Primary undifferentiated pleomorphic sarcoma (UPS) of the pancreas is an exceedingly rare malignant tumor, with only 15 cases have been reported in the medical literature. At present, clinicians have poor recognition of the tumor, the epidemiology, diagnosis, and treatment of this disease have yet not been established.In this report, we depict the clinical and imaging characteristics of a 37-year-old man presenting with a primarily cystic UPS. The patient complained of epigastric pain and distention over 20 days. Abdominal CT and pancreatic magnetic resonance imaging revealed cystic and cystic solid masses in the pancreatic body and tail. An abdominal ultrasound echogram revealed the mass in the body of the pancreas to be cystic with separation echo inside, and the wall was thick, not smooth. Besides, a hypoechoic mass was seen in the tail area of the pancreas with an inhomogeneous echoic pattern, containing small patches of no echo zone in the central. Microscopically, spindle fibroblast-like cells are arranged in a characteristic storiform pattern with pleomorphic and multinucleated cells. Immunohistochemically, tumor cells were positive for CD68 and vimentin. Seven months postoperatively, he was diagnosed with pulmonary lymph node metastasis and died 5 months later. Combined with this case report, we also reviewed the literature regarding UPS of the pancreas.As we know, this is the first report on ultrasonography findings of pancreatic UPS. Despite there are no distinctive manifestation of UPS, a solid cystic lesion on ultrasonography or a hypodense area in the lesion on T2-weighted imaging, should be considered for differential diagnosis with pancreatic UPS. We believe this article may add some ideas into the diagnosis and therapy of patients with this tumor. Undifferentiated pleomorphic sarcoma (UPS) or previously known malignant fibrous histiocytoma (MFH) is considered the most common type of soft tissue sarcoma. It often occurs in the limbs, trunk, and retroperitoneal tissues. However, it has been rarely observed in the digestive organ . PrimaryA 37-year-old man was admitted to our hospital for further evaluation and treatment of pancreatic body and tail masses. He had been suffering from epigastric pain and distention for more than 20 days. The patient had no history of weight loss. The routine hematological laboratory values including tumor markers, hematocrit and liver function tests were within normal limits. He was evaluated by abdominal ultrasonography (US), computed tomography (CT), and pancreatic magnetic resonance imaging (MRI). The abdominal CT scanning revealed two hypodense masses in the body and tail of the pancreas Fig. A. MRI scAfter consulting with our hospital’s multiple disciplinary team, the patient was initially diagnosed as benign pancreatic mass and underwent a resection of pancreatic body and tail to relieve the symptom. At laparotomy, two cystic masses were seen in the body and tail of the pancreas measured 5 × 4 cm and 2.5 × 2 cm respectively. The mass was completely resected and the specimen was submitted for pathology evaluation. On pathology, the resected lesion was macroscopically a cystic structure containing sanguineous fluid, the cyst wall was approximately 3 mm wide, and no signs of malignancy were seen. However, microscopic examination demonstrated a malignant neoplasm predominantly consisted of spindle fibroblast-like cells are arranged in a characteristic storiform pattern with pleomorphic and multinucleated cells (Fig. On immunostaining, the tumor was positive for CD68, vimentin and p53 protein, and negative for EMA, CEA, S-100 protein, cytokeratin, Desmin, CD34, CD99, CD117, SMA, MDM2, and CDK4. The tumor Ki-67 expression was about 30%. Based on morphology and immunohistochemical staining, The tumor was diagnosed as UPS. The patient refused to perform further surgery and was discharged 11 days later. Unfortunately, during the follow-up visit at 7 months, a chest CT scan revealed abnormally enlarged lymph nodes. After comparing the preoperative CT scans, those nodes were suspected of being metastatic lesions. The patient died of the metastasis 12 months after surgery.Primary sarcomas of the pancreas are extremely rare, including leiomyosarcoma, epitheloid sarcomas, granulocytic sarcoma, etc. –4. PancrThe clinical profiles of these patients are summarized in Table Primary pancreatic UPS is more prevalent in men than in women. However, due to the limited number, other risk factors have not been determined. It grows fast, and its clinical features described in literature are varied, but upper abdominal discomfort is the most common presentation. At present, there is no reliable laboratory test contributing to diagnosis, and the preoperative tumor markers in this case are negative. In the previous reports, pancreatic UPS presented a large, nonhomogeneous, hypointense or multinodular lesion with possible intratumoral calcification  and massIn general, it is difficult to diagnose pancreatic UPS without obtaining tumor tissues. Endoscopic ultrasonography-guided needle aspiration has become the most accurate method for the diagnosis of pancreatic malignant tumors . HoweverSurgery is the mainstay of treatment in primary localized retroperitoneal sarcomas . PrimaryIn addition to surgery, radiotherapy may have a certain effect on pancreatic UPS. Studies have found that when the tumor cannot be completely removed, radiotherapy is an important adjuvant treatment for UPS in other parts . HoweverIn summary, primary UPS is an exceptionally rare but distinct malignant neoplasm of pancreas. Despite there are no distinctive manifestation of UPS, a solid cystic lesion on ultrasonography or a hypodense area in the lesion on T2-weighted imaging, should be considered for differential diagnosis with pancreatic UPS. Radical resection is the most effective treatment at present. Postoperative radiotherapy and chemotherapy may improve the survival of patients, which needs to be verified by more clinical cases. This case emphasizes the clinicopathological, imaging, and immunohistochemical finding of pancreatic UPS. Long-term follow-up researches are required to know the exact biological behavior of these neoplasms.


BRAF and KRAS. Four pituitary glomus tumours have previously been reported, none of which showed features of malignant glomus tumour. Similar to our two patients, three previous examples displayed aggressive behaviour.Primary non-neuroendocrine tumours of the pituitary gland and sella are rare lesions often challenging to diagnose. We describe two cases of clinically aggressive primary glomus tumour of the pituitary gland. The lesions occurred in a 63-year-old male and a 30-year-old female who presented with headache, blurred vision and hypopituitarism. Neuroimaging demonstrated large sellar and suprasellar tumours invading the surrounding structures. Histologically, the lesions were characterised by angiocentric sheets and nests of atypical cells that expressed vimentin, smooth muscle actin and CD34. Perivascular deposition of collagen IV was also a feature. Case 2 expressed synaptophysin. INI-1 (SMARCB1) expression was preserved. Both lesions were mitotically active and demonstrated a Ki-67 labelling index of 30%. Next-generation sequencing performed in case 1 showed no mutations in the reading frame of 37 commonly mutated oncogenes, including  Primary non-neuroendocrine tumours of the pituitary gland and sellar region are rare and are often challenging to diagnose preoperatively and at pathological examination. If craniopharyngiomas and meningiomas are excluded, the remaining 1% of sellar non-neuroendocrine neoplasms includes the group of TTF-1 expressing tumours of the posterior pituitary, neuronal and paraneuronal, germ cell, haemopoietic, melanocytic, sellar salivary gland-like tumours, the spectrum of mesenchymal neoplasms including chordoma, and bone and cartilage tumours, sellar atypical teratoid rhabdoid tumour (ATRT), perivascular epithelial cell neoplasm (PEComa) and glomus tumour (GT) .Glomus tumour is a perivascular neoplasm that occurs more commonly in the distal extremities and more often involving the nail bed where glomus bodies are present. Glomus tumours have also been documented in visceral organs such as the lung, stomach, pancreas, liver, gastrointestinal and genitourinary tract , 6. The We describe two patients with primary pituitary GT presenting as large, invasive sellar and suprasellar lesions, one with a fluctuating clinical behaviour due to tumour apoplexy and the second with a short history of visual loss and amenorrhoea.A 63-year-old male sought clinical attention in 2008 for gynaecomastia without galactorrhoea. Hormonal tests revealed normal prolactin, growth hormone deficiency and hypogonadotropic hypogonadism, for which he was commenced on replacement therapy. Magnetic resonance imaging (MRI) at that time documented an expanded sella and a bulky pituitary gland but no tumour was observed. He presented again in August 2018 with worsening intermittent headaches and blurred vision. During that admission, he was diagnosed with adrenal insufficiency and hypothyroidism. Replacement therapy with hydrocortisone and levothyroxine was therefore administered. His headaches gradually deteriorated and developed blurred vision. Visual fields were full with no evidence of ophthalmoplegia. Investigations demonstrated worsening of anterior hypopituitarism and additional adrenal insufficiency. An MRI demonstrated a sellar lesion with suprasellar extension, left deviation of the stalk and slight optic chiasm displacement without compression. The patient was referred for pituitary surgery but the preoperative MRI showed spontaneous volumetric reduction with features consistent with apoplexy Fig. . HeadachThe patient underwent endoscopic transsphenoidal surgery. Intraoperatively the tumour appeared firm and rubbery with a central area of relatively avascular, soft tissue consistent with necrosis. The sellar floor was eroded; the tumour was attached to the right side of the sphenoid septum. A residuum was left in the right cavernous sinus. Headache and diplopia resolved. He presented again 6 weeks later with diplopia and deteriorating visual acuity. An MRI showed a regrowth of the sellar and suprasellar components causing optic chiasm displacement. Further surgery was felt inappropriate and he was referred for adjuvant radiotherapy, which was completed in November 2019. A follow-up MRI documented shrinkage of the lesion following radiotherapy. He was re-admitted in May 2020 with complete third nerve palsy and sudden visual failure. An MRI demonstrated tumour progression with further invasion of the cavernous sinuses and involvement of the anterior cranial fossa Fig. . FurtherA 30-year-old female presented with a 10-month history of progressive bilateral visual deterioration and amenorrhoea. She initially refused an endocrinological assessment and a brain MRI. She later sought clinical attention for a week history of severe headache and vomiting, suggesting acute increased intracranial pressure or apoplexy. Clinical examination showed complete bilateral blindness with optic disc atrophy. Oculomotor movements were preserved. A brain MRI showed a solid cyst, heterogeneously enhancing, intra- and suprasellar lesion with pituitary fossa enlargement, optic chiasm compression, third ventricle extension and bilateral cavernous sinus invasion. Oedema of the frontal lobes was also present Figure . HormonaCandida Albicans, with secondary hydrocephalus that required an external ventricular drainage and then a ventriculoatrial shunt. She remained hypopituitary on replacement therapy. She refused gamma knife treatment and decided for transcranial surgery in another centre. Surgery was complicated by a suprasellar haematoma, subarachnoid haemorrhage and stroke. She developed aphasia, right-sided hemiparesis and left-sided crural paresis and died 6 months after the second operation.The patient underwent an endoscopic endonasal transsphenoidal approach. Intraoperatively, the tumour was firm and difficult to remove. A subtotal resection was accomplished with decompression of the optic chiasm. In the postoperative course, she developed meningitis from The specimens were formalin fixed and embedded in paraffin. Five-micron thick sections were stained with haematoxylin-eosin (H&E). Immunohistochemistry was performed on a BenchMark ULTRA Slide Staining System  using the primary antibodies directed against synaptophysin , FSH , LH , TSH , PRL , ACTH , GH , steroidogenic factor 1 , PIT1 , TPIT , pancytokeratin , cytokeratin 7 , cytokeratin 8 , TTF-1 , vimentin , smooth muscle actin , desmin , myogenin , CD21 , CD23 , CD31 , CD34 , ERG , Collagen IV , STAT6 , GFAP , S-100 protein , p63 , HMB45 , PAX8 , alpha-fetoprotein , INI-1 , BRAFV600E , CD68  and TFE3 .The Ki-67  proliferative index was evaluated by counting the number of positive cells in at least 