Hydatidosis or Echinococcosis is a parasitic disease caused by infection with the larval stage of Echinococcus granulosus. Primary isolated pancreatic hydatidosis is very rare even in countries where echinococcosis disease is highly endemic. The objective of this case report is to highlight this unusual and rare hydatid cyst presentation to avoid incorrect diagnosis and management. In our case, preoperative evaluation guided the diagnosis toward cystic pancreatic neoplasms, leading us to contemplate a radical surgical approach (Pancreaticoduodenectomy). Pancreatic hydatid cysts can be confused with cystic pancreatic neoplasms, it should always be considered as a differential diagnosis in endemic areas, to prevent misdiagnosis and inappropriate management. Hydatid disease is a parasitic disease caused by infection with the larval stage of Echinococcus granulosus. Theoretically, echinococcosis can involve any organ. The liver is the most common organ involved, followed by the lungs. These two organs account for 90% of cases of echinococcosis . PancreaHere, we present a case of a primary hydatid cyst located in the head of the pancreas causing dilatation of the main pancreatic duct.A 33-year-old male was referred to our Department of General Surgery, with a 3-month severe epigastric pain radiating to the back, associated with several episodes of green vomiting. Pain is episodic, relieved by painkillers, has no relation to food or movement. No history of changes in bowel habits or urine color. The patient reported a history of early satiety and an observed unmeasured weight loss. The past medical and surgical histories were irrelevant. Abdominal examination showed tenderness in the epigastric region, with no other signs. Laboratory tests including complete blood cell count, renal and liver function tests were within normal levels. Immunologic methods (ELISA) and indirect hemagglutination (IHA) assays were negative. Serum Amylase and CA 19-9 were within normal levels. Computed tomography (CT) scan revealed a multilocular cystic lesion in the head of the pancreas measuring 9.5 × 8.2 × 11 cm, causing external compression on the distal part of the CBD. The main pancreatic duct near the cystic mass is dilated measuring 0.5 cm. The gallbladder is distended  and 2.Percutaneous US-guided fine-needle aspiration was performed and the result revealed malignant cells. Such findings orient the diagnosis toward Intraductal Papillary Mucinous Neoplasm (IPMN) and Mucinous Cystic Neoplasm (MCN). The patient was admitted to the hospital, and an exploratory laparotomy was carried out both to diagnose the condition and to perform the necessary therapeutic procedure. Intraoperatively, a cystic structure was found, located in the head of the pancreas and was adhered to the superior mesenteric vein . The cysThe cystic fluid was aspirated, after that a scolicidal agent  was instilled into the cyst cavity, then it was re-aspirated. The cyst was then opened, followed by evacuation of the germinative membrane and daughter vesicles .Careful examination of the cyst showed no communication with the pancreatic duct. Partial cystectomy was performed along with omentoplasty. A drain was placed in the residual cavity. Histopathological examination confirmed the diagnosis of pancreatic hydatid cyst (PHC). The post-operative course was uneventful and the patient was discharged on oral Albendazole therapy for 4 months. The drainage tube was removed 5 days later. During 6 months of follow-up, the patient was in good condition with no complications or recurrences.Hydatid disease is a parasitic infestation by the tapeworm Echinococcus granulosus in its larval stage. Hydatidosis is more predominant in sheep-raising communities, where dogs have access to infected offal. Human infection occurs by ingesting food or water contaminated with infected-dog feces containing viable tapeworm eggs.PHCs, despite being rare, have been reported with an incidence of 0.14–2% , 4. TheyVarious imaging modalities are used in the diagnosis of hydatid disease such as ultrasonography, CT scan, MRI and endoscopic US. Accurate diagnosis cannot be established based on radiological findings alone, and this is especially true in pancreatic cystic lesions in which imaging features such as multilocular cysts, internal septations, calcifications and wall enhancement are encountered in both benign (hydatid cysts) and malignant . In this report, our patient showed worrisome radiological features such as dilatation of the MPD.Serological testing is a common method for the diagnosis of hydatid cysts. Enzyme-linked immunosorbent assay (ELISA) for echinococcal antigens results is positive in ~85% of infected patients with hepatic hydatidosis, whereas the seropositivity rate is 54% for PHC cases , 12. ELIThe occurrence of hydatid cysts in unusual abdominal sites presents a major diagnostic challenge, and this is particularly true for primary, isolated lesions with no lung or liver involvement. In addition to the previous features, our patient had alarming radiological findings (dilatation of the MPD), malignant result in the pre-operative FNA and negative serological testing. All of which were consistent with pancreatic cystic neoplasms. The preoperative FNA result represented a major dilemma in deciding whether to explore the cyst or not, as it carried the potential risk of peritoneal dissemination of viable neoplastic cells. However, the surgeon’s decision to obtain an intra-operative FNA of the cystic lesion was of great value. The outcome had an enormous impact on the course of management, by preventing major procedures with high morbidity and mortality rates such as Pancreaticoduodenectomy, as well as adopting a safer and less invasive surgical approach.While surgery remains the only definitive diagnostic and therapeutic tool in the management of PHC , 8, a vaPHC is a rare condition, often confused with cystic pancreatic neoplasms. Although PHC presents a unique challenge in pre-operative diagnosis, it should always be considered in the differential diagnosis of cystic pancreatic lesions from endemic areas. Awareness of this phenomenon helps prevent misdiagnosis and inappropriate treatment.


Small bowel adenocarcinoma (SBA) is a rare disease and presents with intermittent abdominal pain, weight loss, nausea, vomiting, and gastrointestinal bleeding. In cases with delayed diagnosis, intestinal obstruction or bowel perforation can also be observed. In our case, the patient presented with ileus after an operation that was diagnosed with SBA. After six cycles of chemotherapy, the patient went into complete remission. Small bowel adenocarcinoma (SBA) is a rare disease, the diagnosis of which remains challenging due to nonspecific symptoms that mimic Crohn's disease (CD). The predisposing diseases include Crohn's disease (8.7%), Lynch syndrome (6.9%), familial adenomatous polyposis 1.7%), celiac disease (1.7%), and Peutz-Jeghers syndrome (0.6%) [.7%, celiCertain vital suggestions in the CD guidelines can help to detect SBA. First, all newly diagnosed patients with CD should undergo small bowel assessments , and/or capsule endoscopy). Second, a patient with suspected clinical CD and a colonoscopy with normal findings should be evaluated with capsule endoscopy. Alternatively, cross-sectional imaging or device-assisted enteroscopy may be performed for endoscopic or histologic confirmation . MoreoveHerein, we reported a patient with insufficient histological and endoscopic evidence of CD and was treated with mesalazine rather than undergoing further evaluation. One year after diagnosis, she developed ileus and was diagnosed with SBA. We want to emphasize the importance of small bowel assessment, especially for unresponsive treatment and an inefficient diagnostic assessment.A 34-year-old woman was admitted to our emergency department with stomachache and vomiting. She was treated with mesalazine, as Crohn's disease was suspected for one year, without ulcers on her colonoscopy, and her terminal ileum biopsies were suspicious for inflammatory bowel disease Figure .It was not clearly understood why the patient with follow-up at an external centre was considered a Crohn's disease. Her vital parameters were normal, and her bowel sounds were hypoactive. Air fluid was observed on imaging, and computed tomography was compatible with the ileus. Thus, a surgeon was consulted, but an operation was not considered at this time. The patient was admitted to the gastroenterology clinic for the subileus. She was followed up without nasogastric drainage because she did not accept nasogastric tube insertion. During the follow-up period, complaints of vomiting and abdominal pain continued, and the patient could not eat entirely. No findings suggestive of paralytic ileus were found in her examinations. After ten days of observation, no clinical response was observed. CT was re-evaluated, a mass image was noticed Figure .The decision to perform surgery was made. There was a 2cm stricture at a 60cm distance from the ileocecal valve. The diameter of the bowel was 2cm proximal and 5cm distal to the stricture. A 4.5*2.5*1.5cm tumour that invaded the subserosa and lymphovascular invasion was also observed Figure , 4.The small intestine proximal to the tumour was dilated. Microscopically there was mucosal and submucosal ulceration and skip areas. No granulomata are identified figure .No loss of mismatch repair (MMR) protein expression was observed on pathological examination. Following surgery, Positron Emission Tomography - Computed Tomography (PET-CT) was performed, and focal metastases in liver segment five and two metastatic implants of 12mm and 13mm in size were observed in the left lower quadrant of the abdomen. From September 2020 to February 2021, she underwent six cycles of chemotherapy with 5-fluorouracil and oxaliplatin. After completion of chemotherapy PET-CT results were normal. Furthermore, in March 2021, a colonoscopy was performed. There was no evidence of Crohn's disease or malignancy.SBA represents 31%-40% of all small bowel cancer diagnoses. These primary tumors are located in the duodenum (60%), jejunum (25%-29%), and ileum (10%-13%) [The relationship between SBA and Crohn's disease remains unclear due to the statistical power of the published studies. In a recent French nationwide cohort study, the incidence of SBA was 0.235 per 1000 patient-years among patients with small bowel CD and 0.464 per 1000 patient-years among those with small-bowel CD lasting for more than eight years. Although there is a high relative risk, the absolute risk of developing small bowel cancer in patients with CD is low .Advanced imaging and endoscopic techniques can facilitate the early detection of SBA, but these are not recommended because they are not cost-effective for routine surveillance. A sacculated loop with asymmetrical thickening or benign fibrostenosis-like short-segment stenosis can be seen on computed tomography or magnetic resonance imaging . CapsuleWe report a case of a patient with SBA that presented with suspected CD but lacked significant diagnostic evidence. Further investigation led to the diagnosis of SBA, which was diagnosed at a late stage. We want to emphasize the importance of early small intestine assessment in patients who are unresponsive to treatment or when CD diagnoses are doubtful.


