Frantz’s Tumor or Solid Pseudopapillary Neoplasm (SPN) is rare with a solid-cystic pattern, and most common in young women.This study based on guidelines for case reports (SCARE) reports a case of SPN in a teenager aged 13 years at the diagnosis time, attended at a teaching public hospital in Brazil, which evolved into liver metastases. Clinical, laboratory, therapeutic and imaging data were collected from the physical chart and analyzed in light of current publications on the topic. The first consultation occurred in January 2012, where weight loss, fever, vomiting, left-sided hypochondrium and epigastric pain were reported. Imaging exams evidenced an expansive heterogeneous process in the pancreatic tail; however, laboratory exams and tumor markers did not present alteration in relation to reference values. In March of 2012, she underwent caudal body pancreatectomy, splenectomy, segmental colectomy and colo-coloanastomosis as a function of the intraoperative findings. After 63 months, right-sided hepatectomy was performed to resect metastases. Currently, she is undergoing outpatient monitoring, without complaint or alterations in imaging and laboratory exams, totaling 100 months of global survival.This is an interesting case report of a rare tumor, in so far as without any adjuvant chemotherapies, an 8-year long survival time could be achieved in this particular type of tumor despite initially large tumor expansion and later liver metastases.Additional epidemiological studies, molecular and clinical trials are required to increase knowledge on SPN. AccorAlthough the malignancy criteria are still not clearly, it occurs in about 15% of the cases, with deep invasion of the pancreatic parenchyma, perineural, angioinvasion or distant metastasis  .The present study, based on guidelines for case reports (SCARE), aimed to report a case of SPN in a 13-year-old child, attended at a teaching public hospital in the Amazon region of Brazil that evolved with liver metastases, but it has a high overall survival thanks to the surgical treatment performed.2A female adolescent, student, aged 13 years, no family history of cancer, realized the first consultation in January of 2012, presented a principal complaint of epigastric and left-hypochondrium pain, weight loss, fever and vomiting. She denies comorbidities, history of alcohol use, tobacco use or cancer in the family. The findings from imaging evidenced a heterogeneous expansive process in the tail of the pancreas (6.5 × 5.6 cm) . The labIn March of 2012, she was submitted to caudal body pancreatectomy, splenectomy, segmental colectomy at 15 cm of the transverse, colo-coloanastomosis and resection of 18 regional lymph nodes . It shouThe histopathology and immunohistochemistry concluded the presence of solid pseudopapillary neoplasm, encapsulated, measuring 4.5 × 4.3 × 3.5 cm, with bulging external surface, accentuated cystic alteration, hemorrhage and extensive area of necrosis, with free surgical margins, without evidence of vascular or neural invasion, absence of lymph-nodal metastasis and positive for Vimentin, Cytokeratins (AE1/AE3) and CD10 .During 30 postoperative months, the patient reported retrosternal “burning” during outpatient accompaniment, with improvement after the use of pantoprazole. The result of Total Abdominal USG showed the liver with irregular form, wrinkled surface and enlarged dimensions, parenchyma with altered echogenicity and heterogeneous echotexture. The liver vessels did not present alterations and serology was negative for viral hepatites. Then in the 38th month, the patient complained of a frequent, diffuse, slight pain in the abdominal region.In the 46th postoperative month, she referenced a sensation of postprandial gastric plenitude and sporadic fever. Abdominal ultrasound showed 4 hypoechoic rounded cystic images with interior trabeculated in segments VI, VII and VIII, measuring between 2 and 2.6 cm. Computed tomography of the abdomen identified the liver of normal size, with heterogeneous attenuations of the parenchyma, the largest of which being 4.8 cm in segment VIII, of lobulated contours and well-defined limits, without significant enhancement after injection of contrast .Fig. 3PoGiven the suspicion of liver metastasis, magnetic resonance of the upper abdomen was solicited (performed 50 months after surgery), which evidenced multiple oval encapsulated formations, diffuse in the liver parenchyma, with an aspect that was solid, cystic and non-characteristic, suggesting relation to secondary neoplastic implants. The tumor markers and thoracic CT did not present alterations.Magnetic resonance was repeated in the 56th postoperative month, and revealed the liver with normal dimensions and contours, but with multiple nodular images, without significant uptake of liver-specific contrast medium, suggestive of cysts . Yet another lesion was noted (5 cm in segment VIII), without significant uptake of contrast medium, possibly corresponding to a cyst with high protein/hematic content. Serology was also solicited for hydactidosis as a differential diagnosis and its result was negative.After 3 months of the same, the patient complained of pain in the right hypochondrium with irradiation toward the right costal gradient and right shoulder, with worsening upon inhalation; she also referenced dyspnea associated with clinical pain, being hospitalized for controlling pain and diagnostic elucidation.Given this presentation, at 63 months after the first surgery, the patient was referred to a surgical center to undergo exploratory laparotomy for enucleation of cystic lesions. However, in the intraoperative period it was possible to observe macroscopically lesioning in liver segments V, VI and VII, with biopsy being carried out by freezing of the lesion in segment V, which confirmed liver metastasis, with the surgeon opting to convert the surgery to right-sided hepatectomy, with removal of segments V, VI, VII and VIII. Immunohistochemistry of liver metastasis revealed expression positive for β catenin clone 14 and CD10 , findings consistent with solid pseudopapillary neoplasm (SPN) [The patient was hospitalized for 27 days and then discharged from the hospital, after which she received monthly outpatient follow-up with clinical oncology and the general surgical team, without complaints and with negative tumor markers , total abdominal CT without alterations.Starting in May of 2017, the outpatient return consultations became quarterly with the surgical team and semiannual with oncology. Adjuvant chemotherapy was not performed while laboratory and imaging exams were maintained within normality patterns, totaling global survival of 100 months after the first treatment, the first surgery.3This study showed a patient who at 13 years of age presented epigastric pain, weight loss and imaging findings suggestive of pancreatic tumor, with surgery being indicated. The literature highlights that SPN is most common in young women, with an average age of 30 years, and is rarer at 13 years ,7,8.She developed left hypochondrium pain and epigastric pain, palpable mass accompanied by nonspecific symptoms secondary to pancreatic compression (nausea and fever), similar to previous studies ,9,10. ThThe initial treatment of SPN is surgical, and it depends on the clinical-pathological characteristics of each patient, for which there is no standardization ,8. Some In this study, the histopathological result evidenced disease limited to the pancreas, with capsule intact and hemorrhagic signals on the external surface of organs adhering to the pancreatic tumor mass. Furthermore, after 30 months, the patient evolved with multiple liver metastases, even in total tumor resection.Local recurrence or distant metastasis is described in about 15% of SPN cases, with the liver being the most affected organ , associaHere, the patient manifested liver images suggestive of metastasis at 30 months after surgery, diverging from the liver metastasis incidence (15%) more commonly described from the early form  .Total tumor resection has ensured prolonged survival above 95% when SPN is limited to the pancreas or even curative in the resection of metastatic liver disease ,8. In adIt is concluded that this case report is important for ratifying the prognostic and clinical polymorphism spectrum of SPN. Furthermore, it is understood to be a borderline tumor with indolent biological behavior, there are still multiple poorly understood factors that can dictate its malignant transformation and prognosis. Therefore, there is a need for a long and rigorous segment to identify possible long-term metastases. It is necessary to conduct additional epidemiological studies, and molecular and clinical trials that can augment the knowledge on TSP.4The present study followed the guidelines for case reports (SCARE)  and concSecondly, the surgical procedure associated with regular and prolonged postoperative follow-up favored the diagnosis and resection of liver metastases in a timely manner, with positive impact on patient survival and quality of life. Currently, she is in the semi-annual and disease-free outpatient segment, with a total of 96 months overall survival. Lastly, it is necessary to conduct additional epidemiological studies, and molecular and clinical trials that can augment the knowledge on TSP.None.Funded by the authors.This case report was elaborated and developed according to the legal and ethical precepts of Resolution Nº466/12 from the National Health Council/Brazilian Ministry of Health and approved by the Committee for Ethics in Research in the Nucleus for Oncology Research under Protocol Nº 2.730.231.Written informed consent was obtained at 18 years old patient to publish this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Gisela: conception or design of the work and acquisition, Project administration; analysis data.Aline: conception or design of the work and acquisition, Project administration; analysis data.Ana: acquisition data.Samia: drafting the work or revising it critically for important intellectual content.Williams: interpretation of data for the work.Helena: acquisition data.Marta: acquisition data.Amanda: acquisition data.Geraldo: interpretation of data for the work.Paulo: Data curation; Formal analysis, drafting the work or revising it critically for important, intellectual content; review & editing.CAAE: 90972818.6.0000.5634.Paulo Assumption and Aline Cruz.Not commissioned, externally peer-reviewed.