500 tumour cells in hotspot areas on camera-captured printed images.AKT1, ALK, AR, ATRX, B2M, BRAF, CDKN2A, CTNNB1, DDR2, EGFR, ERBB2, FGFR3, GNA11, GNAQ, GNAS, H3F3A, H3F3B, HIST1H3B, HIST1H3C, HIST2H3C, HRAS, IDH1, IDH2, KIT, KRAS, MAP2K1, MET, NRAS, PDGFRA, PIK3CA, PTEN, RET, STK11, TERT, TP53 and VHL. The whole coding sequence was not targeted. Mutation and variant calling by custom bioinformatics analysis pipeline validated to detect SNVs and small insertion/deletion mutations (< 40 bp) to 5% mutant allele frequency. Alterations were categorised following the American College of Medical Genetics guidelines and Association for Molecular Pathology tiering.The most representative tumour area of case 1 was circled on a reference H&E-stained section. Five serial 10-micron thick sections were cut from formalin-fixed and paraffin-embedded tissue for DNA extraction. Genomic DNA was isolated from the selected tissue area using the cobas® DNA Sample Preparation Kit (Roche Diagnostics Ltd Burgess Hill UK). Sequence analysis was carried out following PCR enrichment using a QIAseq Targeted DNA custom panel  and Illumina next-generation sequencing . The panel included Both tumours showed moderate to high cellularity and consisted of nests, sheets and ill-defined lobules in a dense network of thin-walled vessels. Neoplastic cells were of small to medium size and showed neat borders, densely eosinophilic cytoplasm and irregular nucleus with fine chromatin and inconspicuous nucleolus. Several nuclei contained optically clear inclusions. Cytoplasmic vacuoles were present in some cells. Tumour cells often seemed to arise from or form themselves vessel walls Figures , 3b, 4a.Neoplastic cells of both lesions expressed vimentin, SMA, which was focal in case 1 and diffuse in case 2 and CD34 that was focal in case 2; the immunoreaction for collagen IV showed pericellular pattern. Synaptophysin was only expressed in case 2. Scattered tumour cells in case 1 demonstrated membranous EMA immunoreactivity. SMARCB1/INI1 was normally expressed, CD68 highlighted macrophages and CD31 highlighted the dense vascular network. All other immunostains were negative. The Ki-67 labelling index was 30% in both tumours Figures .The NGS panel did not identify any mutations in the reading frame of the 37 genes tested.We have documented two primary GTs of the pituitary presenting as a large, invasive sellar and suprasellar masses. Both patients underwent repeated surgeries.Given the site of the two lesions, the diagnosis of GT in our patients was challenging. Angiocentric sheets of tumour cells expressing vimentin, SMA and CD34 were important clues to reach the diagnosis of GT , 6. The BRAF and KRAS, were identified in our patient 1. Strong nuclear INI-1 expression in both lesions indicated that mutations in SMARCB1 were unlikely to have occurred. The genetic features of GT, GT-UMP and mGT are not fully understood [BRAFV600E and KRAS mutations were documented in superficially located GTs including GT-UMP and mGTs [SMARCB1 [No mutations in the open reading frame of commonly mutated oncogenes, including derstood . BRAFV60and mGTs . Dabek a[SMARCB1 ; unlike [SMARCB1 , 19, but[SMARCB1 . Rearran[SMARCB1  but theyGLI1 with the partner genes ACTB1, MALAT1 or PTCH1 [GLI1 aberrations show nests, cords and reticular structures of monomorphic round to epithelioid cells in a dense capillary network that can mimic GT. Unlike our GT, GLI1-fused tumours express S100 protein and are negative for SMA. A metastasis from a malignant GT was excluded clinically because no other lesion was found outside the sella.Given the location of our cases, we considered a broad differential diagnosis. Synaptophysin expression seen in our case 2 and in one published example  could haor PTCH1 . HistoloBRAFV600E status was tested by immunohistochemistry in one instance [Primary pituitary GT with features of glomangioma was first documented by Asa and colleagues in 1984 . Since tinstance ; otherwiIn conclusion, we documented two primary GTs of the pituitary gland displaying aggressive histological features at onset. Glomus tumour should be considered in the differential diagnosis of rare primary non-neuroendocrine tumours of the pituitary gland and sellar region as they present as invasive lesions that are difficult to remove surgically, have the tendency to recur locally, causing severe morbidity to patients, and require adjuvant treatment, predominantly radiotherapy as the evidence of efficacy of chemotherapy protocols remains very limited. Pituitary GTs can be diagnostically challenging, particularly when they show severe cellular atypia and high proliferation. As recommended by Tsang and colleagues , it is e


A thyroglossal duct cyst (TGDC) is one of the most commonly encountered congenital anomalies of the neck. However, it is difficult to diagnose as differentiating it from other cysts like brachial cysts, lymphangiomas, epidermoid cysts, dermoid cysts, and hydatid cysts, is challenging. In this paper, we systematically reviewed the literature of 47 patients—25 males (53.1%) and 21 females (44.7%)—about their TGDC to assess the clinical picture, therapy, and prognosis of the disease. Most of the patients were children under the age of ten (63.8%). All patients had a history of a painless swelling in the anterior midline of the neck that moved in response to deglutition and tongue protrusion, thus interfering with their daily activity. Post-resection recurrence was unusual, with only 3 of 47 patients (6.4%) experiencing recurrence. A thyroglossal duct cyst (TGDC) is the most frequent embryonic-origin cervical mass discovered in the anterior of the neck. The thyroglossal duct, which connects the base of the tongue to the thyroid gland, generally fails to obliterate, resulting in this disease. This anomaly occurs in approximately 7% of people ,2,3, rephttps://www.embase.com) (accessed on 24 December 2021) and NCBI-PubMed (https://pubmed.ncbi.nlm.nih.gov/) (accessed on 24 December 2021). The search results showed 68 results (non-duplicated), but 58 studies were excluded after looking at the research title/abstract because they did not focus primarily on a TGDC. We included all ten remaining publications in this analysis and thoroughly reviewed and analyzed them  were studied with regard to the mass characteristics, treatment, and clinical outcome based on the 10 preselected studies ,13,14,15A 52-year-old male presented with a history of large, elastic, and easily mobile midline neck swelling and difficulty swallowing. There was no hoarseness or shortness of breath. The patient led an active lifestyle. The thyroid gland was normal in size but showed certain signs of inflammation. The patient was advised to reduce his stress and treat the swelling with progesterone gel. But after a while, he complained that those measures were not working as the mass size was fixed and did not change in size. The patient also complained of not being able to button up his shirt (which indicates the large size of the mass).The patient had regular bowel movements, a normal appetite, slight weight loss, and no heart disease. He suffered from hypertension but was not on medication. He experienced some bloating and cramps in the abdomen, without allergy. The patient was a non-smoker but was an occasional drinker. His family history showed that his mother suffered from hyperthyroidism and his father suffered from arterial hypertension.On physical examination, a large (0.041 m × 0.044 m × 0.033 m), regular, non-tender, and easily mobile suprahyoid cystic mass was found in the upper neck area, located above the larynx, which moved with swallowing. The thyroid gland was easy to palpate.Laboratory results showed that the patient was euthyroid, and his anti-thyroid peroxidase (anti-TPO) was negative. Ultrasound imaging showed an echogenic, homogeneous thyroid with a total volume of 7 mL, with no intra-thyroid nodules. It also showed a mass above the thyroid gland, slightly to the right of the midline. The mass was approximately 0.041 m × 0.044 m × 0.033 m with a strong connective tissue capsule and an anechoic internal structure, as shown in Magnetic resonance imaging (MRI) of the neck’s soft tissues (native and IV KM) diagnosed it as a suprahyoid thyroglossal cyst located posterior to the platysma, as it appeared hyperintense in T2 with isointense parts and was also isointense in T1 with an extension of approximately 0.041 m × 0.044 m × 0.033 m. There was minimal displacement of the hypopharynx and epiglottis with no evident connection to the thyroid gland or the tongue’s base. There was also discreet contrast-medium uptake of the wall and protein-rich fluid, indicating possible previous inflammation. We found no cervical lymphadenopathy, and the submandibular gland and parotid gland were inconspicuous. In the cervical spine, as shown in According to the literature review of the study, a total of 47 patients were reviewed, with 25 males (53.1%), 21 females (44.7%), and one instance of undetermined gender (2.1%). They ranged in age from 5 days to 85 years old (mean 29.1 years). The majority of the patients were under the age of 10 (63.8%). The clinical behavior of the TGDC was typically benign, with recurrence after resection uncommon (only 3 of 47 patients (6.4%) recurred throughout the documented follow-up). The mass size at diagnosis averaged 54.4 mm . The majority of the patients had a common case history of painless swelling in the anterior midline of the neck that moved in response to deglutition and tongue protrusion and interfered with their daily activity. Infrahyoid was the most common tumor site, accounting for 33 cases (70.2%), followed by suprahyoid , and two intralingual cases (4.3%).Typically, a TGDC is a mass in the neck that is flexible and located on the midline or a bit to the side (95% err slightly to the left), most often with other symptoms (that are worrisome). Some who are affected by a TGDC suffer from pain in the neck, relapse, or experience dysphagia infections in the throat . The masTGDCs occur in varying locations; they can be located anywhere in the neck between the foramen cecum, the tongue’s base, and the suprasternal fissure. Four common locations are thyrohyoid (60.9%), suprahyoid (24.1%), supra-sternal (12.9%), and intra-lingual (2.1%) . TGDCs aIn our patient, there were no functional impairments, just a neck swelling with some mechanical impairments.A TGDC is usually a painless, mobile swelling in the neck’s midline near the hyoid bone. It also can manifest clinically or radiologically, however, as an unusual lesion, which may make diagnosis a difficult and challenging task ,21.There is a risk of infection with or without an abscess when thyroglossal fragments remain attached to the tongue’s base via the tract, which can represent the start of a thyroglossal cyst’s presentation . In our The abscess of the thyroglossal cyst may drain into the neck’s skin either after an improper incision ending in a fistulous tract, or drain through the sinus tract to the tongue’s base, thus creating a solution containing fibrous residue. In the end, for the true cyst to remain or develop, a healthy capsule must form, but the infected duct remains chronically .There are several methods for diagnosing a TGDC, including ultrasound imaging of the neck, an appropriate and non-invasive procedure. Preoperative fine-needle cytology is also a cheap and safe method . UltrasoHowever, some of the disadvantages of ultrasound imaging (sonography) lie in the fact that it does not visualize hyoid and infrahyoid TGDCs reliably, and cannot reliably measure the base of the tongue in a suprahyoid cyst. MRI is often preferred for an athyroid cyst near the tongue’s base . On T2-wOn reviewing the literature, the presence of a mass in the neck was the only evidence based on which to diagnose the condition during the examination of the case. With laboratory investigation, the final diagnosis indicated a suprahyoid thyroglossal cyst located posterior to the platysma, with an extension of approximately 0.041 m × 0.044 m × 0.033 m.Even in adult patients, it is important to be aware of congenital anomalies that might lead people to develop neck masses such as thyroglossal duct cysts (TGDCs), and their complications, so that proper investigations can be performed to reach a definitive diagnosis. After reviewing the patient’s condition, the data revealed the presence of a mobile mass in the neck area, with no further clinical data other than swallowing difficulties. Thus, we performed a thyroid function test to make sure the thyroid was working properly, which showed that the gland was unaffected. Ultrasound imaging later revealed a mass above the thyroid gland, ruling out intra-thyroid nodules, but the mass remained unclear. Finally, we employed magnetic resonance imaging (MRI), which showed similar results to the typical TGDC, and thus the final diagnosis was made based on MRI data.