Hemangiomas are benign vascular tumors that most often affect the skin, mucous membranes, subcutaneous tissues, bone and on rare occasions muscles. In the head and neck region, the masseter and trapezius muscles are most often affected; the temporalis muscle involvement is extremely rare. It is a childhood pathology that rarely occurs in adults. We report a case of a cavernous hemangioma in a 37-year-old female. Through this case and in the light of literature we focus on the clinicopathological aspects of this tumor and the rarity of this location. Intramuscular hemangiomas are benign vascular neoplasms that represent less than 1% of all hemangiomas and which are often localized in the trunk and extremities. There are three types of hemangioma depending on the size of the affected vessel: capillary hemangioma, cavernous hemangioma, and compound hemangioma [A 37-year-old female, without significant personal or family medical history. The patient presents a swelling of the left temporal fossa that has been evolving for 5 years by gradually increasing in volume leading to a facial asymmetry. The physical examination revealed a soft, painless, non-pulsatile mass (4x2.5cm in size), without thrill, not increasing volume in the declivity position, fixed to the deep plane, mobile to the skin, without inflammatory signs or lymphadenopathy.Contrast-enhanced computed tomography was performed, showing a temporalis muscle mass that extends to the infra-temporal fossa, measuring 33x17x39mm, isodense, unencapsulated, with polylobed contours, that enhanced after contrast injection and respected the subcutaneous fat .The patient underwent surgery, where we performed a hemi-coronal incision. The mass was found in the temporalis muscle and was excised completely without damaging the frontalis branch of the facial nerve. The operating follow-ups were simple, with excellent functional and aesthetic results .Macroscopic mass examination showed a smooth, nodular tumor tissue measuring 5x2.5x1cm, with a purplish-brownish appearance. The histological study found a benign proliferation of dilated blood vessels with variable shape and size often cavernous that were trapped between muscle fibers; they are bordered by a well differentiated endothelium and supported by fibrous interstitial tissue in favor of cavernous hemangioma. Over a two-year period, no signs of recurrence were detected .Hemangiomas are benign tumors characterized by abnormal proliferation of blood vessels. Their intramuscular locations represent less than 1% of all hemangiomas. Only 14% are located at the head and neck region. Masseter (36%), trapezius 24%) and sternocleidomastoid muscles are the most affected, while temporal muscle involvement remains extremely rare. To our knowledge, only 29 cases have been reported in the literature. A slight female predominance was described with an average age of 33 years [4% and stThe etiopathogenesis of these tumors are unknown, yet there are several theories: congenital, hormonal factors and trauma. There are three types depending on the size of the vessel involved: capillary  most common with an estimated incidence of 68%, Cavernous 26%) (Large vessels) followed by compound types (6%). Cavernous hemangioma is most frequently found in the temporal muscle [6%  is the method of choice in defining the vascular nature of the tumor and providing further information on the exact extent of the tumor. Hemangiomas are generally isointense to muscle on T1-weighted images and hyperintense on T2-weighted images. Arteriography allows to identify the feeding vessels of the mass for a preoperative embolization if necessary , 5.Several therapeutic modalities are available ranging from simple observation, injection of sclerosing agents, corticosteroid treatment, radiotherapy, embolization  to reduce intraoperative bleeding, arriving at complete surgery excision which remains the method of choice in the definitive treatment of this tumor. In some cases of voluminous tumor, injection of sclerosing agents, corticosteroid treatment and radiotherapy, can be indicated as alternatives or adjuvants to surgery , 7. In oLocal recurrence is possible after an incomplete resection with rates estimated at 28% for the compound type, 20% for the capillary and 9% for the cavernous. Close and prolonged Clinical and radiological follow up are recommended for at least 2 years to ensure immediate diagnosis of eventual local recurrence [Cavernous hemangioma of the temporalis muscle is a benign and rare entity. The clinical presentation is unspecific. Imaging plays a very important role in the diagnosis, especially the MRI which remains the examination method of choice. Despite the multitude of therapeutic means, surgery retains its place in the definitive treatment of cavernous hemangiomas.


Neuroblastic tumors (NTs) include neuroblastoma, ganglioneuroblastoma, and ganglioneuroma (GN). They are very rare in adults. The Surveillance, Epidemiology, and End Results identified 144 patients ≥20 years old at diagnosis (6.1%) from 1973 to 2002. GNs account for 14% of all localized NT. Since 1957, a total of four cases of GN of the duodenum have been reported. We report a novel case of GN of the periampullary region in the duodenum in a 41-year-old man presenting with chronic upper gastrointestinal bleed. Given the rarity of GNs in this age group and the nonspecificity of radiological features, this diagnosis is often missed until histopathology is done. This may negatively affect the prognosis of an otherwise well-prognosticated disease. A 41-year-old man presented with complaints of passage of tarry black loose stool for the last 6 months. It was intermittent, well tolerated and with no postural hypotensive symptoms. It was associated with dull abdominal pain over the epigastrium which occurred before meals and relieved after taking food. There was no prior history of blood transfusion. There was no history of hematemesis, jaundice, abdominal pain, abdominal distension, hematochezia, fever, loss of weight, loss of appetite, altered bowel, and bladder habits. No history of previous gastrointestinal (GI) bleeds, peptic ulcer, and blood dyscrasia. No history of usage of anticoagulants, iron supplementation, or analgesics. Physical examination revealed pallor with no other localizing signs. Provisional diagnosis at this stage was peptic ulcer disease, variceal bleed, or malignancy.Laboratory evaluation showed hemoglobin of 8.1 g/dL. Other blood counts, liver function tests, renal function tests, and hematological parameters were within normal limits. Stool occult blood was detected. Ultrasonography showed a mildly prominent hepatomegaly with an otherwise normal study. Upper GI endoscopy and colonoscopy were normal. Side view endoscopy showed a large globular periampullary mass with smooth and erythematous overlying mucosa and bleeding point. There were no ulcer seen at the lesion site . ComputDue to the suspicion of malignant growth and need for biopsy, surgical options were explained to the patient. After informed consent, patient was taken up for duodenotomy, D1–D2 junction polypectomy, feeding jejunostomy, and cholecystectomy. Surgery was done under general anesthesia. We found a mass ∼2 cm, with a wide-based pedicle, arising from the medial wall of the D1–D2 junction . Drain Neuroblastic tumors (NTs) include neuroblastoma, ganglioneuroblastoma (nodular or intermixed [GNBN/GNBI]), and GN. These tumors originate from neural crest cells and range from immature, undifferentiated tumors to mature differentiated ones. According to the International Neuroblastoma Pathology Classification (INPC), GNBI and GN represent the mature end of a spectrum.NTs are very rare in adults. The Surveillance, Epidemiology, and End Results identified 144 patients ≥20 years old at diagnosis (6.1%) between 1973 and 2002 and 35 patients ≥60 years old at diagnosis (0.9%) between 1973 and 2010.GN is a well-differentiated benign tumor composed of mature ganglion cells and neurofibers. About 60% of GNs occur in <20 years of age.Radiological investigations are often inconclusive. Although larger and malignant lesion with lymph node involvement can be picked up in cross-sectional imaging such as CT scan, endoscopy may reveal a polypoid mass but in some instances like the above case, due to its small size, lesion may be missed on an upper GI endoscopy and require a side viewing endoscopy. Biopsy via endoscopy may be attempted leading to incomplete resection of the tumor with major residuals (>2 cm). Moreover, biopsy findings at this stage may be misleading as the tumor is submucosal in origin and endoscopic biopsy mostly takes mucosal sample.Treatment options include either local excision or pancreaticoduodenectomy. The latter is reserved for larger tumors where excision is not possible. Radiotherapy has no role in management.In histopathology, careful observation is required for ganglion cells and neurofibrillary background, as they are very important to rule out other close differentials of polypoidal lesions of the duodenum such as GIST, Brunner's gland hamartoma, adenocarcinoma, lymphoid, carcinoid, neuroendocrine tumor, and other NTs.Herein, we have reported the case to highlight the obstacles in coming to a diagnosis. Clinical presentation is often unclear and radiological investigations may be nonspecific leading to a diagnostic dilemma. I-MIBG scan or urinary catecholamines is not necessary, and hence, their value as a diagnostic determiner have diminished. What was novel about this case other than its extremely rare occurrence was the age of presentation, as GNs are usually found in <20 years of age. Therefore, in the adult population, such as the case presented here, an open mind is required to come to the diagnosis. Additionally, there is also a consensus to highlight the need for optimal surgical resection to ensure best prognosis. For surveillance assessment, we performed monthly follow-up with microscopic examination for stool occult blood and annual endoscopy which were normal. Hence, adequate surgical resection ensures less likelihood of recurrence in this tumor.


The cementoblastoma is a rare odontogenic tumor occurring in the mandibular molar and premolar of the patients in the second and third decades of life. Despite its typical benign behavior, this tumor may promote local destruction by perforating the cortical bone and displacing the mandibular canal. This case report shows a 31-year-old man with an aggressive cementoblastoma attached to the apex of the mandibular first molar. Cone-beam computed tomography revealed a hyperdense mass connected to the root of mandibular molar surrounded by a hypodense area. Multiplanar reconstructions showed rupture of buccal bone plate and tumor invasion of the mandibular canal roof. The surgical planning included enucleation of tumor with the first and second molars extractions and the diagnosis of cementoblastoma was confirmed by histopathology. This case report emphasizes the contribution of cone-beam computed tomography on diagnosis and appropriate surgical planning of the cementoblastoma. Key words:Cone-Beam computed tomography, odontogenic tumors, diagnosis. The cementoblastoma remains as a rare odontogenic tumor with less than 300 cases reported in the literature . This tuDespite of typical benign behavior, the cementoblastoma can also behave aggressively. The tumor may promote local destruction by expanding and perforating the adjacent cortical bone, leading to resorption of the surrounding teeth, and, less commonly, displacing the mandibular canal -4.A detailed three-dimensional analysis by cone-beam computed tomography (CBCT) of cementoblastoma might provide the determination of the tumor size, density, presence of root resorption, cortical bone expansion and perforation, in addition to the relationship of the tumor mass with the adjacent anatomic structures ,6. AccorThis case report shows a 31-year-old man with an aggressive cementoblastoma attached to the apex of the mandibular first molar and causing its resorption and perforation of the buccal bone plate. The importance of cone-beam computed tomography on diagnosis and appropriate surgical planning of the cementoblastoma is discussed.A 31-year-old man was referred to the Oral and Maxillofacial surgeon complaining about a persistent and spontaneous pain in the left mandibular first molar (tooth 36). Intraoral examination revealed a hard swelling in the buccal aspect of the referred tooth. The radiographic analysis showed endodontic treatment of the left mandibular first molar. A radiopaque mass was also attached to the apex of the tooth surrounded by radiolucent halo and extending to the mandibular second molar Fig. A. MultipThe cementoblastoma is a rare benign odontogenic tumor with clinical/radiographic and microscopic well established characteristics. This tumor has a high prevalence in the mandibular first molar root of the young patients in the second/third decade of life ,3,4. AllUntil the 1990s, prior to advent of computed tomography scan, most of the cementoblastomas were diagnosed based on radiographic findings, i.e. rounded radiopaque or mixed-density mass attached to the root of vital tooth causing radicular resorption and obliterating the periodontal ligament space ,6,12. InThe clinical diagnosis of cementoblastoma based in radiographic findings can be challenging. In early stage, the tumor can be confused with other benign lesions of inflammatory origin that occur in the dental apex such as focal sclerosing osteitis and osteomyelitis . ConsideDue to the continuous tumor growth associated with pain and expansion of bone cortical, our patient was referred to an Oral and Maxillofacial surgeon and a CBCT of the mandibular lesion was requested. The CBCT images obtained in different planes  and 3D reconstruction played a critical role on diagnosis of the cementoblastoma, in its extension in relation to adjacent mandibular second molar, root resorption, and in the detection of bone cortical rupture Fig. . As the Additionally, as observed in other cases reported of cementoblastoma ,7, the tThe histopathologic analysis of the present case showed distinctive and classical characteristics of the cementoblastoma, according to World Health Organization and described by others ,8,10,12.Concluding, this case report emphasizes that CBCT contributed for accurate diagnosis of the cementoblastoma enhancing the detailed view of the size, extension, density, the relationship of tumor with adjacent mandibular structures as well as for adequate surgical planning reducing the risk of tumor relapse.


