed abnormal elevation of serum AFP in the preoperative period; whereas in this case, the primary lesion was located in the duodenum, which is a rare site, and the elevation of serum AFP was observed in the postoperative period, which made the diagnosis of this case less precise. The reason for reporting this case is to draw the clinician’s attention to the specific pathological type of HAC. When the pathological morphology of the tumor tissue tends to coexist with areas of adenocarcinoma and hepatoid differentiation, the possibility of HAC should be highly suspected and the accurate diagnosis should be identified as soon as possible. Moreover, the normal serum AFP at the time of diagnosis of the primary lesion cannot exclude the possibility of hepatocellular adenocarcinoma.In conclusion, HAC of the duodenum is a rare special type of duodenal cancer with poor prognosis. It is often difficult to make a definite diagnosis in the early stage. The disease was not considered in the first EUS-FNA biopsy and pancreaticoduodenectomy; the diagnosis was made after the fast elevation of AFP and liver biopsy. It is suggested that for middle-aged and elderly patients, if the level of serum AFP increases and there is insufficient evidence for the diagnosis of hepatocellular carcinoma, HAC should be considered. Due to the low incidence rate, the treatment experience for this kind of disease is insufficient; targeted and effective standardized treatment remains to be explored.In reviewing the paper, the following shortcomings remain, as there is no standard treatment regimen for this rare case, which is a common dilemma in the treatment of HAC. Focusing on this case, although the patient’s symptoms were somewhat relieved by the systemic treatment described above, it is undeniable that the patient’s survival was still relatively short and the exact mechanism of this is still to be explored in a pooled analysis of a large number of cases in order to find more effective treatment regimens.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.ND and LL provided ideas for this case. XW, ND, LL, and BL drafted the manuscript and provided figures. LL, XW, JH, and XQ acquired, analyzed, and interpreted the data. ND, LL, XQ, and BL revised the manuscript critically for important intellectual content and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors contributed to the article and approved the submitted version manuscript.This study was supported by the Basic Research Project of Jiangsu Province (No. BK20211007).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Feline pancreatic neoplasia is a rare clinical and post-mortem diagnosis. This report aims to present the pathological findings of a case of pancreatic hemangiosarcoma in a cat with a history of sudden death. A neoplastic mass, covered by numerous small blood clots, was identified within the body of the pancreas, along with multiple metastases within the omentum and liver. The sudden death was most likely caused by the spontaneous rupture of the pancreatic neoplasm, which produced severe hemoperitoneum and anemia. Cytological and histological findings were completed by immunohistochemical evaluation, supporting the diagnosis of hemangiosarcoma.A 15-year-old female, indoor, spayed, domestic shorthair cat was presented for post-mortem evaluation with a history of sudden death. A red-brown 3 cm x 4 cm neoplastic mass was identified within the body of the pancreas, along with multiple similar nodular structures within the omentum and liver. Associated lesions included hemoperitoneum, yellow discoloration of the peritoneal wall, and severe anemia. Spindle-shaped neoplastic cells exhibiting malignancy features, which occasionally contained within the cytoplasm haematoidin crystals and/or red blood cells, were observed during cytological examination. Histologically, the neoplastic cells were organized in short streams forming vascular spaces filled with erythrocytes. Immunohistochemically, the neoplastic cells were immunolabeled for vimentin and CD31, supporting the diagnosis of hemangiosarcoma. This report offers a complete post-mortem and histological evaluation of a rare tumor in cats with an unusual location and a comparative assessment of 3 anti-CD31 antibodies. Hemangiosarcoma (HSA) is an aggressive malignant tumor originating in the vascular endothelial cells. In both dogs and cats, HSA is classified into non-visceral and visceral forms ,2, the lIn cats, sudden death was recorded in 7.9% of cases, and the most common cause of unexpected death was related to trauma, followed by heart disease and neoplastic disease . Currently, there are only a few reports in the literature on the subject of feline primary and metastatic non-epithelial pancreatic tumors, most of them being included in epidemiological studies ,6,7,11. A 15-year-old female, indoor, spayed, domestic shorthair cat with a history of sudden death was presented to the Department of Pathology at the Faculty of Veterinary Medicine (Cluj-Napoca) for post-mortem evaluation. According to the owner, the cat had no clinical signs before death. A gross general pathological examination was performed, and the body score was defined as good.Tissue samples collected during the post-mortem evaluation consisted of pancreatic neoplasm and multiple abdominal metastases. Impression smears were obtained from all nodular masses and stained with Dia Quick Panoptic .TM HM 325 Rotary Microtome), followed by hematoxylin & eosin staining.Neoplastic masses from each affected organ were collected and fixed for 48–72 h in 10% neutral buffered formalin, followed by routine paraffin embedding. Two-three μm thick tissue sections were obtained using a manual microtome . For immunohistochemical evaluation, the panel of antibodies is presented in All histological sections were examined using an Olympus BX-41 microscope. The photomicrographs were obtained using Olympus SP 350 digital camera connected to Stream Basic imaging software . During the external evaluation, the most important changes were pale mucous membranes and moderate abdominal distention. Approximately 300 mL of partially coagulated blood was detected within the abdominal cavity, associated with yellow discoloration of the peritoneal wall and abdominal fat. A mottled red-brown 3 cm × 4 cm neoplastic mass was found A, locateAdditional findings included moderate pulmonary edema, chronic, mild interstitial nephritis, a renal cyst, and follicular gastritis.Cytological examination revealed the presence of neoplastic spindle cells A, approx2, and atypical cell divisions were a frequent finding. Similar histologic features were observed in the hepatic and omental nodular structures. The established presumptive diagnosis was pancreatic hemangiosarcoma with multiple metastases based on gross, cytological, and histological findings. Immunohistochemically, the neoplastic cells showed diffuse cytoplasmic staining for vimentin , the most common pancreatic neoplasms in cats are represented by epithelial tumors, adenocarcinoma is the most frequently identified one %, the mo. Non-neoNon-epithelial neoplasms of the pancreas, such as lymphoma, mast cell tumor, fibrosarcoma, histiocytic sarcoma, and hemangiosarcoma, are rarely encountered and poorly characterized . To the According to the clinical history provided by the owner, the cat presented no clinical signs before death. Considering the large amount of partially coagulated blood found within the peritoneal cavity, the time elapsed between the onset of hemoperitoneum and the death of the cat was very short. Post-mortem findings suggest that spontaneous rupture of the pancreatic neoplasm, producing hemoperitoneum and severe anemia, was responsible for the sudden death. This evidence was supported by the blood clots observed on the surface of the neoplastic mass. Hemoperitoneum may be classified into spontaneous and traumatic , the latEven though the main metastasizing route of HSA is represented by the hematogenous one, the friable nature of this neoplasm may cause small tears making it prone to direct implantation . Histological evaluation of the pancreatic mass, omental and hepatic metastases revealed the presence of neoplastic spindle to polygonal cells organized in streams and bundles, which were delineating variably-sized vascular spaces. Recognized growth patterns of HSA include capillary, cavernous, solid, and epithelioid variants . CurrentFor immunohistochemical evaluation, the markers used for diagnosis of HSA are CD31 and/or von Willebrand factor. Additionally, CD34 can be used if the immunostaining for the previously mentioned markers results in a strong background. CD34 expression was also observed in several types of carcinomas and lymphohematopoietic tumors in cats, and its specificity for HSA is considered lower than the other two markers . Using tIn both dogs and cats, HSA is considered an aggressive tumor with a poor prognosis and high potential for metastasis. This report describes the pathological findings of a case of pancreatic HSA with abdominal metastasis and hemoperitoneum. The gross and histological findings were supported by the immunohistochemical evaluation, with the best results being obtained using CD31, clone JC70A. HSA should be considered as a differential diagnosis of pancreatic masses in cats.


