Carcinoma ex pleomorphic adenoma (CEPA) is an uncommon complication of an untreated pleomorphic adenoma (PA), but one that has a life-threatening significance. This case report documents the clinical, radiological and histopathological features of an extremely rare case of biopsy-proven pineal metastasis, with cerebellopontine and leptomeningeal spread, from CEPA of the parotid gland in spite of the patient having undergone parotidectomy, ipsilateral neck dissection and adjuvant radiotherapy. In spite of the current surgical and oncological treatment of CEPA, the rates of recurrence and distant metastases are high, with a subsequently poor prognostic outcome in most patients. Distant spread is usually to the bones and the lungs; however, more unusual locations have been documented. Our finding of pineal metastasis from CEPA has not previously been reported in the literature. Although this is a rare complication of an unusual condition, the aggressive behaviour of these malignancies warrants close clinical follow-up, with a low threshold for re-imaging and investigation if indicated. We present the case of a 62-year-old female with a high-grade parotid carcinoma ex pleomorphic adenoma (CEPA) that was initially treated with left parotidectomy and ipsilateral neck dissection. The patient originally presented with a 1-year history of a left parotid region mass and co-existing ipsilateral facial nerve palsy, with the diagnosis confirmed using ultrasound, MRI  and coreThe patient was well at initial follow-up, with no clinical or radiological evidence of recurrence. However, approximately 6 months after completing radiotherapy, she started experiencing tinnitus as well as feelings of “drunkenness”. Fine needle aspiration cytology was performed on a suspicious left supraclavicular fossa lymph node during an outpatient visit to the head and neck one-stop clinic. This demonstrated features consistent with a poorly differentiated metastatic malignancy, likely adenocarcinoma. An audiogram performed at the same time confirmed complete hearing loss in the left ear.18F]-fluoro-D-glucose (18F-FDG) positron emission tomography/CT (PET/CT) imaging, which also revealed multiple bone metastases and bilateral metabolically active hilar lymph nodes, also suspicious for metastases  via a right frontal pre-coronal burr hole. A pale pink rounded mass was seen suspended in the posterior part of the third ventricle, overhanging the cerebral aqueduct. Tumour and cerebrospinal fluid samples were sent intraoperatively. There were no perioperative complications and the patient was discharged home on the third postoperative day.1 In this case, when the histology from the pineal gland was compared with the original histology, the intracranial lesion was consistent with a metastatic adenocarcinoma from the parotid gland .4 Malignant degeneration should be suspected in lesions that appear infiltrative with an irregular contour on imaging. Ultrasound evaluation of the cervical lymph nodes is also vital in identifying any evidence of local lymphatic dissemination.Pleomorphic adenoma (PA) is the most common non-malignant tumour to affect the salivary glands, usually involving the parotid glands and less commonly the submandibular and other minor salivary glands, as well as the lacrimal glands.4 Although often asymptomatic, they most commonly present as a firm mass in the parotid region. The patients may also present with facial nerve palsy owing to deep parotid lobe invasion involving the facial nerve, a finding that should raise the suspicion for malignant degeneration in patients with PA. Transformation into CEPA has been linked to an increased pre-operative duration of a PA.3CEPAs comprise approximately 12% of all malignant salivary gland neoplasms, usually occurring in the sixth to eighth decades of life.6 MRI is the imaging modality of choice for post-treatment follow-up of salivary gland neoplasms, with PET/CT being invaluable for the assessment of suspected recurrence and disseminated disease.7Local and regional surgical resection followed by radiotherapy is the most recognized appropriate treatment in cases of high-grade CEPA, although there are no standardized treatment protocols.4 Zhao and colleagues5 demonstrated an overall 5-year survival of approximately 50% in a study of 51 patients with CEPA, who were treated with curative intent.CEPAs are aggressive malignancies with high rates of recurrence and metastases. Owing to the limited number of cases and the variability in histological and radiological staging, survival rates in these patients vary but are regularly reported as less than 1 year.6 The histological grade of the primary tumour, local invasiveness and cervical lymph node involvement are all important prognostic factors, the latter is recognized as the most reliable feature in predicting recurrence or metastasis.5 In this case, although there was no extracapsular extension of the lesion, the tumour was high-grade with multiple cervical lymph nodes also involved. Distant metastases are usually to the lungs and bones, with renal, hepatic and splenic metastases also rarely reported.6 Intracranial or spinal metastatic spread is exceptionally rare, with only very few cases reported in the literature. Sheedy et al2 reported a case of biopsy-proven metastases from CEPA of a parotid primary tumour affecting the brain and intramedullary spinal cord, which they believed to be the first reported case of its kind at the time. Ahn and colleagues8 have reported intracranial and spinal metastases from a lacrimal gland CEPA, with recurrent primary tumour directly extending into the cavernous sinus with leptomeningeal deposits also identified. Our case is exceptional in that, to our knowledge, a pineal gland metastasis from a distant CEPA has never been reported. The presence of a metastatic deposit within the cerebellopontine angle from this primary lesion has also not been previously described. The intracranial and spinal leptomeningeal disease, which rapidly progressed in our patient, has only previously been documented in the report by Ahn et al,8 indicating that this is also a remarkably rare finding.Regional and distant metastases from CEPA appear to occur with an equivalent frequency.9 There are few cases of symptomatic pineal region metastases reported in the literature; however, a case reported by Nemoto et al10 of a patient with an isolated pineal region metastasis from lung adenocarcinoma also presented with obstructive hydrocephalus. As in our case, there was an indolent clinical course, with little in the way of examination findings and no evidence of raised intracranial pressure. The limited literature available documents that the most common primary tumour site for spread to the pineal gland is the lungs, with other primaries including breasts, malignant melanoma and kidneys also cited.10 Pineal metastasis from a salivary gland neoplasm has not, to the best of our knowledge, been reported in the literature.Overall, the pineal gland is an unusual location for metastatic spread, with many cases discovered incidentally at autopsy: reports have indicated that there is a prevalence of 0.4–3.8% in patients with solid tumours.Although this is an extremely rare case report of a pineal gland metastasis, with a cerebellopontine angle deposit and leptomeningeal spread, from a parotid CEPA, it serves to demonstrate how aggressively this primary tumour can behave, as well as serve to illustrate the diagnostic dilemmas that the clinical team may face. A strong, collaborative effort on the part of the multi-disciplinary team looking after these patients is key to the diagnosis and effective treatment of these challenging cases.There is an uncommon yet significant risk of PAs undergoing malignant transformation, particularly if untreated. As such, they should be considered as premalignant conditions.CEPA behaves unpredictably but has high rates of recurrence and metastases, and the discovery of involved lymph nodes is strongly regarded as a poor prognostic sign.Utilization of a variety of imaging modalities in the management of CEPA is strongly recommended. Ultrasound ± needle aspiration or biopsy, MRI and PET/CT investigations will aid in the definitive evaluation of recurrence at the surgical bed, confirmation of local lymph node involvement or distant metastatic spread, and the suspected complications of disease progression.Intracranial metastases are extremely rare in salivary gland tumours and there needs to be close collaboration between clinicians, radiologists and pathologists in ensuring timely diagnosis and treatment.Although an unusual entity, metastases should be a differential diagnosis for any patient with a known malignancy who presents with a pineal region tumour.Informed consent could not be given by the patient as she was deceased at the time of writing this case report. This could not be obtained from the next of kin or a family member despite exhaustive attempts to contact them.


Nonpuerperal uterine inversion is a very rare event. We reported on the first ever case of nonpuerperal uterine inversion due to adenomyosis. Magnetic resonance imaging is recommended in cases with an unusual vaginal mass, so that this possible uterine etiology can be taken into consideration. Nonpuerperal uterine inversion is a very rare event. We reported on the first ever case of nonpuerperal uterine inversion due to adenomyosis. Magnetic resonance imaging is recommended in cases with an unusual vaginal mass, so that this possible uterine etiology can be taken into consideration. Due to poor tolerance (nausea and vomiting) and lack of effectiveness on bleeding, the patient stopped taking the medication. In August 2018, she consulted for a second opinion. A clinical examination showed a retroverted uterus but no vaginal prolapse. Various treatment options  were discussed with the patient but none was immediately chosen. In January 2019, the woman (who worked in the catering industry) returned to our clinic complaining of abnormal bleeding for 3 weeks a month and pelvic heaviness. A pelvic examination revealed a large intravaginal mass; it measured between 6 and 7 cm, protruded from the cervix, and had the appearance of myoma. Given the recurrence of abnormal bleeding, the pain, and the aspect of the vagina, the woman agreed to have a hysterectomy without adnexectomy. Three days after this consultation, the woman was admitted to the emergency department for acute pelvic pain  and bleeding. The very intense pain suggested torsion or necrobiosis of the myoma. The woman's pain was relieved in part by treatment with paracetamol, a nonsteroidal anti‐inflammatory, and a muscle relaxant. This treatment was combined with hypnosis sessions, which increased the patient's pain tolerance. The pain level fell over the following 48 hours, and surgery was performed three days after the patient had been admitted to the emergency department.A 38‐year‐old primiparous woman with an unremarkable medical history consulted in September 2017 for pelvic pain, menorrhagia, dysmenorrhea, and dyspareunia—suggesting the presence of adenomyosis. Magnetic resonance imaging showed the uterus with a longest dimension of 8.6 cm and an anteroposterior diameter of 7.3 cm. The endometrium had a normal aspect and thickness. A nodular lesion (measuring 6.5‐5.3 cm) was present in the posterior myometrial wall. The lesion was heterogeneous (with areas of hyperintensity in a T2‐weighted MRI sequence) and contained a large number of cyst‐like structures. In a T1‐weighted sequence, some of the cysts had a hyperintensity pattern consist with hemorrhage, whereas others appeared to be filled with fluid. Thickening of the junctional zone was suggestive of adenomyotic damage. In contrast‐enhanced sequences, the lesion appeared to be well delimited and had intense but not suspicious foci of uptake. On this basis, we diagnosed interstitial myoma with focal hemorrhagic cysts  surgical procedure, we observed an invagination of the Fallopian tubes and the ovarian and round ligaments. It was impossible to see the uterus Figure A. We theHistopathological assessments of the cervix  urethral obstruction. In a clinical examination with bimanual palpation, the uterine fundus is not palpable. Although our patient had experienced menorrhagia and pelvic pain for more than a year, the intravaginal mass (inversion) and the onset of acute pain  occurred only a few days before surgery. On the basis of the MRI findings, we initially thought that the intravaginal mass was perhaps a prolapsed myoma. The two other differential diagnoses  were, respectively, ruled out by the normal cervical smear test 9 months previously and our inability to see the cervix.3 × the density of blood [1.066]). Our patient did not report any episodes of coughing but had vomited while taking ulipristal acetate (between November 2017 and March 2018), which would have generated abnormally high intra‐abdominal pressure. However, palpation of the uterus was normal at the clinical examinations in March and August 2018.The pathophysiology of uterine inversion is thought to be related to the coincidence of several factors: a thin uterine wall, a large, rapidly growing, pedunculated tumor in the fundus, and gradual dilatation of the cervix after that of the uterine cavity.We did not perform medical imaging (ultrasound or MRI) during the episode of acute abdominal pain that occurred a few days before surgery. Myoma was diagnosed in November 2017 on the basis of the MRI findings and the clinical aspect of the intravaginal mass, which was strongly suggestive of a myoma that had prolapsed through the cervix. We should probably have performed MRI again in January 2018; this might have revealed the signs described by Moulding,4We reported on the first ever case of nonpuerperal uterine inversion due to adenomyosis. Magnetic resonance imaging might have enabled earlier diagnosis of this complication—especially in view of the acute pelvic pain experienced by the patient. The treatment would have been the same, however, given the irreducible nature of the inversion, the presence of hemorrhagic adenomyosis, and the absence of plans for further pregnancies.None declared.Philippe Merviel: served as the gynecologist and the surgeon in this case, and Head of the Gynecology and Obstetrics department of the Brest University Hospital; conceived the manuscript; and revised the article. Sandrine Guilbert: served as the gynecologist at the start of the case history. Coline Abou Hassan: served as the medical student and assistant during the surgery in this case. Isabelle Thomas‐Kergastel: served as the radiologist (MRI) in this case. Virginie Conan‐Charlet: served as the pathologist in this case. Pascale Marcorelles: served as the pathologist referent and Head of the Histopathology department of the Brest University Hospital. Pierre‐François Dupré: served as the gynecologist and contributed to conceive the manuscript.