Situs inversus totalis is a rare anatomical variation of both the thoracic and the abdominal organs. Common bile duct strictures can be caused by malignant and benign diseases as well. 7–18% of the latter ones are 'malignant masquerade’ cases, as pre-operative differentiation is difficult.We present the case of a 68y male patient with known situs inversus totalis and a recent onset of obstructive jaundice caused by a malignant behaving common bile duct stricture. Technically difficult endoscopic retrograde cholangiopancreatography, brush cytology, magnetic resonance cholangiopancreatography, endoscopic ultrasound, and percutaneous transhepatic drainage with stent implantation were performed for proper diagnosis. Cholecystectomy, common bile duct resection with hilar lymphadenectomy, and hepatico-jejunostomy have been performed following multidisciplinary consultation. The final histology report did not confirm any clear malignancy, the patient is doing well.In situs inversus patients, both diagnostic and therapeutic procedures can lead to various difficulties. Benign biliary strictures are frequently misdiagnosed preoperatively as cholangiocellular carcinoma. Surgery is usually unavoidable, involving a significant risk of complications. The co-existence of these two difficult diagnostic and therapeutic features made our case challenging.The online version contains supplementary material available at 10.1186/s12893-021-01155-w. Situs inversus totalis (SIT) is a rare congenital abnormality characterized by a mirror-image transposition of both the abdominal and the thoracic organs. Its incidence accounts for 1/8000 to 1/25,000 of the normal population . Still, A 68-year-old male patient was referred to our tertiary surgical institute having obstructive jaundice, pruritus and subcutaneous haematomas for two months. At the age of 10, chest X-ray revealed the situs inversus totalis  confirmed a normal size liver located on the left side; the intra- and extrahepatic bile ducts and the common bile duct (CBD) were dilated Fig. , 3. VascAfter the surgery, the patient had a minor wound infection treated by bedside wound opening (Clavien-Dindo classification stage I); he did not have any other surgical complications, and was discharged after 21 days. A multidisciplinary oncology team discussed the case again after the procedure, and no oncological treatment was considered necessary. After 3 months of the surgery, the control CT scan was completely tumour-free  is a rare gastrointestinal malignancy , originating from the intra- or extrahepatic bile duct epithelium . Still, The rate of benign strictures misdiagnosed preoperatively as cholangiocellular carcinoma is high, varying data can be found in the literature: about 7–18%, even up to 50% , 16, 17.For middle and distal common bile duct carcinomas, pancreato-duodenectomy was the gold standard procedure, but nowadays bile duct segment resection with lymphnode dissection is also established . CompletIn our case, the intraoperative frozen section proved malignant cells in the samples, but the final histological report came back without any clear malignancy. During the procedure of frozen sectioning, the nuclei of the epithelium cells can be injured: in such a case, it is difficult to discriminate the epithelium for dysplasia or carcinoma in situ . On the This case was rare and challenging in every aspect: the malignancy mimicking biliary stenosis in a situs inversus patient required both diagnostic and therapeutic considerations, as well as performing a major surgery in an unusual anatomic situation. At 6 months of follow up, the patient was doing well: the control CT scan did not prove either recurrence or any other complications.Additional file 1: Timeline of diagnostic and therapeutic steps.


Hairy polyps, also known as dermoid polyps (DPs), are rare benign cystic lesions of bigerminal origin that may occur in several head and neck regions, including the oropharynx. Despite their benign histopathological nature, DPs may be life threatening, due to their upper airway location, and DPs represent one of the most unusual causes of respiratory distress during the neonatal period. In this paper, we describe a case of respiratory failure in a newborn with an oropharyngeal mass that was accidentally found during difficult intubation. Magnetic resonance imaging (MRI) detected a well-defined soft tissue pedunculated mass, arising from the left oropharynx wall, consistent with an oropharyngeal DP. The newborn had a prompt recovery after trans-oral mass removal. Our case underlines the importance of imaging in differential diagnosis of children’s respiratory distress, secondary to a variety of lesions within the region of the skull base or oropharynx. It allowed us to assess the origin of the lesion, as well as its relationship with the adjacent soft tissues, and to exclude intracranial extension, thus providing essential information for the surgical planning.


Juvenile nasopharyngeal angiofibroma is a rare vascular neoplasm, mostly occurring in adolescent males, and representing 0.05% of all head and neck tumors. Nevertheless, it is usually recognized as the most common benign mesenchymal neoplasm of the nasopharynx. Usually, it originates from the posterolateral wall of the nasopharynx and, although histologically benign, classically shows a locally aggressive behavior with bone destruction as well as spreading through natural foramina and/or fissures to the nasopharynx, nasal and paranasal cavities, spheno-palatine foramen, infratemporal fossa and, very rarely, to the cranial cavity. Extranasopharyngeal angiofibroma is considered a distinct entity due to older age at presentation, different localizations  and attenuated clinical course. Extranasopharyngeal angiofibroma has been sporadically described in the oral cavity. We report a case of extranasopharyngeal angiofibroma with primary and exclusive involvement of the adherent gingiva of the anterior maxilla, managed by preoperative diode laser trans-mucosal photocoagulation and subsequent surgical removal. The current literature on primary extranasopharyngeal angiofibroma is also reviewed. Juvenile nasopharyngeal angiofibroma (JNA) is an uncommon vascular tumor, mainly occurring in the nasal and paranasal cavities of adolescent males and representing approximately 0.05% of all tumors of the head and neck ,2,3,4. MIntraoral localization of ENA has been occasionally reported in the lip, palate, tonsil, tonsillar pillar, cheek, retro-molar area and gingiva ,29,30,31In addition, all previously reported cases of ENAs were selected from the literature, collected and discussed. All angiofibromas arising from or involving the nasal/maxillary cavities were excluded, as well as cases with primary manifestation in the cheek as an extension of a tumor arising elsewhere, in order to better define the clinico-pathological features of ENA with exclusive localization in the oral cavity.The patient was a 17-year-old Caucasian male, referred to our attention for persistent gingival swelling. His medical history was unremarkable, with the exception of massive bleeding during an attempt to surgically remove the same gingival lesion performed by his general practitioner two months earlier. The patient referred no previous bleeding at other sites and his blood tests were within normal limits.At intra-oral examination, a soft, firm and painless swelling of the adherent gingiva (between 2.3 and 2.4 teeth) of red-violet discoloration was detected . RadioloBased on anamnestic data, pre-photocoagulation by diode laser, aimed at reducing the intra-lesion vascular component, with a simultaneous punch-biopsy, were suggested before surgical removal. After local anesthesia, a transmucosal photocoagulation was performed by diode laser  with a wavelength of 800 ± 10 nm, a 320 µm flexible fiber and an energy output of 5 W in continuous emission modality. The procedure lasted approximately 20 s and resulted in color variation from red-violet to white-greyish. A small punch biopsy was performed followed by histological examination of the sample. On hematoxylin-eosin stained slides, the tumor was composed by spindle-shaped fibroblast-like cells immersed in a rather loose collagenous stroma with vascular lacunae containing coagulated and fragmented red blood cells, sometimes showing a ghost-like appearance. On these bases, the diagnosis of angiofibroma was rendered. One month later, the lesion appeared slightly reduced in size and reddish in color a, with pThe patient was monitored until complete mucosal healing a and folThis study was carried out in accordance with the code of ethics of the world medical association (Declaration of Helsinki), approved by internal ethical committee . The patient released informed consent on diagnostic and therapeutic procedures and for the possible use of biological samples for research purposes.The first description of a vascular tumor with prominent fibrous stroma most probably dates back to Hippocrates in the 5th century B.C., while Chauveau in 1906 first used the designation juvenile nasopharyngeal angiofibroma . In 1983Conversely, JNAs with maxillary involvement, with or without extension into the oral cavity, are more easily diagnosable, and an incisional biopsy is performable in most cases. Nevertheless, the differential diagnosis remains extremely challenging, especially for the presence of undefined radiological borders, thus including benign/malignant epithelial and odontogenic neoplasms, benign and malignant maxillary/nasal sinuses neoplasms, localizations of hematological malignancies and metastatic tumors ,39,40,41Extranasopharyngeal localizations of angiofibroma are widely documented in the literature, ,4,8,11 aIn a detailed review published in 2018 by Windfuhr et al.,  174 caseAccording to this statement, in the current review of the literature, we exclusively selected cases with primary oral involvement, paying attention to attentively identify those ENAs occurring in the maxilla  that showed no involvement of the nasal/maxillary cavities. To the best of our knowledge and with the exclusion of the current case, a total of 20 cases of ENA matching the aforementioned criteria have been previously reported ,29,30,31Overall, there were 15 males and five females, the age range was 0–60 years old in males and 14–87 years old in females. Two ENAs occurred in newborns. More precisely, one case was reported as congenital and the other occurred at the age of six months. A higher prevalence (eight cases) was detected among adolescent males, with an age range of 10–23 years old. As to localizations, four cases occurred in the buccal mucosa, four in the mandible, five in the tonsil/tonsillar pillar, one in the soft palate, one in the lip, three in the hard palate/premaxilla, and two in the cheek; the latter were intramural lesions without nasal cavity involvement, as confirmed by radiograms.  Foreign The case reported herein show overlapping clinical features with previously reported ENAs, as occurring in an adolescent male (17 years old), showing bleeding at the first attempt of surgical removal and representing a diagnostic challenge based on its clinic-radiological presentation. In fact, the peripheral localization, along with the radiological appearance, pointed at a wide range of clinical diagnoses including several reactive or neoplastic lesions with gingival/periodontal onset  ,44,45,46As to treatment options, it is generally accepted that the introduction of laser therapies in the last three decades has simplified oral surgery and avoiding or consistently reducing bleeding during laser incision of soft tissues, promoting intralesional coagulation in vascular tumors ,48,49,50Overall, the diagnosis of JNA/ENA generally is achieved by conventional histological examination, being characterized by a dense fibrous stroma, containing spindled to stellate stromal cells, and haphazardly arranged vascular channels of variable size ,35,37,54The etiology of JNA and ENA is still debated and unclear. With regard to JNA, different theories have been postulated but a general agreement is still missing. JNA has been alternatively interpreted as a hamartoma ,56,57,58As an extrapolation of this theory, we may assume that ENA may possibly derive from ectopic (erectile) vascular tissue proliferation within the incisive (premaxillary) bone suture, with subsequent local permeation of the periodontum and, finally, vestibular gingival outgrowth, the latter being possibly related with recurrent gingival inflammation.While considering that several studies are still ongoing to better define the overall pathogenesis of angiofibromas ,60,61, w