Congenital hernias occur 70% on the right side, 25% on the left side, and approximately 5% bilaterally. The finding of a congenital Amyand's hernia is of interest, especially in patients who do not present risk factors associated with connective tissue disorders, ascitic conditions, fetal developmental disorders or any condition that increases abdominal pressure.Male patient, 6 months old, was brought to the pediatric surgery department due to a visible mass in the bilateral inguinal region, which protruded with crying. The parents report that he was a 36-week preterm, low birth weight, monochorionic monoamniotic twin with bilateral congenital inguinal hernia. An open herniorrhaphy was performed, showing a left communicating hydrocele with an indirect left inguinal hernia and right communicating hydrocele with indirect inguinal hernia containing cecal appendix with no signs of inflammation.The most common clinical presentation is the presence of a reducible or irreducible mass, erythema and/or inguino-scrotal edema, irritability manifested by crying and recurrent pain in older infants. This condition may be associated with cryptorchidism, intrauterine structural developmental disorder, and the presence of fistulas. Appendectomy and traditional hernia reduction are the most common surgical approach. The evolution of this condition is favorable with extremely low complication rates.Amyand's hernia in the neonate is a rare presenting condition, which frequently involves nearby structures with risk of inflammation, incarceration and perforation, so repair should be performed early. •Currently there are controversies in the approach of congenital inguinal hernias.•The overall outcome in the management of congenital hernia is favorable.•Traditional hernia management for congenital Amyand's hernia has good results. This case report followed the SCARE guidelines for its realization For the summary of clinical presentations, management and outcomes in neonates and infants with Amyand's hernia, a non-systematic search of the literature was performed in the PubMed database over a period of 16 years (2005–2021), with the key terms “Amyand's hernia” and “Neonate” and synonyms, together with the Boolean operator AND/OR, with the aim of gathering the largest number of related articles; finally obtaining 29 articles. Articles consisting of original studies, case reports, and case series were included. We excluded those articles that did not present data on clinical presentations, management and outcomes of Amyand's hernia, as well as those that did not have full text available. After the application of these criteria, 15 studies were finally included [4], [7]2Male patient, 6 months old, was brought to the pediatric surgery department due to a visible mass in the bilateral inguinal region, which protruded with crying. Parents report no changes in skin coloration or size changes over time in a resting condition. The parents also report that he was a 36-week preterm, low birth weight (2400 g), monochorionic monoamniotic twin with bilateral congenital inguinal hernia. During the first days of his birth, he developed bronchiolitis which was treated and completely resolved. During the mother's pregnancy there were no complications, it was a controlled pregnancy, the delivery was vaginal and the other twin did not present any morbidity or perinatal complication. Considering he was a patient at risk, the neonatology department decided to wait a few days to evaluate the management of the bilateral inguinal hernia. However, due to family issues, the parents did not attend until the sixth month after birth..Physical examination confirmed the presence of bilateral movable inguinal masses of semi-soft consistency, which protrudes with Valsalva manipses. Considering the history of the diagnosis of bilateral congenital inguinal hernia, it was decided to perform elective open herniorrhaphy, according to hospital protocol. This procedure is performed with high sac ligation, distal hydrocelectomy and appendectomy with invagination of the stump. During the operation, left communicating hydrocele with indirect left inguinal hernia, and right communicating hydrocele with indirect inguinal hernia containing cecal appendix with no signs of inflammation were evidenced .Fig. 1InThe patient underwent the postoperative period without complications and was discharged after 5 days. The patient returned to the outpatient clinic 15 days later, where the surgical wound with scar tissue was observed, with no signs of acute inflammation or secretions. The parents were satisfied with the approach and the results of the patient.3As reported in the literature, the average age at which surgical repair of Amyand's hernias is performed during the neonatal and infancy period ranges from 5 days after birth to 16 months The most commonly used imaging techniques for the evaluation of hernia are ultrasonography (which can demonstrate the presence of torsion or testicular ischemia) Of the cases evaluated, absolutely all had a satisfactory postoperative course without complications or recurrence Although in this case the approach was made at 6 months of age, in spite of having been diagnosed a few days after birth, there was no evidence of a perforated or inflamed cecal appendix, only the development of hydrocele. In contrast to the manifestations commonly reported in the literature, such as skin erythema, in the present case only the protrusion of the mass was evidenced by crying. The approach was exploratory laparotomy due to the lack of training in the use of laparoscopy in pediatric surgery in low- and middle-income countries, such Colombia. However, the postoperative course was satisfactory. More studies of the highest quality are needed to determine the factors associated with the development of complications and to establish a cut-off time for surgical repair in cases of Amyand's hernia in neonates and infants.As a limitation, due to organizational difficulties it was not possible to obtain more intraoperative photographs, nor was there access to the initial diagnostic images during the perinatal period, due to loss of this data by the parents. Finally, the parents understood her baby's condition and was satisfied with the approach and effort made by the medical team. In contrast to what is currently published in the literature, this manuscript summarizes clinical presentations, management and outcomes in neonates and infants with Amyand's hernia and describes the presentation of a rare case of bilateral congenital inguinal hernia associated with uncomplicated Amyand's hernia.4Amyand's hernia in the neonate is a rare presenting condition, which frequently involves nearby structures with risk of inflammation, incarceration and perforation, so repair should be performed early. Ultrasonography and abdominal radiography are the imaging techniques of choice to evaluate the inguinal and abdominal region, and to rule out any other differential diagnosis or associated complications such as bowel loop perforation, testicular torsion or hydrocele. Appendectomy and traditional reduction are approaches with a favorable yield, and the appendix should always be resected to avoid the development of appendicitis later on. The evolution of this condition presents extremely low complication rates.Non declared.Hospital exempts ethics approval for reported cases.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.All authors equally contributed to the analysis and writing of the manuscript.Not applicable.sabrinaemz25@gmail.comSabrina Rahman. Department of Public Health, Independent University-Bangladesh, Dhaka, Bangladesh. Not commissioned, externally peer-reviewed.The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