The authors report a case of an embryonal tumor with multilayered rosettes (ETMR) in an 18-month-old female infant who presented with gait imbalance and progressive left-sided weakness for 2 months. ETMR is a rare small round blue cell aggressive tumor of the central nervous system characterized by the amplification of the C19MC region on chromosome 19 (Chr19q13.42). This report in detail the clinical–radiologic and histopathological workup and diagnosis. Because ETMRs are newly described rare pediatric central nervous system tumors with only a few reported cases, we aim to document this typical case to add to the existing data on these tumors. This aggressive tumor has a hallmark cytogenetic abnormality with amplification of the C19MC region on chromosome 19 (Chr19q13.42). ETMR usually affects children before their fourth birthday and can occur anywhere in the brain with highly aggressive behavior and poor prognosis. Morphologically, it is characterized by multilayered rosettes and belongs histologically to WHO Grade VI neoplasms. Because ETMRs are newly described rare pediatric CNS tumors with only a few reported cases, we aim to document this typical presentation to add to the existing data on these tumors.Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered, is a rare malignant pediatric brain tumor recently included in the 2016 World Health Organization (WHO) update classification of central nervous system (CNS) neoplasms and is also present in the latest version of WHO classification of CNS tumors 2021.An 18-month-old female initially presented with right eye pain and outward deviation. Evaluation by an ophthalmologist led to the diagnosis of conjunctivitis. Over the next 2 months, the patient developed gait imbalance, frequent falls, and left-sided weakness with drooling of saliva, leading to a referral to a neurologist.The patient was conscious and alert with normal tone, normal reflexes, and no signs of increased intracranial pressure. The patient had an ataxic gait, drooling of saliva on the left, and decreased motor strength on the left.On admission, the temperature was 36.9°C, respiratory rate was 28 cycles/min, blood pressure was 86/56 mmHg, SpO2 was 98%, height was 83 cm, weight was 17.5 kg, and 25.4 body mass index.A head CT was performed, which demonstrated an isodense to hyperdense mass with a well-defined margin involving the right lateral aspect of the pons, middle cerebellar peduncle, and the cerebellopontine angle. In addition, there was mass effect with partial effacement on the fourth ventricle, left deviation of the pons with no herniation or hydrocephalus .T1 weighted images (T1WI), (T2 weighted images (T2WI), , , mildly (T2WI), , isointe(T2WI), , and min(T2WI), . Diffusi(T2WI),  and appa(T2WI),  map show(T2WI), . Based oPre-operative MRI of the whole spine did not demonstrate any drop metastasis. CSF analysis was also negative.Under general anesthesia, tumor debulking surgery was performed via a right suboccipital craniotomy. The specimen was sent for frozen and permanent pathology.The histological features were classically associated with C19MC alteration].lteration. These fUnfortunately, genetic amplification was not available at that time. Nevertheless, two independent histopathologists confirmed the morphological appearance consistent with the diagnosis of an ETMR, NOS (WHO Grade IV).C19MC amplification results in a diagnosis of ETMR, C19MC-altered., While its absence results in the diagnosis of ETMR- NOS. In our report, we document a new case of ETMR in a female infant who initially presented with right eye pain and exophthalmos, then developing gait imbalance and left-sided weakness. These findings were consistent with a posterior fossa lesion. As with other intracranial masses, the most common clinical features of ETMRs are symptoms and signs of increase intracranial pressure and focal neurological deficits. However, clinical features of ETMRs vary according to the tumor’s site and size, which may occur either above or below the tentorium in the cerebral hemispheres or in the cerebellum, brain stem, or even in the spinal cord. In our study, the tumor was in the right side of the pons and middle cerebellar peduncle with mass effect on the fourth ventricle.In 2016, the WHO classification of CNS tumors added molecular parameters to histology to define CNS tumors and incorporates new entities. ETMR includes the previously known embryonal tumors with abundant neuropil and true rosettes (ETANTR) that also referred to ETMR, ependymoblastoma, and, in some patients, medulloepithelioma. The presence of T1WIs with minimal heterogeneous enhancement after contrast administration, high signal intensity on T2WIs with no surrounding high signal intensity edema on FLAIR. It also demonstrated restricted diffusion on DWIs with low ADC value and slightly elevated rCBV on MR perfusion. These findings are consistent with previous studies that reported that ETMRs were frequently hypointense on T1WIs, hyperintense on T2WIs, with weak and heterogeneous enhancement, restricted diffusion, and high cerebral blood volume (CBV). The high rCBV at MR perfusion could be secondary to increased vascular permeability and increased vascular endothelial hyperplasia. Some authors reported more elevated choline at MRS in this type of tumor than in malignant gliomas, reflecting higher cellularity and mitotic activity in ETMRs.On MRI, our patient, the mass was low signal intensity on  In our patient, histopathology examination showed scattered multilayered rosettes with scattered reactive astrocytes. Govindan et al reported that the hallmark for histological diagnosis of ETMR is the presence of ependymoblastic rosettes and small undifferentiated cells. The immunohistochemistry in our patient showed that the tumor cells were positive for CD56 and synaptophysin. Wang et al reported that synaptophysin neuronal marker was positive in the differentiated areas of ETMRs, neurons with positive NeuN were found occasionally, and Ki-67 proliferative index was observed in up to 40–80% of rosettes and undifferentiated cells. Wang et al also reported that Nestin was a positive immunoreactive neural stem cell marker in ETMRs. Shih et al reported that amplification of C19MC and loss of INI-1 expression is necessary to define ETMRs. This is in line with our patient, which showed intact INI-1. The histological features in our patient are consistent with C19MC alteration.Multiple studies reviewed the immunohistochemical and genetic features of ETMRs. On this patient, the histological features were classically associated with C19MC alteration. Dangouloff-Ros et al reported that ETMRs could be suspected histopathologically by LIN28A-positive immunostaining, but this should be confirmed with molecular confirmation of C19MC alteration.C19MC locus at chromosome 19q13.41 miRNA cluster and enrichment of the LIN28A pluripotency factor. However, the pathogenesis and molecular landscape of ETMRs remains idiopathic. Wang et al reported that 19q13.42 amplifications were detected in 30% of ETMRs.ETMRs are highly aggressive brain tumors with amplification of the  Bailey et al reported that 131I-omburtamab is safe with a favorable therapeutic index in ETMRs when used following surgery and chemoradiation therapy.ETMRs have a poor prognosis even after total resection surgery due to CSF dissemination and metastasis. Typical treatment is trimodal with safe resection, adjuvant chemotherapy, and craniospinal irradiation except in children under 3 years old.This case report is limited because the genetic studies were unavailable, and the diagnosis was solely on the typical MRI features and histopathology results.ETMR is a rare, highly aggressive pediatric age group tumor that can occur throughout the brain with variable clinical features based on location. ETMRs demonstrate characteristic MRI features, and multilayered rosettes should be present on histopathology to diagnose it. C19MC amplification is required to diagnose C19MC altered type. However, if this amplification is not confirmed, ETMR-NOS should be the diagnosis.Awareness of the new WHO classification of the brain tumors among the radiologists should be raised.The new tumor classes are highly dependent on the genetic features.ETMR are highly aggressive tumors with typical CT and MRI features.Informed consent in writing was obtained from the child’s father for reporting and publishing the clinical records.


Post-surgical chylous ascites (CA) is extremely rare in colon surgery, known as the extravasation of creamy fluid rich in triglycerides accumulating in the peritoneal cavity. The incidence of CA after colorectal surgery remains between 1 and 6.5%. A 71-year-old female presented to the Emergency Department complaining of generalized abdominal pain, weakness and anorexia for several days ago and episodes of hematochezia which started the day before admission. Biopsy from colonoscopy revealed mucinous adenocarcinoma. Rectal mass resection with Hartmann’s procedure was performed due to obstructive recto-colonic mass. Pathology report confirmed pT4aN0M0 tumor invading through the visceral peritoneum. On post-operative Day number 4, drainage output increased, changing appearance to a whitish color. The diagnosis of CA was confirmed by obtaining >550 mg per dL of triglycerides. Changes to a high-protein, low-fat with medium chain fatty acids were made to her enteral diet. After 48 hours of diet adjustment, the drainage output of CA resolved. Chylous ascites (CA) is known as the extravasation of creamy fluid with a high composition of triglycerides, which accumulates in the peritoneal cavity . It is lA 71-year-old female with a past medical history of Diabetes Mellitus and Hypertension, presented to the Emergency Department complaining of multiple episodes of hematochezia, generalized abdominal pain, weakness and anorexia. Significant physical examination included diffused abdominal tenderness and a digital rectal exam evidencing hematochezia. Laboratory values were significant for hemoglobin 6.3, CEA 13.7 and albumin 2.9. CT scan revealed a large mass lesion within the lower pelvis, suggestive of a neoplastic process, measuring 7.6 × 6.3 cm in diameter. Colonoscopy revealed obstructive rectal mass identified at 10 cm from the anal verge . The patPathology confirmed a mucinous adenocarcinoma 9.3 cm in greater dimension moderately differentiated, inflammatory tumor invading through the visceral peritoneum with gross perforation. No regional lymph nodes for metastasis (0/12). Hemovac started draining milky secretions 4 days after surgical intervention . CA was Diet was adjusted to a low-fat diet based on high-protein content with medium-chain triglycerides via the enteral route. Drainage output reduced significantly within 2 days post-diet adjustment.Some abdominal surgical procedures may cause CA . CA is aIn addition, diagnosis can be more specifically confirmed through fluid sample of CA based on the distinct characteristic of the ascitic fluid’s milky appearance and a triglyceride level of >200 mg/dL, although some authors use a cut off value of >110 mg/dL .In our case during surgery, the tumor was invading the visceral peritoneum surface adjacent to the posterior uterus. Mesorectum dissection with continuous bipolar energy to fuse was difficult to perform due to the presence of inflammatory tissue; this might have resulted in damage to the lymphatic system and the milky appearance of her Hemovac output . Fluid t