Local prostate cancer recurrence is usually treated with salvage radiation (sRDT) with or without adjuvant therapy. However, surgical resection could be an option. We aim to present the surgical technique for robot - assisted laparoscopic resection prostate cancer local recurrence after radical prostatectomy (RP) and sRDT in 2 cases.First case depicts a 70 year - old man who underwent RP in 2001 and sRDT in 2004. Following adjuvant therapy, patient had biochemical recurrence. MRI showed a solid mass in the prostatic fossa close to vesicourethral anastomosis, measuring 2.1 cm and PET / CT revealed hyper caption significant uptake in the prostatic fossa. Second case is a 59 year - old man who underwent RP in 2010 and sRDT in 2011. Again, patient presented with biochemical recurrence. PET / CT showed hyper caption in the prostatic fossa. Biopsy conformed a prostate adenocarcinoma. Both patients underwent robot - assisted extended pelvic lymph nodes dissection and local recurrence resection. A standard 4 robotic arms port placement was utilized.Both procedures were uneventfully performed in less than 3 hours and there were no complications. Pathological examination showed a prostate adenocarcinoma Gleason 7 and 8 in the first and second case, respectively; surgical margins and lymph nodes were negative. After 6 months of follow-up, continence was not affected and both patients presented with PSA < 0.15 ng / mL.Robot - assisted laparoscopic resection of prostate cancer local recurrence after RP and sRDT detected by PSMA PET / CT seems to be safe in experienced hands. It may postpone adjuvant therapy in selected cases.


Liposarcoma of the larynx and hypopharynx is very rare. Computed tomography (CT) and magnetic resonance imaging (MRI) examinations may synergistically disclose suggestive features of a fat-containing tumor with non-fatty enhancing sub-areas. Diagnosis relies on histological examination of a biopsic specimen. This rare pathology should ultimately be kept in mind when dealing with an laryngeal/hypopharyngeal mass. Liposarcoma is the most common form of soft-tissue sarcoma, most frequently found in limbs and the retroperitoneum. Laryngeal and hypopharyngeal localizations are extremely rare; 40 cases have been reported in the literature up to now, three of which were of the dedifferentiated (DDL) histological subtype 3. We repA healthy 67-year-old man presented with a four-month history of progressively worsening dysphonia and breathing discomfort. Neck palpation was unremarkable. Flexible laryngoscopic examination (not shown) revealed a polypoid and pedunculated mass of the right arytenoid and aryepiglottic fold.Contrast-enhanced (CE) computed tomography (CT) demonstrated a fat-containing tumor arising from the right aryepiglottic fold. The lesion appeared well delineated with a mixed pattern. Magnetic resonance imaging (MRI) was performed after CT to better characterize the lesion and more precisely delineate tumor boundaries before surgical work-up.Fatty areas seen on CT Figure  and 1B eSupraglottic laryngectomy was carried out with clinically uneventful post-operative period, and completeness of the resection on MRI (not shown). Anatomopathological examination of the resection specimen demonstrated a large proportion of well-differentiated liposarcoma with a small areas of dedifferentiated liposarcoma, both with the MDM2 gene amplification Figure .de novo. As in our patient, WDL and DDL may share similar genetic signatures  [2Four histological subgroups of liposarcomas are described by the World Health Organization (WHO) classification: (i): differentiated (WDL); (ii): myxoid; (iii): pleomorphic; and (iiii): dedifferentiated (DDL). DDL is defined as non lipogenic sarcoma of variable (from low to high) histologic grades and may occur either as recurrence of WDL or arise of MDM2) . Laryngef MDM2) 25.Liposarcomas are well-delineated masses on CT and MRI. The presence of fat densities or signal intensities within the tumor is the main clue to the radiological diagnosis. Fatty content is a marker of WDL. ‘Mosaic’ appearance of the tumor may reflect the coexistence of lipogenic WDL and non lipogenic low- to high-grade DDL areas. In our patient a large proportion of WDL and small proportion of DDL was found, thereby explaining the prominence of fatty density/signal intensity on imaging. Mild enhancement is usually observed on post-contrast T1-weighted images, more conspicuously using fat saturation Figure .Tumor extension is best assessed by CT or MRI, but the two modalities may act synergistically, with fat-suppressed post-contrast T1-weighted images better disclosing enhanced high-grade DDL areas embedded within fatty WDL that can be seen on CT and pre-contrast T1-weighted MRI. Mapping of such high-grade DDL areas is crucial both for biopsy targeting and overall treatment strategy. Our case illustrates a lipomatous tumor of overall pseudo-benign fatty appearance but containing small foci of high-grade tissue best depicted on post-contrast FatSat T1-weighted images.Resection is the standard treatment option for HN liposarcomas through either external or endoscopic approach . ConservA mosaic tumor pattern mixing fatty and non-fatty enhancing components should trigger the diagnosis of liposarcoma even in very rare locations such as neck spaces.


Isolated cecal necrosis (ICN) is a rare condition which is developed under decreased mesenteric perfusion. Only a few dozen cases of ICN have been reported previously. The patient was a 59-year-old male with a previous history of atrial fibrillation. He presented to our emergency room with the chief complaint of lower abdominal pain. Computed tomography imaging revealed a dilated cecum and presence of free air. With a preoperative diagnosis of perforation of the cecum; an urgent surgery was conducted. Intraoperative findings revealed an ischemic change of the cecum and a laparoscopic-assisted ileocecal resection was performed. The pathological findings showed transmural ischemic change on the anti-mesenteric side of the cecum, and the diagnosis of ICN was achieved. Preoperative diagnosis of ICN is difficult because of its non-specific radiological features. In patients with right lower abdominal pain, ICN should be considered as a differential diagnosis especially if the patient has a comorbidity causing hypotension attack. Isolated cecal necrosis (ICN) is an infrequent variant of ischemic colitis . Poor me3) with slightly elevated C-reactive protein (CRP 0.43 mg/dL).The patient was a 59-year-old male who presented to our emergency department with a complaint of a 5-h duration of lower abdominal pain. He had a previous history of atrial fibrillation and had taken warfarin, digoxin, and bisoprolol. A physical examination revealed tenderness to palpation of the right lower abdomen without signs of peritoneal irritation. Laboratory data showed slight leukocytosis (WBC 8700/mmA computed tomography (CT) image revealed a dilated cecum surrounded by free air, while the appendix was intact . The preDuring the laparoscopy, the cecum was found to be dilated and discoloured .About 10 h after symptoms onset, we performed a laparoscopic-assisted ileocecal resection. After the resection of the damaged intestine, extracorporeal end to end anastomosis was performed. The specimen revealed localized ischemic change on the anti-mesenteric side of the cecum . MicroscThe lesion was diagnosed as isolated cecal necrosis. On day three postoperative, the patient developed a small intestinal perforation caused by sticking on the drainage tube, and he underwent ileostomy. After that, the patient progressed uneventfully, and the patient underwent closure of the ileostomy on the 51st postoperative day.One of the key factors for developing ICN is a presence of the comorbidity which causes decreased mesenteric perfusion ,2,3,4,5.Another key factor in developing ICN is the presence of a variation in cecum blood supply. The cecum is mainly supplied by the anterior and posterior caecal arteries. These arteries often arise from the vascular arcade between the ileal branch and colic branch of the ileocolic artery, while, in the others, these arteries arise directly from the ileal or colic branch ,14,15. IGuitart et al. suggested that preoperative CT findings suggesting ICN are: 1) thickening of the cecal wall; (2) abrupt transition between the ischemic cecum mural thickening and the ascending colon and 3) pneumatosis of the cecal wall . However thickeni pneumatoPrevious reports have suggested that some cases of ICN showed poor prognosis . On the This case report is for academic communication only and is not for other purposes. This paper does not disclose the personal information of the patient and informed consent was obtained. The study protocol was approved by the institutional review board. The study does not include any animal experiments.We reported a case of ICN. Preoperative diagnosis of ICN is difficult because of non-specific radiological features. In the patients with right lower abdominal pain, ICN should be considered as a differential diagnosis especially if the patient has comorbidity causing a hypotension attack.