Pancreatic cancer is one of the leading causes of cancer mortality and one of the most lethal malignant neoplasms worldwide. It is known for its local tumor extension to the liver; other common sites include the lung, distant lymph nodes, and bone. Brain metastases are extremely rare and represent less than 0.6% of all brain metastases.We report the case of a 66-year-old Caucasian female known to have adenocarcinoma of the tail of the pancreas treated with chemotherapy. During follow-up, thoracoabdominal computed tomography scans did not reveal any residual tumor or any metastasis. Moreover, tumor markers were within normal limits. She presented to the emergency department of our institution following an episode of a generalized tonic–clonic seizure 5 years following the initial diagnosis. Brain magnetic resonance imaging revealed an expansive left frontal intraaxial lesion compatible with high-grade glioma. The patient underwent surgical treatment. Histological examination revealed pancreatic metastasis.Thought to be rare, metachronous cerebral pancreatic metastasis should be kept in mind in patients with pancreatic cancer. Early diagnosis and complete surgical resection play a key role in the survival of these patients. Pancreatic cancer is one of the leading causes of cancer mortality and one of the most lethal malignant neoplasms worldwide . PancreaPancreatic adenocarcinoma is known for its local tumor extension to the liver; other common sites include the lung, distant lymph nodes, and bone , 28. BraWe describe herein a case of late metachronous brain metastasis in a patient known for biologically and radiologically silent adenocarcinoma of the tail of the pancreas mimicking a high-grade primary brain tumor.This 66-year-old right-handed Caucasian female was diagnosed 5 years ago Fig.  with unrShe was initially treated with two cycles of FOLFIRINOX and 5-fluorouracil. Due to intolerance to this regimen, the treatment was switched to a combination of Abraxane and gemcitabine with a good oncologic response. She received a total of ten cycles. During the last oncologic evaluation, the CEA and CA19-9 were within normal limits. Of note, adjuvant radiotherapy was not given to the patient. On regular follow-ups 4 years later, thoracoabdominal computed tomography (CT) revealed no detectable tumor or any metastatic lesions Fig. .Fig. 2PoSix months after the last oncologic follow-up, she presented to our institution with an episode of generalized tonic–clonic seizure, which was treated with levetiracetam. Other than headaches, the clinical examination did not reveal any neurological deficit. Neurological and physical examinations were unremarkable. Cerebral magnetic resonance imaging (MRI) showed a 5-cm cortical and white matter mass in the left middle frontal gyrus Fig. . The masThe patient underwent left frontal craniotomy and surgical removal of the tumor. There was a dense, solid part as shown on the figures that was adhered on the dura without any areas of necrosis or any macroscopic evidence of thrombosed vessels.The postoperative course was uneventful. She remained stable with no neurological signs or symptoms. Histopathological analysis revealed cubic to cylindrical cells with focal marked atypia Fig. . ImmunohImmediate postoperative brain MRI revealed no complications Fig. .Fig. 5A Notably, during hospitalization, a repeat CA19-9 was abnormally high (221 kU/l). A repeat thoracoabdominal CT and positron emission tomography (PET)-CT revealed bilateral pulmonary micronodules without any fluorodeoxyglucose (FDG) uptake. It did not, however, reveal any recurrent pancreatic tumor or metastatic disease. She was discharged home on postoperative day 5 on Keppra. She thereafter received a total of 30 Gy of adjuvant radiotherapy on the surgical bed. At 6-month follow-up, she was doing well without any radiological signs of tumor recurrence.Pancreatic cancer, mostly adenocarcinoma, represents around 3% of all cancers diagnosed each year, affecting more frequently males older than 65 years , 23. Dueet al. reported only four cases of cerebral (0.32%) and three spinal metastasis (0.25%) [et al. reported a cohort of 2492 patients with pancreatic cancer undergoing surgical treatment where four patients (0.2%) suffered from cerebral metastasis [Approximately 59% of patients with unresectable pancreatic adenocarcinoma will develop distant metastasis . Forty p (0.25%) . Similar (0.25%) . Recentltastasis . This lotastasis , 19, liktastasis .et al., two patients presented with symptomatic brain metastasis 4–5 years following the initial diagnosis [The interval between initial diagnosis of pancreatic cancer and brain metastases varies between 2 months to more than 6 years . In the iagnosis . These tiagnosis .et al. reported that the cause of death was not due to the nervous system metastasis [et al., resection of distant cerebral metastases originating from pancreatic adenocarcinoma can result in a significant increase in survival in patients with well-controlled primary disease [The treatment of choice for cerebral brain metastases depends on the estimated prognosis and aim of treatment and may include surgical resection, whole-brain radiotherapy, and stereotactic radiosurgery , 14, 24.tastasis . However disease , 10. Las disease . In our MRI findings in patients with pancreatic cerebral metastasis usually revealed a single intraaxial, cyst-like lesion, with an area of necrosis, irregular ring enhancement, and perifocal edema , 19. MorThe incidence of brain metastases from pancreatic adenocarcinoma is currently expected to increase because of prolonged survival due to improved treatment protocols. All patients with suspicion of metastatic CNS lesions should have cerebral CT scan and MRI –14. NeurWe present a rare case of a late metachronous brain metastasis mimicking a high-grade glioma in a patient with a biologically and radiologically cured cancer of the tail of the pancreas. In our experience, surgical resection followed by adjuvant radiotherapy may be a good option in carefully selected patients. However, this remains an isolated case report, therefore further prospective studies need to be carried out. Lastly, clinicians should be aware of this rare entity, since its incidence may increase nowadays because of prolonged survival due to improved treatment regimens.


Caseous mitral annular calcification (CMAC) is a rare liquefactive variant of mitral annular calcification (MAC) and superficially mimics a cardiac vegetation or abscess. CMAC is viewed as a benign condition of MAC, while MAC has clinical implications for patients’ lives. Correctly diagnosing CMAC is essential in order to avoid unnecessary interventions, cardiac surgery or even psychological suffering for the patient.We report on 6 patients with suspected intra-cardiac masses of the mitral annulus that were referred to our institution for further clarification. A definitive diagnosis of CMAC was achieved by combining echocardiography (Echo), cardiac magnetic resonance imaging (MRI) and cardiac computed tomography (CT) for these patients. Echo assessed the mass itself and possible interactions with the mitral valve. MRI was useful in differentiating the tissue from other benign or malign neoplasms. CT revealed the typical structure of CMAC with a “soft” liquefied centre and an outer capsule with calcification.CMAC is a rare condition, and most clinicians and even radiologists are not familiar with it. CMAC can be mistaken for an intra-cardiac tumour, thombus, vegetation, or abscess. Non-invasive multimodality imaging  helps to establish a definitive diagnosis of CMAC and avoid unnecessary interventions especially in uncertain cases. Calcification of the mitral annulus (MAC) is a common finding particularly in elderly patients. MAC is associated with renal and cardiovascular morbidity . In cont9/l). The one male patient (case 6), however, had lived in a tuberculosis incidence area for several years for professional reasons. A tuberculin test was not conclusive due to vaccination in childhood. These uncertain cases therefore persuaded us to perform a successive three-step approach with Echo, MRI and CT for further clarification. The typical imaging characteristics of CMAC are illustrated as an example in Fig. Six out-patients were initially referred to our institution for further evaluation of echocardiographically detected intra-cardiac masses at the mitral annulus. The main clinical characteristics of the patients are summarised in Table First, CMAC typically appeared in Echo as a mostly spherical, static and echo-dense (i.e. hyper-echoic) mass with smooth borders in the posterior-lateral periannular region. It contained one or several central hypo-echoic areas Fig. A, B. CMASecond, the masses appeared larger in MRI than in Echo, but clear differentiation of the CMAC mass from the adjacent myocardium was impaired in the standard steady-state free precision “cine” sequences Fig. C. In conCT scans revealed the typical demarcation of the hyper-dense structure of the “amorphic” and avascular centre without contrast enhancement from the fibrotic capsule with small irregular calcifications Fig. E, F.Nevertheless, the systematic use of these three imaging modalities gave us not only a definitive diagnosis of CMAC, but also information about important “bystander” diagnoses. For example, due to high frailty in cases 1 and 4, CT played a key role in planning the transcatheter aortic valve replacement for severe aortic stenosis initially diagnosed by Echo Fig. A–C. FurtCMAC is a rare variant of the more common MAC . The mosCMAC is a rare condition, and most clinicians and even radiologists are unfamiliar with it. CMAC can be mistaken for an intra-cardiac tumour, vegetation, or abscess. Non-invasive multimodality imaging  in uncertain cases helps establish a definitive diagnosis of CMAC and avoid unnecessary interventions.


Appendicitis after colonoscopy is rare, with an estimated incidence of 3.8 cases per 10 000 colonoscopies. Herein, we report a 56-year-old female who visited the emergency department with a history of diffuse abdominal pain and nausea 8 h after a screening colonoscopy. Abdominal examination disclosed deep tenderness at Mc Burney point and positive Rovsign’s sign. Laboratory studies revealed elevated white blood cells and neutrophils (WBC 15.37 K/Ul and NEUT 86.5%) with normal C-reactive protein (5 mg/l). The initial diagnosis was acute appendicitis, which was confirmed by the ultrasonographic findings. The patient was admitted to the surgical department, and a laparoscopic appendectomy was performed. Post-colonoscopy appendicitis is increasingly recognized as a complication after colonoscopy in the last decade. Early recognition is vital in preventing morbidity and mortality. It may also be worthwhile to include appendicitis after colonoscopy as a possible complication during the consent before the procedure. Colonoscopy is usually performed to diagnose or correct a problem in the large or distal small intestine. Its clinical applications have significantly increased in recent years . IntestiPost-colonoscopy appendicitis is extremely infrequent, with a reported incidence of 3.8 cases per 10 000 colonoscopies . NowadayA 56-year-old female visited the emergency department with a history of diffuse abdominal pain and nausea 8 h after a screening colonoscopy. There was no history of diarrhea, constipation or vomiting. The patient was afebrile and reported no significant past medical or surgical history. In the morning, the patient got a screening colonoscopy. The preparation was excellent, requiring no cecal washing. The colonoscope was advanced to the cecum without great difficulty. It was not impacted against the appendiceal orifice. No biopsy or polypectomy was performed.Abdominal examination disclosed deep tenderness at Mc Burney point and positive Rovsign’s sign, whereas rebound tenderness in the right iliac fossa, psoas sign and obturator sign were all negative. Laboratory studies revealed elevated white blood cells and neutrophils (white blood cell count 15.37 K/Ul and neutrophils 86.5%) with normal C—reactive protein (5 mg/l). The initial diagnosis was acute appendicitis confirmed by the ultrasonographic findings: enlarged noncompressible blind tubular structure in the right iliac fossa (diameter 1 cm) periappendiceal fat stranding. No free fluid was found .The patient was admitted to the surgical department, and a laparoscopic appendectomy was performed. The appendix was identified, exposed and noticed to be enlarged, hardened but without inflammation of the cecum and distal ileum. There was no free fluid, mesenteric lymphadenopathy or Meckel’s diverticulum. Laparoscopic appendectomy was carried out using a harmonic scalpel . No antibiotics were administered post-operatively. The patient recovered uneventfully, and she was discharged after 2 days. The histological report evinced the diagnosis of acute appendicitis: a heavy focal infiltration of the mucosa by neutrophils that spread out of the appendix wall, especially in the serosa, focal edema, and congested vessels of medium and largesize.Colonoscopy is a widely used endoscopic procedure for diagnosing, surveillance or screening various colorectal diseases. Despite its rare complications, colonoscopy is regarded as a safe technique. Perforation is the most severe complication, with a reported incidence of 0.016–0.8% for diagnostic colonoscopy and up to 5% for therapeutic colonoscopy. Bleeding is another significant complication happening in 2.4 cases per 1000 colonoscopies. Other rare complications encompass post-polypectomy syndrome, splenic injury, small bowel perforation, appendicitis, cholecystitis, pancreatitis, cecal volvulus and mesenteric ischemia [Post-colonoscopy appendicitis is considered an extremely rare complication after colonoscopy. It was first described by Houghton and Aston in 1988 and is being cited with increasing frequency recently . The incThe exact mechanism by which colonoscopy induces appendicitis remains unclear. Several theories have been proposed, including pre-existing subclinical appendicitis, the introduction of a fecalith into the appendix, barotrauma resulting from increased airway pressure, direct trauma or exposure of the mucosa to the residual glutaraldehyde type solution for cleaning the endoscope , 8–11.The clinical manifestation of post-colonoscopy appendicitis is the same as common acute appendicitis. Patients usually experience pain in the right lower quadrant or diffuse abdominal pain with or without related symptoms like nausea, vomiting and fever. On physical examination, findings of fever, tachycardia and peritonism  should raise suspicion of abdominal sepsis. The main concern in all patients who experience pain after colonoscopy is perforation of the colon, primarily when polypectomy is carried out. Unfortunately, complications such as bowel perforation and post-polypectomy syndrome may mimic post-colonoscopy appendicitis , 8.Computed tomography is the imaging modality of choice for the diagnosis of post-colonoscopy appendicitis, as post-colonoscopy pain can be brought about by retained gas, colonic spasm or complications . UltrasoThe management of post-colonoscopy appendicitis is not different from common acute appendicitis. In recent years, laparoscopy has gained much popularity and has become the preferred approach for uncomplicated and complicated appendicitis . AdvantaK.T. studied conception and design, drafting of manuscript; P.T. studied conception and design, drafting of manuscript; S.K.S. did literature search and acquisition of data; C.K.S. did literature search and acquisition of data; K.M.N. performed analysis and interpretation of data; V.G. performed analysis and interpretation of data; G.G-P. did critical revision and K.V. did final approval of the version to be submitted.All the authors 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.