Pelvic Organ Prolapse (POP) is defined as the descent of one or more of the pelvic organs from their normal position. This is commonly associated with multiparity, postmenopausal status, and obesity. Most of the cases present as uterine descent with or without cystocele, rectocele, or enterocele. But, the descent of pelvic organs in adolescent and young, nulliparous women is an uncommon presentation. We report a case of a 19-year-old girl with extreme elongation of the cervix without uterine descent. Uterus size was normal, no adnexal abnormality was there. The patient was apprehensive about her future fertility and pregnancy outcome.This is a rare case as it has not been reported in the preceding three decades of literature searches and poses a challenge in management decisions because we must consider future fertility while restoring normal anatomy.  Pelvic Organ Prolapse (POP), which is common in the elderly, has a 40% prevalence in women over the age of 45, with 11-20% requiring surgical intervention, and has a poor quality of life [The incidence of juvenile nulliparous uterine prolapse due to inherent congenital weakness of pelvic support is reported to be 1.5-2% . The inc [Cervical elongation is defined as the presence of cervical length >3.38 cm or cervix to corpus ratio > 0.79 . Ibeanu Uterus-sparing surgery such as the Manchester-Fothergill procedure (MFP) and the Sling procedure using a minimally invasive approach are preferred for reproductive-age POP patients and offer the potential for preserving fertility.A 19-year-old adolescent girl presented in the Gynaecology OPD with a complaint of something coming per vaginum for the last six months, with the size of protruded mass increasing during defecation. There was no history of sexual contact, lifting heavy weights, chronic cough or constipation. She attained menarche at the age of 13 years and her menses were regular, cyclical with average flow and duration. There was no associated urinary complaint.On examination, third-degree cervical descent was noted , cervix was hypertrophied and elongated, firm in consistency and cervical length was approximately 6 cm, and all the fornices were felt. The uterus size was found to be normal.MRI pelvis showed an elongated cervix with a length measuring 5.9 cm, bulging into the vagina inferiorly. The uterus was of normal size and in a normal position, and no abnormality in the adnexa was noted Figure .A cervical length of more than 33.8 mm is defined as cervical elongation and its pathophysiology is not yet clearly understood . A revieOld age, multiparity, congenital weakness of pelvic floor muscles, prolonged labour, instrumental vaginal deliveries, birth trauma, chronic cough, genetic factor, smoking, prior surgery, collagen disorders including myopathies are a few well-known aetiological factors of POP .Conservative management with ring pessary insertion may be considered if surgery is not possible or the pessary is ineffective in cases of uterocervical descent. There is no gold standard surgery to correct POP due to variable combinations of presentation. Different uterus preserving techniques using slings such as sacral cervicopexy or transvaginal sacrospinal fixation provide excellent repair and the possibility of childbearing . A uterus-preserving technique, the Manchester-Fothergill procedure (MFP), is being performed for more than a century. There are several advantages of MFP including preservation of pelvic integrity, minimal blood loss so lesser hospital stay and morbidity, and also less recurrence rate . UterineIn sling procedures, Indian gynaecologists had contributed to developing conservative surgical treatment options with preservation of reproductive functions such as Shirodkar’s Sling and vaginal prolapse operation, Purandare’s Cervicopexy including its modified cervicopexy, Khanna’s Sling operation, Soonawala’s Sling operation and Nadkarni’s Sleeve Excision Anastomosis for cervical elongation . ModifieA systematic review described no difference in outcome among various sling procedures and vaginal hysterectomy . With thA case series studied causes of primary infertility in 3520 patients, 17 patients had extra-long vaginal cervix measuring 4.0 cm or more. Twelve out of 17 patients were treated by amputation of the cervix and 9 (75%) had successful pregnancy within 18 months while the rest five patients remained infertile and were not operated . Manchester-Fothergill procedure is considered the fertility-sparing surgery in young women with extra-long cervix but the true incidence of successful pregnancy after the procedure is not known, probably due to the rarity of the condition. In our case, we had planned the Manchester-Fothergill procedure, but the patient is too young and apprehensive, so after having a detailed discussion, the patient and her family have decided to postpone any intervention till she marries.Congenital cervical elongation without uterine descent is an uncommon finding in an unmarried, nulliparous woman presenting as third-degree cervical descent, which creates apprehension for the patient and presents challenges for the Gynaecologist in deciding on the suitable uterine preserving surgeries. There should be a detailed discussion on future fertility issues and pregnancy outcomes before opting for the operative procedure in such patients.


Blastomycosis is a rare fungal infection that typically presents as a pulmonary infection. Systemic involvement of blastomycosis from the lungs commonly occurs in the skin and bones. Tracheal involvement is an unusual presentation of blastomycosis, which makes it a formidable diagnostic challenge. We herein report an unusual case of an 85-year-old man presenting with tracheal blastomycosis presenting as a primary tracheal tumor. We also highlight the challenges that were faced in the diagnosis of such an uncommon presentation. To the best of our knowledge, this is only the third occurrence of blastomycosis with tracheal involvement. Blastomycosis is an uncommon fungal infection that most commonly occurs after inhalation of fungal spores from soil . BlastomThe patient is an 85-year-old male with a past medical history of chronic obstructive pulmonary disease (COPD) requiring two liters of home oxygen and an extensive smoking history. He initially presented to the outpatient clinics after developing multiple episodes of epistaxis and coughing out blood for a two-week duration. Initial workup, including coagulation studies, complete blood count, basic metabolic profile, and chest x-ray, were all normal. He was referred to the otolaryngology clinic for further evaluation of epistaxis, and a nasal endoscopy was performed; inspection of the nasal cavity, sinuses, and larynx did not reveal any source of bleeding. After the preceding workup, the patient reported feeling blood coming out of his throat rather than the nasal cavity. He was then referred to the pulmonology clinic, and a decision was made to proceed with bronchoscopy to investigate a suspected pulmonary source of bleeding. A bronchoscopy was performed and revealed a 2 cm × 4 cm mid-tracheal bleeding tumor in the posterior tracheal wall that was friable and bleeding with contact. Given the friability of the lesion at that time, it was not biopsied due to a fear of excessive bleeding. A second bronchoscopy was performed that revealed the same 2 cm × 4 cm bleeding tumor in the mid-trachea Figure , multiplA biopsy of the tracheal lesion was taken without significant bleeding and sent to the pathology lab. Nearly one week after the second bronchoscopy, the patient started to develop shortness of breath, cough, and fever that were gradually increasing in intensity. Given the patient’s extensive smoking history and the concerning appearance of his tracheal tumor, we had a very high suspicion of malignancy and decided to order a positron emission tomography/computed tomography (PET/CT) to investigate for possible metastasis. PET/CT showed multiple enhancing pulmonary lesions in the left lobe of the lung and an enhancing lesion in the mid-trachea Figures , 3, 4.Following the PET/CT scan, the biopsy results from the bronchoscopy came out and showed squamous epithelial inflammation and multiple fungi morphologically consistent with blastomycosis. Special stains, including Grocott’s methenamine silver stain, periodic acid-Schiff stain, and mucicarmine block C1 stain, were all used to confirm the presence of blastomycosis. A diagnosis of disseminated blastomycosis was given, and the patient was sent to the emergency department for hospital admission and further treatment. Upon admission, liposomal amphotericin B was started at a dose of 3 mg/kg every 24 hours for a total of two weeks, after which the patient stopped coughing out blood and his fever resolved. He was discharged from the hospital and followed up with the infectious disease outpatient clinic, where oral itraconazole was started at a dose of 200 mg twice daily for a total of 12 months for disseminated blastomycosis, which the patient is still completing. A follow-up CT scan of the chest was performed after two months of discharge and showed significant improvement (but not resolution) of the pulmonary lesions previously detected by PET/CT. The patient is still on antifungal treatment and is being monitored by the infectious disease outpatient clinic.Blastomycosis is a rare infection that most commonly affects the lungs; direct inhalation of conidia from the soil is the primary route to developing an infection. In symptomatic cases, up to 40% of patients will develop extrapulmonary symptoms . AlthougIn the previous two reports of tracheal blastomycosis, patients presented with symptoms of a pulmonary infection, including fever, sputum production, and cough ,7. AlthoThis case report presents a rare case of blastomycosis presenting as a primary tracheal tumor. To our knowledge, this is the third reported case of tracheal involvement in blastomycosis. Clinicians need to acknowledge that blastomycosis may have atypical presentations, especially when dissemination occurs. A very high suspicion is required to diagnose such cases, especially in highly endemic areas of the United States.