Surgical scar endometriosis is typically presented as a slow-growing, painful abdominal mass near the site of a past surgery. Endometriosis on the trocar port site is rare. To best of our knowledge, only 17 cases have been reported in the literature. The nonspecific nature of endometriosis presents a diagnostic challenge, and it is often considered as an incisional hernia or other conditions.Here, we described our experience with a recurrent abdominal scar endometriosis case at the trocar port site of a previous laparoscopy, which was initially thought to be an incisional hernia.Abdominal wall endometriosis should be considered as an important differential diagnosis in females with a positive history of prior abdominal surgery, presented with painful nodule or mass at the site of the surgery. Endometriosis is defined as the presence of functional endometrial glands and stroma in extrauterine locations. The endometrial tissue is usually found within the ovaries, uterine ligaments, fallopian tube, or pelvic side wall , 2. ExtrA 25-yr-old G1L1 women was presented with abdominal pain and swelling at the port site of the previous laparoscopy. The patient was a case of primary infertility and polycystic ovary syndrome. She had received many medications such as clomiphene, human menopausal gonadotropin/ human chorionic gonadotropin, metformin, and levothyroxine without any response. All of her husband’s work ups were normal. Hence, she underwent diagnostic laparoscopy, and both ovarian cauterizations were performed due to polycystic ovary syndrome, seven yr ago. She became pregnant after two months, and with normal vaginal delivery, a boy was born. After one-yr mild abdominal pain began and worsened during the upcoming years. So, she was referred to a general surgeon with the impression of incisional abdominal wall hernia, therefore, herniorrhaphy and mesh repair was done for her. However, her abdominal pain continued after surgery with the cyclic pattern, and after seven months a large mass palpated at the site of abdominal scar. In a clinical examination, a painful, round, firm mass was detected near the previous site of operation. The patient underwent a second operation, and an irregular fibro-fatty tissue measuring 4x4x1.5 cm was excised. Serial cut sections of the specimen showed yellow-brown color with some foci of hemorrhage. Microscopic examinations showed endometrial glands and stroma in favor of endometriosis . After tEthical considerationWritten informed consent was obtained from the patient for the publication of this case report as well as accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.Abdominal wall endometriosis (AWE) is characterized by the presence of a nodule along the surgical scar. There is history of previous surgery such as C-sections, hernias, laparotomies, laparoscopy port sites or hysterotomies when the endometriosis involves the abdominal wall -6. FrequSeveral theories for endometrioma formation has been proposed, which include the hematogenous, lymphatic and iatrogenic spread of endometrial cells, metaplastic change of pluripotent cells at any site to endometrial cells, the retrograde spread of endometrial cells to the pelvis during menstruation, and immune system dysfunction . Scar enThe diagnosis of abdominal wall endometriosis is mostly clinical when there is a mass at the surgical scar site, which becomes painful during menstruation , 6. HoweFine-needle aspiration cytology has been shown to be a fast, accurate, and inexpensive method to make a diagnosis before surgery. The most specific findings are endometrial-like epithelial cells, stromal cells, and hemosiderin-laden macrophages . SeveralIn our case, the presence of endometriosis and its sign and symptoms were 7 yr after the diagnostic laparoscopy at the trocar port site that was first operated as an incisional hernia, and it recurred twice. At first, it reoccurred seven months later and once again after second surgery. To best of our knowledge, there are only a few cases of recurrent scar endometriosis after excision . In a reHowever, the patient received gonadotropin-releasing hormone-agonist as treatment. Gonzalez-Fernandez presented a case report of a 44 yr old woman with recurrent abdominal wall endometriosis, noted on the fascia, which was resected 9 yr prior to its recurrence . The risAbdominal wall endometriosis should be considered as an important differential diagnosis in females with a positive history of prior abdominal surgery, presented with painful nodule or mass at the site of the surgery.


The most prevalent type of soft tissue sarcoma is undifferentiated pleomorphic sarcoma (UPS) or previously known as malignant fibrous histiocytoma. It accounts over 20% of all soft tissue sarcomas and occurs most frequently in the extremities, trunk, and retroperitoneum. However, it has been rarely observed in the digestive system. Pancreas sarcoma represents less than 1% of all pancreatic tumors, and primary UPS of the pancreas is even rarer. It exhibits high recurrence and poor prognosis. In this case, a 72-year-old woman with a UPS tumor which was located in the pancreas head and neck without adhesion to the retroperitoneum will be discussed. Undifferentiated pleomorphic sarcoma (UPS) or previously known malignant fibrous histiocytoma is the most common type of soft tissue sarcoma of middle and late adulthood –3. It isA 72-year-old woman was referred to our hospital with pancreatic head and neck carcinoma for more evaluations and surgical operation. She had been suffering from abdominal pain for about 1 year, and the pain has become more intense in the last 6 months. The pain was postprandial and localized to the right upper abdomen. She has become icteric with generalized spread plus itching since past 2 months. The patient had no history of alcohol consumption or smoking. The patient was referred to us with a highly probable diagnosis of pancreatic head and neck carcinoma to be consulted for surgery. Also, a plastic stent was implanted for her in the previous health center due to intra- and extrahepatic duct dilation for the relief of patient symptoms and signs. After admission to our hospital, she was evaluated by abdominal computed tomography (CT), endosonographic imaging, and diagnostic ampullary biopsy. The abdominal CT scan with contrast exhibited an 18 mm × 20 mm hypodense mass at the head and neck of the pancreas . Also, ePostoperative pathological studies were established and indicated a tumor with the greatest dimension of 4 cm which was extended to the duodenum. Invasion of the venous, lymphatic vessels and perineural sites was seen; however, no exact evidence of distant metastasis was found. The periampullary occlusion had hindered the bile flow over time, leading to chronic cholecystitis and pancreatitis which was confirmed by histopathological assessments. The hematoxylin and eosin staining revealed the presence of two cellular populations including spindle fibroblast-like and pleomorphic cells within the tumor. In addition, the proliferation pattern of the mesenchymal cells was storiform . There wThere are currently two hypotheses regarding the etiology of UPS. The first one suggests these tumors do not actually represent a type of cancer but rather a common “morphologic pattern” shared by many neoplasms. This common morphologic pattern is probable to be the fate of a final common malignant progression pathway as tumors become progressively more undifferentiated. Therefore, UPS can originate from not only sarcomas but also carcinomas. The second less common one hypothesizes that undifferentiated sarcomas are originated from malignant transformation of mesenchymal stem cells which do not express differentiation markers from the beginning , 8.Primary UPS of the pancreas as a subtype of pancreatic sarcoma is extremely rare , 9. It uImmunohistochemical exams can exclude other mimic tumors and finalize the UPS diagnosis. The absence of characteristic epithelial markers such as desmin, keratin, S-100P, alpha-fetoprotein, and CEA can exclude the epithelial origin. In addition, UPS exhibits characteristic that have strong reactions to vimentin, alpha 1-antichymotrypsin, CD68, and lysozyme as this case did , 16. MosThe UPS genetic aspects are hard to assess because of the shifting diagnostic criteria used throughout the recent years. In general, their karyotypes have high complexity, with extensive intratumoral heterogeneity. Considerable percent of cases exhibit triploid or tetraploid chromosomes, and a few cases are near-haploid , 23. AltThe origin of the term “malignant fibrous histiocytoma (MFH)” dates back to the early 1960s. Extraction and culturing of cells from some tumors exhibited a storiform pattern which contained pleomorphic and giant cells with phagocytosis-like movements, these features seemed reminiscent of histiocytes. Upon further growth, these cells became elongated and assumed a fibroblastic appearance. Based on these observations, the hypothesis that pleomorphic soft tissue tumors arose from the fibroblastic transformation of histiocytes was suggested . TherefoTo the best of our knowledge, 21 cases of pancreas UPS have been reported in the English-language literature up to now . They coOf these 21 UPS cases, one patient died of perioperative complications. Five patients died of the main disease 5, 7, 11, 12, and 24 months after surgery. 2 patients have not been followed up, and 13 patients were alive without evidence of recurrence, with a median follow-up period of 15 months (mean: 17.5 months and range: 2 to 48 months). Only 8 patients survived for more than one year and just 3 for more than two years. It is apparent that exact conclusions can not be made from such a diverse and limited number of cases that lack appropriate follow-up data. Nonetheless, this evaluation of patients with primary pancreas UPS suggests a poor prognosis for long-term survival. This can be explained by the high malignant potential as well as delayed diagnosis. Furthermore, from 21 introduced UPS cases, only 1 case represented evidence of calcification  and 7 caNone of the 21 patients with pancreas UPS had pulmonary, hepatic, or other distant metastasis at the time of surgery. Only one case was reported with lymph node metastases . Also, oIn summary, our knowledge about UPS of the pancreas is really inadequate especially because of its rarity. More cases with definitely follow-ups are needed to perform a representative clinical analysis.