We present a case of an eight-month-old girl who was brought to the emergency department with bloody stools. An initial ultrasound reported a mass in the left iliac fossa that was further characterized by magnetic resonance imaging (MRI) as a hypervascular ovarian tumor. Prior to surgical resection of the tumor, abdominal arteriography with selective embolization and vessel occlusion was performed. Pathology reported epithelioid hemangioendothelioma of the left ovary. This condition has not been previously reported in girls. In this case report, we describe the ultrasound, MRI, and arteriographic findings with a histopathologic correlation of an adnexal tumor that is unknown in the pediatric female population until now. Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, originating from endothelial or pre-endothelial vascular cells  and mainAn eight-month-old girl patient with no personal medical history was transferred to the emergency department of a highly complex children's hospital in Medellín, Colombia, due to fever and “red currant jelly” stools. Initially, an ultrasound was performed, where a solid mass was observed in the left iliac fossa with heterogeneous echogenicity, without calcifications, and with significant central arterial and venous vascularization in color Doppler mode Figure .A magnetic resonance imaging with intravenous contrast was performed Figure , identifAlpha-fetoprotein (AFP) and human chorionic gonadotropin (B-HCG) were normal. Prior to surgical resection, abdominal arteriography was performed with selective embolization and occlusion of the vessels. Two vascular blushes originating in branches of the internal iliac artery and the left gonadal artery were observed irrigating the tumor Figure .A left salpingo-oophorectomy was performed with the removal of a solid mass Figure . The patEHE is a rare vascular tumor, originating from endothelial or pre-endothelial vascular cells  and mainIts involvement is more frequent in the lungs, liver, bones, and soft tissues, although it can occur in any tissue, and in 10% of cases, it can be multifocal. Involvement of the female genital organs, such as the clitoris, vulva, and ovaries, has been described in adult case reports, but no cases have been reported in the pediatric female genital system ,5,7. TheThe etiology is not yet clear. No clear predisposing factors have been described, although a possible association with trauma, radiation therapy, and hormonal factors has been reported . AnotherThe clinical and histological characteristics of EHE are intermediate between benign vascular tumors such as hemangiomas and malignant tumors such as angiosarcomas, although it is part of the malignant tumors according to the 2018 International Society for the Study of Vascular Anomalies (ISSVA) classification ,8.The definitive diagnosis is made with immunohistochemistry. The tumor is made up of endothelial cells with minimal nuclear pleomorphism, arranged in cords, short strands, or small nests, with minimal vascular differentiation. In the most aggressive cases, there is increased mitotic activity, significant atypia, focal cell excision, and necrosis. On immunohistochemical staining, tumor cells are positive for at least one vascular marker: CD31, CD34, factor VIII, vimentin, and Ulex europaeus agglutinin 1 (UEA-1); and most cases are negative for cytokeratin . CD31 has a sensitivity of 100% and a specificity of 62% for the diagnosis of EHE [The characteristics of ovarian EHE images are not well defined due to their rarity; only one case has been previously described in the literature of an adult woman with ultrasound findings of a solid tumor with heterogeneous echogenicity and venous and arterial vascularization in color Doppler mode. No previous description of computed tomography, magnetic resonance imaging, or arteriographic features has been performed .To date, ovarian EHE has not been described in the pediatric population. Due to the lack of knowledge of the characteristics of the image, the diagnosis is currently made by histological confirmation and immunohistochemical staining. However, in the presence of a solid adnexal hypervascular tumor, it is important to think of EHE as a possible differential diagnosis.The differential diagnosis includes multiple ovarian tumors such as hemangiomas, teratomas, dysgerminomas, choriocarcinomas, sex cord-stromal tumors, and superficial stromal epithelial tumors. Tumor markers and the age of the patient are very helpful in making a proper differential diagnosis . Mature EHE is usually indolent; however, there is a risk of recurrence or metastasis. In some cases, EHE remains stable for many years with further progression or has an aggressive clinical course from diagnosis ,3,5. OveEHE is a rare and low prevalence tumor. This is the first report of ovarian EHE in a pediatric patient. Imaging findings are poorly understood due to the rarity of the disease but it should be a differential diagnosis in patients with hypervascular ovarian tumors. Diagnostic images are essential for the characterization and stratification of the lesion. In this patient, arterial embolization prior to surgical resection decreased the risk of bleeding, facilitating the procedure and reducing complications.


Paraneoplastic or ectopic Cushing's syndrome (CS) is a rare cause of endogenous hypercortisolism. It is due to ectopic adrenocorticotropic hormone (ACTH) secretion and has been reported in association with a variety of neuroendocrine tumors such as small-cell lung carcinoma, carcinoid tumors, and medullary carcinoma of the thyroid. Paragangliomas (PGLs) are rare neuroendocrine tumors that can secrete catecholamines. Case reports and reports of ectopic ACTH secretion from metastatic PGLs causing CS are exceedingly rare. We present a case of a 38-year-old female, who presented with typical signs, symptoms, and complications of CS, secondary to a PGL with widespread metastases, which eventually led to her demise. Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors, collectively termed pheochromocytoma–paraganglioma (PPGL). PCCs usually arise from the adrenal medulla, whilst PGLs arise from chromaffin tissue outside of the adrenal medulla and can secrete neuropeptides and catecholamines so are also referred to as extra-adrenal PCC.Cushing's syndrome (CS) is a collection of signs and symptoms due to prolonged exposure to either endogenous or exogenous excess glucocorticoids (GCs) resulting in a disruption of the normal hypothalamic-pituitary-adrenal axis . CS is bEctopic CS is a rare cause of endogenous hypercortisolism accounting for 5–20% of all CS cases and 10–20% of ACTH-dependent CS patients [A 38-year-old female presented to hospital with a long-standing history of progressive abdominal pain and distension. Functional inquiry revealed complaints of amenorrhoea, worsening malaise, proximal muscle weakness, insomnia, weight gain, and persecutory delusions.She had a background history of Human Immunodeficiency Virus (HIV) infection, for which she was on second-line antiretroviral therapy (ARV)  due to previous virological failure, with no history of any prior opportunistic infections. In addition, she was diagnosed with hypertension and type 2 diabetes mellitus a few years prior, which was insufficiently controlled (glycated haemoglobin (HbA1 c) level of 7.6%) on admission. She had no micro- or macrovascular complications. Her medication included amlodipine and metformin; however, she was not on any medication for her mental health complaints. There was no history of headaches, palpitations, or hyperhidrosis. She denied any symptoms of sleep apnoea. She was a nonsmoker and denied any history of exogenous corticosteroid use . There was no family history of Cushing's syndrome/disease or pheochromocytoma.2. Physical examination revealed typical cushingoid features including moon facies, facial plethora, and prominent truncal, facial, and nuchal adiposity, as well as multiple areas of bruising. Abdominal distention was noted, with visible violaceous striae and no palpable masses. The rest of the clinical examination was normal.Her body weight and height were 183 pounds (83 kg) and 5 foot 4 inches (165 cm), respectively, with a body mass index of 30.5 kg/m3 with an undetectable viral load. Fasting blood glucose levels were elevated. Biochemical investigations revealed an elevated random serum cortisol level of 1118 nmol/L (133–537 nmol/L), with an inappropriately normal adrenocorticotrophic hormone (ACTH) level of 4.6 pmol/L (1.6–13.9 pmol/L) (Her latest CD4 lymphocyte count was 131 cells/mm pmol/L) . In addi pmol/L) . The resBased on her history, clinical examination, and investigations, a diagnosis of CS was made. To determine the underlying etiology, further imaging was carried out.Abdominal sonography showed hepatomegaly span of 188 mm) with multiple hypodense hepatic lesions suggestive of metastatic disease. The adrenal glands, pancreas, spleen, and kidneys were normal. In addition, the pituitary gland was normal on imaging. Contrast-enhanced computed tomography (CT) of the chest and abdomen confirmed the liver lesions and multiple lesions in the pancreas and lungs, with no significant abdominal or thoracic lymphadenopathy . Numerous tumor deposits were noted in the liver, with smaller deposits seen in the thyroid, heart (endocardium), liver, pancreas, spleen, left kidney, and peritoneal lymph nodes. The brain (pituitary) and adrenal glands were normal. Right kidney pyelonephritis was confirmed as the source of sepsis.On histology, all the metastatic tumor deposits, including the tumor in the left lung, were similar to the tumor in the liver. The liver section showed a nesting  pattern of cells within a prominent vascular network . The celImmunohistochemistry stained positive for chromogranin  and synaBased on the histological and immunohistochemistry findings, a diagnosis of metastatic paraganglioma with paraneoplastic phenomena resulting in CS was made. The cause of death was deemed as systemic sepsis.Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors, collectively termed pheochromocytoma-paraganglioma (PPGL). PGLs are grouped based on their origin in the parasympathetic or the sympathetic nervous system. Sympathetic PGLs are most commonly found in the para-aortic region of the abdomen, pelvis, and chest , 4. ParaThe most common sites for PGLs with catecholamine hypersecretion are localized in the abdomen and pelvis. The catecholamine secretion causes paroxysmal episodic hypertension, often accompanied by palpitations, headache and/or hyperhidrosis. Nonfunctional or silent PGLs most commonly manifest as abdominal pain or an incidentally found mass.25–40% of PPGLs have an underlying germline mutation, of which a mutation in the succinate dehydrogenase (SDH) family is most common. Patients with a mutation in the B subunit of SDH (SDHB) are more likely to have metastatic disease. Other frequent syndrome-associated PPGLs secondary to germline mutations include Von Hippel–Lindau disease (VHL), Multiple Endocrine Neoplasia 2 (MEN2), and Neurofibromatosis type 1 (NF1) .The histological features of PGLs are similar to those of adrenal PCCs. The tumor cells usually show chief cells arranged in nests, alveolar-like, and stereo-like structures surrounded by sustentacular cells partly or entirely with delicate fibrovascular stroma , although they may also grow in a diffuse pattern .Synaptophysin is an integral membrane protein of small synaptic vesicles in the brain and endocrine cells and is abundant in neuroendocrine cells of diffuse origin and tumor tissues with neuroendocrine function , 9. ChroCytokeratins are keratin proteins found in the intracytoplasmic cytoskeleton of epithelial tissue . They arThe combination of cytokeratin negativity and chromogranin and synaptophysin positivity on immunohistochemistry is required for a definitive diagnosis of PGLs.The natural history of PPGLs varies from a benign tumor cured with surgery to the development of rapidly progressive metastatic disease with approximately 10% of PCCs and 25% of PGLs being malignant . The oveOur patient's immunohistochemistry stained positive for both chromogranin and synaptophysin and negative for cytokeratin. The combination of Zellballen pattern on histology, a cytokeratin negative stain, and positive chromogranin and synaptophysin staining on immunohistochemistry confirm that our patient had a PGL. On morphological examination, all the tumor deposits in the various organs were identical. The finding of tumor deposit in sites where neuroendocrine tissue is not usually present confirmed the diagnosis of disseminated malignant PGL in our patient.CS is a rare endocrine condition with an estimated incidence of 2-3 cases per million people per year , 16. CS Whilst the clinical features of CS may be subtle, the metabolic abnormalities are more upfront. Hypokalemia may be present in up to 58% of cases . Other cThe exact biological mechanism of ectopic ACTH secretion is unknown . Whilst Prior to this case, only a few cases of ectopic ACTH secretion associated with PGLs have been described in the literature , 28. As In our case, biochemistry failed to demonstrate excess ACTH in serum. This may suggest that the tumor cells produced a small amount of ACTH, which stimulated the synthesis of cortisol. Excessive cortisol would then inhibit the secretion of ACTH in the pituitary as a negative feedback. A normal ACTH level in the face of an extremely elevated cortisol level is inappropriate and suggests ACTH secretion independent of the control of the hypothalamic-pituitary axis (HPA).In our patient, postmortem specimens failed to stain for ACTH. This may have been due to the severe necrosis and autolysis of the tumor. Normal pituitary and adrenal glands on autopsy , elevated serum cortisol, elevated 24-hour urinary free cortisol level and ACTH levels, and failure to suppress on a high-dose dexamethasone suppression test support the diagnosis of ectopic ACTH production. Immunostaining for CRH is not available in our setting, nor are we able to measure CRH levels in plasma.We present a 38-year-old female, with widespread metastatic PGL, with CS due to ectopic ACTH secretion. Whilst the diagnosis of CS was confirmed while the patient was alive, the diagnosis of metastatic PGL was made on postmortem as the patient demised whilst still being investigated. Autopsy findings showed multiple tumor deposits with histological examination revealing a Zellballen pattern in keeping with a PGL. Positive immunohistochemistry staining for chromogranin and synaptophysin combined with negative staining for cytokeratin sealed the diagnosis of a PGL. The nondemarcated, widespread autolytic degradation of the tissue is likely responsible for the loss of demonstrable hormone expression (ACTH staining).It is possible that our patient may have had an underlying genetic mutation predisposing her to develop metastatic PGL; however, due to her demise prior to the diagnosis; we were unable to offer her genetic testing. Her prognosis was poor in view of such widespread disease. The hypersecretion of cortisol may have resulted in hyperglycemia and suppression of her immune system, which predisposed her to severe infections, leading to her demise. Whilst her underling HIV may have contributed, we considered this unlikely due to her undetectable viral load.This case represents a very rare cause of ectopic CS, most likely caused by an ACTH-producing metastatic PGL.