This report describes the first potential case of seeding after fine-needle aspiration (FNA) of a rib osteosarcoma in a dog. An 8-year-old, 28-kg female spayed Golden Retriever was presented to her primary veterinarian with a 3-week history of a 3-cm firm, unpainful, immobile mass arising from the 9th rib. The mass was aspirated and submitted for cytological examination. A subcutaneous nodule developed several days after the FNA was performed in a location immediately overlying but distinct from the primary rib tumor on palpation. Both the primary mass and the newly diagnosed subcutaneous nodule were biopsied and were consistent with an osteosarcoma. Although it cannot be ruled out that the subcutaneous lesion was metastatic, seeding was a reasonable explanation based on where the new mass was located and how quickly it appeared after the FNA was performed. The aim of this case report was to describe the possibility of tumor seeding during FNA for osteosarcoma. It is the authors' opinion that utility of cytological diagnosis of bone tumors outweighs the risk of possible seeding and should continue to be used as a routine diagnostic test for the diagnosis of aggressive bone lesions. Osteosarcoma (OSA) is an aggressive tumor accounting for 85% of primary bone tumors in dogs . This tyvia ultrasound guidance has been described and may increase the ability of obtaining a diagnostic sample (A preoperative diagnosis of bone tumors can be obtained by performing fine-needle aspiration (FNA) or incisional biopsy of the lesion. Collecting an FNA sample c sample . While nc sample –19, to tAn 8-year-old, 28-kg female spayed Golden Retriever was presented to her primary veterinarian with a 3-week history of a 3-cm firm, unpainful, immobile, and ill-defined mass on the left lateral thorax. No other abnormalities were noted on physical examination. Radiographs of the thorax were obtained to identify the origin of the mass and revealed an expansile, lytic, and proliferative bone lesion arising from the 9th rib. A blind FNA sample was collected for cytology and evaluated by a veterinary clinical pathologist. The pathological description was consistent with a malignant neoplasia with primary differentials including osteosarcoma, chondrosarcoma, and plasma cell tumor. Three-view thoracic radiographs revealed no evidence of pulmonary metastatic disease. The dog was then referred to a specialty hospital for further diagnostics and discussion of treatment options.via blind FNA and showed the presence of a malignant neoplasia with certain cellular features suspicious for osteoblasts in both samples, but other tumor types such as histiocytic sarcoma and hemangiosarcoma could not be ruled out. A biopsy for histopathology was recommended.Three weeks following the original presentation, the dog was assessed in our institution. On physical examination, a new 1-cm erythematous, freely movable, and subcutaneous nodule located directly over the rib mass was noted. According to the owner, the nodule developed a few days after the FNA sample of the mass was obtained. The size of the rib mass had also increased from 3 to 5 cm. The remainder of the physical examination was otherwise unremarkable. Bloodwork was performed with biochemical analysis showing a mild elevation in alkaline phosphatase of 153 IU/L (15–140 IU/L), with no other abnormalities, and complete blood cell count was within normal limits. Cytology of both the subcutaneous nodule and the rib mass was performed Thoracic radiographs were offered to the client for staging purposes but were declined at this time because of a preference to have them performed by the primary veterinarian. Flouride-18 fludeoxy glucose positron emission tomography and computed tomography (18F-FDG PET/CT)  was also offered to more thoroughly stage the patient but was declined for financial reasons. Three-view thoracic radiographs were obtained the following day by the primary veterinarian. The images did not show evidence of pulmonary metastatic disease, intrathoracic lymphadenopathy, or mediastinal masses.max) 16.3] centered around the left 9th rib. An avid subcutaneous mass separated from the primary rib lesion was identified (max 7.4), a very avid left sternal lymph node (SUVmax 9.8) compared to the right (SUVmax 1.9), and many small soft tissue pulmonary nodules throughout the pulmonary parenchyma were identified. The remainder of the study found no significant abnormalities. During the same anesthetic event, Tru-cut biopsies with a 16-gauge needle were obtained from the mass on the 9th rib, as well as the associated subcutaneous nodule. No aspirations of the other abnormalities noted on imaging were performed. The histopathology of the subcutaneous nodule showed haired skin and subcutaneous adipose tissue with an inner neoplastic osseous core . The dog also had a new onset of exercise intolerance. A total body F-18FDG PET/CT was performed after injection of 5 microcuries of 18F-FDG. The study showed a large, aggressive, osteolytic, and osteoproductive mass, with high avidity [degree of uptake of 18F-FDG measured as maximum standardized uptake value (SUVentified . Additioous core . Cells iDue to the advanced stage of the tumor, palliative radiation therapy with or without the addition of chemotherapy was offered to the client but was declined. The patient was maintained on NSAIDs for pain control. Two weeks following the last appointment, the patient was humanely euthanized due to rapid progression of clinical signs causing decreased quality of life combined with a grave prognosis.Cytology is a practical and useful technique for obtaining a definitive diagnosis of many tumor types in small animals . Bone tuThe subcutaneous lesion identified in this patient cannot be ruled out as metastasis from the primary rib lesion. However, seeding was thought to be a reasonable explanation, since the lesion appeared a few days after the FNA was performed and was in a location immediately overlying but distinct from the primary rib tumor. This theory is supported by the findings from a case report where seeding was detected 2 weeks after obtaining an FNA sample in a cat with a lung carcinoma . Needle The possibility of seeding after core biopsies using Tru-cut needles in humans with sarcomas has been briefly described in case reports and small case series, but the low sample size and limited patient follow-up do not allow for definitive conclusions on the true frequency of this event , 28. OneThe aim of this case report was to describe, for the first time in veterinary medicine, the possibility of tumor seeding during FNA for OSA. It is the authors' opinion that utility of cytological diagnosis of bone tumors outweighs the risk of possible seeding and should continue to be used as a routine diagnostic test for the diagnosis of aggressive bone lesions.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Ethical review and approval was not required for the animal study because no experimental procedures were performed. Written informed consent for participation was not obtained from the owners because no experimental procedures were performed. The owner consented to all diagnostics performed.TF wrote the first draft. GT, BS, DW, and MG managed the patient and contributed to conception of the case report. All authors contributed to manuscript revision, read, and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Internal hernias through the foramen of Winslow are considered as rare events. A 66-year-old female patient presented to our emergency department with epigastric and right upper quadrant pain associated with abdominal distention, nausea and vomiting and signs of shock. A computed tomography scan showed bowel strangulation with distended loops identified within the lesser sac. The diagnosis was confirmed by a midline laparotomy procedure. The ileum, the caecum and the ascending colon were found to herniate into the foramen of Winslow. A right hemicolectomy with a resection of the necrotic segment was performed. The patient recovered well postoperatively and was discharged on 10th day after surgery. The diagnosis and management of this rare disease remains challenging. Cross-sectional imaging with reconstruction is considered as the diagnostic modality of choice. Moreover, right hemicolectomy is a preferred procedure in order to decrease the rate of recurrence. Internal hernias are protrusions of the abdominal viscera through a pre-existing intraperitoneal orifice. They are a common cause of acute intestinal obstruction with an ischemic component by vascular strangulation . They ha3 and C-reactive protein (CRP) of 500 mg/l and procalcitonin of 96 ng/ml. Her lactate on venous blood gas sampling doubled within an hour from 1.64 to 2.3 mmol/l. An urgent abdominal computed tomography (CT) was performed and revealed a dilated loop of bowel within the left upper quadrant and the lesser sac through the foramen of Winslow  22 000 elem/mm Winslow . Further Winslow . There w Winslow .MCTD) and died 2 months later from a severe pneumonia.Our patient underwent an emergency laparotomy and the internal hernia was reduced manually after coloepiploic detachment. The herniated ileum and colon was found to be ischemic and was subsequently resected  and a riFWHs are unknown, which may delay the diagnosis. This life-threatening surgical emergency has a rapid evolution and can lead to strangulation, gangrene of the bowel and peritonitis , 4. Few The most reported mechanisms implicated in the FWH pathogenesis are changes in the intra-abdominal pressure by pregnancy, immediate postprandial periods or overeating, distal obstructive lesions of the colon, in addition to, excessive viscera mobility with a ‘wandering cecum’ found at 10–20% of people, an abnormally long small bowel mesentery, the defects of the gastrohepatic ligaments or cholecystectomy , 9, 10.Diagnosis of FWHs is made per-operatively in the majority of reported cases. Nevertheless, CT cross-sectional imaging with multiplanar reformation is considered the diagnostic modality of choice , 9, 10. Regarding the management of FWHs, the operative approach is the treatment of choice , 7, 11. et al. [Prophylactic measures to prevent recurrence are discussed such as the fixation of the hypermobile colon with cecopexy to the lateral abdominal wall, or, the closure of the foramen by simple stitches and mobilized great omentum , 11, 13.et al. , 14. To To sum up, there is a consensus throughout the literature that ileo-colic resection is a good surgical solution allowing easy reduction of the hernia and reliable prevention of recurrence.FWH is a rare type of internal hernia with flat belly occlusion that is largely ignored by physicians and radiologists, which leads to a delay in diagnosis and may be dangerous for an acutely ill patient. The advent of CT with multiplanar reformation aid in making an early diagnosis and an appropriate surgery before ischemia occurs. Therapeutic strategies still needs to be established through larger cohort studies, yet, a prompt intervention allows uneventful recovery and reduce the morbidity and mortality rates of this uncommon disease.All authors contributed toward data analysis, drafting and revising the paper, gave final approval of the version to be published and agree to be accountable for all aspects of the work.The authors declare no conflict of interest.The author(s) received no financial support for the research, authorship and/or publication of this article.