Intracranial lipomas represent approximately 1% of intracranial lesions generally felt to represent the abnormal persistence of the meninx primitiva and are commonly accompanied by various developmental brain abnormalities. We report a case of midline intracranial lipoma and evolving frontal lobe fluid-attenuated inversion recovery (FLAIR) abnormality concerning for glial neoplasm in a patient with intractable epilepsy. Our case shows evolving magnetic resonance imaging (MRI) features over two decades raising suspicion for low-grade neoplasm which was ultimately found to represent cortical dysplasia. Intracranial lipomas are rare lesions generally felt to represent the abnormal persistence of the meninx primitiva. They represent approximately 1% of intracranial lesion and are commonly accompanied by various developmental brain abnormalities. Most lipomas reported are located near the midline and are pericallosal in nature with agenesis of the corpus callosum commonly observed ,3. We reOur patient is a 43-year-old ambidextrous male with a 20-year history of intractable seizures. His seizure semiology typically included a hot flash and other sensory auras with evolution into focal motor activity predominately on the right. Workup for seizure etiology included magnetic resonance imaging (MRI) revealing the large midline lipoma, partial callosal agenesis, and an adjacent lesion in the left frontal lobe presumed to represent a glial neoplasm based on the radiographic appearance. The left anterior medial frontal lobe lesion consisted of nodular calcification with peripheral enhancement and extensive FLAIR signal changes involving bilateral cingulate gyri and the left frontal lobe Figure .Medical management efforts had failed to control his seizures and he was referred for surgical treatment options. The enhancing lesion was noted to progress with additional imaging and the extent of edema also expanded. The concern for progressive glial neoplasia and poor seizure control prompted a recommendation for surgical resection. The decision was made to proceed with neuronavigation-guided resection of the left frontal enhancing mass and partial frontal lobectomy utilizing intraoperative electroencephalogram (EEG), cortical mapping, and somatosensory evoked potentials (SSEP) monitoring.Intraoperative samples sent for frozen pathology were found to have Rosenthal fibers and focal calcification and felt to likely represent glial neoplasm. Permanent pathologic evaluation revealed a lipoma and focal cortical dysplasia, Palmini Type IA, in the adjacent brain. The mild cortical architectural abnormalities and associated white matter gliosis can be seen on hematoxylin and eosin (H&E) and glial fibrillary acidic protein (GFAP) stains and neurofilament protein (NFP) staining in the areas of gliosis shows no evidence of dysmorphic neurons, giant neurons or balloon cells Figure .Post-operative imaging Figure  shows a Post-resection recovery included a moderate supplementary motor area syndrome including hemiparesis, delayed speech, and changes in effect. These changes gradually resolved and the patient was found to be neurologically intact at outpatient follow-up. Seizure control occurred immediately after resection. Antiepileptic agents were continued and gradually removed over subsequent months without recurrence. The patient remained seizure-free three years post resection. 18-month follow-up imaging found stable T2/FLAIR changes with small unchanged areas of perilesional enhancement Figure .Intracranial lipomas are commonly accompanied by other abnormalities of development which are likely a result of a defect in differentiation of the meninx primitiva during neural tube flexion. Although various cortical abnormalities have been described in association with intracranial lipomas, the underlying pathology remains poorly understood ,5. A fewWhile concurrent abnormalities are often related to a common underlying developmental pathology, this is simply unknown. In the setting of lipomas, cortical dysplasias may change over time.


A 74-year-old female with hypertension, coronary artery disease, was hospitalized due to an acute myocardial infarction. A percutaneous transluminal coronary angioplasty (PTCA) and stenting were performed successfully. After six hours of PTCA, an intense abdominal pain localized on right iliac fossa started. On physical examination, a palpable, painful, firm, nonpulsatile abdominal mass was observed. Fothergill’s and Carnett’s signs were present; there were not Cullen's and Grey Turner's signs. Abdominal computed tomography (CT) showed oval collection, isodense in relation to the muscular planes, located in almost all the extension of the rectus abdominis muscle on the right, measuring in its greater axial axes 8.6 x 6.0 cm. Patient did not present drop of the hemoglobin levels nor hemodynamic instability. The diagnosis of hematoma of the rectus sheath was performed and conservative management was elected. Rectus sheath hematoma (RSH) is an uncommon cause of acute abdominal pain and can be life threatening if diagnosis and management is delayed. RSH occurs due to the accumulation of blood within the rectus abdominis sheath secondary to bleeding from damaged inferior or superior epigastric arteries, or through direct tears of the rectus abdominis muscle. Abdominal pain and fall in hemoglobin with presence of risk factors  constitute important clinical clues to the diagnosis. Management is guided by the size of the haematoma and haemodynamic stability of the patient. Clinicians should consider RSH in the differential diagnosis of acute abdominal pain.


Idiopathic pneumoperitoneum (IP) is a rare phenomenon which merits special attention. It is defined as free intraperitoneal air which is not caused by viscus perforation and does not require surgical intervention. It is generally considered a diagnosis of exclusion and often poses a genuine diagnostic dilemma.We present an unusual case of persistent pneumoperitoneum with no identifiable cause which was found incidentally on a computed tomography (CT) scan in a patient with chronic cough and no prior surgical history. Serial consults revealed no abdominal symptoms or signs. He was managed conservatively and remains asymptomatic despite having a persistent IP on serial radiology.To our knowledge, our case is the first in the literature of an idiopathic pneumoperitoneum that persisted on subsequent radiology and was managed successfully without surgical intervention. This is a highly important case for all practicing general surgeons to learn from as knowledge of this phenomenom may help avoid unnecessary surgical intervention and potential morbidities associated with this.IP is a diagnosis of exclusion which should only be made after surgical and non-surgical causes have been outruled. In the absence of signs of peritonitis and evidence of gastrointestinal perforation on CT, a conservative approach is warranted, allowing patients to avoid unnecessary surgical intervention. In overWe present an exceedingly rare case of persistent IP in a 71-year-old patient which was found incidentally on a thoracic CT scan during investigation of chronic cough that has persisted on serial radiology. Our case is unique as we demonstrated a persistent pneumoperitoneum at 4 month interval which was managed conservatively successfully. The following case has been reported in line with the SCARE criteria .2A 71-year-old Caucasian male presented to the emergency department with productive cough and dyspnoea on a background of recurrent lower respiratory tract infections (LRTI). Of note, he had no prior history of abdominal surgery. He reported being a lifelong non-smoker. His physical examination was unremarkable and his vital signs were all within normal limits. Laboratory investigations revealed a CRP of 102 nmol/L but were otherwise within normal ranges. His chest radiograph demonstrated no gross abnormality. He was initially treated as a presumed LRTI with intravenous antibiotics. A CT thorax demonstrated multiple pockets of sub-diaphragmatic intraperitoneal free air just anterior to the liver and close to the gastric antrum, raising concern for gastric perforation . Despite3Pneumoperitoneum is typically thought to represent a surgical emergency. This is with good reason, as over 90% of reported cases are due gastrointestinal perforation ,6. PneumNon-surgical pneumoperitoneum (NSP) is found less frequently . Intra-tWe present a rare case of an incidental finding of a pneumoperitoneum with no identifiable cause that has persisted on radiological follow-up 4 months after it was initially detected. What is unique is our case is the persistence of pneumperitoneum over a period of months in a clinically-well and asymptomatic patient. Only a handful of cases of in the literature described a recurrent pattern of IP, but in these cases, all underwent negative exploratory laparotomies, while ours was successfully managed conservatively ,5,13. ToSuch a finding certainly poses a significant challenge to the clinician with regards to investigation and management, particularly as the initial concern upon finding a pneumoperitoneum is of an acute gastrointestinal condition requiring urgent surgical intervention ,8,9. TheA detailed history and examination is essential in distinguishing surgical from nonsurgical pneumoperitoneum, thus avoiding unnecessary surgical intervention ,9,14. Th4IP is a diagnosis of exclusion which should only be made after surgical and non-surgical causes have been outruled. We describe a rare case of persistent IP which was successfully managed conservatively. Our case is unique as the pneumoperitoneum persisted radiologically at a 4 month interval and was successfully managed conservatively. Although pneumoperitoneum is often associated with significant intra-abdominal pathology, in those without clinical signs of peritonitis and without evidence of gastrointestinal perforation on contrast-enhanced abdominal CT, a conservative approach is warranted, thus allowing patients to avoid unnecessary surgical intervention.No funding was sought or received for the purposes of this study.Not applicable. This case report is exempt from ethical approval in our institution.Fully informed written consent was obtained from the patient and documented in the medical notes. No identifying information has been used in this article.All authors read and approved the final manuscript.S Hoashi identified the subject and treated the patient.D Toomey treated the patient and acted as senior author.E Saad performed the literature review.E Hannan wrote the final draft and assisted in the literature review.This is a case report and does not refer to research involving human studies.Mr Enda Hannan.Not commissioned, externally peer-reviewed.The authors declare no conflict of interests.