A gastrocolonic fistula is a rare surgical presentation, typically in the setting of gastric or colonic malignancy. This report presents the first reported Australian case of a gastrocolonic fistula following upper gastrointestinal surgery.A middle-aged woman presented to emergency with a short history of severe abdominal pain, faeculent vomiting, profuse diarrhoea and weight loss. This was in the setting of a previous pylorus-preserving pancreaticoduodenectomy complicated by marginal ulceration, for which a distal gastrectomy and Roux-en-Y reconstruction was required.The rarity of gastrocolonic fistulae and non-specific presentation with diarrhoea, vomiting and weight loss can make the diagnosis challenging. The mainstay of management is surgical resection in both benign and malignant disease. A gastrocolonic fistula is a pathological communication between the stomach and the colon. It is most commonly seen in the setting of gastric or colonic malignancy, and in exceedingly rare cases, gastrocolonic fistulae can also develop as a complication following upper gastrointestinal surgery. Here we present the first Australian case of a gastrocolonic fistula following upper gastrointestinal surgery.A 48-year-old lady presented to the emergency department (ED) of a metropolitan tertiary hospital with a short history of severe abdominal pain, faeculent vomiting, profuse diarrhoea and weight loss. This was on the background of a complex surgical history characterized by a pylorus-preserving pancreaticoduodenectomy 6 years beforehand for a gastrinoma at the pancreatic head. This was complicated by recurrent ulceration at the duodenojejunostomy anastomosis for which the patient underwent a distal gastrectomy and Roux-en-Y reconstruction 1 year prior. Despite this aggressive surgical management, repeat endoscopy 6 months prior demonstrated persistent marginal ulceration at the gastrojejunostomy as well as development of a further jejunal ulcer despite strict adherence to a twice-daily regimen of high-dose proton-pump inhibitor as well as the addition of sucralfate. As such, the patient was planned for a total gastrectomy however this was delayed due to the COVID-19 pandemic and, as it would be, she presented to ED before this elective surgery could be performed. Her other past history was significant for Crohn’s disease, gastroesophageal reflux, Raynaud’s disease and a previous right hemithyroidectomy for a neuroendocrine tumour.On presentation to ED, the patient appeared unwell and was tachycardic with a heart rate of 122 bpm; other observations were within normal limits. Her abdomen was soft with generalized tenderness but no guarding or peritonism. A computed tomography (CT) scan of the abdomen and pelvis demonstrated diffuse mural thickening of the stomach and increased distension of the proximal small bowel, however no obstruction, collection or specific cause for the patient’s symptoms. She was admitted to the acute surgical unit for further investigation.A repeat CT scan was performed, this time with oral contrast, which revealed a large gastrocolonic fistula into the transverse colon . The patMacroscopically there was no suggestion of malignancy or mass lesions; instead, it was felt that the fistula was likely caused by the advanced ulceration previously demonstrated on gastroscopy. The patient was commenced on total parenteral nutrition to optimize her nutrition preoperatively, and then 1 week after admission was taken to the operating theatre for definitive surgical management. At laparotomy, an inflammatory mass involving the distal stomach, transverse colon and Roux limb of the previous Roux-en-Y reconstruction was found. An uncomplicated near completion gastrectomy and segmental colectomy was performed, with the partially resected Roux limb able to be mobilized sufficiently to allow for the near-total gastrectomy to be appropriately reconstructed to the alimentary canal. Histopathology of the resected tissue was consistent with chronic inflammation and ulceration, with no evidence of malignancy. The patient recovered well post-operatively and a gastrograffin meal did not show any suggestion of ongoing fistulous communication or anastomotic leak . She wasGastrocolonic fistulae form when a pathological communication develops between the stomach and colon, most commonly due to malignancy of either gastric or colonic origin. Benign causes include peptic ulcer disease, non-steroidal anti-inflammatory use, Crohn’s disease, chronic pancreatitis and, in rare cases, as a complication of upper gastrointestinal surgery . To our Classically gastrocolonic fistulae present with a triad of diarrhoea, feculent vomiting and weight loss, however this triad is only seen in ~30% of cases .The limited literature on gastrocolonic fistulae suggests barium enemas are the most accurate imaging modality for diagnosis, with a close to 100% detection rate . AlthougSurgical resection of the fistula tract is the mainstay of treatment for gastrocolonic fistulae. A margin of adjacent tissue is typically taken to allow for both disease-free margins and a decreased risk of recurrence in the settings of malignant and benign disease respectively , 3. In rThis is the first reported Australian case of a gastrocolonic fistula following upper gastrointestinal surgery. The rarity of these fistulae and non-specific presentation can make the diagnosis challenging, however the presence of feculent vomiting in the absence of intestinal obstruction should raise suspicion of a gastrocolonic fistula.