Schwannomas are the most common benign tumors of the peripheral nerves, but represents only 5%-8% of all soft tissue tumors. Their diagnosis is usually delayed due to their slow growth and the nervous adaptation to their increased volume. Ultrasound sonography and magnetic resonance imaging (MRI) images usually ease the diagnosis. Correct enucleation offers very good postoperative outcomes and avoids recurrences. We report an unusual case of schwannoma of the radial nerve (RN) that remained asymptomatic for one year and a half, and treated well, had good outcomes. Schwannomas are the most common benign tumors of the peripheral nerves, composed exclusively of Schwann cells derived from the neural crest. They are solitary in 90% of the cases, occasionally there can be multiple lesions or a lesion associated with neurofibromatosis. The diagnosis is usually delayed due to their slow growth and the nervous adaptation to their increased volume . We repoPatient information: a 20-year-old male patient, non-smoker, non-alcoholic with no particular medical or family history.Clinical findings: the clinical examination found an ovoid tumefaction of the external face of the lower third of the left arm, measuring 5 cm in length, solid, freely mobile. Percussion over the mass produced Tinel's-like paresthesia in the radial nerve territory. The neurological examination showed preserved motor and sensory functions. The rest of the exam was normal.Timeline of current episode: the beginning of his disease goes back to a year and a half before his admission by the occurrence of a swelling of the external face of the lower third of the left arm gradually increasing in volume and causing dysesthesia on contact (electric shocks) with an evolution marked by motor discomfort after repeated movements of the limb.Diagnostic assessment: soft tissue ultrasound revealed a fusiform mass, homogeneous, well defined and hypoechoic resting on the biceps muscle with no sign of invasion or rupture of its fibers, measuring 27mm/30mm/19mm  are benign, well-encapsulated, slow-growing nerve sheath tumors composed exclusively of Schwann cells derived from the neural crest ,3. They The ultrasound sonography and MRI tests play an important role in guiding preoperative diagnoses, since they do not have 100% accuracy. On ultrasound, schwannomas are usually seen as homogeneous, well-defined hypoechoic masses, often ovoid, and can show the origin and relationship of the tumor with the affected nerve. MRI can provide useful information about morphological data on the tumor; however, it cannot provide dynamic information , MRI alsSurgical excision is the treatment of choice. Schwannomas are theoretically removable because of their eccentric, noninfiltrating growth, thus allowing their enucleation without or with only slight damage to fascicular structure ,13. SomeIn our case, it's a schwannoma located on the radial nerve occurring in a 20-year-old male patient with a delayed diagnosis to one year and a half, causing swelling and a Tinel's-like paresthesia, the diagnosis was made on ultrasound and MRI, as the diagnosis of these tumors is often delayed, and sometimes done too late when the neurological deficit is irreversible. Hence, the early and the correct enucleation without injuring nerve's fascicules has better clinical outcome and avoids postoperative sequels.Schwannomas are benign tumors, that have low incidence, slow growth and atypical symptoms often leading to misdiagnosis or delayed diagnosis, as in our reported case. Ultrasound sonography and MRI images usually ease the diagnosis. Correct enucleation offers very good postoperative outcomes and avoids recurrences. The patient in this case report had an excellent clinical outcome and no recurrence at 12 months follow-up.


Paragangliomas are highly vascularised and often heritable tumors derived from the paraganglia. They are typically discovered in the retroperitoneal space as well as the head and neck region but are rarely encountered in the liver parenchyma. We report a case of a primary functioning hepatic paraganglioma and provide an up-to-date literature review of patients with such tumors. We present a case of functioning paraganglioma in a 34-year-old female patient who suffered a solitary lesion in her left lateral lobe with symptoms of hypertension since pregnancy. She did not have any family history and her pre-pregnancy examination was negative. An abdominal CT imaging revealed a 6.5 × 5.7 cm liver lesion in segments II and III. Laboratory investigations identified elevation in plasma-free catecholamines. With sufficient preoperative preparation, the patient underwent laparoscopic left hemihepatectomy. Immunohistochemical staining revealed Syn (+) tumor cell nests surrounded by S-100 sustentacular cells (+), providing a definitive diagnosis of paraganglioma. The patient recovered uneventfully without signs of recurrence during a 1-year follow-up period. Our case demonstrates that primary refractory hypertension in pregnancy should be screened for paraganglioma through abdominal ultrasound and plasma free catecholamines. On the other hand, laparoscopic surgery is technically safe and feasible for the treatment of patients with hepatic paragangliomas in favorable locations. Paragangliomas (PGLs), also known as extra-adrenal pheochromocytomas, are tumors derived from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of the thorax, abdomen, and pelvis. Moreover, they also originate from parasympathetic ganglia located along the glossopharyngeal and vagal nerves in the neck and at the skull base . As rareThese tumors are frequently associated with an excessive secretion of catecholamines, predisposing patients to multiple endocrine abnormalities of migraine-like headache, sweating, palpitations, episodic or sustained hypertension, pallor, or postural hypotension . HoweverHere, we report a case of laparoscopic technique use in a 34-year-old woman who had hypertensive symptoms since pregnancy and was eventually diagnosed with primary functional hepatic paraganglioma. To our knowledge, there are no published cases of successful use of laparoscopy in a patient with hepatic functioning paraganglioma. Additionally, we performed a literature review on hepatic paraganglioma to update the clinical features of this rare disease. A 34-year-old female patient presented to our hospital with a solitary lesion in her left lateral lobe A, who suAbdominal enhanced computed tomography further revealed an irregularly shaped and poorly circumscribed lesion, measuring 6.5 × 5.7 cm in segments II and III of the liver with mild dilatation of intrahepatic bile duct. The single liver lesion was hypervascular and markedly enhanced in the early arterial phase, with slight washout in the portal phase B–D. The According to the anesthesiologist and endocrine consultations, an alpha-adrenergic receptor blocker  was used for 10 days preoperatively. In addition, a high-sodium diet and adequate rehydration were simultaneously performed to prevent unpredictable instability in blood pressure during surgery. After adequate preoperative preparation, a left hemihepatectomy was performed under laparoscopy depending mainly on the location of the tumor and the relatively mature laparoscopic technique. The lesion was completely encapsulated and had gross features of high vascularization from a serosal laparoscopic view A,B. In aAs the surgery progressed, it was noteworthy that a rapid decrease in blood pressure was present since the total dissection of the tumor. Postoperatively, the patient was transferred to the intensive care unit on a metaraminol infusion, and her blood pressure was stable at 113/70 mmHg. Grossly, the surgical specimen of paraganglioma displayed a solitary, fish-fleshy-like mass with a clear margin. Moreover, pathological examination of the surgical specimen indicated a solid tumor measuring 5.0 × 3.5 × 2.3 cm located in the left lateral lobe C,D. MicrThe incidence of paraganglioma was 0.33 cases per 100,000 people per year. Moreover, pheochromocytoma and paraganglioma in pregnancy is rare, occurring in only 0.007% of all pregnancies . Women gNevertheless, paragangliomas may be clinically silent and detected as incidentalomas. As a result, it is difficult to determine the correct diagnosis for hepatic paragangliomas, especially nonfunctioning paragangliomas. In the present case, refractory hypertension in a young woman without any family history since the beginning of pregnancy is noteworthy. On the other hand, preoperative biochemical testing showed elevated plasma norepinephrine, normetanephrine, and 3-methoxytyramine levels, which could be valuable in assisting the clinical diagnosis. Based on these clinical features, hepatic paraganglioma was suspected preoperatively. According to the patient’s negative pre-pregnancy examination, we simultaneously speculated that the tumor is stimulated by hormones, which allowed tumor growth and stimulated epinephrine production by tumor parenchymal cells during pregnancy ,14. ThusTo date, a total of 13 cases of hepatic paraganglioma have been reported by a thorough search of the PubMed database between 1995 and 2022 ,25,26,27According to an improved understanding of catecholamine metabolism, measurements of plasma-free metanephrines or urinary fractionated metanephrines are commonly recommended ,29. SevePer the present clinical guidelines, perioperative medical management, including alpha-adrenergic receptor blockade, a high-sodium diet, and fluid intake, was used to prevent perioperative cardiovascular complications and severe hypotension after tumor removal . ParticuSurgery should always be the preferred treatment for locoregional hepatic paraganglioma. The combination of tumor location and maturation in laparoscopic technology was a pivotal factor enabling us to perform the left hemihepatectomy. Open resection for paragangliomas was recommended, but laparoscopic resection can be performed for small, noninvasive paragangliomas . The lapIn clinical practice, the presence of these features in patients with paraganglioma and pheochromocytoma indicate a high probability of heredity, including a positive family history, multifocal, bilateral, and metastatic disease ,43,44. OIn conclusion, routine screening of abdominal ultrasound and detection of catecholamines are necessary in atypical pregnancy-induced hypertension. Additionally, our review provides the first case in the world with a functioning hepatic paraganglioma to be resected laparoscopically. We contemplate that the safety and effectiveness of laparoscopic approaches could be thoroughly evaluated and discreetly selected to achieve complete resection. Appropriate expertise including anesthesia, endocrinology, and intensive care is ideally suited to manage patients perioperatively. Furthermore, we should be aware of the presence of hepatic paraganglioma even in asymptomatic individuals, and adequately master perioperative management for this situation.