Leiomyosarcoma is a malignant smooth muscle neoplasm, which is traditionally divided into superficial and deep tumors. Superficial leiomyosarcomas are quite rare entities, accounting for approximately 7% of soft tissue neoplasms and 0.04% of all cancers. Here we describe a rare case of advanced primary cutaneous leiomyosarcoma (PCL) in a 93-year-old woman, highlighting the considerable size of the lesion and the correct surgical and oncological management. The clinical story began about 4 years ago, and the neoplasia was treated only with local radiotherapy, but the patient suffered from a dramatic volumetric increase of the right arm sarcoma one year ago. Then, an amputation of the limb was performed without following adjuvant chemotherapy. Currently, she does not show signs of recurrence and is in good shape. Leiomyosarcoma is a malignant smooth muscle neoplasm, which is traditionally divided into superficial and deep tumors. Superficial leiomyosarcomas are quite rare entities, accounting for approximately 7% of soft tissue neoplasms and 0.04% of all cancers . Based oA 93-year-old woman presented herself to the Complex Operating Unit of Orthopedics of Aldo Moro University of Bari for a worsening of her health conditions. About 3 years earlier, he had been diagnosed with a primary cutaneous leiomyosarcoma at another hospital, which had been treated with local radiotherapy. In recent months, he had noticed a progressive increase in the size of the lesion. On clinical examination, the woman showed a lesion of 10.3 × 9.1 × 2.4 cm located in the right arm, extensively ulcerated, with polycyclic contours, with hemorrhagic zone A. RadiogAfter an interdisciplinary consultation, it was decided to amputate the limb as the patient’s age did not indicate the use of systemic chemotherapy, a much-debated point in the Literature see . PatholoAfter tissue processing, paraffin embedding, microtome cutting and routine haematoxylin-eosin staining, the microscopic examination showed at low magnification , at the At higher magnification, the neoplastic cells showed pleomorphic, nucleolated, hyperchromatic nuclei, and moderate to abundant eosinophilic cytoplasm: numerous typical and atypical mitotic figures, extensive necrosis, and hemorrhages were present together with areas of apparent dedifferentiation A,B. The Cutaneous leiomyosarcoma is a rare soft tissue sarcoma that has traditionally been interpreted to originate in the normal pilo-erector muscles of the dermis . It is aIn fact, about 3 years after the initial diagnosis, the patient reported an almost “dramatic” volumetric increase in the previous 6 months with a general decline in clinical conditions. In the Literature , it is cClinically, PCL can be misinterpreted as a keloid, granulomatous lesion  or as a benign neoplastic lesion  or malignant . From the histological point of view, PCL must be differentiated from fibrosarcoma, melanoma, malignant tumor of the peripheral nerve sheath, synovial sarcoma, malignant fibrous histiocytoma, pseudosarcomatous squamous cell carcinoma, and dermatofibrosarcoma protuberans . TherefoStandard treatment is surgical resection with free lateral margins of 3–5 cm including subcutaneous tissue reaching the fascia . Kraft ap = 0.02) [Adjuvant chemotherapy is a controversial issue. Administration of doxorubicin and ifosfamide is the most widely used therapy, showing an increased overall survival (OS) and odds ratio (OR) for local recurrence of 0.73 (95% CI 0.56–0.94; In the metastatic setting, polychemotherapy includes doxorubicin plus ifosfamide or doxorubicin plus dacarbazine, and after progression with an anthracycline, trabectedin is the common second-line therapy .Alternative treatment can be pegylated liposomal doxorubicin or epirubicin. Gemcitabine plus docetaxel or gemcitabine alone achieve disappointing results . Orally Sorafenib and regorafenib showed no clinical benefit in LMS , whereasCombination blockade with nivolumab plus ipilimumab demonstrates efficacy with a response rate of 18% and a median OS of 14.3 months. Nevertheless, the data are scarce, because of the rarity of this cancer . Hence,


An inflammatory myofibroblastic tumor originating from the aorta is extremely rare. Here, we report a case involving a 41-year-old female patient with an aortic inflammatory myofibroblastic tumor. Although preoperative imaging showed a mediastinal mass indicative of invasive thymoma, surgical findings revealed that this mass originated from the aorta. The patient underwent partial resection of the aorta, including the mass with patch angioplasty. Based on the postoperative histological findings, the patient was diagnosed with an aortic inflammatory myofibroblastic tumor and is currently under outpatient follow-up. Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm consisting of myofibroblastic spindle cells and various inflammatory cells. Although these tumors are usually considered benign, local recurrence and distant metastasis are commonly observed, suggesting the intermediate biological potential of these tumors. Since the first report of a pulmonary IMT, these tumors have been reported mostly in the lungs; however, extrapulmonary lesions, such as in the abdomen, retroperitoneal space, pelvis, and extremities, have also been reported .3). Cardiac magnetic resonance imaging (MRI) was performed, and while the boundaries of the mass were relatively clear and distinct, the possibility of its invasion into the aorta and pulmonary artery could not be completely ruled out  revealed a 5.5-cm mass in the anterior mediastinum, along with pericardial effusion , partial resection of the aorta including the remnant mass and Dacron patch angioplasty was successfully performed. Total cardiopulmonary bypass time was 45 min (aortic cross-clamp time = 20 min).The patient was discharged on postoperative day 8, without any complications. Final pathology revealed that the tumor was composed of bland-appearing spindle cells and scattered inflammatory cells—mainly lymphocytes and plasma cells Fig. a, b. On To date, IMT has been ambiguously known as an inflammatory pseudotumor, fibromyxoid tumor, plasma cell granuloma, inflammatory fibrosarcoma, or pseudosarcomatous myofibroblastic tumor. However, Gleason et al. suggested that IMT should be clearly distinguished from various neoplastic and reactive lesions and that the unqualified designation of “inflammatory pseudotumor” should be avoided .IMT mostly develops in children and young adults, with the main site of onset being the lungs and abdominopelvic cavity. Several studies have reported the onset of IMT in various somatic soft tissues. As described in this report, an IMT originating from the aorta is extremely rare, although less than 50 cardiac IMTs have been reported thus far .The clinical symptoms of an IMT can vary depending on the infiltrated organ and mass effect. In 15–30% of the patients, general inflammatory symptoms, including fever, weight loss, and malaise, are commonly observed; however, asymptomatic development of the mass is also a distinct possibility . CanteraA definitive diagnosis of IMT is made by histologic examination. The characteristic feature of IMT is proliferation of myofibroblastic spindle cells in a myxoid to collagenous stroma with prominent inflammatory infiltrates, including plasma cells and lymphocytes and/or eosinophils. Immunohistochemical analysis can be helpful, and the tumor is often positive for smooth muscle actin, vimentin, and desmin. Approximately 50% of the IMTs exhibit positivity for ALK, and this is reported to affect the prognosis; however, there is no consensus on this association, and ALK positivity is not always necessary for diagnosing IMTs .While there are various treatment options, the gold-standard treatment for IMT has not been clearly established. The majority of the previous studies have recommended complete surgical resection, if possible . While lIn this case report, we described a rare case of IMT originating from the ascending aorta in a female patient, which was successfully treated through surgery. Although this tumor originated from a vital organ, we expect a good prognosis after successful complete resection. Long-term follow-up is required for this patient.


Colorectal cancer is commonly diagnosed among the Japanese population, and various strategies in treating the colorectal liver metastasis have been introduced over the years. Here, we present a case of colorectal liver metastases in which we devised a multidisciplinary treatment plan for a better prognosis.We report a case of a 44-year-old female who developed rectal cancer with advanced synchronous liver metastases and was treated by a liver-first surgical approach following neoadjuvant chemotherapy. At diagnosis, there were 12 bilobular lesions in the liver, and the primary rectal cancer was asymptomatic and unprogressive. We adopted a liver-first strategy because the control of the liver metastases was considered the key prognostic factor. Furthermore, because the lesions were highly progressive, we planned neoadjuvant systemic chemotherapy first to provide an observational period to identify potential new metastatic lesions that were refractory to systemic chemotherapy or contraindicative for surgical resection. We administered two courses of S-1 + oxaliplatin (SOX)+ bevacizumab (BV) and an additional course of SOX without BV as neoadjuvant chemotherapy in preparation for surgery. This resulted in a prominent minimalization of colorectal liver metastases, and no other remote metastasis was observed. Then, surgical resection of the colorectal liver metastases was performed safely, and the pathological result revealed complete remission of all tumors by neoadjuvant chemotherapy. The primary tumor in the colon was successfully resected 2 months after the hepatectomy. Although the patient experienced a recurrence in two different sites in the lungs 10 months after resection of the primary rectal lesion, these metastases were successfully resected after diagnosis. The patient is alive with no signs of recurrence 3 years after the diagnosis of colorectal cancer with synchronous liver metastases.The combination of a liver-first strategy and neoadjuvant chemotherapy is a possible treatment of choice to cure colorectal cancer with simultaneous advanced colorectal liver metastases. Globally, colorectal cancer (CRC) is the third most commonly diagnosed cancer among men and second among women. This is mirrored in the Japanese population . The proIn this article, we presented a case of colorectal cancer with advanced synchronous liver metastases which were treated by a combination of neoadjuvant chemotherapy and a liver-first surgical approach.2) + BV (7.5 mg/kg); day 1–14: S-1 (70 mg/m2/day)), and an additional course of SOX without BV was performed in preparation for surgery. At this point, the patient presented low white blood cell count (grade 2), generalized weakness (grade 2), loss of appetite (grade 1), and constipation (grade 1). The enforcement of chemotherapy resulted in a considerable reduction in the size of the tumor in both the rectum  + BV 7. mg/kg; dIn the recent years, the prognosis of CRC has been improving . SeveralIn CRLM treatment, hepatic resection remains to be the only curative option. If macroscopic surgery is determined to be feasible and if the maintenance of at least 30% of future liver remnant may be sustained under R0 resection, surgery should be the first-line therapy . If the Although hepatic resection remains the only curative option for CRLM, long-term survival or curing the condition is reported to be realized in only 20–50% of patients who have undergone R0 resection of CRLM . The metChemotherapy must be planned accounting for the patient’s liver function. Chemotherapy for over 16 weeks before surgical resection increases the risk of liver injury and post-operative complications, without pathologic improvement . Long-teAdam et al. reported an analysis of CRLM patients who underwent surgery in their institution, concluding that complete remission may occur in almost one-third of objective responders 60 years or younger with metastases ≤ 3 cm and with low carcinoembryonic antigen (CEA) values . Our casIn our case, both the primary and the metastatic sites seemed to have a great sensitivity to neoadjuvant chemotherapy upon CT and colonoscopy. However, final pathological findings of the primary lesion revealed viable cells existing adjacent to the muscularis propria with vascular invasion, while the pathology for the liver lesions presented complete remission. Gervaz et al. compared histological response in colorectal liver metastases and the primary lesion in 29 patients, in which all were treated with a neoadjuvant chemotherapy followed by liver-first surgery, and then the primary lesion surgery . There wIn conclusion, neoadjuvant chemotherapy and a liver-first surgical approach could be a possible curable treatment for asymptomatic colon cancer with simultaneous advanced CRLM, especially for young patients with no rise in CEA and with no CRLM lesions over 3 cm in size. Neoadjuvant chemotherapy played a key role in treating unresectable, but potentially curable, metastatic CRC.