Melanosis peritonei is an unusual and uncommon condition characterized by pigment deposition in the peritoneum. It is usually associated with other conditions. During a right inguinal hernia open repair for an 86-year-old gentleman, widespread black spots were observed on the hernial sac. After appropriate histopathological and immunohistochemical studies, the diagnosis of melanosis peritonei was established. The past medical history of the patient included an endoscopic excision of a sigmoid polyp which was found to be an adenocarcinoma with <1mm clearance margin on histopathology. Being recognized as an extremely rare condition, melanosis peritonei should be differentiated from other conditions including metastatic melanoma. Melanosis Peritonei is an unusual condition characterized by focal or diffuse pigment deposition in the peritoneum . PreviouAn 86-year-old gentleman was referred from his general practitioner (GP) with a computed tomography (CT) proven large right inguinal hernia containing small bowel loops. His past medical history included hypertension and chronic obstructive pulmonary disease (COPD) for which he was on inhalers. Following the appropriate clinical examination and proper discussion, he was added to the waiting list for an elective open mesh repair.During the hernia repair, widespread black spots were noted on the hernial sac Figure . The herIt was noted that the patient was being followed up by the colorectal team because of a 25 mm distal sigmoid polyp which was endoscopically resected four months prior to his hernia procedure. Histopathological assessment of the polyp reported a moderately differentiated adenocarcinoma with a <1mm clearance margin. A computed tomography (CT) scan of his chest, abdomen, and pelvis was performed as part of the staging of his sigmoid cancerous polyp and this showed no evidence of lung, liver, or bony metastases.The colorectal multidisciplinary team earlier concluded that there was more than a 20% risk of having residual disease and that would favour the need for undertaking a sigmoid colectomy or high anterior resection depending on his fitness.Due to his age, comorbidities, and his baseline fitness, the patient eventually decided not to proceed with surgical resection. Therefore, a follow up was arranged in the form of regular carcinoembryonic antigen (CEA) levels, lower gastrointestinal (GI) endoscopy, and a repeat CT scan in six months.To our knowledge, only 17 cases of melanosis peritonei have been reported in the English literature to date , two witPeritoneal melanosis commonly affects females from 18 to 28 years of age, with only one male case reported . It alsoThe pathogenesis of this condition is still unclear . One theSeveral other theories were introduced. One theory stated that excessive migration of neural crest cells prior to the 10th week of development leads to the formation of pigmented mesothelial cells and dendritic melanocytes in the peritoneum . MoreoveThe main differential diagnosis of melanosis peritonei is metastatic melanoma which may present with the same picture but with a very poor prognosis ,16. AlthPeritoneal endometriosis is another differential diagnosis that should be considered. It is usually dark brown in colour and can be easily diagnosed histologically by the presence of endometrial glands, stroma, and hemosiderin-laden macrophages. Intraperitoneal spillage and spread of India ink from preoperative endoscopic tattooing can also be misinterpreted as peritoneal melanosis although it is of no pathological significance .In this paper, we present an extremely rare condition known as melanosis peritonei, which was incidentally found during an open inguinal hernia repair. Melanosis peritonei is almost exclusively associated with other conditions, and in our case, the patient was previously diagnosed with adenocarcinoma of the sigmoid colon.The pathogenesis of melanosis peritonei is still debatable. This could be attributed to the rarity of this condition, the limited number of studies performed, and the variable associated pathologies. Some argued that the cause could be melanin pigment disposition which was previously formed inside ovarian teratomas. Others speculated that it is linked to neural crest cells’ developmental errors.The main differential diagnosis is metastatic melanoma, which carries a far worse prognosis. Despite their gross resemblance, they can be differentiated on microscopic examination. Other differential diagnoses include peritoneal endometriosis and ink spillage during endoscopic tattooing.


Leydig cell tumors, most often benign, are a rare cause of isosexual gonadotropin-independent precocious puberty in boys due to secretion of testosterone. Very rarely do these tumors produce estrogen, causing gynecomastia. Testicular sparing surgery is the mainstay of treatment currently although radical orchidectomy was the choice in the past. Following surgery, clinical signs improve along with a revision of biochemical changes. Occasionally, it has been reported few children are progressed to gonadotropin-dependent precocious puberty (GDPP) after initial clinical and biochemical recovery. Gonadotropin receptor analogs have been successful on them to halt the progression of puberty, and growth hormone administration has been used to optimize the adult height. Here, we report a case of a 10-year-old boy who presented very late due to failure in recognition of features of puberty due to a Leydig cell tumor. Even though he underwent successful radical orchidectomy, just within 1 month following surgery, he entered GDPP in contrast to the published cases where it was earliest detected at 3 months. Leydig cell tumors (LCTs) are a rare cause of gonadotropin-independent precocious puberty in boys . OccasioA 10-year-old boy presented with incidental detection of testicular asymmetry by his father complaining the left testes being smaller than the right testes for an unknown duration. The parents did not have any other concerns. Direct questioning revealed that he has had secondary sexual characteristics for a longer period than expected but was assumed to be normal by his parents. He had not encountered any medical professional during this time and had missed the routine school medical inspection at the age of 9 years due to schools being closed following the COVID-19 pandemic. He has been the tallest in the class from preschool. Acne, body hair, and pubic hair have been there for more than 2 years and progressed throughout and deepening of voice for more than 1 year. He has experienced erections for the last 4 months. There were no concerns regarding his behavior.He had not had any features suggestive of raised intracranial pressure. There was no history of central nervous system infections including tuberculosis, head trauma, or exposure to cranial irradiation.He denied any abdominal pain or abdominal distension, recently increased skin pigmentation, or salt craving.His father has achieved puberty at the age of 14 years, and there is no family history of precocious puberty.On examination, his height was 160.5 cm which was well above the 95th centile and his weight was lying on the 90th centile. His height has exceeded the midparental height range shown in . He was The biochemical evaluation revealed a luteinizing hormone (LH) level of 0.01 IU/L and a follicular-stimulating hormone (FSH) level of 0.05 IU/L, both being suppressed to a greater degree. Beta-HCG, alpha-feto proteins, and dehydroepiandrosterone sulfate (DHEAS) were normal.Testosterone levels were extremely high (69.45 nmol/L), almost double the upper margin of an adult male reference range (9.7–38.14).X-ray bone age was 14–15 years according to the Greulich and Pyle method at the chronological age of 10 years.USS scrotum showed an enlarged right testis with a well-defined heterogeneous hypoechoic lesion measuring 3 × 1.8 cm. Multiple calcifications were seen within the lesion. Contrast-enhanced CT abdomen, pelvis, and scrotum further confirmed a well-localized testicular mass without any evidence of metastasis.The child underwent a right-sided orchidectomy. Testicular sparing surgery was not possible due to the size of the lesion. Histology revealed a macroscopy of a well-circumscribed solid tumor measuring 30 mm in diameter. On microscopic examination, there was nuclear atypia and bizarre nuclei, but no other features associated with malignant behavior, mitoses <3/10hpf, clear tumor margins, no necrosis, and no vascular invasion. The adjacent seminiferous tubules as well as the distant seminiferous tubules were prepubertal without evidence of spermatogenesis. The immunohistochemistry report was suggestive of a Leydig cell tumor.1 month following surgery, even though the serum testosterone level has come down to 12.69 nmol/l, hormonal assay revealed that the child has now entered gonadotropin-dependent puberty with an LH value of 10.61 IU/L and FSH 9.59 IU/L.As the bone age was already advanced, it was decided not to proceed with GnRH analogs.α-hydroxyprogesterone, and sometimes estrogen either by peripheral aromatization of testosterone or else directly produce estradiol. When androgens are the predominant secreting hormone, boys present with features of gonadotropin-independent precocious puberty, penile enlargement, pubic hair growth, masculinization, advanced bone age, and voice changes. Boys who have an estrogen-producing LCT will present with gynecomastia and breast tenderness [A Leydig cell tumor (LCT) is a sex cord-stromal tumor and accounts for 1–3% of all testicular tumors in adults and 4–9% in prepubertal children . Even thBiochemically, LCT will show elevated serum testosterone levels and increased estradiol levels if estrogen is secreted, along with prepubertal levels of FSH and LH. In pure LCT alpha-feto proteins, beta-HCG levels would be typically normal . USS of The histology and the immunohistochemistry are important aspects, deciding the future management and treatment modalities. In contrast to the index case where the adjacent nonneoplastic testicular tissue was prepubertal, there are reported cases where the adjacent seminiferous tubules were positive for spermatogenesis with distant infantile seminiferous tubules , 6.Orchidectomy is the mainstay of therapy with an overall good prognosis and regression of clinical signs though recently testicular sparing surgery has become a choice of treatment , 4.In most instances, following treatment, the signs of puberty have regressed, but occasionally, following surgery, some have entered gonadotropin-dependent precocious puberty (GDPP). A review conducted in 2015 reported 8 cases , and onlIn all these reported cases, patients have presented much earlier and received gonadotropin-releasing analog  except for the oldest patient who was 9 years at the presentation with a bone age of 13.5 years. GnRH analogs have successfully withheld the progression of puberty, but the already advanced bone age resulted in growth deceleration, and growth hormone has been administered to some patients to optimize the adult height . The exaThe long-term follow-up in adult males with LCT has found low sperm counts and abnormal sperm morphology with low testicular volume and has been a known cause for male infertility .It is important to recognize features of precocious puberty by caregivers to prevent delaying presentation and diagnosis of sinister conditions. Gonadotropin-dependent puberty should be considered when clinical signs failed to regress following surgery, and therefore, these children require short-term as well as long-term follow-up.


Extraosseous Ewing’s sarcoma (EES) is a malignant tumor that is classified as a rare disease. EES is common in children and adolescents, with a rarer incidence being present in the elderly. ES of the primary intestine is rare, with only a few reports described in the literature. Here we report a case of a 69-year-old male patient who was experiencing abdominal pain for over 3 months. Ultrasonography (US) revealed a solid hypoechoic lesion with multiple irregular necrotic areas in the left lower abdomen close to the dome of the bladder. Contrast-enhanced ultrasonography (CEUS) showed that the lesion exhibited heterogeneous enhancement and quick peripheral enhanced tissue wash-out classifying this mass as malignant. PET–CT showed a high metabolic mass in the lower abdomen, multiple metabolic nodules in the mesentery, considered as a small intestinal stromal tumor with lymph nodes metastasis, and that a diagnosis of lymphoma should be excluded. Esophagogastroduodenoscopy performed at another hospital 1 month prior to CT showed an abnormal density in the pelvic cavity that was considered as a colonic diverticulum with an abscess. The endoscopy showed no obvious occupying lesions. The mass was removed and postoperative pathology confirmed ES of the small intestine. The patient avoided receiving chemotherapy. After 2 months, skull metastasis was diagnosed and surgical intervention was done. His survival was only six months after the second surgery. To our knowledge, our case is the first report of ultrasound and CEUS manifestation of EES in the small intestine in elderly. The Ewing’s sarcoma (ES) family of tumors is highly aggressive and includes the extraosseous ES (EES), peripheral primitive neuroectodermal tumor (PNET), Askin’s tumor and atypical ES . ES main). The tumor contained multiple necrotic areas and close contact with the bladder wall. Heterogeneous enhancement, and wash-out time of 54 s on CEUS , malignant gastrointestinal stromal tumors (GISTs), and intestinal lymphoma. 1. SBA: It has been reported the most frequent histologically malignant tumor of the small intestine; most SBA arises in the duodenum; it can also arise in the jejunum, ileum, or in unspecified location. It often occurs at 60–70 years old. The most frequent symptoms are abdominal pain, obstruction, and occult gastrointestinal bleeding. Typically SBA gives an annular constriction to the intestine and grows into the cavity; infiltration into surrounding structures and distant metastases appears early , 27. 2. EES/PNET is not only a big challenge for imaging diagnosis but also poses challenges for pathology. EES masses often present with extensive hemorrhaging and necrosis; a fine needle biopsy is usually inadequate for diagnosis , 32. EESEES is clinically characterized by rapid growth of the soft tissue mass, which is often manifested early in the lung, lymph nodes, and bone metastases . The treWe report a rare case of senile, small bowel primary ES showing rapid skull metastasis. A variety of preoperative imaging showed malignant features but it was difficult to distinguish it from common intestinal malignancies. Surgical resection is a conventional treatment, but due to its highly aggressive biological behavior has limited effects on improving the survival rate of EES. Thus, it is necessary to explore multimodality treatment approaches to achieve a better favorable outcome for elderly EES patients.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.The studies involving human participants were reviewed and approved by Shengli Clinical Medical College of Fujian Medical University, Fujian Provincial Hospital. The patients/participants provided their written informed consent to participate in this study.JY and HW analyzed the data and image acquisition, revised the manuscript, have contributed equally to this work. SW designed the study and revised the manuscript. All authors of this manuscript have actively participated in the data acquisition, and they all commented and approved the final version of the manuscript.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.