Intrahepatic cholangiocarcinoma (ICC) is a highly lethal hepatobiliary neoplasm, which originates from the bile ducts proximal to the second-order division. ICC can be anatomically divided into two subtypes: the large duct type  and the small duct type (mixed-ICC) origins from hepatic progenitor cells (HPCs). The immunoreactivity of S100P and neural cell adhesion molecule (NCAM) are useful biomarkers to distinguish the two subtypes. In this study, we report a difficult-to-diagnose case of metastatic retroperitoneal tumor of occult hepatolithiasis-associated ICC. Besides, this case was both positive for S100P and NCAM, considered as a rare muc-ICC with the HPCs features. Tumor whole exome sequencing detection results by Genetron (China) revealed that there were 41 gene mutations in this patient. The SMAD4-p.His530ThrfsTer47 and KRAS-p.Gly12Val mutation might promote the occurrence and distant metastasis of the tumor. Intrahepatic cholangiocarcinoma (ICC) has an increasing incidence worldwide and is the second most common primary hepatic malignancy after hepatocellular carcinoma. Cirrhosis, viral hepatitis, obesity-associated liver disease, diabetes, and the diseases leading to hepatobiliary fibrosis, such as primary sclerosing cholangitis, Caroli’s disease, hepatolithiasis, and liver fluke infractions, are risk factors for ICC. However, only 50% of ICC patients have identifiable risk factors . Surgery2. On physical exam, she was of middle nutrition and had no jaundice. Her abdomen was soft, while an approximately 6 cm hard mass without clear boundaries in mid-upper abdomen was found. The laboratory data revealed that the patient had normal routine blood test, hepatic function, renal function, and coagulation function. The serum levels of carbohydrate antigen 19-9 (CA19-9) (8.01 U/ml), cancer embryonic antigen (CEA) (2.24 ng/ml), CA-125 (19.71 U/ml), CA-242 (1.24 U/ml), alpha-fetoprotein (AFP) (1.39 ng/ml) were all within the normal range. Intraoperative ultrasonography did not detect fatty liver. The abdominal contrast-enhanced computed tomography (CT) scan and CT angiography showed a huge irregular neoplasm located at the retroperitoneum with edge intensifying  after fixation. Tissue sections were deparaffinized, subjected to antigen retrieval, and blocked with 5% bovine serum albumin (BSA) in phosphate-buffered saline (PBS) for 30 min. CA19-9, CK19, CK7, neural cell adhesion molecule (NCAM), S100P, CK20, AFP, and GATA-3 primary antibodies were purchased from Abcam Plc . CEA, Muc-1, CK8/18, Ki67, P40, P53, c-kit, Mucin-2, SATB, and Villin primary antibodies were purchased from Cell Signaling Technology  and Hep-par1primary antibody was purchased from Affinity Biosciences . The sections were incubated with primary antibody overnight at 4°C, followed by the appropriate secondary antibody at room temperature for 1 h. All images were captured by confocal microscopy. Hematoxylin and eosin (HE) staining revealed that chronic inflammation of the intrahepatic bile duct and high-grade intraepithelial neoplasia of the glandular epithelium were commonly seen in the left hepatic lobe of 10 different layers , frame shift mutation , missense mutation  and splicing mutation (KCND2)  of this patient, in the conserved G box domain of KRAS protein, results in the activation of KRAS. KRASG12D activation leads to the development of invasive ICC with low penetrance and long latency. Latency was shortened by combining KRASG12D activation with heterozygous or homozygous deletion of p53, which also resulted in widespread local and distant metastasis  for the publication of any potentially identifiable images or data included in this article.CL and SJ were involved in histopathological diagnosis. QF and PL wrote the first draft of the manuscript. TQ and MH were involved in surgery and tissue collection. YW and XZ performed laboratory experiment. TQ and HZ reviewed and edited the manuscript before submission. All authors contributed to the article and approved the submitted version.The prevalence study was funded by the National Natural Science Foundation of China (31671440).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Osseous choristoma is an uncommon benign lesion characterized by the presence of ectopic mature bone within soft tissue. In most cases, these lesions occur on the dorsum of the tongue in patients in their third and fourth decades of life. This article describes a case of lingual osseous choristoma in a pediatric patient. An eleven-year-old girl with a lingual mass was referred to our hospital from a dental clinic. Total excisional biopsy and histological examination were performed, and osseous choristoma was diagnosed. The postoperative course was uneventful with no signs of recurrence during the 12 months after surgery. Moreover, a literature review focusing on pediatric cases with lingual osseous choristoma was performed to know the etiology, clinicopathological characteristics, and course of treatment of the lesion. Choristoma is defined as a tumor-like lesion that is composed of normal tissue in an abnormal location. In 1971, Krolls et al. proposed the term “osseous choristoma” for soft tissue osteoma in the head and neck region . LingualAn 11-year-old Japanese girl told her dentist about a mass in her tongue and was referred to our hospital. She had noticed an asymptomatic nodule at the dorsum of the tongue. However, the fear of being diagnosed with a malignant condition prevented her from consulting a doctor, at least for a while. She had been aware of its existence for 2–3 years before her first visit. She was diagnosed with pneumonia at the age of one year but had no other remarkable medical history. She was not on any long-term medications. Her clinical examination revealed a pedunculated mass covered with normal mucosa in the tongue's posterior portion . The lesLingual osseous choristoma is rare among pediatric patients. In this manuscript, we present another pediatric case of lingual osseous choristoma and review the relevant literature.In Japan, most children attend pediatric clinics until the age of ~12 years. Thus, we focused on patients below the age of 13 years with osseous choristoma in our literature search. To our knowledge, in the literature, 62 cases have been described on patients above the age of 13 years  1, 3, 6–45. On tWe summarized the characteristics of the 17 cases with pediatric lingual osseous choristoma including our case in The pathogenesis of osseous choristoma is not yet known, a “developmental malformation hypothesis” and a “chronic trauma-associated reactive hypothesis” were proposed , 54, 58.Finally, it cannot be overlooked that the 30-day waiting period until the histological diagnosis of the patient's lesion was stressful for the patient and her family. Although the reason for the long waiting time was the need to decalcify the bone tissue, the psychological care we provided was insufficient. It was speculated that the information provided by imaging modalities can help reduce patients' anxiety. Diagnostic imaging was conducted in five pediatric cases. Given that no attending surgeons considered the possibility of an osseous choristoma, we did not conduct any imaging examination in this case. An earlier study reported computed tomography (CT) images are useful for the radiological diagnosis of lingual osseous choristoma . Also, YIn conclusion, we presented a pediatric case of lingual osseous choristoma and conducted a review of the literature to identify the characteristics of pediatric cases of the lesion.