Leiomyosarcomas are rare and heterogeneous group of tumors that account for less than 1% of adult malignancies. More than 50% of all vascular leiomyosarcomas occur in the lower part of vena cava. Since the first description of Perl and Virchow in 1871, only approximately 450 cases have been reported in literature.The patient presented due to abdominal pain and weight loss. Based on the imaging evaluations a retroduodenal tumor with compression of the inferior vena cava was observed. In the explorative laparotomy a leiomyosarcoma originating from the inferior vena cava was identified. Considering the extensive intramural and intraluminal tumor manifestation, the patient underwent a segmental resection of the vena cava. Reconstruction was achieved by implanting a polytetrafluoroethylene (PTFE) prosthesis. Postoperatively a stenosis developed due to a pericaval haematoma with consecutive compression of the prosthesis. An angiographic implantation of a stent was successfully performed. In the 24-month follow-up, the patient is free of symptoms and tumors.Leiomyosarcomas of the vena cava are classified anatomically according to their relationship to the liver and renal vessels. The clinical symptoms depend on the affected segment. The therapy of choice is radical en bloc tumor resection. After resection, the options for reconstruction include placement of a synthetic graft, primary repair and patch repair.Due to a variety of topographic and tumor biological sarcoma manifestations, no standard has been established for the resection of this entity. The extent of resection should be planned individually. They grSince the first description of Perl and Virchow in 1871 , only apDue to the slow growth, the tumors remain clinically inapparent for a long time and are usually diagnosed in a locally advanced tumor stage. This work has been reported in line with the SCARE criteria .2The patient presented due to abdominal pain and weight loss. According to the preoperative computer tomography (CT) imaging evaluation, a retroduodenal tumor with compression of the inferior vena cava was observed. Clinical examinations did not show any sign of lower extremity edema or deep vein thrombosis. In the explorative laparotomy a tumor located in the infrarenal vena cava was identified . Subsequ3Postoperative histopathological evaluations identified a leiomyosarcoma originating from the inferior vena cava (grad 2 based on the FNCLCC classification) with a tumor stage of pT1b V1 R1 M0. Postoperatively the patient suffered from an abdominal pain. Subsequently, she underwent CT examination, which showed a stenosis developed due to a pericaval haematoma with consecutive compression of the prosthesis . To reso4Leiomyosarcomas of the vena cava are classified anatomically according to their relationship to the liver and renal vessels. The clinical symptoms depend on the affected segment. Segment I  will cause lower extremity edema, deep vein thrombosis and abdominal pain; segment II  causes nephrotic syndrome and abdominal pain; and segment III (suprahepatic to right atrium) causes weight loss, nausea, Budd-Chiari syndrome and cardiac arrhythmia ,8. DespiLeiomyosarcomas originated from vena cava are the extremely rare diseases. Based on the available literature, the radical en bloc tumor resection seems to be the treatment of choice as the only therapeutic approach that can improve patient survival . R0 reseAfter resection, the options for reconstruction include placement of a synthetic graft, primary repair and patch repair. According to the literature the material of choice for a caval replacement is PTFE . Studies5Due to a variety of topographic and tumor biological sarcoma manifestations, no standard has been established for the resection of this entity. Taking these characteristics into account, the extent of resection should be planned individually.This study is not fundedThis is a case report. Therefore, an ethical approval is not required.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.-The conception and design of the study: KK and GAS-Acquisition of data: BJ and MJ-Drafting the article: KK and MJ-Revising it critically for important intellectual content: BJ and GAS-final approval: All authorsNA.Dr. Kira Kellerkikeller@klinikum-saarbruecken.deWinterberg 1, 66119 Saarbrücken, Germany, Phone: +49 (0)6 81 963 34168, Fax: +49 (0)6 681 963 2417, Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer-reviewed.The authors declare no conflict of interest.


Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an under-recognized, benign fibroinflammatory disease most commonly seen in the submandibular gland of older adult males. Sialolithiasis or bacterial infection was first suspected as an etiology, but CSS is now considered an immunoglobulin G4-related disease (IgG4-RD). IgG4-RD can affect almost every organ in the body, characterized by organ fibrosis with IgG4-positive plasmacytes. Numerous autoimmune-related diseases have been unified under IgG4-RD, including Mikulicz disease (MD), autoimmune pancreatitis, Reidel’s thyroiditis, and others. In any organ, IgG4-RD can present similar to malignancy. Due to the ability to mimic malignancy, it is crucial to be aware of this under-recognized clinical entity. CSS is currently of broad and high clinical interest due to increased understanding, multiorgan involvement, and more clearly defined criteria. To increase awareness of this disease, we describe a rare presentation of CSS with a literature review. Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an uncommonly recognized, benign fibroinflammatory disease of the major salivary glands. It primarily occurs in the submandibular gland of middle-aged and elderly adults, typically affecting males more than females . ImmunogAlthough the majority of pediatric salivary gland tumors are benign, when a parotid mass is discovered in a pediatric patient, it can be a more ominous sign. This is due to the higher rate of salivary gland malignancy, roughly 15%-32% when compared to adults .CSS is currently of broad and high clinical interest due to increased understanding, multiorgan involvement, and more clearly defined criteria. Karim et al. presented the first dedicated pediatric literature review of IgG-RD in any organ in order to highlight the fact that IgG4-RD is not limited to adults . In an aA 15-year-old African-American female presented to the community otolaryngology clinic for the evaluation of a left neck mass, progressively enlarging over the past 10 months. The patient stated that the mass was firm and largely non-tender with periods of sensitivity, mildly relieved with nonsteroidal anti-inflammatory drugs (NSAIDs). She denied fevers, chills, weight changes, or any other masses in the head and neck. The mass failed to respond to multiple courses of antibiotics prescribed by her primary care physician. Past medical history was reported as unremarkable. On physical exam, there was a 4 x 3 cm mass overlying the left mandible angle and ramus, which was mobile, soft, and swollen appearing. Contrasted computed tomography (CT) was obtained, which revealed a large, loculated, cystic, parotid mass ~6 x 4 x 3cm , Sjögren's disease, sarcoidosis, and neoplastic processes such as lymphoma and carcinoma . The hisImagingAlthough the diagnosis of CSS is difficult with imaging alone, there have been reports of diagnosis solely through sonography . CSS canCytology, histology, and immunohistochemistryIn CSS, FNA findings may be non-specific but can suggest CSS in the appropriate clinical setting . Due to CSS was originally differentiated into four stages based on histologic features, with stage 4 being the loss of lobular architecture, which is what this histology indicates . Later oTherapeutic optionsConservative treatment has been proposed versus surgical. However, corticosteroids are the first line of treatment for IgG4-RD with a good prognosis; some report up to 61% remission at one year, but relapse is not uncommon, especially when steroids are discontinued . Dosing CSS or Küttner tumor is currently considered to be a manifestation of IgG4-RD, which can present in several different organs solitarily or as part of multi-organ systemic disease. CSS most commonly presents bilaterally in the submandibular glands of middle-aged males. It is imperative to differentiate this disease from ominous malignancy. A multidisciplinary team is necessary and the patient should be referred to a rheumatologist and possibly a hematologist. A strong pathologic diagnosis can be made with histology alone. Additionally, as awareness of IgG4-RD continues to increase, its recognition and diagnosis is likely to follow.


Lipomas are a rare cause of compressive neuropathy and they lead to atypical clinical presentation that can mimic carpal tunnel syndrome.The authors describe a rare presentation of a carpal tunnel syndrome recurrence after a hand giant lipoma, presenting with rapidly compression neuropathy of the median nerve, 6 months after de primary surgery.Lipomas are common benign soft tumours. Their occurrence in the hand remains rare and they rarely cause secondary entrapment neuropathies. Carpal tunnel syndrome is mostly idiopathic and bilateral. Local factors should be suspected when these neuropathies present with atypical symptomatology or even when they recur after primary conventional surgical release. Investigation should consider images studies as this correct preoperative assessment leads to successful diagnosis and treatment. They arCarpal tunnel syndrome (CTS) is one of the most common compression neuropathy  and in aThis case report describes a rare presentation of a hand giant lipoma causing secondary carpal tunnel syndrome treated in a tertiary referral public hospital. This work has been reported in line with the SCARE criteria .2A leucodermic sixty-three-year-old female patient, was referred to an orthopaedic consul presenting with a numbness sensation of the right hand. She had a medical history of hypothyroidism, hypertension and hypercholesterolemia and her medication included levothyroxine, bisoprolol and atorvastatin. The patient was diagnosed and treated for an idiopathic CTS 6 month before, with a palmar mini-open surgical decompression of the median nerve. The patient confirmed complete clinical improvement in the early post-operative period.On presentation the patient complaint of recurrence of numbness in the median nerve distribution of the right hand. On examination, there was a mass on the thenar eminence . It was Standard X-rays showed no abnormalities. Ultrasound (US) and magnetic resonance image (MRI) of the hand revealed an occupying-space lesion of adipose density, capsulated with well-defined contours , Fig. 3.en bloc marginal resection carried out  are useful to determine the location and severity of the compression . StandarDifferential diagnosis for extra-neural adipose neoplasms is mostly fibrolipoma of the median nerve and liposarcoma . The hig“Giant” lipoma is classically defined by a diameter greater than 5 cm and neoplasms exceeding this size should always raise suspicion for malignancy . For thiIn the majority of the patients the pathogenesis of CTS is unclear (idiopathic) . In someAlthough rare, the surgeon must consider unusual causes of CTS when patients present with atypical symptomatology or postoperative recurrence. Further investigation may be necessary prior to intervention, usually US and MRI. In addition, these patients are bad candidates for endoscopic and minimally invasive techniques that limit surgical visualization of the nerve and possible space-occupying lesion. A correct preoperative assessment of each patient is the key for proper management, successful treatment and reduced risk of recurrence of CTS entrapment neuropathy.The authors report no declarations of interest.None declared.Not applicable.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Mariana Magalhães Barreira, MD MsC – Study concept, data collection, drafting, revision, approval of final manuscript.Nuno Frederico Ramos Marques, MD MsC – Data collection, drafting, revision.Vicente Carlos da Silva Campos, MD MsC – Revision.Guilherme André de Paiva Marques, MD MsC – Revision.Sérgio Rodrigues Gonçalves, MD MsC – Study concept, drafting, revision.Sandra Vitoria Stefanova Alves, MD MsC – Study concept, revision, approval of final manuscript.N/A.Mariana Magalhães Barreira.Not commissioned, externally peer-reviewed.