Uterine leiomyomas are a common finding in medical practice, but their frequency changes drastically when contextualized in a syndrome, as in the following case. A 50-year-old woman with a known Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome presented at our clinic with abdominal pain located in the lower quadrants and scarcely responsive to analgesic therapy. A twisted gynecological pelvic mass was diagnosed, and management for prompt resolution was adopted. Histologically the mass was described as a leiomyoma. The aim of the present study is to share our experience and to review the literature to compare different manifestation of the disease and different approach used in the various centers. The additional novelty of the paper is the immunohistochemical study we carried out on the leiomyoma that is contrasted with the current etiopathogenetic theories. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is one of the most common causes of primary amenorrhea, second only to gonadic dysgenesis, having a frequency of 1:4000–5000 women . The karThe syndrome has three different forms: type 1, typical form, is characterized by congenital absence of the uterus and the upper two-thirds of the vagina with regular adnexa; type 2, atypical form, manifests alterations to the genital apparatus (ovaries) and renal defects; type 3 shows aplasia or hypoplasia of the uterus and vagina in association with renal malformations  as well as skeleton, cardiac, ocular and hearing alterations, known as MURC syndrome [The presence of leiomyomas in patients with MRKH syndrome is rare and, according to a recent review, only one other case has been described in Italy . The majLeiomyomas are benign tumors derived from smooth muscle cells forming the myometrium. They represent the most common benign female genital neoplasia with an estimated frequency of 20% of the population between 30- and 60-year-old women . In the A 50-year-old woman presented in the emergency room who had been experiencing abdominal pain for one week. Initially the pain was spread throughout the abdomen and was similar to cramps; later on, it started to be confined and localized in the abdominal inferior quadrants and was scarcely responsive to an analgesic therapy. There were no accompanying symptoms or increased inflammatory markers. The patient was aware of being affected by MRKH syndrome, which was diagnosed in puberty during a follow-up for a primary amenorrhea. The patient was also aware of a pelvic mass of unknown origin discovered 5 years prior during a routine ultrasound. During the anamnesis, the patient denied having menstrual flows and pelvic pains in the past. At the medical examination, the abdomen was soft and non-tender except for a strong tenderness corresponding to a hardened hypogastric tumefaction. The Blumberg sign was negative. Blood tests were requested and came out normal. White blood count and C reactive protein were negative. An abdominal ultrasound was performed as well as a computed tomography, with identification of both kidneys, normal in size and position, with smooth profiles and a regular echogenicity and parenchymal thickness, without any signs of cystic alterations in their context. There was no sign of hydronephrosis on the right side; mild pyelectasis was found on the left kidney. The bladder was expanded with regular walls. A neoformation of unknown origin was identified in the pelvic region described as a voluminous pelvic expansive formation  likely referred to a voluminous uterine partially calcified leiomyoma. Mild perihepatic and perisplenic effusion were also detected; liver, pancreas, spleen, adrenal glands and kidneys were normal. At the gynecologic examination, the vagina was atresic, and a hard hypomobile tumefaction was identified in the pelvic region with its extension reaching 2 cm above the transverse umbilical line.At transrectal and transvaginal ultrasonography examination , the uterus was not recognizable nor was the endometrium visible, and the whole pelvis was occupied by a 15 cm diameter solid bilobate “C-shape” well-defined lesion  with mixIn addition, we described a ureterocele image in the left kidney, possibly due to extrinsic compression by the pelvic malformation or by primary renal malformation, as described in MRKH disease. Moderate peritoneal effusion was present in the pelvis.Due to the worsening of the pain and no response to the analgesic therapy, a surgical approach was chosen, with a supposed diagnosis of an unknown origin pelvic lesion in a patient with MRKH syndrome. An initial laparoscopic approach was undertaken with the insertion of an umbilical 10 mm optical trocar and three 5 mm ancillary trocars. A rudimental uterus was highlighted on the right side of the pelvis with bilateral regular ovaries. A 15-cm dark-red left formation was found, departing from the rudimental uterus and twisted on its peduncle . Considering the size and hardness of the mass due to calcifications present in its context, as well as the uncertain histologic nature of the mass, we decided to convert the surgery from laparoscopy to laparotomy by a transverse suprapubic mini-laparotomy. A small quantity of peritoneal liquid was drained and sent for cytological evaluation. After identification of the left ureter by retroperitoneal access, we proceeded with the removal of the entire mass with its vascular peduncle. The mass  was, theA search was carried out on PubMed and Scopus (until December 2020) to identify articles involving patients with MRKH and leiomyomatosis. The manuscripts considered were published from 1999. The research was focused on original articles in English. Nine manuscripts were detected through the references of the works that had been identified with the research on PubMed and Scopus. A total of 35 cases were found describing the presence of myomatosis in patients with MRKH where 27 were not complicated and 8, instead, had an acute manifestation similar to our case.Apparently, there are only 27 cases described of uterine leiomyoma in MRKH not complicated by acute abdomen  2,3,4,9,29,30,31Focusing our attention on cases with acute onset similarly to ours, we found only eight cases where a torsion of the lesion was described ,35,36,37To better understand the manifestation of the disease and the different management of it worldwide, we proceeded to analyze a few features, as follows. In almost all cases, the first suspicion was given by ultrasound since it is the faster instrument used in the emergency room. After that, magnetic resonance and computed tomography were used to confirm the diagnosis. The average age of onset of the disease was 45.5 years old, ranging from 28 to 55 years old and in line with the typical age of leiomyoma manifestation. The volume of the masses is similar in almost all cases, around 10 cm × 15 cm. Only in one case, the dimension of the pelvic mass was smaller, and in two studies, there is no mention of the volume of the myomas. The mass origin was from a rudimentary uterus in four cases, paraovarian or adnexal in three cases and not clarified in the remanent two cases. Regarding the surgical approach, all the surgeons preferred laparotomy access with respect to a laparoscopic one, with the only exception of Yi et al.  who, unfThe presence of a pelvic mass in a woman with MRKH can be a diagnostic dilemma due to a subverted gynecologic anatomy and the frequent association with renal malformations. The low incidence of the disease makes the diagnosis even more difficult. Our experience and a review of the literature demonstrate that leiomyomas may develop in the context of a rudimental uterus, but it is a rare finding. Specifically, our case falls within the classic form of the syndrome, with the presence of a rudimental unilateral uterus. Clinically, it seems that the mass can be completely asymptomatic until its volume reaches great dimensions (10–15 cm). Larger or smaller myomas, generally, do not lead to an acute complication. This is probably due to the poor mobility and, therefore, to the reduced possibility of torsion of myomas of these sizes.The age of onset is the same of the population with normal gynecologic development, and surgeons almost unanimously prefer the laparotomic approach in emergency cases such as the ones analyzed in the current review.On ultrasound examination, leiomyomas are hypoechoic or heterogeneous masses. Sometimes there can be an internal cystic degeneration with necrosis or hemorrhage that appears ultrasonographically as a cystic component with internal echogenic material; differently, calcifications appear as hyperechoic foci . The pre-surgical differential diagnosis between an ovarian lesion and a lesion of a rudimental uterus may be difficult since the usual anatomical landmarks are subverted. For example, a large pelvic mass with possible inner myxomatous degeneration may be easily confused with a cystic ovarian lesion .If the mass is associated with pelvic pain, adenomyosis and other pathologies should be considered in the differential diagnosis, as described in rare cases ,38. In oAmong the few cases described in the literature, apparently only eight are reported to be the manifestation of the lesion as a twisted mass, and six patients had an acute abdomen. Furthermore, only in two reports, the torsion involved the mass exclusively, sparing the adnexa ,6, similRegarding the surgical procedure, the literature describes various approaches. Laparoscopic, as well as laparotomic techniques, have been chosen according to the patient’s clinical evaluation, imaging, number of masses, mass localization and characteristics (size of the mass or complicated nature of its feeding arteries). Removing the remnant uterus may be a choice in order to prevent the risk of recurrence ,32 and aIn our review, only one case is described as a mitotically active leiomyoma , and theIn women with Mayer-Rokitansky-Küster-Hauser syndrome presenting with pelvic pain not responsive to the analgesic therapy, the presence of a gynecological complication should always be considered among the possible diagnosis. The use of ultrasonography is recommended, adding a CT or an MRI for higher anatomical detail and better pre-surgical stadiation. However, the proper and final diagnosis, due to the rarity and the anatomical complexity of these lesions, can be given only through surgical procedure and histological evaluation. We demonstrated that the etiopathogenesis of leiomyomas still remains unclear, and the cluster of agonists and antagonists with their receptors involved in the development needs to be clarified. Finally, from our review, it emerges that the occurrence of new myomatous neoplasms in patients with MRKH, starting or not from uterine rudiments, could cause an acute abdomen requiring urgent surgery. For this reason, patients who undergo pelvic surgery could benefit from preventive remotion of uterine residues by minimally invasive surgery with minimal discomfort to the patients.