Ossifying fibromas are rare, benign, nonaggressive fibro-osseous lesions that manifest in the craniofacial region. Ossifying fibromas are benign tumors of bone, commonly involving the posterior dentate mandible in middle-aged individuals with a female predilection. Clinical manifestations are an asymptomatic expansion of the dentate mandible, with infrequent maxillary lesions. Benign fibro-osseous lesions of the maxilla exhibit similar histopathological findings. Cone-beam computed tomography scan plays an important role in diagnosing and understanding the invasiveness of this lesion. This case report describes an uncommon presentation of ossifying fibroma in the posterior maxilla in an adult male patient. Treatment consisted of surgical excision, and wound healing was uneventful during the one-year postoperative follow-up period. Fibro-osseous lesions are characterized by a normal bone being replaced by fibrous tissue forming a newly mineralized product. Lesions with osseous and fibrous components include ossifying fibroma (OF), cementifying fibroma, fibrous dysplasia, cemento-ossifying fibroma, and cemento-osseous dysplasia . Fibro-oA 22-year-old male patient reported to the Department of Oral and Maxillofacial Surgery complaining of pain and swelling in the left posterior region of his upper jaw for the past six months. The patient had difficulty in mastication due to the presence of swelling. On medical examination, the patient did not have any systemic problems. Extraoral examination revealed facial asymmetry due to the presence of mild swelling on the left middle third of the face. The skin overlying the swelling was normal. Intraoral examination revealed expansion of the maxillary jaw on the left posterior region, extending from the upper left second premolar to the left maxillary tuberosity region, measuring 4.5 × 3.5 cm in size Figure . Buccal The axial section of the cone-beam computed tomography (CBCT) scan revealed a unilocular, mixed (predominantly radiopaque) expansile lesion, with well-defined borders on the left posterior maxilla Figure . The corUnder general anesthesia, a vestibular incision was placed in the 22-27 region, the mucoperiosteal flap was raised, and the lesion was exposed Figure . SurgicaAlthough there are differences in classifying and diagnosing fibro-osseous lesions in the craniofacial region, certain clinical features are common to these lesions. These lesions demonstrate the replacement of normal bone tissue with fibroblasts, collagen fibers, with altering amounts of mineralized materials . PluripoOssifying fibroma has a female predilection and occurs predominantly in middle-aged individuals. The mandibular premolar-molar area is the most prevalent site, with only 30% of lesions occurring in the maxilla . Due to Radiography plays an important role in distinguishing OF from other fibro-osseous diseases of the jaw. Only a few lesions are radiolucent, while the vast majority appear as distinctly mixed density lesions (predominantly radiopaque), and it is determined by the maturity of mineralized contents. Few aggressive ossifying fibromas of the maxilla resemble ground-glass appearance . The typHistologically, OF is made up of fibrous tissue with varying degrees of mineralized material and cellularity. The bone may have osteoblastic rimming and spherical calcified deposits that are very acellular and resemble cementum. In fibrous dysplasia, the absence of coherent rimming of osteoblasts in the bony trabeculae is utilized to discern it from this lesion, which is more typically lined by plump osteoblasts .The composition of stroma in OF is highly cellular, reflecting the aggressive nature of the tumor, and necessitating thorough surgical excision to prevent local recurrence. Treatment of small lesions involves enucleation and curettage; however, larger lesions need radical resection . ConservThe maxillary OF is a very uncommon benign tumor. Because OF is clinically asymptomatic, esthetic and local dental issues are frequently the first signs. CBCT plays a vital role in diagnosing and understanding the invasiveness and extent of this lesion into the adjacent structures. Irrespective of the treatment modality employed, the elimination of the possible etiologic factor is of utmost importance. As OF is a well-circumscribed lesion, the majority of lesions should be treated by surgical excision in a conservative method to minimize facial deformity and loss of function. Radical resection is reserved for aggressive lesions invading adjacent structures. Thorough removal of the lesion is essential to prevent recurrences.


Ileosigmoid knotting is a rare case of intestinal obstruction that later leads to bowel necrosis. This is more common in males than females and seen more in areas with increased rates of sigmoid volvulus. The main clinical presentations are abdominal distention, abdominal pain and tenderness, vomiting, and obstipation. Definitive diagnosis is challenging due to its rarity and overlapping symptoms with other more common abdominal conditions. Delay in diagnosis and management can lead to peritonitis, necrosis of the bowel, sepsis, and eventually septic shock. Surgeons should consider ileosigmoid knotting in cases of acute abdomen, as it requires fast decision-making and intervention for a good prognosis. We present two cases of ileosigmoid knotting presenting with acute abdomen requiring emergent laparotomy with resection of necrotic bowel. Ileosigmoid knotting is a rare surgical emergency in which the ileum makes a knot with the sigmoid colon, causing a closed-loop intestinal obstruction and bowel necrosis . IleosigCase oneA 54-year-old male presented with a sudden onset of non-projectile, non-bilious vomiting and loss of appetite for two days, associated with abdominal distention. The review of other systems was unremarkable. On physical examination, he was sick-looking and afebrile. Vital signs were within normal limits. The patient was noted to have mild conjunctival pallor and skin tenting, but no pedal edema. The abdomen was mildly distended with tenderness in all four quadrants with rebound and guarding. Rectal examination revealed a swollen, non-tender, smooth, and rubbery prostate with no stool. Laboratory findings were significant for an elevated white blood cell count of 15.49 109/L. Sodium was decreased to 126 mmol/L (ref. 136-145 mmol/L) and chloride was 91 mmol/L (ref. 98-107 mmol/L). All other serum electrolytes were within normal limits. Abdominal ultrasound demonstrated dilated loops of bowel with reduced peristalsis . It is less commonly encountered among white populations but more commonly seen in African, Asian, and Middle Eastern countries. Common causes include a long sigmoid with a narrow pedicle, a long small bowel mesentery with a freely mobile small bowel, and consumption of a bulky diet in the presence of an empty small bowel. Meckel’s diverticulum, internal herniation, malrotations, and postoperative adhesions are other rare predisposing factors ,5. It isThe diagnosis of ileosigmoid knotting can be difficult preoperatively as there are no specific blood tests or radiographic imaging for the diagnosis. A computed tomography (CT) scan is the most preferred imaging modality. CT scan typically demonstrates a whirlwind sign with a median deviation of the cecum, the convergence of the left colon, and mesenteric vessels toward the whirlwind, with signs of ischemia ,8. Due tIleosigmoid knotting is a rare condition with a high mortality rate if not managed early. Timely intervention is required to avoid the development of peritonitis and gangrene. A high index of suspicion is necessary for patients who present with an acute abdomen, suspicious radiographic findings, and concerns for bowel ischemia and gangrene.