Synovial sarcoma (SS) is a rare soft tissue tumor. Among different anatomical locations where it can be found, gastric localization is a very uncommon one. Based on soft tissue sarcoma guidelines, complete tumor excision is considered the main treatment approach. Depending on size and localization of the tumor, both wedge and major gastric resections have been performed in the past for the treatment of this condition.We present the case of a 43-year-old woman who underwent a laparoscopic intragastric excision of a gastric 10-mm SS located nearby the esophagogastric junction. Pathology examination confirmed the presence of a SS. The resected specimen confirmed margin-free excision of a monophasic spindle cell neoplasm invading the submucosa and presenting the rearrangement of SS18 gene at fluorescence in situ hybridization (FISH). No adjuvant treatment was offered, and 18 months after surgery, the patient was alive and disease free.This represents the first case reported in literature of a laparoscopic intragastric resection for a gastric SS. This approach allowed to obtain a full thickness radical tumor resection with the advantages of minimally invasive and organ preserving surgery.The online version contains supplementary material available at 10.1186/s12957-021-02172-y. Synovial sarcoma (SS) is a rare malignant tumor derived from mesenchymal tissue and has an incidence of 8 to 10% among all soft tissue sarcomas [It occurs primarily in the extremities of arms and legs, often in proximity to joint capsules and tendon sheaths, but it has been rarely observed in the head and neck region, mediastinum, blood vessels, heart, liver, and in gastrointestinal tract –8.When located in the gastrointestinal tract, it can be easily mistaken for a gastrointestinal stromal tumor (GIST). What sets these two entities apart is the presence of an SSX-SS18 chromosome translocation .Interestingly, only 40 cases of SS have been reported to have a gastric location . As per We present the first case of laparoscopic intragastric surgery for the treatment of a gastric SS.A 43-year-old woman was referred to our Tertiary Care Center for a submucosal ulcerated lesion of the upper third of the stomach. Her past medical history was unremarkable, with the exception of a 1-year history of epigastric pain and dyspepsia which failed to improve on standard PPI treatment. Esophagogastroduodenoscopy (EGDS) showed atrophic gastritis with multiple amartomatous polyps and a 10-mm submucosal round-shaped lesion with central ulceration located in the upper third of the stomach in proximity of the esophagogastric junction  which failed to identify the lesion and a fluorine-18-labeled fluorodeoxyglucose positron emission tomography (18F-FDG PET/TC) which demonstrated the presence of a single metabolically active mass in the upper third of the stomach (Fig. Given size and location of the tumor near the cardial region, a laparoscopic intragastric approach was used. This approach was employed also based on the fact that no formal lymphadenectomy was deemed necessary. The day before surgery, a further endoscopic evaluation with clip tumor marking was carried out to facilitate intraoperative tumor identification Fig. c.The patient was placed on the operating table in the supine position with parted legs, 20° reverse Trendelenburg, and slight left-side tilt. A 5-mm camera was used. A 5-mm trocar was placed at the umbilicus, and further, two 5-mm balloon trocars and one 12 balloon trocar were placed in the mid-left upper quadrants of the abdomen considering the position of gastric body in relation to the abdominal wall (Fig. Additional file 1. Supplemental Digital Content 1.Postoperative course was uneventful. Soft diet was resumed on postoperative day 1, and patient was discharge on postoperative day 3.Histopathology examination confirmed the presence of a monophasic spindle cell neoplasm. Four out of ten mitoses on high magnification field was shown, no necrosis was present, and lympho-vascular invasion was seen. The tumor involved the submucosa. Circumferential and deep margins were tumor free. Fluorescence in situ hybridization established the rearrangement of SS18 gene confirming the diagnosis of SS Fig. a, b, c.No adjuvant treatment was offered, and 18 months after surgery, the patient is alive and disease free.Primary gastric SS is an exceptionally rare disease. The first case was described in 2000, and 40 cases have been reported since , 10.At endoscopic examination, gastric SS resembles a GIST tumor, though diagnosis should be suspected when a white submucosal plaque-like lesion with central depression is observed , 14.On hematoxylin and eosin staining, histology demonstrates a similar pattern or mono or biphasic spindle cell in both SS and GIST tumors. On immunohistochemistry, however, c-kit positivity is lacking in gastric SS, and the fusion products of the SS18 gene combined to either SSX1, SSX2, or SSX4 gene are found . Three hWith regard to treatment, no definite guidelines are available for gastrointestinal SS. Based on NCCN and ESMO guidelines for soft tissue sarcomas, complete tumor resection is considered the main goal of surgery and no lymphadenectomy is indicated , 12.In previous series, different surgical approaches have been described in 29 cases: a major gastric resection was performed in 16 cases, and a wedge or tumor resection was carried out in 13 cases , 17–27. In our case, despite the small size of the lesion, a proximal or total gastrectomy would have been required to achieve tumor clearance due to its location nearby the esophagogastric junction. The choice of a laparoscopic intragastric approach allowed us to perform a complete tumor resection within the objective of organ preservation. This approach has been successfully performed in GISTs as well as other tumors located on the posterior wall of the upper third of the stomach when no lymphadenectomy is required , 29. ThiThis is the first case reported in literature of laparoscopic intragastric resection for a synovial sarcoma of the stomach. This approach allowed to obtain a full thickness complete resection of the tumor with the preservation of the stomach, hence combining the advantages of minimally invasive and organ-preserving surgery.


Lycanthropy is a rare variant of a delusional misidentification syndrome specifically reverse inter-metamorphosis where patients believe that they are experiencing transformation or have transformed into an animal. A case report of this phenomenon is discussed. We report the lycanthropy phenomenon of a 25-year-old male who believed himself to be transformed into a buffalo after bestiality along with developing obsessive-compulsive features. A case report along with a literature review forms the basis of discussion. Clinical lycanthropy has been reported with various neuropsychiatric conditions including primary psychotic and affective conditions, drug intoxication and withdrawal, cerebrovascular disease, traumatic brain injury, dementia, delirium, and seizures, but its association in the context of obsessive-compulsive and related disorders (OCRDs) is a very rare finding. A differential of OCRD should be given due importance and managed accordingly when dealing with lycanthropy, which may help in early identification and management.  Lycanthropy is an uncommon form of delusion or unusual belief that one has transformed into a non-human animal or displays behaviour or feelings indicative of such a belief. It is a rare variant of delusional misidentification syndrome (DMS) specifically reverse inter-metamorphosis where patients believe that they are experiencing transformation or have transformed into an animal. DMS is seen in association with several neuropsychiatric conditions, including primary psychotic and affective conditions, drug intoxication and withdrawal, cerebrovascular disease, traumatic brain injury, dementia, delirium, and seizures . A “two-The patient was a 25-year-old milkman from a low socio-economic class and residing in an urban area. The patient was brought to the Department of Psychiatry by family members with complaints of excessive hand washing, irritable behaviour and decreased sleep along with recurrent cleaning of genital areas, and acting like a buffalo for four months. During an interview with the patient, he reported engaging in sexual activity with his own buffalo a few times six months ago and since then he believes that a few cells of the buffalo have entered into his body and he would turn in a buffalo. To avoid this, he recurrently washes his hands and genitals. Gradually his belief strengthened, and he was convinced that he had become a buffalo due to his bestiality. Along with this, his obsession with contamination and compulsive hand washing continued. About two months after the act of bestiality, he started believing his body had transformed into a buffalo beginning from his lower half towards torso and face. When he looked at himself or in the mirror, he visualized his body parts like a buffalo and kept on screaming about his repugnant state and was constantly preoccupied about his looks Figure . HoweverA diagnosis of obsessive-compulsive disorder (OCD) and body dysmorphic disorder with delusional beliefs was formulated as per Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM V). As per Yale-Brown Obsessive-Compulsive Scale (YBOCS), the obsession score was 17 and compulsion score was 15  [Lycanthropy is a rare psychiatric phenomenon seen in the context of various neuropsychiatric conditions and not a disorder itself. Its differentials include schizophrenia, delusional disorder, bipolar disorder, psychotic depression, epilepsy, organic causes; however, its presence in the context of OCRDs is rare. It can be viewed as a delusional misidentification of own body parts . The preThe lycanthropy phenomenon has been reported in various major psychiatric disorders such as schizophrenia, delusional disorders, affective disorders, and other organic states. However, cases of secondary clinical lycanthropy in OCRD are rare. We urge the clinicians to also consider clinical lycanthropy a symptom secondary to OCRDs, which should be given due importance and which may help in early identification and management.