Gastrointestinal lipomas are rare, often colonic tumors. The stomach is an unusual site of involvement of lipomas, accounting for less than 5% of all gastrointestinal lipomas and less than 3% of all benign gastric neoplasms. They are usually asymptomatic, and symptoms develop as the tumor grows. Gastric lipomas can present with massive bleeding from an ulcerating tumor and can be life-threatening if left untreated. We present a case of an ulcerating gastric lipoma that presented as an acute upper gastrointestinal hemorrhage. The patient was treated with Billroth II procedure and final pathology showed an ulcerating submucosal lipoma. The diagnosis of gastric lipoma is often suspected incidentally on imaging, then confirmed via biopsy. Definitive treatment of large lesions typically requires surgery, however, newer endoscopic techniques are being utilized for resection of these benign tumors. Lipomas are benign tumors that form from mature adipose tissue . GastroiA 65-year-old male with a known past medical history of hypertension, gastroesophageal reflux disease, and hyperlipidemia presented with complaints of intermittent, non-localized abdominal discomfort associated with early satiety, decreased appetite, and shortness of breath on exertion for six months duration. His symptoms acutely worsened two weeks prior to presentation and were associated with black, tarry stools of four days duration. Further history revealed alcohol consumption of 1-2 beers per day and a previous 60 pack-year cigarette smoking history. He denied ever having an esophagogastroduodenoscopy (EGD) but did have a colonoscopy 12 years prior to presentation which revealed diverticulosis and a single polyp that was successfully removed. Vital signs were stable and physical examination revealed no abdominal tenderness, abdominal distension, or conjunctival pallor. Laboratory results revealed a hemoglobin level of 9.7 mg/dL , hematocrit level of 28.7%, and a mean corpuscular volume (MCV) of 95.5 fL. The remainder of his laboratory values including platelet count, white blood cell count, serum creatinine, blood urea nitrogen (BUN), potassium, sodium, and bicarbonate were within normal limits. He was started on pantoprazole twice daily, and intravenous fluid resuscitation was initiated. The patient underwent computed tomography (CT) scan of his abdomen and pelvis with intravenous contrast that revealed evidence of a 5 cm fat-attenuating mass at the gastric antrum protruding through the pylorus and into the first portion of the duodenum, suggestive of lipoma. Hemoglobin level dropped to 8.2 mg/dL during his admission. The patient underwent an EGD which revealed a large, ulcerated mass in the antrum of the stomach with central necrosis that appeared like an atypical ulcer Figures . Given tBiopsy results showed benign gastric mucosa with hyperplastic changes consistent with a hyperplastic polyp, and no malignant or dysplastic process was identified. Given the symptomatic nature of the mass and ongoing blood loss, the patient underwent an exploratory laparotomy. This revealed a gastric antral mass 5-6 cm in diameter with three sites of ulcerations without any evidence of lymph node or liver metastasis. A distal gastrectomy with gastrojejunostomy (Billroth II) procedure was performed. Surgical pathology revealed an ulcerated submucosal lipoma with six benign lymph nodes Figures -6. ImmunLipomas are tumors of mesenchymal origin that are slow-growing, benign, and surrounded by a thin fibrous capsule ,6,7. TheGastric lipomas arise from the submucosa in 95% of cases and are typically found in the gastric antrum (75%) ,9. AlthoThe diagnosis of gastric lipoma is often made incidentally when obtaining imaging for an unrelated issue or complaint. CT scan is specific for the diagnosis of lipomas, which are often described as well-circumscribed submucosal mass lesions with fat attenuation -5,7. If There are multiple modalities for the treatment of gastric lipomas. Asymptomatic lesions that are incidentally identified on imaging can be observed with no further treatment or evaluation ,2,6. SmaGastric lipomas are rare, benign, typically submucosal tumors that predominately occur in the gastric antrum. They are often found incidentally but can also present with symptoms of gastric outlet obstruction or gastrointestinal hemorrhage, such as in our case. Diagnosis of these lesions can be done based on typical CT findings alone, but further investigation with endoscopic or surgical biopsy may be necessary. Treatment options vary depending on the size of the lipoma and the availability of advanced endoscopic techniques. Small (< 2 cm), asymptomatic lesions can be observed or removed endoscopically. Larger (> 2 cm) lesions typically require surgical excision by a provider trained in advanced endoscopic procedures. Histopathologic evaluation of these tumors reveals mature fat cells with varying shapes and sizes, which are typically larger than the surrounding fat. All fatty tumors, both benign and malignant, will stain positively for vimentin and S-100 protein. Prognosis varies depending on the size of the lesions and symptoms. All bleeding lipomas should be removed to prevent potentially life-threatening hemorrhage.


Myxomas, being the most common primary benign tumor among all cardiac tumors, occur rarely with a very low incidence rate. Myxomas can cause various clinical manifestations or can be diagnosed incidentally. Some patients with cardiac myxomas are completely asymptomatic. Cardiac myxomas cause life-threatening complications, thus early diagnosis is imperative. We are reporting a case of atrial myxoma in a 38-year-old female without any significant past medical history, who came to our clinic for cardiology evaluation prior to plastic surgery. The elliptical mobile mass, located in the left atrium with its attachment to the interatrial septum, was diagnosed by transthoracic echocardiography. The patient was referred for surgery and a minimally invasive surgical approach was chosen. A histological report confirmed the diagnosis of myxoma. The patient recovered well. Three years of follow-up did not reveal any signs of recurrence of the tumor. We are also analyzing 20 previously published cases of asymptomatic myxomas and myxomas treated with a minimally invasive surgical approach, reported in the PubMed database for the last 20 years (2001-2021) in the adult patient population (age 19 and over). The aim of this study is to highlight the asymptomatic presentation of cardiac myxomas and to underline the advantages of a minimally invasive surgical approach. In summary, asymptomatic cardiac myxomas are rare incidental findings. Attention to subtle symptoms during a physical exam and scrupulous history-taking can provide a clue to this diagnosis. Early diagnosis of cardiac myxomas is crucial to prevent life-threatening complications. Minimally invasive surgery is a promising alternative approach to standard open-heart surgery for treating cardiac myxomas, providing faster recovery and higher patient satisfaction with surgical care.  Cardiac tumors are differentiated into primary and secondary. The prevalence of primary heart tumors is 0.001-0.03%, as reported in the autopsy series . Only onA 38-year-old white asymptomatic female without any significant past medical history or risk factors came to our clinic for cardiology consultation prior to cosmetic facial surgery. The patient, who recently returned from a mountain climbing trip in the Himalayas, was in good physical shape. On a physical examination a holosystolic murmur, best heard at the left lower sternal border, was noticed. The patient was sent for transthoracic echocardiography (TTE), which showed elliptical, mobile homogeneous hyperechoic mass, 28x24 mm, with smooth surface, located in the left atrium Figure .The mass did not prolapse through the mitral orifice during diastole, did not obstruct diastolic filling of the left ventricle, was attached to the interatrial septum, although the peduncle was not clearly visualized Video . The lefThe patient underwent a right lateral mini-thoracotomy under general anesthesia. Peripheral cardiopulmonary bypass using right femoral artery and vein and right internal jugular vein was established. Induced ventricular fibrillation was used. Left and right atria were opened. The tumor was excised with a part of an interatrial septum Video , which wThe histopathological report confirmed a diagnosis of cardiac myxoma. Macroscopically, the specimen consisted of a small fragment of the left atrial wall and the oval-shaped tumor, presented by gelatinous tan-white tissue with the friable surface Figure .Microscopically, hematoxylin and eosin-stained sections showed a neoplastic lesion with two components. The first, a cellular component, consisting of stellate and spindle cells with scant eosinophilic cytoplasm, round to oval nuclei (some multinucleated), and mild nuclear polymorphism Figure . No signThe second, extracellular component, was represented by an edematous eosinophilic stroma with foci of hyalinosis, numerous vessels, surrounded by accumulations of hemosiderin granules. Small vessels of the tumor had capillary type structure and resembled vascular slits and wide tubular formation. Large vessels consisted of only several layers of myxomal cells. The basement membrane, smooth muscle cells, and adventitia were absent Figure . It shouThe postoperative period was uncomplicated. The patient was followed up annually for three years with no signs of recurrence of the tumor.We present a rare case of a completely asymptomatic patient with left atrial myxoma, which was incidentally revealed during preoperative cardiac evaluation prior to plastic facial surgery. Twenty previously published cases of asymptomatic myxomas and cases of myxomas treated with a minimally invasive surgical approach, reported in the PubMed database for the last 20 years (2001-2021) in the adult patient population (age 19 and over) were analyzed. We summarized information to present a comprehensive review, including information about the first authors of the studies, year of publication, demographics, initial presentation, imaging tests, size of myxoma, localization, and site of its attachment, type of surgery, and postoperative follow-up.Asymptomatic myxomas, described in previously published case reports, were found either during routine check-ups or due to work-ups for other medical conditions, such as hypertension, chronic liver disease, bronchiectasis disease, or due to necessity to be admitted to the hospital because of cancer, bronchocele surgery, elective gastric banding. One of the patients underwent TTE screening as her daughter has been diagnosed with hypertrophic cardiomyopathy (HCM) and was found both HCM and myxoma . One of Myxoma in the reported case was located in the left atrium, attached to the interatrial septum. About 75% (60-88%) of myxomas occur in the left atrium , 15-20% (4-28%) occur in the right atrium ,10. AtypTumors vary widely in size, ranging from 1 to 15 cm in diameter and weighing between 15 g and 180 g . MyxomasTransthoracic echocardiography is a simple and non-invasive screening method of diagnosing heart masses, detecting 95% of cardiac masses . HoweverThe standard approach in treating cardiac myxoma is the median sternotomy. Two-thirds of patients in the previously published cases underwent standard surgery. However, standard median sternotomy is associated with unsatisfied cosmetic outcomes and risk of sternal infection. In our case, the patient underwent a less frequently used minimally invasive surgery and it was successful without any postoperative complications. The patient recovered well, staying in the hospital only for four days, and was satisfied with the surgical care, expedited wound healing, and small size of the cosmetic scar. Right anterolateral minithoracotomy with incision of 4 to 6 cm is gradually applied in the surgery of cardiac tumors and is a good alternative technique, compared to standard open-heart surgery, for treating cardiac myxomas. It is associated with reduced trauma and pain, low complication rates, significantly fewer arrhythmia events, a shorter intensive care unit, and hospital stay: median duration of hospital stay in the minimally invasive group is 10.4 ± 1.5 days vs sternotomy group 17.5 ± 5.6 (p = 0.004), according to Sawaki S. et al. ,14. PatiDetailed information is presented in the table Table .Limitations of the study: a small sample size, we were limited to full-text English language case reports only, we did not include population under age 19 as well as articles not containing enough information about variables we were gathering.Asymptomatic cardiac myxomas are rare primary cardiac tumors. However, they should be included in the differential diagnosis of non-specific cardiothoracic symptoms, as myxomas cause life-threatening complications. Noticing mild signs and symptoms during a physical examination and taking a detailed history is imperative to diagnose this tumor. The minimally invasive surgical approach has multiple advantages comparing to standard open-heart surgery: lower complications rates, lower risk of infection, less blood loss, fewer arrhythmia events, shorter intensive care unit stay and hospital stay, higher patient satisfaction with cosmetic healing of the wound, scar appearance, and surgical care. Minimally invasive surgery is a promising technique for treating cardiac myxomas. Further studies are needed to better estimate the advantages of the minimally invasive cardiac approach.