Aneurysmal bone cysts rather than local aggressive lesions of the bone which may arise in any part of the axial or appendicular skeleton. Although several theories are available in the literature, the pathogenesis is still conflicting. We report an exceptional case of an aneurysmal bone cyst in the distal femur of a female cerebral palsy patient who underwent bilateral distal femoral derotational osteotomy and plate-screw fixation operations when she was 11 years old. Twenty-four months after the operation, radiographs showed a cystic lesion in the distal portion of the right femur around the osteotomy site. The diagnosis of Aneurysmal Bone Cyst (ABC) was made and the lesion was treated by curettage with cement application. After 36 months of follow-up, there was no recurrence. This is the first case reported in literature which raises the possibility that an osteotomy could be a cause in the development of an aneurysmal bone cyst. The etiology of ABCs is still conflicting. Generally, ABC exists as a primary bone lesion. However, a predisposing lesion is present in 30% of the cases; a finding suggesting that ABCs are reactive lesions to other pathological changes, rather than a distinct de novo tumour. Moreover, trauma may also act as a pathogenic trigger for the development of ABCs2. The purpose of this case report is to present an exceptional case of an ABC of the distal femur occurring after a femoral derotational osteotomy.Aneurysmal bone cysts (ABC) are benign but not local aggressive lesions of bone which most commonly occur within the first two decades of life, with a slight female predominance. Although these lesions are seen predominantly around the knee joint, any part of the axial or appendicular skeleton may be involvedAn 11-year old girl was referred to our department with a ten-year history of difficulty in walking diagnosed as cerebral palsy (CP). The history obtained revealed that the patient was followed up regularly by paediatric neurologists for her CP, and she had multiple botulinum injections when she was 8 years old. The physical examination based on the walking analysis illustrated limited range of motion in addition to 55° of increased femoral anteversion in either hips. From the data of walking analysis, physical examination and radiological findings, the increased femoral anteversion and excessive soft tissue contractures due to CP were considered the primary reason for her walking difficulty. To improve her gait pattern, a combination of soft tissue procedure and distal femoral derotational osteotomy were planned.Under general anaesthesia, the tendons of adductor longus, iliopsoas, semitendinosus, semimembranosus, gracilis, and gastrocnemius were released from the musculotendinous junction bilaterally. Bilateral distal femoral derotational osteotomy was then performed just above the metaphysis level with a lateral approach, and osteosynthesis was obtained by dynamic compression plate and screw fixation. Bilateral long leg casts were applied postoperatively to maintain osteosynthesis. The patient was discharged on the third post-operative day, casts were removed one month later and routine rehabilitative physiotherapy was instituted.At one-year routine follow-up, there was complete union of the osteotomy. Although pre-operative radiographs of the right knee showed no evidence of any cystic or tumoral lesions around the distal femur , the secIn consideration of the possible progression of the lesion and the risk of fracture associated with ABC, an operation for plate removal, curettage, and cementing procedure was performed under general anaesthesia. Histopathological examination of the curettage specimen obtained during surgery, reported as containing irregular blood-filled chambers with islands of bone and fibrous tissue, re-confirmed the diagnosis of ABC . No recuThe most significant finding in the present study was that the previous osteotomy could be an underlying cause in the development of an aneurysmal bone cyst and this case is being presented as the first such case in the literature.2. However, current data suggests that two thirds of all ABCs are primary bone lesions with a neoplastic nature3. Furthermore, some studies suggested that ABCs may occur as a result of a hemorrhage or degeneration in patients with other bone lesions such as giant cell tumor, chondroblastoma, osteoblastoma, non-ossifying fibroma2. Additionally, it has been claimed that there may be a role of trauma as a trigger in the pathogenesis of ABC2. But, a limited number of studies have addressed such cases and the role of trauma in the pathogenesis is still poorly understood.In previous studies, available data regarding the pathogenesis of ABC remain controversial. Numerous theories have been proposed to explain its pathogenesis, ranging from post-traumatic, reactive vascular changes to genetically predisposed bone tumors. Traditionally, ABCs are thought to arise from local circulatory disturbances leading to increased venous pressure and production of local hemorrhage3. However, complications related to open surgery and high recurrence rates after curettage have resulted in the evolution of less invasive techniques and adjuvant modalities. Common adjuvant agents used in order to minimise the risk of recurrence are bone cement, high-speed burr, cryotherapy, phenol and argon beam3. Recently, less invasive surgical techniques including curopsy, arterial embolisation, radiotherapy, sclerotherapy and novel medical treatments such as denosumab and bisphosphonates have been investigated in order to avoid complications related to aggressive surgical procedures3. However, there is a lack of high-evidence study in the literature regarding the efficacy of these emerging modalities and their usage depends on the clinician’s preference. In our case, the patient was successfully treated with curettage of the lesion and application of cement without any complication or recurrence.Traditional and standard treatment of ABCs include curettage of the lesion and reconstruction of the defect area with bone graft2. Even though the knowledge is limited to a few case reports, surgical trauma also might play a trigger role for ABC formation. Dagher et al reported a case of an ABC which appeared five years after repairing an acute anterior cruciate ligament rupture in the distal femoral metaphysis. The patient was treated with curettage and bone grafting. No recurrence was seen after ten months of follow-up4. Even though ABC was seen after surgery in this reported case, it is not possible to claim a certain causative relation between ABC formation and surgical trauma since there is a prior trauma which might also have played a role. Yamamoto et al reported another case of an ABC in the distal humerus which occurred after resection of an intra-articular nodular fasciitis of the elbow. The authors claimed that ABC and nodular fasciitis are characterised by similar genes and that ABC occurred predominantly secondary to nodular fasciitis. However, they admitted that surgical trauma cannot be excluded in the pathogenesis of this case5. In our case, surgical trauma was the sole possible etiologic factor for the formation of the ABC.Trauma has been reported to be a possible etiological factor of ABCs due to the reactive vascular changes and the remodeling process of the traumatised tissuesAn argument can be made that the present study, which addresses the issue of ABC in the distal femur after femoral derotational osteotomy, makes an important contribution to the existing literature; because, to our knowledge, osteotomy is not yet cited as a cause of ABC in the current literature. We treated this patient successfully with curettage and cement application. After 36 months follow-up we did not see any recurrence of the ABC.ABCs are mostly seen as primary bone lesions, however, orthopaedic surgeons should keep in mind that postsurgical trauma like osteotomy may be an etiologic factor in the development of ABCs, as in this report of our case.


The differential diagnosis and therapy of retroperitoneal masses suspicious for soft tissue tumors remains difficult and needs individual decisions as to the best management of patients. We report an unusual case of retroperitoneal schwannoma (RS).We report on a 57-year-old female patient with a retroperitoneal space-occupying lesion with displacement, but no infiltration of surrounding vessels, especially the inferior vena cava. As presence of malignancy could not be completely excluded by imaging and biopsy, we performed an open resection of the tumor with plastic reconstruction of the right renal vein. Curative resection of the tumor could be achieved and benign RS verified.Preoperative work-up, including a broad spectrum of differential diagnoses, and consecutive appropriate surgical resection are challenging. Characteristic features of the pre-therapeutic diagnostics of retroperitoneal schwannomas are discussed and differential diagnostic considerations as well as surgical therapeutic options are outlined.Deeper understanding of retroperitoneal schwannomas gained from preoperative radiologic diagnostics may serve to underline the need for targeted biopsy. This and its histopathological examination necessitate expert interventional radiologists and pathologists. Thus, even the pre-therapeutic diagnostic management should be performed in a specialized center. If doubts remain regarding the entity of the retroperitoneal lesion and findings are still controversial after thorough assessment, surgical oncologic resection represents the therapy of choice, if the surgical risk is adequate. Soft-tiA conclusive pre-therapeutic radiologic differentiation between benign and malignant retroperitoneal space-occupying lesions is challenging and not always possible, even though imaging criteria as, e.g. the configuration of the borderline of a space- occupying lesion with regard to the surrounding tissue, or of their internal structure(s) may give rise to the suspicion of a specific lesion . In acco2A/B). [18 F]FDG-PET/CT confirmed the presence of the described right lateral paraaortic lesion, situated at the level of the renal hilus with a malignoma-typical increase of metabolic activity in the marginal areas . The tumor together with the intact capsule was resected curatively (R0-resection). The postoperative course was uncomplicated and the patient was discharged from hospital on postoperative day 8. No adjuvant therapy was indicated and regular aftercare only was recommended by the interdisciplinary tumor board.The observed displacement of both, the inferior vena cava and the right renal vein, as well as the malignoma-typical increase of metabolic activity in the [18 F]FDG- PET/CT led to the assumption – in spite of negative histology - that the presence of malignancy could not be excluded. Consecutively, we performed an open resection of the tumor with plastic reconstruction of the right renal vein, while the inferior vena cava could be completely preserved, as it was unaffected A. MacrosFig. 43Approximately 90% of schwannomas occur solitarily and sporadically, while about 4% of these are associated with neurofibromatosis type 2 . The cli18F] FDG uptake by non-gastrointestinal schwannomas and tumor size, cellularity as well as microvascular density in the [18F]FDG-PET or [18F]FDG-PET/CT has been controversially discussed [18F]FDG-PET/CT scan confirmed the presence of a para-aortic lesion located on the right, at the level of the renal hilus with a malignoma-typical increase in metabolic activity in the border region. There was no evidence for metastatic spread.Differential diagnosis is challenging – and the meticulous assessment, whether to operate or to pursue conservative management - is to be outlined in our report. Specific laboratory or tumor markers do not exist. The detection of a retroperitoneal space-occupying lesion in the course of an ultrasound examination for clarification of upper abdominal pain, as performed in our patient, is the exception rather than the rule. Frequent CT-morphologic findings include circumscribed round and oval tumors, or spindle-shaped soft-tissue tumors with a lower density than, e.g. muscle tissue, which is characterized by a higher myelin and consequently also by a higher lipid content . Althougiscussed ,16. In tBoth, the image-supported biopsy and the histopathologic evaluation therefore were of utmost relevance for further pre-therapeutic diagnosis. In the performance of a differential diagnosis, consideration should be given not only to space-occupying lesions of neighboring retroperitoneal organs and structures, in particular to those arising from the adrenal glands, such as pheochromocytoma, the pancreas, the sympathetic trunk (e.g. paragangliomas), or the inferior vena cava (e.g. leiomyosarcoma), but potential hemato-oncologic conditions (e.g. lymphoma) or retroperitoneal, extragonadal seminoma also require careful evaluation as differential diagnoses. In all of the described instances and, in particular in the presence of the above mentioned suspected diagnoses, the performance of a pre-therapeutic biopsy is an essential prerequisite, even in cases of apparent resectability, to avoid the (primary) surgical intervention. However, preoperative biopsy is contraindicated, if pheochromocytoma is suspected.Although findings were controversial in our patient, we decided to operate. The lesion should always be resected oncologically, if there are any doubts of malignancy left and the surgical risk is adequate. In view of the poor response of retroperitoneal schwannomas to chemo- or radiation therapy, primary surgery represents the procedure of choice. The surgical therapy always requires complete removal of the tumor, inclusive of the capsule and the associated pseudocapsule (neovascularization zone). The described removal is also indispensable to prevent the development of local recurrence in cases where the preoperative biopsy does not permit the definitive exclusion of malignity. In our patient, the inferior vena cava was displaced, but not infiltrated and the tumor was dissected without complications. The right renal vein required reconstructive plastic surgery, which eliminated the need for a simultaneous en-bloc resection of the respective vessels and the right kidney itself. Postoperative aftercare is indicated, in particular since the incidence of metastases and local recurrence has also been reported for primary benign retroperitoneal schwannomas ,17.4Retroperitoneal schwannomas (RS) are a rare entity and the preoperative diagnostic workup represents a challenge, because image-morphological properties of these lesions are frequently unspecific and radiologic and nuclear medicine diagnostic procedures might be discrepant.Deeper understanding of retroperitoneal schwannomas gained from preoperative radiologic diagnostics may serve to underline the need for targeted biopsy. This and its histopathological examination necessitate expert interventional radiologists and pathologists. Thus, even the pre-therapeutic diagnostic management should be performed in a specialized center. Correct diagnosis exerts an influence on the pre- and intraoperative therapy algorithm, thereby obviating the need for possible extended multivisceral resections.If doubts remain regarding malignancy of the retroperitoneal lesion and findings are still controversial after thorough assessment, surgical oncologic resection represents the therapy of choice, if the surgical risk is adequate.The authors declare no financial competing interests related to this work.There are no sponsors and there was no special funding for this case report. We acknowledge support from the German Research Foundation (DFG) and Leipzig University within the program of Open Access Publishing.The ethical approval for the publication of this case was exempted by our institution because all of the data were collected from clinical records and imaging systems for routine perioperative planning.Written and informed consent was taken from the patient for publication of this case report and the accompanying images.Writing, editing, review of the literature: IG, SN, RT, CW.Performed surgery: IG, SN, HS.Performed diagnosis: SU, KTH, OS, KS, CW.Editing: IG, RT, CW.All authors have read and approved the manuscript.Not applicable.Prof. Dr. Ines Gockel, MBA.Not commissioned, externally peer-reviewed.