Buccal space tumors constitute rare pathologies with significant histological diversity. They may pose serious diagnostic and therapeutic challenges for the head and neck surgeon.  A case of buccal space tumor diagnosed and treated in a tertiary center is presented. Clinical presentation, imaging, and surgical approach are discussed, followed by review of the literature.  A 79-year-old male patient with a slowly growing painless mass on the right cheek presented to a head and neck reference center. Imaging revealed a tumor of the right buccal space with nonspecific characteristics. Imaging studies revealed extended infiltration of the masseter muscle as well as the anterior border of the parotid gland. FNA biopsy was performed but was nondiagnostic. The decision of surgical excision with a modified parotidectomy incision was taken. The lesion was completely excised with preservation of neighboring facial nerve branches and ipsilateral Stensen's duct. The postoperative course was uneventful. Histological examination showed CLL/Lymphoma, and the patient was referred to the hematology department for staging and further management.  Differential diagnosis of buccal space masses is very diverse. Despite challenges in the diagnostic and therapeutic approach, these entities may be managed surgically with minimal morbidity. Buccal space pathologies are relatively rare and thus seldom encountered in the literature. Despite occult epidemiology, buccal space masses show significant clinical and etiologic diversity , 2. In aA 79-year-old male patient presented with a painless tumor of the right cheek, located below and laterally to the right zygomatic bone. The mass had existed for at least six months and was slowly increasing in size. Hypertension, diabetes, hyperuricemia, and hypercholesterolemia were mentioned in the medical history. No previous significant pathology or surgery was reported in the head and neck area. Computed and magnetic tomography, with 1.25 mm thickness cuts in the transverse plane and with image reconstruction cuts of 1.25 and 2.5 mm in coronal and sagittal planes, respectively, revealed a lesion of 1.64 cm × 4.8 cm in dimensions which occupied the right buccal space, originating from the right major zygomaticus muscle, and extending to the right anterior part of the parotid gland Figures . The pat4Permanent histology described a solid white/brown ulcerated neoplastic mass with a maximum diameter of 5.3 cm . InfiltrThe buccal space is a small potential space on the lateral aspect of the face that forms the infrastructure of the cheek , 2. ContA variety of diseases may occur within the buccal space, including developmental lesions, inflammatory conditions, neoplastic lesions, either primary or metastatic, and miscellaneous diseases such as Kimura disease and foreign body granuloma , 6. In aDevelopmental lesions of the buccal space include dermoid cysts and hemangiomas . AbscessStaphylococcus aureus, Fusobacterium spp., and Streptococcus spp [Mycobacterium tuberculosis usually in the context of extrapulmonary tuberculosis in an immunosuppressed patient [The most common pathogens associated with acute viral parotitis are paramixovirus, adenoviruses, and rarely influenza A (H3N2) or coxsackie A or B –11. Herpccus spp –12. Less patient . Finally patient , 10.Neoplastic lesions of the buccal space include primary, benign or malignant, and metastatic. Salivary gland tumors, rhabdomyosarcoma, neurofibroma, and lymphoma constitute the majority of primary neoplastic lesions of the buccal space. Salivary gland tumors originate from the parotid gland in 80% of cases, and their epidemiology and clinical course are well established and beyond the scope of this report , 15. RhaMetastatic lesions in the buccal space occasionally derive from squamous cell skin carcinomas of the face and less frequently from lung, breast, and kidney cancer . In rareKimura disease is a chronic inflammatory disorder of unknown etiology and is histopathologically characterized by lymph-folliculoid granuloma and pronounced eosinophilic infiltration , 23. It Following clinical detection of a buccal space mass, detailed patient history and clinical examination are required. It is important to demonstrate location, depth, size, consistency, and mobility of the mass. Pain and signs of inflammation should also be noted. Apart from physical examination, an imaging study of the buccal region such as ultrasound, CT, or MRI will be needed. Ultrasound is used predominantly for superficial structures in order to distinguish solid from cystic lesions, gives information regarding vascular supply, and is able to detect enlarged regional lymph nodes , 25. UltBranches of the facial nerve and the parotid duct are closely associated with tumors of the buccal space. Therefore, careful intraoperative mapping of the tumor and the neighboring regions is necessary. Several surgical techniques have been proposed at times. The incision of the ideal surgical approach should provide complete exposure of the surgical field, allow the conversion to radical surgery if it is needed, and produce a satisfactory cosmetic result . AlthougIn conclusion, buccal space masses are rare and at the same time quite diverse, making their diagnostic approach very challenging. Whenever surgical management is decided, it may be successfully performed as long as adequate respect to the anatomy is shown, with careful identification and preservation of structures such as the facial nerve branches and parotid duct.


Malignant chondroid syringomas (MCSs) are extremely rare and aggressive skin tumors, and wide surgical excision is the main treatment. They can progress with local recurrence and nodal and distant metastasis. The role of radiotherapy is uncertain, but may enhance local control after surgical approach. We report a case of a 60-year-old female with this disease that, four years after surgical resection, presented with nodal metastasis and was managed with surgery and adjuvant radiotherapy. Chondroid syringomas (CSs) are rare benign tumors originated from sweat gland and ectopic salivary glands. They are usually asymptomatic, indolent and occur usually in the head and neck ,2. MaligHerein, we present a case of a 60-year-old female diagnosed with MCS on the lateral aspect of the left knee, treated with surgical resection. She was found to have inguinal and iliac nodal metastasis four years later and was successfully managed with radiation therapy.A 60-year-old female presented with a nodular lesion in the lateral side of the left knee, measuring 2 cm, painful on palpation, that appeared two years before. She underwent a resection in 2013 and the histology confirmed MCS, with positive margins , has no definite age predilection, with median age of 49 years, ranging from 13 to 83, and has no evidence of a specific risk factor [The histological features that are suggestive of this pathology, differentiating from the benign form, include nuclear pleomorphism, cytologic atypia, increased mitotic activity, focal necrosis and lymphatic or vascular invasion . ImmunocAdequate wide surgical excision is the main treatment for MCS. From cases reported so far, 50% have local recurrences. Nodal metastasis was observed in 39% of the cases, while distant metastases  were observed in 36% of them. 27% of patients died from this disease within nine weeks following surgery; one patient survived 13 years after diagnosis .There are only three cases report of the use of radiation therapy on MSC. Schvili and Rothem  reportedIn this reported case, as there is no specific treatment for MCS lymph metastasis, we based the treatment on the intergroup multicenter randomized trial (ANZMTG 01.02/TROG 02.01), which includes adjuvant radiotherapy for patients with melanoma after lymphadenectomy at high risk of recurrence (according to the size of involved nodes and the presence of extracapsular extension) .A total dose of 4800 cGy in 20 fractions of 240 cGy with three-dimensional conformal radiation therapy was performed on the left inguinal and iliac region. The only complication the patient presented was mild lymphoedema, that is expected after this sort of treatment (surgery + radiotherapy). No sign of residual disease was found after one year and three months of follow-up and no systemic therapy was used.MCS is an extremely rare condition and its management remains controversial. It is more aggressive than the benign form and, as there are only a few cases reported and each one has differences among them, systematic approach lacks. Complete resection is the main treatment and the role of radiotherapy on this disease remains uncertain, but it seems to be a reasonable option as adjuvant treatment for optimizing local control after nodal metastasis resection. Clinicians, including primary care physicians and dermatologists, should be aware of the existence of this entity and try to biopsy any suspicious lesion to rule out possible underlying malignancy.