Colonic lipomas are rare, benign neoplasms, typically asymptomatic, and predominantly found incidentally during autopsy or routine surveillance. Symptomatic lesions are usually those greater than 2 cm in diameter while “giant” lesions are characterized as those over 4 cm. Presentations can vary from asymptomatic to more severe sequelae, including obstruction, gastrointestinal bleeding, or intussusception. Resection of these lesions has historically been restricted to large or symptomatic lesions. However, recent reports suggest lipomas may retain the ability to grow and can become symptomatic over time despite being inconsequential initially. This series provides a review of the clinical manifestations of colonic lipomas, radiographic characteristics, and a treatment recommendation for management of these lesions using minimally invasive surgical techniques whilst advocating for consideration of resection prior to the development of symptoms or more emergent complications. Colonic lipomas are rare, benign tumors composed of adipose tissue, with a peak prevalence of 4.4% , second Case 1A 71-year-old male with a history significant for benign prostatic hypertrophy and nephrolithiasis was referred for surgical evaluation after the identification of a giant colonic lipoma causing colocolonic intussusception of the sigmoid colon. The patient reported the recent development of colicky abdominal pain, bloating, and constipation, which prompted him to seek evaluation by his gastroenterologist. Previous colonoscopies had identified a colonic lipoma, but he never experienced symptoms. A review of systems revealed no pertinent findings, and he denied any family history of colon cancer. On examination, the patient’s abdomen was soft, non-tender, and non-distended. Prior to evaluation, an outpatient computed tomography (CT) scan was obtained, demonstrating a large, pedunculated mass in the sigmoid colon with possible intussusception. An urgent colonoscopy was performed, which demonstrated an ulcerated, pedunculated lesion with the head measuring 8 cm in diameter, neck 10 cm long, and the base occupying roughly 75% of the luminal circumference. No evidence of intussusception was observed endoscopically. Multiple attempts were made to resect the mass using an endoloop, but they were unsuccessful, as it was not possible to pass the endoloop over the head of the mass.In the setting of persistent symptoms, failed attempts at endoscopic resection, and concern for intussusception, the patient was taken for operative exploration. Intraoperatively, an area of intussusception was identified near the splenic flexure. With the diagnosis of colonic lipoma confirmed preoperatively, the decision was made to proceed with a limited resection of the mass and associated intussuscepted colon. A successful laparoscopic, segmental colectomy of the descending colon was performed with the creation of a stapled, isoperistaltic, side-to-side colocolonic anastomosis. Postoperatively, he recovered as expected and was successfully discharged on postoperative Day 2 following tolerance of an oral diet, oral analgesic medication, and return of bowel function. A pathologic review of the specimen identified a tan-yellow, ulcerated, partially infarcted, pedunculated submucosal lipoma. The head measured 6.8 x 4.7 x 4.5 cm and the stalk measured 6.0 x 3.6 x 3.0 cm with evidence of intussusception of the neighboring colonic tissue as well as 14 reactive lymph nodes  into a distal segment (intussuscipiens), leading to a telescoping of one segment into another. This pathology is of great concern, as it restricts perfusion to the involved segment of the bowel, which can result in ischemia, necrosis, or perforation. Colocolonic intussusception is regarded as especially concerning, as anatomical attachments of the colon are typically protective and approximately two-thirds of cases are resultant due to a primary malignancy . With thSurgical intervention has long been the standard of treatment for intussusception in adults. The traditional operation for colocolonic intussusception due to a giant colonic lipoma has been either a segmental colectomy or a formal hemicolectomy. These techniques enable adequate resection of the colon and associated lymphatic tissue while ensuring adequate margins are obtained when concerned for an underlying malignancy. As minimally invasive techniques have developed, the incorporation of laparoscopic and robotic approaches has become increasingly common, which is exemplified in our series of patients. However, some alternative approaches to consider include colotomy with enucleation and laparoscopic-assisted submucosal excision . These aEndoscopy remains the gold standard for the identification and diagnosis of colonic lipomas. Hallmark findings, such as the “tent sign,” “pillow sign,” and “naked fat sign” are diagnostic criteria for colonic lipomas ,16. HoweAside from determining the best technique, defining when and which lipomas merit intervention has been a topic of debate. Historically, the intervention has been driven by symptomatology and the size of the lesion. However, as surgical techniques continue to be refined, a more proactive approach to resection would likely help minimize detrimental complications and emergent presentations. Traditionally, the intervention has been limited to symptomatic lipomas, typically with a diameter > 2 cm, as the slow-growing nature of lipomas meant small, asymptomatic lesions had a low likelihood of becoming clinically relevant ,18. HoweIn conclusion, we present a series of giant colonic lipomas managed safely and effectively with minimally invasive surgical resection. As our series and other reports have demonstrated, colonic lipomas may retain the ability to grow and become symptomatic over time. It may thus be pragmatic to consider resection of colonic lipomas prior to the development of symptoms or more emergent complications.


Background: The apparent absence of a uterus upon imaging women with primary amenorrhea appears to lead to a high risk of misdiagnosis, which will lead to significant mental distress in patients. Case: Three young females with primary amenorrhea were referred with a diagnosis of Mayer–Rokitansky–Kuster–Hauser syndrome based on radiological findings of an apparently absent uterus. In two patients, the absence of the uterus could be confirmed, but with various diagnoses. The other patient had a normal but unstimulated uterus due to her hypoestrogenic state. Summary and Conclusion: The presented cases illustrate the broad differential diagnoses and the specific pitfalls of primary amenorrhea with an apparently absent uterus upon imaging. A well-established diagnosis was only possible through a thorough correlation of imaging findings with clinical history, biochemical findings and physical examination. Primary amenorrhea is a common problem that leads to severe distress in adolescents. Normal puberty starts with breast development at the age of eight to thirteen years. Menarche usually occurs two years after the first signs of puberty, at age eleven to fifteen years. Primary amenorrhea is defined as the absence of menarche by age fifteen or more than three years after the start of breast development . The cauUterus absence in otherwise healthy females with normal external genitalia can be caused by different conditions within differences in sex development (DSD) conditions. Therefore, a DSD condition, such as Müllerian agenesis, testosterone synthesis defect or complete androgen insensitivity syndrome (CAIS), should be considered as the underlying cause of primary amenorrhea.Pelvic ultrasound and magnetic resonance imaging (MRI) are good methods to visualize Müllerian structures ,4. When In this paper, we present three cases of young women who were referred to our hospital presenting with primary amenorrhea and an apparently absent uterus. Our aim is to point out the importance of correlating imaging findings with clinical history, biochemical findings and physical examination, and provide guidance for distinguishing the various diagnoses of primary amenorrhea with an absent uterus upon imaging. This is important because these cases are rare, but various medical practitioners could encounter them and misdiagnosis will lead to significant mental distress in patients.A seventeen-year-old girl was referred to the local gynecologist because of primary amenorrhea. Puberty had started with breast development and pubarche at the age of ten. She had used the oral contraceptive pill, but no withdrawal bleeding ever occurred. According to the patient, penetration during sexual intercourse was possible without pain. However, from a physical examination and vaginal ultrasound, a short and blind-ending vagina was found. The MRI suggested an absent uterus, normally located ovaries and normal kidneys . With thA seventeen-year-old girl presented with primary amenorrhea after a normal start to puberty. Breast development started at the age of twelve years and she had a growth spurt two years later. The physical examination showed the normal anatomy of the vulva and shaved pubic hair, and the vaginal depth upon digital palpation was 3–4 cm  and very sparse axillary and pubic hair. After the specialized radiologist reviewed the MRI, the previously described ovaries actually appeared as atypical gonadal structures with a large solid part, suspected of being (ovo)testicular tissue b. A hormA sixteen-year-old girl presented with delayed puberty and primary amenorrhea. Breast development started at age fourteen, but no further progression occurred after tanner stages 1–2. Axillary and pubic hair also developed up to tanner stage 2. There were no signs of virilization. She had a short stature and low weight . Bone agShe was referred to our expert center with a diagnosis of MRKHS. Ultrasound and MRI revision by our radiologist showed a small uterus with visible myo- and endometrial tissue, which was interpreted as an unstimulated hypoestrogenic uterus . A furthThis study presents three patients with primary amenorrhea, who were referred to our expert clinic with a (mis)diagnosis of MRKHS because of radiological findings of an apparently absent uterus. In two patients, the absence of the uterus could be confirmed, but with various diagnoses (MRKHS resp. CAIS). The other patient had a normal but unstimulated uterus. Our case report shows that the apparent absence of a uterus upon imaging in women with primary amenorrhea appears to lead to a high risk of misdiagnosis. The presented cases illustrate the broad range of diagnoses of primary amenorrhea and specific pitfalls. A well-established diagnosis was only possible through considering the combination of clinical history, physical examination, biochemical evaluation and assessment of imaging by an experienced radiologist.During the prenatal development of 46,XX individuals, the Müllerian ducts fuse in absence of AMH to form the uterovaginal canal. In syndromes with Müllerian agenesis, such as MRKHS, this process fails, resulting in the absence of the uterus and upper part of the vagina . In 46,XThe first step in the evaluation of primary amenorrhea is carefully looking through clinical history and physical examination. Women with MRKHS experience a normal start of puberty with age-appropriate breast development and pubarche, but primary amenorrhea as an isolated symptom . In CAISThus, assessing patient history and physical findings carefully an accurate diagnosis can be made. However, as the impact of these diagnoses is great, biochemical analysis, imaging or karyotype analysis is conducted to support this. A careful consideration of necessary diagnostics will prevent misinterpretation and misdiagnosis.LH, FSH, progesterone, estradiol, TSH and prolactin can guide in finding possible endocrine causes of primary amenorrhea . In respIn women with normal secondary sexual characteristics but primary amenorrhea, pelvic ultrasound is advised to search for possible anatomical causes ,3. In caIn women with delayed breast development, uterus imaging is not advised, since mostly estrogen deficiency will be the cause of primary amenorrhea. An important consideration is that, in the hypoestrogenic state, the uterus is small and unstimulated and can therefore be difficult to visualize. Berglund et al.  describeIn case of absent uterus upon imaging, a progestin challenge test  will proThe diagnosis of an absent uterus and vagina leads to a negative impact on the affected women’s level of psychological distress and self-esteem . AdditioThe presented cases illustrate the broad differential diagnosis and the specific pitfalls of primary amenorrhea with an apparently absent uterus upon imaging. It is important not to draw premature conclusions from findings of an apparently absent uterus from imaging alone. A well-established diagnosis was only possible with a thorough correlation of imaging findings with clinical history, biochemical findings and physical examination.