Idiopathic spontaneous intraperitoneal hemorrhage (ISIH) is a rare event associated with high mortality. There have been multiple case reports of spontaneous rupture of middle colic pseudoaneurysms in the literature. Herein, we present a case of a 51-year-old female that presented with spontaneous rupture of the middle colic artery and associated massive intraabdominal hematoma without findings of a pseudoaneurysm. The patient underwent a computed tomography (CT) scan as an outpatient 24 hours prior to the onset of the bleeding due to abdominal pain without findings of hematoma or aneurysm of the mesenteric vessels. Subsequently, the patient underwent emergent exploratory laparotomy with findings of a massive hematoma in the lesser sac and spontaneous bleeding from the middle colic artery that was ligated. The patient had an uneventful postoperative course and fully recovered. To our knowledge, this is the second reported case of idiopathic bleeding from the middle colic artery without evidence of a pseudoaneurysm based on a current review of the literature. Idiopathic spontaneous intraperitoneal hemorrhage (ISIH), also referred to as abdominal apoplexy, is a rare and potentially fatal condition  that is The patient is a 51-year-old female who presented to the emergency department with the sudden onset of generalized abdominal pain and distention. She had also reportedly fainted at home, according to a family member. The patient denied any recent trauma, however, she did mention diarrhea for the past 24 hours and vague abdominal pain for one week prior to the episode. The patient reported being seen earlier in the day by her primary care provider, and that she had a computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast done as part of her workup, with findings of a mildly dilated stomach and no findings of an intra-abdominal hematoma. Her past medical history was notable for breast augmentation, but no history of vasculitis, connective tissue disorder, or hypertension was noted. On admission, she was hypotensive, with a blood pressure of 85/40, a pulse of 98, and a body temperature of 96.3°F. On physical examination, her abdomen was distended and tenderness was diffusely present on palpation. Her complete blood count results were as follows: white blood cell count 19 × 109/L, hemoglobin 10.4 g/dL, and hematocrit 30.9%. The prothrombin time, partial thromboplastin time, platelets, liver function tests, amylase, and lipase were within normal limits. The patient received crystalloid resuscitation and underwent a CT scan of the abdomen and pelvis without contrast, which demonstrated a 6 cm x 15 cm mixed density collection around the region of the pancreas and adjacent mesentery, displacing the stomach anteriorly  . ConsideThe treatment of idiopathic spontaneous intra-abdominal hemorrhage revolves around patient resuscitation and management of the source of bleeding. In case of a ruptured aneurysm of the middle colic artery, surgical management includes an emergency laparotomy, arterial ligation, and resection of the aneurysm. Endovascular embolization has been suggested as a safe and less invasive alternative approach. To our knowledge, this is the second reported case of idiopathic bleeding from the middle colic artery without evidence of a pseudoaneurysm, based on a current literature review .Idiopathic spontaneous intraperitoneal hemorrhage (ISIH), also referred to as abdominal apoplexy, is a rare and potentially fatal condition whose etiology remains unclear in most cases. We present the case of a 51-year-old female with ISIH that, to our knowledge, is the second such case without evidence of a pseudoaneurysm, based on a current literature review. We hope that by presenting this case, we may add to the body of literature to further elucidate improved methods of diagnosis and early recognition and to reduce morbidity in future cases.


Undifferentiated pleomorphic sarcoma (UPS) as a primary cardiac tumor is rare, with extremely poor prognosis owing to high recurrence and invasion. We encountered a patient who presented with a primary cardiac tumor incarcerating the mitral valve and who was in a shock state.A 41-year-old man was transported emergently to our hospital owing to acute respiratory distress and hemoptysis. He was diagnosed with acute left heart failure caused by a large mass in the left atrium (LA) that obstructed cardiac blood flow, as revealed by imaging study findings, and he underwent an emergency open-heart surgery for tumor resection. He was pathologically diagnosed with UPS invading the muscle layer of the LA. However, after receiving combined therapy for local recurrence and distant metastasis, including proton beam radiotherapy and chemotherapy with molecularly targeted drugs, he could return to work for 2 more years after surgery.In this study, we reported the case of a patient who was in a state of shock state owing to the presence of UPS in the LA. The patient underwent an emergency surgery and received combined therapy. He survived for 2 more years after an initial diagnosis, without active local recurrence and distant metastasis. In this study, we report the case of a patient who was in a shock state owing to the presence of a large undifferentiated pleomorphic sarcoma (UPS) in the left atrium (LA). The patient underwent an emergency surgery and received combined therapy. He survived and returned to work for 2 more years after surgery despite local recurrence and distant metastasis.A 41-year-old man suffering from the bilateral knee and ankle arthralgia for several months was transported emergently to our hospital owing to acute respiratory distress and hemoptysis. Upon arrival, he was in a shock state. Chest roentgenography revealed severe pulmonary congestion; cardiac echogram revealed a large mass in the LA that incarcerated into the mitral valve. Additionally, chest computed tomography (CT) revealed a tumor in the LA; thus, he was diagnosed with acute left heart failure caused by the mass that obstructed cardiac blood flow , which invaded the atrial muscular layer Fig. . SubsequHowever, local recurrence in the LA was observed on positron emission CT (PET) and other imaging studies 7 months after surgery Fig. a. A tumoAfter 6 months, the second local recurrence at a different site in the LA and distant metastasis to the left adrenal gland were simultaneously observed on the results of several imaging tests. In the left adrenal gland, a large solid tumor with an irregular surface and abundant blood flow was observed on enhanced CT scan Fig. a, and reTwenty-seven months after surgery, no active tumor was noted on any imaging result, and he returned to work without symptoms, including arthralgia of the lower limbs.UPS as a primary cardiac tumor is extremely rare and accounts for approximately 1.7% of all cardiac tumors . The proIn our case, the patient presented with acute and severe left heart failure and underwent emergency surgery. However, local recurrence occurred twice in the LA, and distant metastasis to the left adrenal gland was observed. Next, he was treated with proton beam radiation for the recurred tumors, and oral chemotherapy with tyrosine-kinase inhibitor (pazopanib), which is the only molecularly targeted drug authorized for treatment for sarcoma in our country, was additionally selected to suppress tumor activity , 6. WhilAn effective treatment for UPS except for surgical resection has not been thus far reported. Proton beam radiation is described as available for malignant tumors, including sarcoma . HoweverIncidentally, our patient was suffering from arthralgia of both lower limbs several months before the initial diagnosis, and local recurrence and metastasis were observed; however, his pain disappeared after surgery and radiotherapy. There were no specific tumor markers on the blood test that indicated an activity of UPS. Thus, arthralgia may be a significant indicator of the tumor activity on clinical scenes.A patient with a primary cardiac tumor of UPS, which caused acute heart failure, was rescued by an emergency open-heart surgery. After receiving combined therapy for local recurrence and distant metastasis, including proton beam radiotherapy and chemotherapy with molecularly targeted drugs, he could return to work. Then, he survived for 2 more years after surgery under control of UPS.