Congenital tumours are rare conditions in both veterinary and human medicine. Undifferentiated sarcomas represent a recently introduced section that encompasses unclassified sarcomas that have no distinct histologic, immunohistochemical, or genetic features. The aim of this study is to describe the clinical and pathological phenotype of a 3-day-old male crossbred calf presenting a congenital suborbital mass that infiltrated the underlying muscle and bone, and suggestive of undifferentiated sarcoma. To the best of our knowledge, our report constitutes the first clinical, ultrasonographic, radiographic, endoscopic and pathological study of a congenital undifferentiated sarcoma, a condition that has been rarely reported in the veterinary literature.Undifferentiated sarcomas are rare conditions that represent a group of unclassified sarcomas. The purpose of this study is to describe the clinical and pathological features of a calf showing a congenital infiltrating suborbital mass suggestive of undifferentiated sarcoma. The animal was referred because of respiratory distress and the presence of a right suborbital mass since birth. At ultrasonography, the mass displayed an irregular shape with multiple cavities. Radiographs revealed a diffuse, poorly defined mass with different densities overlying the bony structures of the skull. Endoscopy showed a co-involution of the mass in the right side with extension into the nasopharynx. Post-mortem examination showed a round, poorly demarcated neoplasia infiltrating the nasal turbinate and displacing the nasal septum. Histologically, the subcutis was expanded by lobules and bundles of densely cellular neoplastic spindle cells. The neoplasm infiltrated the underlying muscles, bone and the right retromandibular lymph node. The neoplastic cells had a diffuse intense cytoplasmic immunexpression to vimentin, and were negative to cytokeratin AE1/AE3, desmin, MUM1, IBA1, melan A, chromogranin and synaptophysin. Congenital tumours are rare conditions that arise from tissue rudiments undergoing development and are detected during pregnancy or within the first three months of life . These tIn cattle, the only reported case of an undifferentiated sarcoma dates back to Görig in 1893 . It was Therefore, we aimed to typify the clinical and pathological features of a crossbred calf displaying lesions suggestive of undifferentiated sarcoma.A 3-day-old male crossbred calf, with a body mass of 37 kg, was referred to the teaching hospital of the Department of Veterinary Medical Sciences (DIMEVET), University of Bologna, because of respiratory distress and presence of a right suborbital mass since birth. The calf was subsequently clinically examined, on which the mass, estimated to be approximately 10 cm in diameter, was recognized to be within the subcutaneous tissue a,b. The A complete blood count (CBC) and blood chemistry profile were performed. The blood investigation of the calf showed a moderate hypoproteinaemia (5.19 g/dL) with hypoalbuminemia (2.51 g/dL) and hypogammaglobulinemia (gamma globulins = 0.64 g/dL), hypercreatinemia (7.51 mg/dL), and hyperphosphatemia (10.7 mg/dL). No abnormalities were detected in the CBC.Blood samples from the calf were also sent for routine viral and parasitological analysis  using antigen-enzyme-linked immunosorbent assay (ELISA) and antibody-polymerase chain reaction (PCR). These analyses revealed positivity for bovine Schmallenberg virus using antigen-ELISA, and tested negative for all the remaining viral and parasitological analyses.Ultrasound examination (US) of the infraorbital mass and radiography of the head were performed. At US the mass displayed an irregular shape with a multiple cavities a. RadiogMoreover, endoscopy of nasal cavities and larynx was performed with a flexible endoscope. The larynx displayed a slight thickening of the medial portion of the left arytenoid in absence of laryngeal aditus reduction. The right nasal cavity, from the middle meatus and extending dorsally, was filled with a mass, characterized by smooth surface and dark colour. Endoscopy showed that the mass heavily infiltrated the nasopharynx (Video S1). On the left nasal cavity, no abnormalities were noticed.The clinical findings were compatible with an invasive neoplasm.The animal was euthanized at 9 days of age and was subsequently submitted to necropsy.Macroscopically, the mass was round, poorly demarcated, infiltrating the ventral conca and disrupting the morphology of the nasal cavities and palate . All rigHistologically, the subcutis was expanded by the neoplasm, multilobular, densely cellular, not demarcated, unencapsulated, with infiltrative growth in the underlying skeletal muscle and bone, with lysis and resorption a. The neThe pathological and immunohistochemical findings were suggestive of undifferentiated sarcoma.Congenital undifferentiated sarcomas have been poorly described in veterinary medicine. To the authors’ knowledge, only one case has been so far reported in cattle and dates back to 1893 . It was The crossbred calf presented in this study showed a congenital suborbital lesion in the subcutaneous tissue with infiltration of the underlying muscle and bone, and metastasis in the right retromandibular lymph node. The pathological and immunohistochemical results were overall suggestive of congenital round to spindle cells undifferentiated sarcoma.A diagnosis of undifferentiated malignant neoplasm was considered and a supportive IHC was required. The negative staining to desmin, MUM1, IBA1, Melan A, chromogranin and synaptophysin excluded the diagnoses of rhabdomyosarcoma, pleomorphic plasmacytoma, histiocytic sarcoma, melanoma and malignant neuroendocrine tumour. The vimentin only positive expression suggests the mesenchymal origin of the neoplasm and therefore the diagnosis of undifferentiated sarcoma.Rare form of spindle-cell undifferentiated sarcoma is described in adult cattle. This late-onset form of undifferentiated sarcoma may be observed in a multicentric form  or in thIn human medicine, cases of congenital undifferentiated sarcomas are rare ,11,12,13Peribunyaviridae, of the newly established order Bunyavirales that has been associated with congenital malformations of the nervous and musculoskeletal systems and stillbirths in ruminants [The affected calf reported in this study was seropositive for Schmallenberg virus (SBV) and we speculate that the calf has been infected in utero. SBV is an arthropod-transmitted pathogenic orthobunyavirus of the family uminants ,15. Receuminants . HoweverWe performed a complete clinical and pathological investigation of the crossbred calf revealing a suborbital mass with infiltration of the underlying soft tissues and bone, which was compatible with a congenital form of undifferentiated sarcoma. To the best of our knowledge, our report constitutes the first clinical, ultrasonographic, radiographic, endoscopic and pathological study of a congenital undifferentiated sarcoma, a condition that has been rarely reported in the veterinary literature.


The malignant potential and the appropriate treatment of uterine tumor resembling ovarian sex-cord tumor (UTROSCT) is controversial. Although these tumors generally have benign outcomes, several reports have described recurrences, metastases, and deaths associated with this disease.A 57-year-old Japanese woman  was referred to our hospital for the evaluation and treatment of uterine fibroids. Magnetic resonance imaging revealed a right ovarian mass and multiple fibroids in the uterine myometrium.The patient was diagnosed with UTROSCT with sarcomatous features.She initially underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy, followed by second-stage surgery comprising pelvic and para-aortic lymphadenectomy and subtotal omentectomy.No postoperative recurrence was observed in the patient in 36 months.In this case, extended radical surgery prevented the development of recurrent disease in a patient with UTROSCT with sarcomatous features. These clinicopathological findings suggest that UTROSCT is associated with several risk factors, including older age, presence of necrosis, lymphovascular invasion, significant nuclear atypia, and significant mitotic activity. This lesion type should be considered malignant and treated with curative intent. These tumors are considered endometrial stromal tumors. Tumors in the other subgroup contain more than 50% sex cord-like cells, with no recognizable endometrial stromal component. Unlike ESTSCLEs, these uterine tumors resembling ovarian sex-cord tumor (UTROCST) are clearly separated from the surrounding tissues, exhibit distinct clinicopathological features, and are generally benign. Nevertheless, several cases of UTROSCT with recurrence and metastasis have been reported.–9 Therefore, the malignant potential of UTROSCT, as well as the appropriate treatments, remains controversial.Uterine tumors infrequently exhibit ovarian sex cord-like features. In 1976, Clement and Scully further classified the tumors into two subgroups. Specifically, positivity for calretinin plus one of the other three stains is considered diagnostic. Researchers have attempted to identify the risk factors associated with recurrence and metastasis of UTROSCT. Moore et al reported several features associated with a UTROSCT with malignant behavior, including older age, larger mass, presence of necrosis, lymphovascular invasion, cervical involvement, significant nuclear atypia, and significant mitotic activity. Nevertheless, only the presence of necrosis and significant mitotic activity were statistically significant. Lee et al reported that older age, larger mass, and significant mitotic activity were risk factors for malignant behavior. Here, we report a case of UTROSCT in an older patient who exhibited the presence of necrosis, lymphovascular invasion, significant nuclear atypia, and significant mitotic activity. However, no recurrence of this tumor occurred following the extended radical surgery.The immunohistochemical expression profile of UTROSCT is polyphenotypic, and a diagnostic panel of four stains  has been proposed.2A 57-year-old Japanese woman  had previously visited another clinic because of lower abdominal pain. A right ovarian mass and multiple uterine masses were detected using transvaginal ultrasonography, and she was referred to our hospital for evaluation and treatment. She had a history of hypertension and a family history of stomach cancer (father) and uterine leiomyomas and auditory tumor (mother). Magnetic resonance imaging (MRI) revealed a 50-mm mass in the right ovary and multiple fibroids in the uterine myometrium that were suspected to be an ovarian serous adenoma and uterine leiomyomas, respectively Fig. A,B. No mThe patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. The uterine body contained a tumor measuring 25 mm × 20 mm located in the posterior wall that was not continuous with the endometrium. It had a mixed white, yellow, and black cut surface Fig. C, D. HisAn immunohistochemical analysis of the surgical sample revealed that the tumor cells were positive for calretinin (diffuse), α-inhibin , CD99 , AE1/AE3 (diffuse), CD10 , estrogen receptor (diffuse), progesterone receptor (diffuse), S-100 protein (diffuse), vimentin (diffuse), CD56 , chromogranin A , and synaptophysin  with UTROSCT developed extrauterine metastases to a variety of sites, including the pelvic and abdominal peritoneum, ovary, lymph nodes, bone, liver, and lung. Moreover, three patients (8.8%) died as a result of the tumor.or Table ,[2–9] an In particular, the presence of necrosis and significant mitotic activity was statistically significant. The present case exhibited mitotic activity, a high MIB-1 labeling index, necrosis, and lymphovascular invasion and hence, was considered high-risk for recurrence. Nevertheless, the patient underwent a second extended radical surgery, including lymphadenectomy, and progressed without recurrence. These results suggest that, for a high-risk UTROSCT, both hysterectomy and extended radical surgery, including salpingo-oophorectomy, parametrium resection, and pelvic and para-aortic lymphadenectomy, should be considered. Although no effective chemotherapy regimen has been identified, high-dose progesterone therapy may be effective for UTROSCTs which often exhibit diffuse positivity for hormone receptor expression.In previous studies of UTROSCTs, malignant behavior was associated with older age, larger tumors, the presence of necrosis, lymphovascular invasion, cervical involvement, significant nuclear atypia, and significant mitotic activity. For this reason, the initial treatment often does not involve sufficiently extensive surgery. Because UTROSCTs are often located within the uterine myometrium and do not involve the endometrium, they are not detected by endometrial biopsy. Takeuchi et al reported that UTROSCTs exhibited high signal intensity on MRI diffusion-weighted images in contrast to the generally low signal associated with leiomyoma. Therefore, this modality is useful for a differential diagnosis. A combination of diffusion-weighted imaging and apparent diffusion coefficient maps are useful for distinguishing benign leiomyomas from leiomyosarcomas and smooth muscle tumors of uncertain malignant potential. Because the tumor in the present case exhibited high signal intensity on MRI diffusion-weighted images, we should have considered the possibility of malignancy. The initial surgery led to a diagnosis of UTROSCT, and a second-stage extended surgery was performed. We believe that second-stage surgery should not be avoided when a UTROSCT associated with several risk factors is encountered.UTROSCT is challenging because it is often detected incidentally after surgery for other benign lesions.JAZF1-SUZ12 fusion that is characteristic of endometrial stromal tumors, indicating that these lesions are unlikely to be of endometrial stromal origin. Instead, we confirmed the presence of the characteristic immunohistochemical features of UTROSCT, the absence of morphological features of the endometrial stroma, and the absence of continuity with the endometrium.This study was limited by a lack of molecular analysis. UTROSCTs lack the In conclusion, we encountered a patient with UTROSCT with sarcomatous features who was successfully treated by extended surgery and did not develop recurrent disease. These clinicopathological findings suggest that a UTROSCT associated with several risk factors  should be considered a malignant tumor and treated with curative intent.www.editage.jp) for English language editing.We thank Tomomi Katoh and Masanori Yasuda, Department of Pathology, Saitama Medical University International Medical Center, for their technical support. We also thank Editage (MS: conception and writing of the manuscript. SS, YA, SA, HM: collection of clinical data. IY: pathologic diagnosis and immunohistochemical analysis. KN: collection of clinical data and revision of the manuscript. MY: conception, pathologic diagnosis, immunohistochemical analysis, and revision of the manuscript. All authors read and approved the final manuscript prior to submission.Mitsutake Yano orcid: 0000-0002-4436-838X.