Germ cell tumors in infants are most frequently extragonadal, benign, and amenable to surgical resection. An unusual feature of germ cell tumors is the potential coexistence of malignant with benign disease which makes it possible for patients with incompletely resected tumors to experience either a benign or malignant recurrence. A challenge to postoperative surveillance is the interpretation of serum alpha fetoprotein, a marker of malignancy, that is physiologically elevated during the first year of life. A rare subset of germ cell tumors occur in the retroperitoneum. Although the vast majority are benign, these tumors are often large and distort normal anatomy, and may demonstrate local invasiveness that increases risk of resection. The intent of these reports is to caution readers about these unusual features of germ cell tumors of infancy. Germ cell tumors (GCT), often referred to as teratomas account for ~3% of neoplasms in children under 15 years . RetropeMalignant GCT elaborate alpha fetoprotein (AFP), which serves as a marker of malignancy, but can presents challenges in interpretation during the first year of life when serum AFP levels are physiologically elevated. Pathologic assessment of resected tumors must take into account the potential for coexistence of benign with malignant histology and underscores the importance of postoperative AFP surveillance as a marker for residual or recurrent malignant tumor.For localized tumors with benign histology, primary resection is curative. Although most benign teratomas are encapsulated and usually amenable to complete extracapsular resection, retroperitoneal teratomas (RT) have a tendency to cause anatomic distortion and infiltration of vital structures including the abdominal aorta, its visceral branches, and the inferior vena cava.We present three cases which highlight: (i) pitfalls in postoperative AFP surveillance leading to a delay in detection of a malignant recurrence after resection of a benign sacrococcygeal tumor (SCT); and (ii) challenges in the surgical management of RT which emphasize the importance of preoperative imaging and intraoperative risk-assessment in balancing treatment goals with surgical safety.A healthy term female underwent a spinal ultrasound on day of life 3 because of asymmetry in her intergluteal cleft. The ultrasound identified several small cysts adjacent to the coccyx, consistent with a small cystic SCT. Spinal magnetic resonance imaging (MRI) confirmed a 12 mm × 10 mm × 26 mm multiloculated cystic lesion in the pre- and post-coccygeal space. Serum AFP was measured to be 150 ug/L on day of life 104 which was appropriate for normal physiologic elevation at 3 months of age  demonstrated bilateral pulmonary metastases . A videoA 20 month-old female presented to hospital with abdominal pain and features of gastric outlet obstruction. Abdominal and pelvic CT scan delineated a 11.4 cm × 8.0 cm × 7.7 cm, mixed solid and cystic retroperitoneal mass obstructing the gastric outlet with areas of calcification and fat, consistent with a mature teratoma . AFP wasGiven the invasive nature of this apparently benign tumor, two cycles of carboplatin, etoposide, and bleomycin, were given to see if any cytoreduction could be achieved. Post-chemotherapy imaging revealed no change in the areas of invasion with slight overall enlargement of the tumor.Magnetic resonance cholangiopancreatography demonstrated a margin of normal duodenum below the Ampulla of Vater and above the site of duodenal invasion by tumor. A second laparotomy was undertaken with a goal of complete tumor resection. The tumor capsule was densely adherent to the duodenum, inferior mesenteric artery, left renal hilum, and aorta. A predominantly extracapsular tumor resection included the 3rd and 4th parts of the duodenum , mesentery of the transverse colon, and the inferior mesenteric artery near its origin. The dilated 2nd part of duodenum was anastomosed end to end to the proximal jejunum, and the transverse and descending colon were resected, leaving an ascending colostomy and distal Hartmann pouch. Procedural blood loss was 500 cc and an intraoperative transfusion was required. The child returned to the operating room 2 weeks later for duodenojejunal anastomotic revision, and then several months later for colostomy closure. Pathological examination of the tumor was that of mature teratoma without immature elements, with a positive epithelial margin noted on the posterior aspect. She remains free of recurrent disease at 24 month clinical and radiological follow-up.A previously healthy 2 month-old male presented to hospital with a large abdominal mass and 1 week history of non-bilious emesis. Abdominal MRI demonstrated a 11.3 cm × 10.5 cm × 8.1 cm, mixed solid-cystic, encapsulated retroperitoneal mass . The tumThe patient underwent bilateral ureteral stent placement followed by an exploratory laparotomy which revealed a large, partially encapsulated tumor. The tumor was adherent to the mesentery of the transverse and descending colon, and superiorly to the root of the small bowel mesentery, but these attachments were carefully dissected exposing the anterior aspect of the tumor. Posteriorly the tumor was adherent to the aorta and IVC, and it became clear that a complete extracapsular dissection would not be feasible or safe. Notably, the tumor encased the left proximal ureter and was densely adherent to the left renal artery and vein. Following tumor resection, efforts were made to go back and excise areas of remnant capsule. The patient received a transfusion for an estimated intraoperative blood loss of ~300 cc.Pathology on the resected specimen was predominantly immature teratoma, grade 2–3, with focal yolk sac tumor. There was a positive left retroperitoneal margin (immature teratoma) corresponding to areas of adherence to the left kidney and ureter. Two weeks after surgical resection, his AFP had dropped to 3,000 ug/L, and it continued to decrease thereafter, reaching a normal range within 2 months of surgery.Three months after surgery, a recurrence in the left retroperitoneum was noted on follow-up ultrasound and confirmed on MRI. Serum AFP remained within normal limits for his age. The patient was taken back to the operating room for excision of recurrent tumor which was again encasing the left ureter and hilar vessels, and infiltrating the descending colon mesentery, requiring a left nephrectomy and partial left hemicolectomy with a primary anastomosis. Pathology on the resected recurrence was that of mature teratoma with no immature or yolk sac elements. At 15 month follow-up, he remains clinically and radiologically disease-free.The treatment sequence, intermediate and final outcomes for each case are summarized in Germ cell tumors arise from molecular aberrations in early germline progenitors known as primordial germ cells. These cells migrate from the yolk sac along the gonadal ridge in the midline of the developing embryo. Disruption of the migration process is the likely explanation for the midline propensity of extragonadal GCTs. The pluripotent nature of these cells accounts for the range in observed histology. Immature teratomas contain less differentiated tissue, including neuroectoderm, and are assigned a grade (I-3) according to the degree of dedifferentiation. Although mature and immature teratomas are benign tumors, the need for thorough histologic evaluation of immature teratomas, particularly those with high grade features, is important given the known association with microscopic foci of malignancy .There are several factors which increase the likelihood of SCT recurrence after resection, including incomplete resection, failure to remove the coccyx and tumor rupture/spillage during surgery . BecauseA recently published meta-analysis of 22 studies identified a total of 999 patients with mature histology following SCT resection, from which there were 102 recurrences (10.2%). Of these, 57 (56%) proved to be malignant recurrences, and almost all were yolk sac tumor . Other sRecently published guidelines for follow-up surveillance of resected SCT with benign (mature or immature) histology emphasize the importance of physical examination, selective imaging and serum AFP monitoring until at least 3 years after initial treatment . FollowiThese rare tumors usually present as an abdominal mass in infancy, although they can also be diagnosed prenatally . PreoperOther reports describe similar challenges in the surgical management of RT. Jones and Burns describe 6 children (5 infants) with benign RT that displaced and/or enveloped the aorta and IVC with infiltration of the mesentery and adjacent viscera . They reIn summary, we report three cases of GCT (2 in infants) which highlight some of the unusual biologic tendencies of these rare tumors. An awareness of the estimated 10% frequency of malignant recurrence of presumed benign GCT underscores the importance of early and frequent postoperative tumor marker surveillance to ensure timely detection of recurrence. An appreciation of the propensity for RT to be locally invasive despite their benign histology, should inform preoperative preparation and guide intraoperative decision-making. Finally, pre- and post-operative multidisciplinary discussion of these unusual tumors increases the likelihood of care optimization.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Written informed consent to participate in this study was provided by the participants' legal guardian/next of kin. Written informed consent was obtained from the minor(s)' legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.All authors listed have made a substantial, direct and intellectual contribution to the work, and approved it for publication.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Primary pulmonary artery sarcoma (PPAS) is a rare malignancy arising from mesenchymal pulmonary artery cells and mimics pulmonary embolism. Palliative chemotherapy such as anthracycline- or ifosfamide-based regimens and targeted therapy are the only options. However, the evidence of clinically beneficial systemic treatment is scarce. Here, we report a case of disseminated PPAS achieving clinical tumor response to olaparib based on comprehensive genetic profiling (CGP) showing genetic alterations involving DNA repair pathway. This provides supportive evidence that olaparib could be a promising therapeutic agent for patients with disseminated PPAS harboring actionable haploinsufficiency of DNA damage repair (DDR). Primary pulmonary artery sarcoma (PPAS) is a rare malignancy, with an incidence rate of 1–30 per 100,000 people ,3. Due tAlthough surgical resection has been demonstrated to prolong survival ,7, the iMDM2 amplification; CDKN2A homozygous deletion; and RAD50, PTCH1, PTEN, CHEK1, MRE11, BRCA2, RB1,and BLM hemizygous deletion, treated with next-generation sequencing (NGS)-guided olaparib (Lynparza®) and achieving a transient partial response.Herein, we present our experience of treating a patient with PPAS harboring ®+) for molecular-matched therapeutic options [A 71-year-old woman had progressively exertional dyspnea associated with productive cough and chest tightness for half a year. A computed tomography (CT) scan of the chest revealed a left main pulmonary arterial embolism and multiple metastatic nodules over both lung fields . A CT-gu options .® + test  to detect mutations in the coding region of 440 cancer-related genes and fusion of 31 genes. NGS was conducted at a mean depth of 1298x and 91% uniformity. Twelve nonsynonymous mutations were identified, while none of them was considered clinically significant (The FFPE lung tumor was subject to next-generation sequencing (NGS) using the ACTOnconificant . The gerMDM2, classified as an oncogene, was identified and confirmed by immunohistochemical (IHC) staining, suggesting that MDM2 is a potential driver in sarcoma. MDM2 inhibitors may be the optimal treatment; however, they are not available outside clinical trials. The homozygous deletion of CDKN2A and hemizygous deletion of multiple tumor suppressor genes (TSGs) with haploinsufficiency, including RAD50 (chr 5), PTCH1 (chr 9), PTEN (chr 10), CHEK1 (chr 11), MRE11 (chr 11), BRCA2 (chr 13), RB1 (chr 13), and BLM (chr 15), were determined by observed copy number < 2 copies and zygosity of SNPs located on the genes  revealed that the lung tumors remained stable . After oThis report was approved by the Institutional Review Board of Chang Gung Medical Foundation (202100118B0). Written consent was obtained from the patient’s legal guardian to use the images included in this report.TP53 (47%), CDKN2A (36%), CDKN2B (25%), and RB1 (13%). In contrast, 11% of PPAS harbored clinically relevant genomic alterations that affected PDGFRA, RICTOR, CDK4, and KIT [EGFR, TSC2, ALK, and BRAF was considered actionable [In this case report, a patient with disseminated PPAS was treated with CGP-guided olaparib and achieved a transient partial response. Studies on CGP in PPAS are rare and only reported in abstracts ,12. In 2 and RB1 %. In contionable . No micrMDM2 gene in this tumor, as confirmed by IHC, might be one of the primary drivers [MDM2 amplification was observed in 18.6% of sarcoma cases based on the TCGA database (PanCancer Atlas). Notably, MDM2 amplification is most common in well-differentiated liposarcoma/atypical lipomatous tumors, dedifferentiated liposarcoma, intimal sarcoma, and low-grade osteosarcoma [In this case, several identified clinically relevant copy number variants provided the rationale for using targeted therapy . All of  drivers . MDM2 amosarcoma .CDKN2A was also found, implying that the p14/MDM2/p53 axis played an essential role in tumorigenesis in this particular case. MDM2 has been reported to be associated with tumorigenesis in multiple solid tumors and sarcoma, as it inhibits the function of p53 (a tumor suppressor) via ubiquitination, leading to proteasomal degradation. Targeting MDM2 alone or in combination with other agents could be a therapeutic strategy in cancer treatment [MDM2 inhibitors are still under investigation in early clinical trials and are clinically unavailable [Moreover, a homozygous deletion of reatment ,17. Howevailable . In addivailable . As a reRAD50, BRCA2, BLM, CHEK1, MRE11, PTEN, ATM, and H2AX) might highlight a possible therapeutic route. According to Sanmartin et al., haploinsufficiency of DDR genes localized in 11q may confer higher sensitivity to olaparib treatment in neuroblastomas [Alternatively, the observed hemizygous deletions of several DDR genes (lastomas . The collastomas . This relastomas . This deBRCA1/2 pathogenic mutations and the loss or disruption of crucial HRR genes [MRE11, CHEK1, RAD51, PTEN deficiency; knockdown of RAD50; and BLM have all conferred sensitivity to PARP inhibitors across different tumor types [BRCA1/2 mutations [Clinical trials have demonstrated the effective induction of synthetic lethality in tumors harboring RR genes ,24,25. Tor types ,30,31,32or types ,34 and cor types . These putations . No docuGiven the lack of targeted therapies for patients with PPAS, olaparib could be a promising therapeutic agent for patients with disseminated PPAS harboring actionable haploinsufficiency of DDR genes. Future studies using NGS-based CGP to guide the treatment of PPAS-harboring haploinsufficiency of DDR genes are eagerly awaited.