Endobronchial lipomatous hamartoma with hilar extension is a very rare benign disease of the airway. A 78-year-old male with coronary artery disease and a coronary stent presented with worsening shortness of breath. The workup included a computed tomography (CT) scan of the chest that showed a nearly obstructive lipomatous endobronchial lesion in the right bronchus intermedius with 2 cm hilar extension of the lipomatous mass. We performed a robot-assisted resection of the lipomatous mass using the “five on a dice” port placement for the Da Vinci Xi robot. In order to fully obtain the exposure necessary to resect the mass and the endobronchial lesion, we performed a right lower lobe superior segmentectomy. The margin of the parenchyma was determined using indocyanine green angiography and divided with the robot blue load stapler. The mass was carefully separated from the pulmonary artery and divided from the endobronchial portion. The frozen section showed that it was a lipomatous mass without any signs of malignancy. We then resected the endobronchial mass with robot scissors and the pathology was consistent with benign lipomatous hamartoma. Two sutures were placed as retraction sutures to pull the airway away from the pulmonary artery. We then closed the opening with absorbable sutures in an interrupted fashion. There was no air leak at the end of the case. The patient went home on postoperative day 3 without any complications and with his shortness of breath resolved. The use of the Da Vinci Xi robot allows for a successful minimally invasive method of resecting a rare endobronchial hamartoma with hilar extension. Pulmonary hamartomas represent approximately 70% of all benign lung tumors. Endobronchial lesions represent a much rarer variant of the disease. Many of these endobronchial lesions are amenable to endobronchial resection -4, howevA 78-year-old male patient with coronary artery disease status post coronary stent placement was found to have a lung nodule on the chest radiograph at that time. The patient underwent a computed tomography (CT) scan, and bronchoscopy, and was found to have a 9 mm fatty endobronchial lesion in the bronchus intermedius above the middle lobe with 2 cm extraluminal fatty lesion into the right hilum. An endobronchial ultrasound with biopsy of the mass was performed, which showed benign bronchial epithelial cells. Since endobronchial resection of the mass would lead to a large defect in the right bronchus intermedius, the decision was made to perform robotic-assisted resection of the lesion Video .We used the Da Vinci Xi robot to perform resection of the endobronchial lesion and hilar mass with right lower lobe superior segmentectomy to remove the lesion. The patient had a “five on a dice” port placement for the operation , 7. FirsIn order to reconstruct the airway, we placed two 3-0 vicryl stay sutures at the proximal and distal ends of the airway and placed the suture through the posterior ports to pull the airway posteriorly away from the main pulmonary artery. We closed the opening with 4-0 PDS (polydioxanone) in an interrupted fashion eight times. This provided good closure of the opening. We performed a bronchoscopy that showed no abnormalities and the air leak test demonstrated no appreciable air leaks.The patient went home on postoperative day 3 without any complications. The final pathology report was lipomatous hamartoma.Pulmonary hamartomas represent the most common benign lung tumor, however, the endobronchial variant is very rare. Many of these lesions may be resected via bronchoscopy, but the large size and hilar involvement of the tumor precludes safe removal of the tumor through an endoscopic procedure. Surgically, these tumors have been removed with thoracotomy , 9. We fThe robot allowed us to perform resection of rate endobronchial lipomatous hamartoma with hilar extension with superior right lower lobe segmentectomy and airway reconstruction in minimally invasive fashion that translated to excellent patient outcome.


Primary schwannoma is extremely rare in the trachea, and its optimal treatment has not yet been established. Previous literature have indicated that traditional resection by thoracotomy is an effective surgical procedure but with huge trauma, and endoscopic excision is a minimally invasive surgical method but with possibility of recurrence. Window resection was usually utilized for selected patients with trachea invasion by thyroid carcinoma, but video-assisted thoracoscopic window resection for trachea schwannoma has not been reported previously.A 23-year-old woman was admitted to hospital due to dyspnea, coughing and wheezing that had persisted for 2 months with aggravation for 1 week.Chest computed tomography (CT) scan revealed a well-circumscribed soft-tissue mass located on the right lateral posterior wall of the trachea. Bronchofibroscopy (BFS) showed a whitish, smooth and round mass with a wide base in the trachea. Immunohistochemical staining demonstrated cells labeled with Vim (+), S-100 (+), SOX-10 (+), SMA (–), CK (–). Histopathological examinations showed that the mass was a schwannoma.The tumor was nearly completely excised via BFS, but relapsed 2 times at 12 days and 3 weeks after endoscopic resection. Finally, the patient underwent video-assisted thoracoscopic window resection of trachea.The patient recovered rapidly and no recurrence was observed over 6 months of follow-up.The treatment of tracheal schwannoma depends on the characteristics of tumor and the condition of patient. Surgical resection is a preferred alternative for sessile or transmural tumors and recurrence after endoscopic excision. Tracheal window resection by video-assisted thoracoscopy is beneficial for some appropriate patients with a small and sessile tumor. Previous literature have indicated surgical resection and endoscopic excision have been successfully utilized in the treatment of this disease,,3 especially endoscopic surgery is widely used with the advance of equipment and technology. It is undeniable that endoscopic surgery has the advantages of less trauma and faster recovery, but the possibility of local recurrence should not be ignored.–4 Circumferential resection and end-to-end anastomosis of the trachea by thoracotomy is usually adopted in the treatment of tracheal schwannoma,,2 but the surgery is hugely traumatic and risky, and may lead to severe complications and bilateral recurrent laryngeal nerve injury. Window resection was usually used for selected patients with trachea invasion by thyroid carcinoma with favorable outcomes,,6 but it has not been reported in the treatment of tracheal schwannoma so far. Therefore, we attempted to resect a primary tracheal schwannoma, following recurrence after endoscopic excision two times, by video-assisted thoracoscopic window resection. In addition, we emphasize the selection of treatment along with a literature review.Schwannomas originate from Schwann cells and they are benign lesions usually. Primary schwannoma is extremely rare in the trachea being more frequently reported in the lungs and bronchi and the exact frequency is unknown.2The informed consent for publication of this case report and images were obtained from the patient. Since the personal information and images presented in the article are entirely unidentifiable, the Ethics Committee of our institution waived the requirement for ethical approval.3A 23-year-old woman was admitted to department of respiratory and intensive care unit on September 29th, 2018 due to dyspnea, coughing and wheezing that had persisted for 2 months with aggravation for 1 week obviously. The patient denied chest pain, fever and palpitation. Physical examination revealed a temperature of 36.4°C, a heart rate of 80 to 90 beats/minute, a blood pressure of 113/76 mmHg, a respiratory rate of 16 to 20 breaths/minute and a transcutaneous oxygen saturation 99% on room air. Lung auscultation showed wheezing in the both inspiratory and expiratory phases and a prolonged expiratory phase on the cervical trachea. In the Outpatient Clinic, the chest x-ray did not show any abnormality 1 month ago, but the following chest plain computed tomography (CT) scan revealed a ∼1.5 cm, well-circumscribed soft-tissue mass located on the right lateral posterior wall of the trachea at the level of the brachiocephalic veins  searches of the PubMed, there were only 51 cases of primary tracheal schwannoma were identified from 1950 to 2013, and after that to October 2018, only 9 cases had been reported in addition.,8–15Schwannoma is a benign tumor arising from Schwann cells of the peripheral nerve, and it usually occurs in the head, neck, retroperitoneum and extremities. The tumor is more frequently reported in lungs and bronchi but not trachea in respiratory system.,14,16 the therapeutic method of tracheal schwannoma includes surgical resection and endoscopic excision based on the size, the location and the extension of the tumor. According to the tumor's site and extension, Kasahara et al proposed a classification of the pulmonary schwannomas. They divided the pulmonary schwannomas into 2 types: central type and peripheral type. According to the relationship between tumor and tracheal luminal space, the central type is classified in to the following 2 subtypes:Because of the rarity of tracheal schwannoma, its optimal treatment has not yet been established. In the published literature,(1)intraluminal type which tumors exist only in the intraluminal space and(2)transmural type which tumors occur in both intraluminal and extraluminal spaces Table .–4 In these 4 excellent case reports with literature review, about 30% patients were underwent endoscopic excision and 70% patients were underwent surgical resection by traditional thoracotomy. For tumors with pedicle and only in intraluminal space (pedunculated type),,18 and in those patients with surgical contraindications or in emergency situation due to severe obstruction by tumor, endoscopic excision may be an appropriate option for radical resection or debulking surgery relieving airway obstruction. For tumors with broader base (sessile type) or both intraluminal and extraluminal , and in those patients without surgical contraindications, surgical resection is a preferred alternative. However, there were some recurrent cases with endoscopic treatment (the recurrence rate from 4% to 21%),,3 while there was no recurrent case with surgical resection in these reports. The results indicated that endoscopic treatment had the possibility of local recurrence, therefore, careful selection of treatment for appropriate patients should be considered for reducing the risk of recurrence. Recurrent case after endoscopic excision was strongly recommended surgical resection rather than endoscopic treatment repeatedly.,11The treatment for primary tracheal schwannoma should consider the characteristics of tumor and the condition of patient. In the published literature, surgical resection and endoscopic excision are utilized in the treatment of tracheal schwannoma mainly.In the present patient, a transmural tumor both in intraluminal and extraluminal space of trachea was detected by CT initially, and it was confirmed and partial resected via BFS to relief severe airway obstruction. It was seen to be a reasonable treatment option due to the urgent condition. According to the above principles, in such a case with high risk of recurrence, surgical resection of the residual tumor should be planned in time. Unfortunately, the patient was not to be advised surgical resection. Consequentially, the tumor recurred 2 times, respectively, in 12 days and 3 weeks later after endoscopic excision. Finally, the patient was admitted to our hospital and received surgical resection without relapse in 6 months of follow-up.,11 but this approach is a high-risk surgery due to intraoperative transthoracic tracheal intubation, difficult tracheal end-to-end anastomosis and huge trauma, and it may lead to recurrent laryngeal nerve injury. Window resection was usually used for trachea invasion by thyroid carcinoma with good outcomes,,6 and the above problems were overcome. This is useful if tumor invasion is less than one quarter of the tracheal circumference and <2 cm in its vertical dimension. In this case, though the tumor occupied about one third of the tracheal circumference, but it was a benign tumor and only 1.4 cm in its vertical dimension. Therefore, we attempted to performe video-assisted thoracoscopic tracheal window resection and longitudinal suture in this case and succeeded. Preliminary identification of this method could resect a small tracheal schwannoma completely and safely. Compared to circumferential resection by traditional thoracotomy, this surgical procedure overcomes intraoperative transthoracic intubation and reduces the trauma and the risk of surgery. In addition, the patient did not need to maintain neck flexion and recover quickly. But this approach has some limitations. For example, it is not suitable for the large tumor or the tumor involved carina. We consider that the surgery approach is suitable for the tracheal tumors which occupy less than one third of the tracheal circumference and <1.5 cm in its vertical dimension above the carina. Long-time follow-up is required to evaluate outcomes and complications, such as recurrence and airway stenosis. And this approach needs to be further explored by studies of larger patient samples.Tracheal resection and end-to-end anastomosis by traditional thoracotomy are usually adopted in tracheal schwannoma,–21 So, whether malignant transformation of tracheal schwannoma after stimulation , long-time follow-up is needed and surgical resection is recommended after endoscopic excision with recurrence.Meanwhile, we found the pathology had more mitotic figures and the ki-67 index raised from 3% to 20% after endoscopic treatment repeatedly. Though malignant transformation of schwannoma after APC, cryotherapy or electrosurgical snaring has not been reported, but there were some cases about malignant transformation of vestibular schwannoma spontaneously or after radiation.5Primary tracheal schwannoma is extremely rare. The treatment depends on the characteristics of tumor and the condition of patient. Surgical resection is a preferred alternative for sessile or transmural tumors and recurrence after endoscopic excision. Tracheal window resection by video-assisted thoracoscopy is beneficial for some appropriate patients with a small and sessile tumor, but long-term follow-up is required.Conceptualization: Huihuo Chen, Weibin Wu.Data curation: Huihuo Chen, Kai Zhang, Mingjun Bai, Lijia Gu.Visualization: Mingjun Bai, Haifeng Li, Jian Zhang.Writing – original draft: Huihuo Chen, Jian Zhang.Writing – review & editing: Kai Zhang, Weibin Wu.