Superficial temporal artery (STA) aneurysms are very rare compared to vascular aneurysms of other regions. They are divided into two as true and pseudo. Pseudoaneurysm were much more common and often depend on an etiological factor but spontaneous true aneurysms are extremely uncommon and the etiologic causes are not clear yet.We present a 91-year-old female patient who consulted to us with swelling in front of the ear; there was no history of previous surgery or any trauma. The patient had a pulsatile mass in the preauricular region, which started 4 years ago and growed faster for the last 2 months.There was a mass consistent with a saccular type aneurysm whose continuity was observed with the temporal artery in imaging studies. The mass was excised under general anesthesia. The patient whose pathological examination was a true STA aneurysm was discussed in the light of the literature. Dear Editor, aneurysms of the superficial temporal artery (STA) are a scarce kind of aneurysms. The majority of this artery's aneurysms are pseudoaneurysms of posttraumatic origin, mostly due to blunt traumas, surgery, or even hair transplantation. Spontaneous true aneurysms are extremely uncommon,We present the case of a true STA aneurysm of 35 mm in diameter.Our case; caucasian female, 91 years old, presented with a pulsatile mass over the right preauricular area, which was gradually getting more prominent for the last four years but expanded faster over the previous two months, and the pain was added. There wasn't any history of trauma or surgical intervention.Examination revealed a 4 × 4 cm, partially mobile mass with pulsation superior to the right parotid gland. The skin over the mass was intact; no other aneurysms in any other region of the patient's body was found. There was no sign of inflammatory diseases, including several kinds of vasculitis.Doppler Ultrasound examination showed a saccular aneurysm with dense echogenic thrombus material inside next to the parotid gland. Continuity with temporal artery and blood flow through the mass was seen Surgical excision of the aneurysm after dissection and ligation of the STA proximal and distal through a preauricular skin incision under general anesthesia was done Aneurysms associated with STA are only less than 1% of all arterial aneurysms reported up to date.In the review made by Kawai et al., In 2014, only 28 true spontaneous aneurysms were found and the oldest patient was 85 years old [10]. Considering that a few cases are added to this number of cases nowadays, this is around 30–35 cases and it is quite low. However no case as old as our case has been reported so far.,Spontaneous aneurysms of STA mean that there shouldn't be any kind of trauma in the patient's history. These true aneurysms were usually related to atherosclerosis, congenital weakness of vascular layers, or chronic arteritis.There weren't any history of trauma, metabolic disorder, infection, or systemic illness in our patient.Most of the patients were presented with a pulsatile mass over the temporal area at the physical examination; some might be non-pulsatile due to thrombosis.Doppler USG is usually enough for diagnosis if there is a single and small aneurysm. If there are neurological symptoms, then detailed radiological evaluation of intracranial structures with angiography, computed tomography, or magnetic resonance imaging is indicated. The risk of stroke and neurological injury due to angiography must be kept in mind. These invasive techniques must be preserved for chosen patients with the probability of AVF or AVMs. Doppler USG may reveal native arteries with a fusiform dilatation, the presence of turbulent intraluminal blood flow, and partial or total thrombosis.Treatment options include interventional embolization or surgery. Expanding mass, pain, cosmetic issues, and degeneration of the skin or bone around the aneurysm indicate surgical excision.True aneurysms of the STA are rare disorders, but they must be kept in mind when a pulsatile or non-pulsatile mass is seen in the temporal region. Although it can be seen in all age groups, there are no elderly patients as much as our case in the literature.This research did not receive any specific grant from funding agencies in the public, commercial, or not for- profit sectors.No conflict of interest.This is a retrospective case report and required no ethical approval. An informed consent to publish the data and pictures was obtained from the patient.YO: Drafting the work or revising it critically for important intellectual content. UH, TS: Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. OEK: Final approval of the version to be published. All authors have critically reviewed and approved the final draft and are responsible for the content and similarity index of the manuscript.


A tailgut cyst (TGC) is a rare congenital lesion that occurs due to failure of involution of the distal hindgut, leading to the development of a mucus-secreting cyst. The clinical presentation is nonspecific, and often the diagnosis can be missed. We present the case of a 20-year-old female with a TGC in the perianal region. Surgical excision of the cyst was performed, followed by an uneventful recovery. The young age of our patient and the anatomical location of the TGC make our case a rare entity, highlighting the need for practicing surgeons to keep TGC as a differential in mind while examining masses in the perianal region. Tailgut cysts (TGCs) are rare congenital lesions that occur due to failure of involution of the distal part of the hindgut, which normally regresses in the embryonic period. This leads to the development of mucus-secreting cysts . These cA 20-year-old female patient presented to the outpatient department of Benazir Bhutto Hospital, Rawalpindi, with the complaint of something coming out of her anus since birth, which had increased in size over the past year. It was associated with mild, dull pain in the perianal region. The pain did not exacerbate on defecation. There was no associated history of fever, constipation, per-rectal bleeding, or any urinary complaints. Her past medical and surgical history and systemic inquiry were unremarkable. There was no family history of cancer, and the patient’s drug history was insignificant. On examination, 6 x 4 cm irreducible swelling was seen arising from the anal verge with its pedicle at the 6 o’clock position. The margins of the mass were regular with a smooth surface. The overlying skin was not attached to the swelling. There was no active bleed or discharge Figure .The MRI of the pelvis revealed a 5.9 x 5.5 x 3.1 cm circular cystic lesion arising just posterior to the external anal sphincter on the left side with an external mild mass effect on the left ischiorectal fossa and an intact interface. Inferiorly, it protruded out of the anal canal. The rest of the pelvic and abdominal vessels and viscera were normal on MRI. The differential diagnosis of this pedunculated anal swelling included anal polyp or anal lipoma Figure .Proctoscopy and sigmoidoscopy done during the preoperative planning demonstrated that the lesion lay externally around the anal canal, with no involvement of deeper structures. Therefore, examination under anesthesia (EUA) and excision was planned. Perioperatively, a broad-based, fluid-filled, pedunculated swelling was seen arising from the anal verge, 2 cm below the dentate line. Using a circumferential incision, skin and subcutaneous tissues were incised, and the pedicle was separated. After the lesion was excised, the structures were closed in reverse order Figure .On dividing the specimen into two halves, the lesion showed a large cyst measuring 4.5 cm in diameter and a smaller cyst measuring 2 cm in diameter; both filled with brown-colored amorphous material. The dissected specimen was labeled accordingly and sent for histopathology. Fluid for culture and sensitivity was also sent. The patient was discharged the next day on analgesia.The fluid culture report showed no growth of organisms. The histopathology sections revealed cystic lesions lined by stratified squamous keratinized epithelium with transitional-to-pseudostratified ciliated columnar epithelium. It was surrounded by focal areas of fibromuscular stroma showing infiltration by foamy histocytes, lymphocytes, and plasma cells. No evidence of malignancy was seen , or they may be detected incidentally during imaging. They are often large, measuring up to several centimeters in diameter . MicroscVarious imaging modalities can detect TGCs, including USG and CT. However, MRI remains the gold standard imaging technique for detecting and guiding surgical intervention . On MRI,There are no established guidelines for the management of TGCs. However, complete surgical removal has been recommended for these lesions due to the risk of infection and malignancy. The most common surgical approach used worldwide is open surgical excision using the posterior approach . PostopeThere is extremely limited data regarding the post-excision follow-up of these patients. Some case series have highlighted the risk of recurrence after surgical removal. However, such recurrence has been attributed to incomplete excision, and the actual recurrence rates are low ,4. Due tThere is a broad list of differentials for TGCs, and more often than not, these lesions are misdiagnosed. The possible list of differentials includes dermoid and epidermoid cyst, rectal duplication cyst, teratoma, chordoma, meningocele, and lipoma, to name a few ,5,9. In Our patient was initially misdiagnosed as having a perianal pedunculated lipoma, with the subsequent differential being external hemorrhoids. Looking at it in retrospect, the diagnosis should have been made preoperatively based on the multilocular cystic nature of the lesion and intraoperatively by the thick brownish fluid drained from the specimen. Grossi U et al. described these findings as being pathognomonic for perianal cysts, with all cases of perianal TGCs reported till date presenting similarly .Our findings are consistent with the published literature with respect to epidemiology, diagnostic difficulty, and management approach. However, there are certain unique aspects to our case. First, our patient had a TGC situated in the perianal region, with its origin 2 cm beneath the dentate line. Most of the TGCs reported in the literature are located in the retrorectal or presacral space , and to Due to the fact that perianal TGCs are an extremely rare entity, they are often not included in the list of differential diagnoses for perianal swellings. TGCs are often asymptomatic; however, the late diagnosis can be harmful due to the risk of malignant transformation in long-standing TGCs. Therefore, TGCs should always be considered for multilocular cystic swellings in the presacral and perianal regions in female patients, especially if they contain thick, brownish fluid on aspiration. The preferred management is complete surgical excision to avoid recurrence and postoperative histopathology to confirm the diagnosis and rule out neoplasia.