Colonic lipomas (CLs) are a rare benign neoplasm of the gastrointestinal tract and the second most common tumor of the colon after adenomatous polyps. The clinical presentation of lipomas depends on the size of the tumor. Lesions that are less than 2 cm are mostly asymptomatic and are incidental findings during procedures like colonoscopies, imaging, or surgery. CLs that are greater than 2 cm can present with abdominal pain, nausea, intestinal obstruction, diarrhea, bleeding, and intussusception. Barium enema and colonoscopy can provide diagnostic clues; however, abdominal computed tomography (CT) confirms the diagnosis. Treatment modalities range from observation to segmental colectomy and endoscopic or laparoscopic procedures. Surgical resection is preferred for CLs that are either symptomatic or greater than 2 cm. Giant lipomas are often symptomatic and amenable to treatment, with surgical resection, laparoscopy, and endoscopic resection being the preferred treatment modalities. In this case report, we discuss a case of a 71-year-old white female who presented with abdominal pain and was found to have a relevant intestinal obstruction due to a 7.2 x 4.7 x 5.3 cm fatty mass arising from the transverse colon. Right hemicolectomy was performed, and histopathological examination revealed a lipoma of the transverse colon. Intestinal lipomas are one of the rare types of gastrointestinal tumors ,2. The i3), normal platelet count , potassium levels (3.1 mmol/L), alkaline phosphatase (136 units/L), and normal aspartate transaminase and alanine transaminase. The serum carcinoembryonic antigen, total protein, and serum creatinine levels were within the normal range. Abdominal CT of the bowel and mesentery demonstrated a remarkably abnormal transverse colon. There was a large fatty mass within the colon that measured 7.2 x 4.7 x 5.3 cm. The right colon was fluid filled suggestive of relative intestinal obstruction , white blood cell count  . EndoscoA colonoscopy reveals various characteristics features of lipoma such as elevation of the mucosa stretched over the lipoma (“tenting sign”), an impression of soft mass under the biopsy forceps (“cushion sign”), and finally the visualization of yellow fat after performing a biopsy (“naked fat sign”) ,17. An aTreatment options vary from observation to endoscopic to laparoscopic or surgical removal . Choice CLs are rare alimentary masses that can cause intestinal obstruction and intussusception. Colonoscopy and CT are useful diagnostic tools. The surgical approach is considered for lesions greater than 2 cm. Laparoscopy and mini-laparotomy could be future considerations for management. However, in some cases, the endoscopic reduction might be the optimal primary treatment modality. There is little to no chance of recurrence once CLs are removed.


Abdominal ultrasound showed gallstones. Although initially cholecystitis was suspected and cefmetazole was administered, the fever lasted for 1 week. Contrast-enhanced computed tomography (CECT) revealed enlarged spleen, multiple ill-defined splenic hypodense foci, and no lymphadenopathy ((1). An ultrasound-guided splenic core needle biopsy was performed, which led to the diagnosis of diffuse large B-cell lymphoma . However, splenic imaging studies may help establish the diagnosis. In particular, if a patient presents with persistent fever and splenomegaly, both of which are common symptoms in daily practice, clinicians should keep a high index of suspicion of PSL and perform CECT (3). To arrive at the correct histopathological diagnosis, although splenectomy has generally been performed, splenic biopsy can nowadays be used as an effective diagnostic method with low complication rates (4).The spleen is frequently recognized as the “forgotten organ” among clinicians NoneHF acquired data and drafted the manuscript. AK, KW, and MS reviewed and supervised the manuscript.We have obtained informed consent for this manuscript.In this study, IRB approval was not required.


Adenomatoid tumor (AT) is an uncommon benign neoplasm of mesothelial origin, usually occurring in the female and male genital tracts. Extragenital localization such as the adrenal gland is extremely rare. Until now, only 39 cases of adrenal AT have been reported in the English literature. Here we report two novel cases of adrenal AT that occurred in male patients aged 30 and 31 years. The tumors were discovered incidentally by computed tomography (CT). Macroscopically, the tumors were unilateral and solid, and the greatest dimension of the tumors was 3.5 and 8.0 cm, respectively. Histologically, the tumors consisted of angiomatoid, cystic, and solid patterns and infiltrated the adrenal cortical or medullary tissue. The tumor cells had low nuclear/cytoplasmic ratio, with no pathological mitosis or nuclear pleomorphism. Thread-like bridging strands and signet-ring-like cells could be seen. Immunohistochemically, the tumor cells were positive for epithelial markers  and mesothelial markers . The Ki-67 index was approximately 1 and 2%, respectively. The differential diagnosis of adrenal AT includes a variety of benign and malignant tumors. The patients had neither local recurrence nor distant metastasis at 21 and 8 months after removal of the tumor. In the literature review, we comprehensively summarized the clinical, morphological, immunohistochemical, and prognostic features of adrenal AT. Adrenal ATs are morphologically and immunophenotypically identical to those that occur in the genital tracts. Combining the histology with immunohistochemical profiles is very supportive in reaching the diagnosis of this benign tumor, helping to avoid misdiagnosis and overtreatment. Adenomatoid tumor (AT) is a benign neoplasm originating from mesothelial cells. It is commonly encountered in the genital tracts, especially the uterus and fallopian tube in females and paratesticular sites in males , 2. ExtrCase No. 1: A 30-year-old man admitted to the hospital complained of palpitation and dizziness for one year. The laboratory results, including routine blood examination, serum cortisol, and ketosteroid levels, were within the normal range. Computed tomography (CT) examination showed a relatively well-demarcated, oval mass measuring 34 × 24 mm in the right adrenal gland. A laparoscopic right adrenalectomy was performed. After surgery, the patient was discharged from the hospital and had an uneventful recovery. No recurrence or metastasis was observed during 21 months follow-up.Case No. 2: A 31-year-old man presented to the hospital for a routine physical examination, and a mass lesion was incidentally detected by CT. He was asymptomatic, and CT demonstrated a left adrenal mass measuring 77 × 43 mm which was regarded as an adrenal adenoma. Preoperative laboratory examinations revealed that the urinary vanillylmandelic acid, serum cortisol, and ketosteroid levels were within normal limits. A laparoscopic left adrenalectomy was performed. The postsurgical recovery was also uneventful. The patient had no signs of recurrence or metastatic lesion after 8 months follow-up.via electronic searches through PubMed for articles that contained the following search terms, adenomatoid tumor and adrenal gland. Only those written in English were included.All the resected specimens were routinely fixed in 10% neutral buffered formalin and embedded in paraffin. Sections of 4 μm in thickness were stained with hematoxylin and eosin for histopathological examination. Immunohistochemical analyses were performed using the Ventana Ultra View Universal DAB Detection Kit, and primary antibodies were obtained from different companies see Table 1.The excised adrenal tumor specimens measured 3.5 × 2.0 × 1.0 cm and 8.0 × 6.5 × 3.0 cm in size, respectively. The tumors were well circumscribed. On cut surface Figure 1Histologically, the tumor cells infiltrated and compressed the normal adrenal cortical or medullary tissue, imparting an appearance of an infiltrative growth pattern. The invasion of the capsular or periadrenal adipose tissue was not detected. The tumors consisted of different patterns: angiomatoid, cystic, or solid. The angiomatoid pattern was composed of anastomosing glands or small to medium sized tubules lined by flattened or cuboidal cells with scant-to-moderate amounts of eosinophilic cytoplasm , with more than half of the patients younger than 40 years of age. There are 18 cases of adrenal AT located in the left adrenal gland and 22 cases located in the right adrenal gland, and the location of one case was unavailable. ATs primarily occurred in males, accounting for 39 of 41cases.All tumors presented in patients as incidental radiological, surgical, or autopsy findings. The tumor in thirty-four patients was discovered incidentally during radiological examinations, six patients during autopsy and one patient during surgery for resection of rectal adenocarcinoma. Most of the patients were asymptomatic and physical examination was non-specific. Some patients presented with symptoms or comorbidities. Ten cases of adrenal AT were found to be associated with hypertension. Three patients had nephrolithiasis. Two patients had a history of chronic abdominal pain. One patient suffered from painless gross hematuria. One patient of our case complained of palpitation and dizziness. The tumors in other patients were discovered during the investigation in the course of Cushing syndrome, sarcoma staging, acute cholecystitis, pneumonia, diverticulitis, inguinal hernia, and acquired immune deficiency syndrome (AIDS). Six cases of adrenal AT were found during autopsy, and the cause of death of four patients was not available. One patient with HIV-1 infection developed acute bilateral pneumonia with severe respiratory failure and died. The immediate cause of death was disseminated coccidioidomycosis. Another patient died of acute coronary thrombosis resulting in heart failure after drinking alcohol.Macroscopically, the tumors ranged from 0.5 to 19.0 cm  in greatest dimension. The dimension of two cases was unavailable. Twenty-three cases were presented as a solid mass, whereas twelve cases have been described as mixed solid and cystic, and only six cases as entirely cystic.Histologically, extension of tumor into cortex/medulla or periadrenal adipose tissue was observed in 58.5% (24/41) of cases and one tumor enwrapped the ipsilateral renal artery and vein. Most of tumors contained multiple histologic patterns, such as adenoidal, angiomatoid, cystic, papillary, and solid patterns. Adenoidal, angiomatoid or cystic patterns were common in AT. Only 11 cases of tumor had solid patterns, and four cases had papillary patterns. All the tumor cells had low nuclear/cytoplasmic ratio, with no pathological mitosis or nuclear pleomorphism. Signet-ring-like cells were described in 23 cases, and lymphocytes infiltration or aggregates were observed in 25 cases. Ultrastructural study was done in 10 cases, and all displayed numerous long, bushy, and slender microvilli that were typical of mesothelial-derived cells.Immunohistochemically, the tumor cells were positive for epithelial markers, such as AE1/AE3, CAM5.2, and CK7, and mesothelial markers, such as calretinin, D2-40, and WT-1. However, the tumor cells were weakly or focally positive for CK5/6, which was also the marker of mesothelial cells. Negative staining was observed for CD34, CD31, HMB45, Melan-A, Actin, Desmin, Syn, and CgA.No local recurrence or metastasis of adrenal AT has ever been reported in 18 patients who were followed up for 3–177 months, and one patient died of pulmonary carcinoid.Adenomatoid tumor (AT) of the adrenal gland is extremely rare. From 1988 to 2020, only 39 cases of adrenal AT were reported in the English medical literature, among which only four authors reported more than one case of adrenal AT , 14, 18,Adrenal ATs tend to occur in adults in their fourth decade