Synchronous occurrence of benign cystic mesothelioma and adenomatoid tumor of uterus (UAT) are very rare and few cases have been published in the English literature. They are easily misdiagnosed as malignant by clinicians, due to the lack of reports.A case of benign mesothelial combined with uterus adenomatoid tumor (UAT) in a 48-year-old Chinese woman was reported. Our patient presented with abdominal pain and surgery showed a large subserous mass (12.0 × 11.4 × 9.8 cm) combined with a small intramural solid nodule (2.0 × 1.0 × 1.0 cm), and multiple minute neoplastic growth on the ovary.Due to the patient's symptoms, pathological findings, she was diagnosed with synchronous occurrence of benign mesothelioma and UAT.We treated her with a total hysterectomy and bilateral adnexectomy.The patient is now in stable condition, without any signs of recurrence during 1 year of follow-up.Most mesotheliomas are malignant, synchronous occurrence of benign mesothelioma and UAT are extremely rare. And they are often misdiagnosed as malignancy by clinicians. Our case report can improve the awareness of the disease and avoid excessive treatment. The lesions are limited, but have a high propensity for local recurrence and some reports suggested that adenomatoid tumors of the uterus may coexist. Clinicians often mistakenly believe that it is malignant, so there are often cases of overtreatment, including excessive surgical range, and unnecessary postoperative chemotherapy.Mesothelioma is a very rare tumor of mesodermal origin that covers the surface of the body's organs such as the peritoneum, pleura, and pericardium. Moreover, the malignancy accounts for a large majority, and its main characteristics present as increased cellularity, cytologic atypia, papillary formations, and even invasion. On the contrary, benign mesothelioma is much rarer, multicystic peritoneal mesothelioma (MPM), a type of benign ones, the incidence of which was reported only at 0.15/100000.To improve the clinicians’ awareness of the disease, here we reported a synchronous occurrence case of benign cystic mesothelioma and adenomatoid tumor of uterus (UAT). This case report was approved by the ethics committee of Maternal and Child Health Care Hospital of Ninghai County and Women's Hospital, School of Medicine, Zhejiang University. The informed consent form was signed by patient. The pathology was confirmed by Department of Pathology, Women's Hospital, School of Medicine, Zhejiang University.2A 48- year-old Chinese woman, gravida 4, para 2, was admitted to our hospital due to abdominal pain for 1 month. The clinical features of the patient at baseline were summarized in Table Fifteen years ago, the patient was diagnosed with a uterine myoma, which was about 8 cm in diameter. Instead of undergoing surgery, she opted for regular check-ups. One month ago, she developed abdominal pain and ultrasonography showed a 12 × 11.4 × 9.8 cm heterogeneous mass behind the uterus, with a cystic dark area of 3.4 × 2.3 cm . Secondly, synchronous occurrence of UAT was detected postoperatively in pathology. AT is also a rare benign tumor, with the incidence of 0.04% to 1.74%, and its morphology is similar to that of adenoma, but its origin was considered as mesodermal. Adenomatoid tumors may coexist, suggesting that hysterectomy would be the appropriate choice.Through literature review, only 10 cases of benign mesothelioma have been reported so far. Hatano et al had already summarized 6 cases of benign mesothelioma combined with UAT in 2011. However, because of the risk of local recurrence, whether bilateral adnexectomy need to be performed during surgery requires further investigation, especially in young patients with ovarian metastases. Perhaps the patient's informed choice should be considered before a final decision was made. We treated our patient with a total hysterectomy and bilateral adnexectomy, after obtaining her informed choice. The patient did not receive any postoperative chemotherapy. And she is now in stable condition, without any signs of recurrence during nearly 1 year of follow-up.According to the literature review, postoperative chemotherapy was not generally recommended, although intraperitoneal chemotherapy has been reported in extremely rare cases.4Synchronous occurrence of benign mesothelioma and UAT are extremely rare and are easily misdiagnosed as malignancy by clinicians. Understanding these diseases can improve the skills of both clinicians and pathologist.We are grateful to the patient, who gave her informed consent for publication.Data curation: Jin Cen, Yuan-Ming Shen.Supervision: Xue-Qian Qian, Xiao-dong Cheng.Writing – original draft: Ping-Rong Shen.Writing – review & editing: Xue-Qian Qian, Xiaoyun Wan.


Salivary gland tumors are relatively common in the junction of the hard and soft palate area of the oral cavity. Pleomorphic adenoma is considered the most common benign salivary gland tumor in this location. Some of the rarer subtypes of this tumor may have a misleading clinical presentation. Recognition of these variants is important since long-standing pleomorphic adenomas have the potential to become malignant.  A healthy 24-year-old male was referred for a painless, large, slowly growing, exophytic swelling of the right hard and soft palate. Interestingly, the lesion was yellowish in color and soft to palpation, suggestive of an innocuous lipoma or cystic lesion. An incisional biopsy was performed and the diagnosis was consistent with pleomorphic adenoma with a significant adipose tissue component. The patient was referred to an oral surgeon and underwent a complete surgical excision. Upon two-year follow-up, the patient is doing well with no recurrences.  This case highlights a rare microscopic variant of pleomorphic adenoma with altered clinical presentation that led to an erroneous clinical diagnosis. The importance of taking a biopsy for definitive diagnosis and appropriate management is reinforced. Pleomorphic adenoma (PA) is by far the most common benign salivary gland neoplasm, accounting for 70-80% of total salivary gland tumors . This tuPleomorphic adenoma is categorized microscopically into 3 broad types: classic, cellular, and myxoid . HoweverIn the following report, we present an uncommon case of a pleomorphic adenoma of the palate that clinically presented in an unusual manner. Due to the significant lipomatous component, the lesion had a yellowish color and felt soft to palpation, leading to an erroneous clinical diagnosis of lipoma. We stress the importance of biopsy, especially utilization of the punch biopsy technique, for definitive diagnosis and the need for oral health care providers to be aware of variations of the typical clinical appearance of pleomorphic adenomas.A 24-year-old male of Asian descent reported to the oral medicine clinic at NYU College of Dentistry. His chief concern was a painless, slowly growing mass on his hard palate that he noticed a few weeks ago. The patient denied any significant medical issues or medications. He also denied any significant family history or any similar lesions in any of his immediate family members. The extraoral examination was within normal limits. Intraoral examination revealed a large exophytic mass of the right hard palate extending to the soft palate, yellowish in color, and soft to palpation . The les3Pleomorphic adenoma (PA) is the most common benign salivary gland tumor with the parotid gland being the most common site. Histopathologically, it is a benign neoplasm with myoepithelial and epithelial components arranged in diverse morphological patterns. The epithelial cells typically form duct-like structures or pools of plasmacytoid myoepithelial cells. The stromal element demonstrates varying degrees of myxoid, hyaline, cartilaginous, or osseous differentiation . A lipomThe term “lipomatous” pleomorphic adenoma was first used by Seifert et al. in 1999. According to Seifert et al., a lipomatous pleomorphic adenoma (LPA) consists of a stroma that is more than 90% lipomatous . In anotOnly a few cases of lipomatous pleomorphic adenomas and pleomorphic adenomas with extensive lipomatous metaplasia have been reported and well documented in the English literature –9.  TablThe histogenesis of the LPA is not yet fully understood. The transformation of myoepithelial cells into fat cells (metaplasia) or the entrapment of adipose tissue during tumor expansion are both possible mechanisms , 6.Pleomorphic adenomas, including the lipomatous variant, are generally treated by complete surgical excision. However, the tumor has a significant tendency to recur. PA recurrence can be related to multiple factors. Incomplete and intraoperative rupture of the tumor capsule, especially in cases of large parotid tumors involving the facial nerve, is an important factor  as is thAs mentioned previously, there is a concern that a long-standing pleomorphic adenoma can undergo malignant transformation. The transformation rate has been reported to range as low as 1.5% and as high as 13.8% ; the WHOIf malignant transformation of a PA to a carcinoma ex pleomorphic adenoma (CA ex PA) does occur, the treatment and prognosis radically change. Surgery followed by radiation treatment is considered as the standard of care for a patient with CA ex PA. Generally, patients with CA ex PA have a poor prognosis with common regional metastases and high mortality . The WHOOur case is also unique in that it is the only case of LPA initially diagnosed through punch biopsy to our knowledge. We advocate this technique as a quick and easy method to obtain the correct diagnosis so that appropriate treatment is not delayed.Since the lipomatous pleomorphic adenoma is a fairly rare neoplasm, there were no reports of recurrence or malignant transformation in any of the reported LPA cases . The patRecognizing the varying presentations of salivary gland tumors is paramount for early diagnosis and treatment of the patient. Pleomorphic adenoma is the most common salivary gland tumor in the oral cavity, and it is of great importance to know its different clinical presentations. Performing a biopsy on any abnormality is the first step for treatment, especially since a lipomatous PA could mimic other more innocuous lesions such as lipoma and be missed on a CT scan due to the high fat content. Obtaining the definitive diagnosis was especially important in this case since long-standing pleomorphic adenomas have the potential to become malignant.