Renal cell carcinoma (RCC) with invasion into the renal vein is well described; however, invasion into the gonadal vein is a rare event with less than five cases reported in the literature. RCC occasionally presents with metastasis to the ovaries or the fallopian tubes, although this is also a rare occurrence. We present a case of locally advanced left RCC with direct extension into the ipsilateral gonadal vein with extension into the bilateral ovaries and uterus, which has not been previously described. Computed tomography (CT) in a 72-year-old female with a 35-pound weight loss indicated the presence of a 16-cm left renal mass with caudal tumor extension through the left gonadal vein and regional lymphadenopathy. There was no evidence of distant metastasis, and she underwent an open left radical nephrectomy. Intraoperatively, she was found to have direct extension of the tumor through the left gonadal vein into the uterus, bilateral ovaries, and the left fallopian tube. All visible disease was resected, and retroperitoneal and pelvic lymphadenectomy were performed. The patient had an uneventful hospital course. Pathology revealed clear cell RCC, Fuhrman grade 3. The final pathologic stage was pT4N1M1. The patient was ultimately noted to have pulmonary metastasis and was treated with immunotherapy with no evidence of disease progression. With 403,262 annual cases around the world, kidney cancer is the sixth most common cancer diagnosis in the United States , 2. RenaA 72-year-old female presented to her primary care physician reporting progressive fatigue, abdominal pain, and a 35-pound unintentional weight loss. She was noted to have a palpable left abdominal mass, and laboratory work was notable for hypercalcemia, thrombocytosis, and elevated alkaline phosphatase. Subsequent computed tomography (CT) imaging revealed a 16-cm left-sided renal mass with caudal tumor extension and bulky regional lymphadenopathy along the left gonadal vein . The patThe patient’s medical and surgical history was unremarkable except for insulin independent diabetes mellitus. A left-sided abdominal mass was palpable on physical exam. Further evaluation revealed no evidence of distant metastatic disease on CT imaging of the head, chest x-ray, recent CT chest, or CT abdomen and pelvis. Magnetic resonance imaging of the abdomen, obtained approximately 1 month prior to her surgery, did not reveal any evidence of left renal vein involvement. After extensive counseling and a thorough discussion of the risks involved and the benefits, she opted for surgical extirpation of her locally advanced renal mass and regional lymphadenopathy. She underwent an open left radical nephrectomy, total abdominal hysterectomy with bilateral salpingo-oophorectomy, and retroperitoneal and pelvic lymph node dissection. Intraoperatively, the mass was found to be large and bulky, extending toward the pelvis and invading the local structures. The ascending colon was reflected medially, at which time the mass was noted to be involving part of the mesentery, which required resection. Once the hilum was controlled, the mass was dissected down toward the pelvis and was noted to be involving the uterus and the bilateral ovaries. A supracervical hysterectomy with bilateral salpingo-oophorectomy was performed. The left gonadal vein with tumor thrombus was mobilized and the mass was dissected off the lateral abdominal wall, which allowed the whole specimen to be elevated en bloc until it was completely free. A retroperitoneal and left pelvic lymph node dissection was completed, and all specimens were sent for pathologic examination. Estimated blood loss was 750 mL, and there were no intraoperative or postoperative complications. The patient had an uneventful 5-day hospital course and was discharged to a subacute rehabilitation facility. Upon further review of the abdominal and pelvic CT scan, there was a heterogeneous appearance of the uterus, which had been assumed to be related to fibroids .Repeat CT chest postoperatively revealed several suspicious bilateral lung nodules, which were not seen preoperatively. Further staging was performed, which showed no other evidence of metastasis. Given the new finding of distant metastasis, the patient elected for combination immunotherapy with four cycles of ipilimumab and nivolumab, which was recently completed. The most recent imaging showed resolution of the right-sided lung nodules, stable left lung nodules, and no new sites of metastatic disease.The left kidney, supracervical uterus, bilateral fallopian tubes, and ovaries were sent for histopathological examination. The left kidney measuring 21.0 cm in greatest dimension showed a 15.6 × 11.2 × 11.1 cm yellow, gelatinous, hemorrhagic, and necrotic mass that obliterated the lower pole. The mass invaded the perinephric fat abutting Gerota’s fascia, the hilar fat, calyces, and distorted the renal sinus . The proMicroscopic examination revealed a tumor composed of nests of clear cells surrounded by interconnecting vascular framework. Cells with eosinophilic cytoplasm were also noted in combination with the clear cells. Immunohistochemical stains, including vimentin, were positive, and CD10 showed diffuse membranous positivity. Eosinophilic nucleoli were identifiable at 100×, consistent with Grade 3 tumor using the International Society of Urological Pathology (ISUP)/WHO grading scheme . No sarcTo our knowledge, there have been no previously reported cases of RCC with direct contiguous invasion into the ipsilateral fallopian tube, uterus, and bilateral ovaries occurring along the pathway of the gonadal vein. The mechanism for the involvement of the contralateral ovary in this case is unclear, as there was no evidence of invasion into the associated fallopian tube. There may have been an extension from the uterus to the ovary, or it may have been a result of hematogenous metastasis similar to the nodal disease noted at initial imaging and the distant pulmonary metastasis, which were ultimately discovered postoperatively. Metastasis to the ovaries or fallopian tubes, although rare, has been described and appears to be reported more commonly than direct extension of the tumor , 12. TheWe presented a case of local extension of RCC through the gonadal vein into the ipsilateral ovary, fallopian tube, and uterus, which has not been previously described. Based on the available histopathologic data, we suspect that the uterus, left ovary, and left fallopian tube were involved by contiguous spread, while the right ovary, lymph nodes, and pulmonary metastases were likely a result of hematogenous spread. This tumor was managed with surgical resection followed by adjuvant immunotherapy. At the most recent follow-up, the patient was doing well without evidence of new metastatic disease.


Gastric adenocarcinomas with low grade atypia may be difficult to diagnose as gastric cancer by preoperative biopsy. We report an extremely well-differentiated adenocarcinoma (EWDA) of the stomach which appeared like a submucosal tumor diagnosed by preoperative endoscopic submucosal dissection.A 70-year-old male was referred with a 3-month history of a submucosal-appearing lesion in the gastric wall found on endoscopy. Biopsies of the lesion were performed and were inconclusive for neoplasia. Endoscopic ultrasonography showed a low echoic tumor growing into the fourth layer of the gastric wall. It was difficult to identify the tumor by repeat biopsy. Endoscopic submucosal dissection of the lesion was performed and revealed adenocarcinoma, and laparoscopic total gastrectomy was performed. Histopathologic evaluation showed that the tumor was stage IIA (T3N0M0). There is no recurrence 12 months after resection.Gastric EWDAs are rare lesions, accounting for 0.6% of all gastric cancers. It is difficult to diagnose gastric EWDA especially if it appears like a submucosal tumor. This lesion was finally diagnosed by endoscopic submucosal dissection.Endoscopic submucosal dissection may facilitate establishing the preoperative diagnosis of a tumor thought to be a gastric EWDA based on its endoscopic appearance and pathological findings. Gastric extremely well-differentiated adenocarcinoma (EWDA) is defined as a neoplastic lesion consisting of highly differentiated neoplastic epithelium which mimics normal gastric mucosa or intestinal metaplastic mucosa with mild nuclear atypia, but has the ability to invade the gastric wall . It alsoA patient presented with a lesion that could not be characterized despite repeated preoperative biopsies including a boring biopsy. Gastric EWDA was suspected based on the endoscopic appearance like a submucosal tumor in the proximal gastric body and the lesion was finally identified after endoscopic submucosal dissection. To the best of our knowledge this is the first report of a gastric EWDA which appeared like a submucosal tumor diagnosed by endoscopic submucosal dissection.This work is reported in accordance with SCARE criteria .2Helicobacter pylori eradication therapy which was confirmed negative 3 months later. The first endoscopic examination revealed a lesion with depressions in the center on the surface, but protruding as a whole and appeared like a submucosal tumor, located on the posterior wall of the proximal gastric body  with gastric EWDA with an elevated lesion appearing like a submucosal tumor have been reported. The pathological findings, operative procedure, and initial diagnostic procedures for these patients are summarized in 4In patients with lesions without definitive pathological findings on biopsy, the diagnosis of a lesion suspected to be a gastric EWDA can be made based on endoscopic findings using endoscopic submucosal dissection to assess the findings in the submucosa. Due to their growth characteristics, formal resection may subsequently be needed.The authors declare no conflicts of interest or competing interests.None.Review of this case report was waived by the Jichi Medical University Institutional Review Board.Written and signed consent was given by the patient for this case report.Katsushi Suenaga: Conception of Study, Acquisition and analysis of data, drafting and critical revisions of the article, approval of final version.Shiro Matsumoto: Acquisition of data, critical revisions of the article, approval of final version.Alan Kawarai Lefor: Acquisition of data, critical revisions of the article, approval of final version.Yoshimasa Miura: Acquisition of data, critical revisions of the article, approval of final version.Yoshinori Hosoya: Acquisition of data, critical revisions of the article, approval of final version.Daigo Kuboki: Acquisition of data, critical revisions of the article, approval of final version.Hidenori Haruta: Acquisition of data, critical revisions of the article, approval of final version.Kentaro Kurashina: Acquisition of data, critical revisions of the article, approval of final version.Atsushi Kihara: Acquisition of data, critical revisions of the article, approval of final version.Daisuke Matsubara: Acquisition of data, critical revisions of the article, approval of final version.Yasunari Sakuma: Acquisition of data, critical revisions of the article, approval of final version.Joji Kitayama: Acquisition of data, critical revisions of the article, approval of final version.Naohiro Sata: Acquisition of data, critical revisions of the article, approval of final version.Not Applicable.Katsushi Suenaga MD.Not commissioned, externally peer-reviewed.