of life, but there is a wide age range. The tumor has a significant male predilection . A possible explanation related to the different roles of mesonephric ducts in males and females in embryological development together with the hypothesis that ATs arise from primitive mesenchymal cells associated with the Mullerian tract , 34. TheUsually, adrenal AT is non-functioning, asymptomatic and discovered incidentally during radiological examinations, surgical procedures for other unrelated reasons, or at autopsy. Radiological examinations are not specific for adrenal ATs. In rare occurrences, it is associated with symptoms or comorbidities, such as hypertension, AIDS, disseminated coccidioidomycosis, hematuria, Cushing syndrome, adrenal cysts, and kidney stones. About 24.4% (10/41) of adrenal AT was associated with hypertension. We speculate that adrenal AT may be associated with hypertension. However, the inherent relationship of hypertension and adrenal AT needs further investigation. Angeles-Angeles et al.  reportedMacroscopically, the tumors are usually solid, but rarely, they present as solid-cystic or completely cystic tumors, which may probably be misdiagnosed as lymphangioma , 36. On Microscopically, the tumors may extend into the adrenal capsule, cortex, medulla, or extra-adrenal adipose tissue. We should note that this is not the diagnostic criterion of malignancy. The tumors have multiple patterns of growth, including adenoid, angiomatoid, cystic, solid, and papillary. About half of the cases showed two or more patterns . In our Immunostaining of tumor cells is usually diffusely positive for epithelial markers  , 36, 38 Because of the unusual location, lack of specific clinical and radiological features, the blurred boundary between the tumor and the adjacent tissue, existence of various growth patterns, adrenal ATs may lead to difficulties in diagnosis and need to be differentiated from other tumors. Differential diagnosis covers a variety of entities of benign and malignant tumors.Benign tumors mainly include lymphangioma and angiomyolipoma. Lymphangioma is a benign hamartomatous tumor which is characterized by abnormal proliferation of lymphatic vessels that usually present as cystic masses, with hemangioma, cystic architecture, and lymphocyte aggregates similar to ATs, but lymphangioma shows absence of a solid pattern and is negative for epithelial markers, WT-1 and calretinin . AngiomyThe malignant tumors include epithelioid hemangioendothelioma, malignant mesothelioma (MM), and metastatic adenocarcinoma. Epithelioid hemangioendothelioma is a vascular neoplasm with cords or small nests of round endothelial cells with abundant eosinophilic cytoplasm . Tumor cAs for treatment, surgery is the main treatment, and postoperative adjuvant treatment is not required. Although only limited clinical follow-up data is available, no local recurrence or metastasis has been reported in adrenal ATs. In our two cases, patients have a good prognosis after surgery.Adrenal AT is a benign neoplasm of mesothelial derivation. The tumors tend to occur in adults in their fourth decade of life and have a significant male predilection. Most of the tumors are asymptomatic, discovered incidentally by imaging during surgery or at autopsy. The tumors are well circumscribed and solid, with a grayish yellow cut surface. Histologically, the tumor may extend into the adrenal capsule, cortex, medulla, or extra-adrenal adipose tissue. There is a mixture of multiple patterns of growth, including adenoid, angiomatoid, cystic, solid, and papillary. No cellular atypia, pathological mitosis, or necrosis can be seen. Signet-ring-like cells and thread-like bridging strands can be found. Lymphocyte infiltration and aggregates also exist. The tumor cells are positive for epithelial markers  and mesothelial markers . The differential diagnosis includes lymphangioma, angiomyolipoma, epithelioid hemangioendothelioma, malignant mesothelioma, and metastatic adenocarcinoma. Complete excision is the main treatment. No recurrence or metastasis has been reported. Histology and immunohistochemical profiles are very supportive in reaching the diagnosis of adrenal ATs, helping to avoid misdiagnosis and overtreatment.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.JG and CZ: conceived the idea of the work, contributed significantly to analysis and manuscript preparation, performed the data analyses, and wrote the manuscript. HL: contributed to the manuscript preparation and reference collection, performed the data analyses. WZ and WL: stained sections of 4 μm in thickness with hematoxylin and eosin, performed the experiment of immunohistochemical analyses. LT: provided revision opinions and revised the manuscript. JC: supervised the study, funding support, and revised the manuscript. All authors contributed to the article and approved the submitted version.This work was supported by the Natural Science Foundation of Guangdong Province (2018A030313650), the Guangzhou Science and Technology Project (202102010156) and the NSFC cultivating grant of The Third Affiliated Hospital, Sun Yat-sen University (2020GZRPYMS01).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


A 29-year-old male presented with a seven-year history of a slow-growing, painless, firm, mobile mass in the right upper back that was bothersome when supine or with direct pressure. On initial presentation, a clinical diagnosis of lipoma was given. The mass progressively increased in size over several years but remained painless. The mass measured 15 x 10 cm on examination. Excision of the lesion was performed, which revealed a white cut surface with cystic degenerative changes. Histologically, the lesion revealed spindle cell morphology with occasional mitosis. Diffuse immunohistochemical staining with MUC4 supports a diagnosis of low-grade fibromyxoid sarcoma (LGFMS). Tumor was present with focal extension into the deep margin. However, serial magnetic resonance imaging studies performed suggest no residual disease and negative regional lymph node involvement. This case demonstrates the growth pattern of LGFMS, but also denotes the importance of correlating radiological and pathological features to accurately diagnose and treat these tumors in a timely fashion. Low-grade fibromyxoid sarcoma (LGFMS) is a rare form of malignant tumor that often presents as a painless, slow-growing mass in the deep soft tissues of the trunk or proximal extremities of young adults ,2. This A 29-year-old male with no significant past medical history presented to his primary care provider for an enlarging mass on his upper back, inferior and lateral to the right scapula. He complained of discomfort when lying in the supine position and with direct pressure on the mass. He otherwise had no local pain, erythema, warmth, or drainage from the mass. This was originally noticed by the patient about seven years prior and was described as firm, mobile and about 5 cm in diameter. He was given a working diagnosis of lipoma pending further workup. After referral to dermatology, the mass was noted to be firmer than a characteristic lipoma, and the patient was scheduled for excision with the department of surgery. At that time, the patient was lost to follow up. On return visit seven years later, he presented with the complaint that the mass had grown. On examination, the mass remained firm, mobile, and non-fluctuant.Surgical excision of the mass resulted in retrieval of a 15 x 10 cm cystic mass adherent to the superior portion of the trapezius muscle, but non-adherent to deeper structures or the scapula. On gross examination, the outer surface was tan-white and smooth. Serial sectioning revealed a tan-white cut surface with cystic degeneration Figure  and negaUpon pathological analysis, the deep margin was positive for the tumor. This case was discussed by a multidisciplinary cancer tumor board and close follow-up with imaging was recommended. Post-operative magnetic resonance imaging (MRI) at one month showed focal nodular enhancement within a small fluid collection and nodularity thought to be either post-surgical change or residual tumor Figure . The patLGFMS is a potentially challenging diagnosis, as its indolent growth pattern and benign appearance on small biopsy could be mistaken for benign soft-tissue tumors due to its late metastatic potential ,2. Most Regarding potential for late metastasis in LGFMS, this is most commonly seen with involvement of the lungs. Of note, in Evans' (2011) study following 33 LGFMS cases with long-term follow-up, metastasis occurred in half of the patients and there was a 42% mortality rate [The main differential diagnosis of LGFMS includes myxofibrosarcoma (MFS), dermatofibrosarcoma protuberans (DFSP), desmoid fibromatosis, nodular fasciitis, malignant peripheral nerve sheath tumor (MPNST), and sclerosing epithelioid fibrosarcoma (SEF).MFS is a malignant fibroblastic tumor typically found in elderly populations. It is commonly located in the dermis and subcutis of extremities. MFS usually has abundant myxoid stroma and less fibrous areas compared to LGFMS. MFS is also pleomorphic and hyperchromatic compared to LGFMS, which has comparatively bland cytology. Low-grade MFS most closely mimics the presentation of LGFMS. Due to high risk of local recurrence, treatment generally includes adjuvant radiotherapy to improve outcomes ,6.DFSP typically presents as an asymptomatic, slow-growing violaceous nodule or plaque. Highest incidence is noted in patients 30-50 years old, and incidence is nearly double among blacks compared to white patients. These tumors are often asymmetric, and detailed mapping has shown long tentacle-like projections that extend beyond 3 cm . For thiLGFMS may also show areas histologically similar to desmoid fibromatosis. These tumors, however, are not well circumscribed, show infiltrative borders, and exhibit myofibroblastic differentiation. There is a significant association of these tumors with familial adenomatous polyposis. Surgical intervention with active surveillance has now been suggested as a reasonable initial strategy in most patients with desmoid fibromatosis. Other non-operative options include nonsteroidal anti-inflammatory drugs, hormone therapy, cytotoxic chemotherapy, and targeted agents .Nodular fasciitis is a benign tumor that often originates in the extremities, head, neck, and trunk. These tumors are most often also solitary, well-circumscribed lesions found in adults 20-40 years old. Diagnosis can be aided by positive staining for alpha-smooth muscle actin. Simple excision is the most common treatment and post-resection recurrence is noted to be very rare .MPNST is yet another form of soft-tissue sarcoma. One-half of these tumors arise in patients with neurofibromatosis type I, and these tumors may be preceded by peripheral neuromas or atypical neurofibromas. They often show positive staining for S100, which is not present in LGFMS. While workup and treatment of these precursor tumors vary, once diagnosed with MPNST, complete surgical resection with wide tissue margins is required. Chemotherapy and radiation therapy may also be done as indicated .SEF is a diagnosis that has been thought to exist on a spectrum with LGFMS, but represents a much more aggressive tumor with high rates of recurrence and metastasis. Patients with SEF are often of older age, found in the lower extremity, and have involvement of bone. Due to the rarity of the tumor, no clear treatment guidelines have been established, but amputation, wide excision, radio- and chemotherapy, and various combinations have been attempted .LGFMS is a slow-growing soft-tissue tumor with low-grade morphology, but metastatic potential. Thus, clinicopathologic correlation and adequate sampling are required for proper diagnosis. Long-term radiologic follow-up is also needed to monitor for metastasis.


Anaphylaxis is a potentially life-threatening condition caused by a variety of triggers. However, anaphylaxis following an abdominal trauma is an exceptionally rare condition and could be the first and only sign of hepatic hydatid cyst, especially when no obvious etiology is present. Here, we present such a rare case and discuss relevant diagnostic and management strategy in light of the literature.This case report refers to a 17 year-old previously healthy girl admitted in our pediatric emergency department (ED) for syncope after a minor blunt abdominal trauma. She was hypotensive on admission and shortly after she developed urticaria and angioedema. She was diagnosed with anaphylaxis and treated immediately. Possible etiologies including drug or food ingestion, insect bite, and previous allergy/anaphylaxis history were excluded. After stabilization abdominal imaging was performed, which revealed a ruptured large hepatic hydatid cyst in the vicinity of biliary tree. Albendazole treatment was started and surgical resection was performed after clinical stabilization, which confirmed the cyst rupture into the biliary ducts. Patient recovered without complications after surgery and was discharged uneventfully.This case report highlights that hydatid cyst rupture should be included in the differential diagnosis of anaphylaxis without obvious etiology, particularly in regions where hydatid disease is endemic. Ruptured hydatid cyst leading to anaphylaxis requires timely diagnosis, management and emergent intervention. Anaphylaxis is a severe and potentially life-threatening allergic reaction . It is eHydatid disease also known as cystic echinococcosis is a zoonotic disease. Infection occurs by ingestion of parasite eggs in contaminated food, water or soil, or through direct contact with animal hosts. Hydatid disease is endemic in certain parts of the world including Turkey. Infections with Echinococcus granulosus lead to one or more hydatid cysts located most often in the liver and may remain asymptomatic for years until hydatid cysts cause clinical signs . Most frHere, we report an unusual case in which anaphylaxis following mild abdominal trauma was the only presentation of hydatid disease in a 17-year-old female patient.Previously healthy 17-year-old female patient was admitted to the pediatric emergency department because of syncope and hypotension after walking into the classroom door at school when she had hit upper right side of her abdomen. Emergency medical services were noticed and she was transported to the ED via an ambulance. She seemed weak and confused. Her vital signs at admission were as follows: blood pressure 80/35 mmHg, heart rate 120 beats/min, temperature 38.5 °C. Her oxygen saturation on room air was 98%. Upon arrival, she demonstrated an urticarial rash mainly on her face and trunk together with angioedema of the eyelids and lips, and had severe abdominal pain, which occurred in approximately 15-20 min after the trauma. We treated her immediately with 0.5 mg intramuscular 1/1000 epinephrine for anaphylactic shock and initiated intravenous fluid resuscitation. Her response to the initial dose of epinephrine was poor, so that a repeated dosing was required after 15 min. Eventually, her blood pressure was maintained around 110/70 mmHg, approximately 10 min after the second epinephrine dose, with this treatment regime and her hemodynamic status was stabilized soon after. She also received intravenous prednisolone (50 mg) and diphenhydramine (50 mg) to prevent late reaction. Laboratory work-up revealed leukocytosis without eosinophilia and a high C-reactive protein level. Her bilirubin level was mildly elevated and she had hypoalbuminemia. The other laboratory tests were within normal limits  located in the fifth and sixth segment of liver was evident on both abdominal ultrasonography (Fig.  × 68 mm We present an adolescent girl with previously unknown hydatid disease of the liver, which manifested with anaphylaxis due to cyst rupture following a minor blunt trauma to the abdomen. This patient was asymptomatic until this event. Most patients with hydatid disease remain silent for several years until cysts enlarge enough to trigger clinical symptoms mainly due to mass effect in a small space or until complications arise , 6. AnapIn our case, rupture and leakage to the biliary tree was evident in accordance with the previously reported cases. Like most authors argued, we think that antigen release following cyst rupture may trigger the anaphylactic reaction , 11–16. This case report depicts a very rare case of minor blunt trauma-induced anaphylaxis caused by a rupture of a previously unknown hydatid cyst. We think that it is an informative and illustrative case for pediatricians, emergency and primary care physicians. This paper presents the diagnostic workup and clinical management process in detail. However, we were not able to obtain intraoperative picture or video, which could be demonstrative as well.Hydatid cyst rupture should be considered in the differential diagnosis of anaphylaxis following blunt trauma as a missed or delayed diagnosis may lead to severe complications and sudden death.


A 35-year-old homeless man presented with a sudden loss of left limb strength with brachial predominance and a recent history of weight loss and cough. Brain magnetic resonance imaging (MRI) revealed patchy foci of leptomeningeal nodular enhancement and acute ischemia in the right lentiform nucleus . Vessel The patient was HIV positive, with immunosuppression stigmas, such as oropharyngeal candidiasis, and off antiretroviral therapy . Chest computed tomography revealed diffuse miliary lung nodules; a cerebrospinal fluid polymerase chain reaction test was positive for neurotuberculosis.,,Cerebral vasculitis, which corresponds to the inflammation of blood vessel walls, may occur as a complication of neurotuberculosis and lead to secondary strokes,Magnetic resonance vessel wall imaging is a non-invasive method capable of detecting vascular inflammation, and arterial abnormalities may persist after treatment. Moreover, residual vascular lesions may be a source of disease recurrence. Therefore, follow-up studies may be helpful,Vessel wall enhancement with the absence of vascular complications, such as infarction, may significantly improve prognosis, with early treatment initiated to prevent complications


A colon lipoma is defined as a benign tumor made of adipose tissue present in the submucosa. Lipomas of the colon are unusual with a frequency of 0.035%-4.4%. We present an uncommon case of a 41-year-old female with left colonic lipoma causing intussusception. The patient presented with abdominal pain at the left iliac fossa. She underwent an emergency exploratory laparotomy plus left hemicolectomy and was followed in the surgical out-patient clinic postoperatively. She was found doing well taking a normal diet and having normal bowel habits. The overall prognosis depends on the complete removal of the tumor. A colon lipoma is defined as a benign tumor made of adipose tissue present in the submucosa . Lipomas3, and white count 7,500/mm3.A 41-year-old female with no previous comorbidity and no significant medical history presented with a history of abdominal pain at the left iliac fossa (LIF), which was relieved by defecation. She had four previous lower segment cesarian sections (LSCS) the last one being seven years back and an umbilical hernia repair six years back. The patient had constipation for over a few weeks. She felt nauseated and had episodes of vomiting and passed a bloody diarrhea stool. On physical examination, there was tenderness at the LIF with a mild abdominal distention. The patient was stable, appeared to be in pain, afebrile, the abdomen was flatulent, the heart rate was 95/min, and the respiratory rate was 19/min. Laboratory findings were as follows: hemoglobin 13.8 g/dL, hematocrit : 41.3%, platelets 462,000/mmA CT abdomen with oral and IV contrast showed an intussusception of the proximal part of the descending colon into its distal part Figure , with a A colonoscopy was done and the finding was a large mass lesion in the descending colon obstructing the lumen and the scope could not be passed beyond the mass. The patient was taken for surgery and underwent exploratory laparotomy and left hemicolectomy. The specimen was sent to the pathology lab and it confirmed a benign lipoma.The patient stayed few days in the hospital and the post-operative period was uneventful. The histopathology showed an ulcerated lipomatous lesion with hemorrhagic necrosis and perforation. Thirteen reactive lymph nodes were identified with the specimen. The margins were clear. The patient was followed in the surgical out-patient clinic postoperatively and was found doing well taking a normal diet and having normal bowel habits.Lipomas of the colon were described for the first time by Bauer in 1757 ,4. They In conclusion, the patient presented with signs of abdominal obstruction. Further investigations including a CT scan showed an intussusception with a posterior abdominal lipoma forming the lead point in this case. The patient was treated surgically, which is the treatment of choice for such conditions. Even though colonic lipomas are rare, they should be put in the differential diagnosis of bowel tumors presenting with symptoms.