TSC1 or TSC2 alterations, which result in the activation of the mTOR pathway, or TFE3 fusions. Given these molecular characteristics, mTOR inhibitors have recently been approved by the FDA in the treatment of malignant PEComas, particularly in those with TSC1/2 alterations. Therefore, molecular analyses may be helpful for both the diagnostic workup of and predicting response to mTOR inhibitors in cases of malignant PEComas.Malignant perivascular epithelioid cell tumors (PEComas) are exceedingly rare malignant mesenchymal neoplasms with characteristic morphological and immunohistochemical (IHC) patterns. However, some malignant PEComas are poorly differentiated with atypical histopathological features, making a definitive diagnosis difficult. PEComas are most commonly found in females and often show either TP53 and TSC2, supporting a definitive diagnosis of malignant PEComa. The patient was then switched to nab-sirolimus, with initial stabilization of the disease.Here, we report a case of an aggressive, 23 cm mesenteric malignant PEComa with multiple peritoneal metastases in a young male patient. Pathological examination of the initial biopsy showed a malignant epithelioid neoplasm with high-grade morphology and atypical immunoprofile, which precluded a definitive diagnosis. Because of the patient’s excessive transfusion requirements due to intra-tumoral hemorrhage, a palliative R2 resection was performed. Histopathological examination of the tumor revealed focal immunoreactivity for Melan-A, HMB-45, desmin, and CD117. Although a diagnosis of malignant PEComa was favored, other entities such as epithelioid gastrointestinal stromal tumor (GIST) or melanoma could not be definitively ruled out. Given the favored diagnosis, the patient was started on sirolimus, an mTOR inhibitor, rather than chemotherapy. Molecular analyses were performed and the tumor was found to harbor mutations in TSC1/2 alterations, for both the definitive diagnosis of malignant PEComas and predicting their response to nab-sirolimus.This report details a multidisciplinary approach for the diagnosis and management of a highly aggressive, metastatic malignant PEComa in a young male patient. The basis for the treatment of malignant PEComas with the recently FDA-approved mTOR inhibitor, nab-sirolimus, is also reviewed. In summary, this case highlights the importance of molecular analysis, particularly The online version contains supplementary material available at 10.1186/s13000-023-01323-x. Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with characteristic perivascular distribution of epithelioid cells which are immunoreactive of both smooth muscle and melanocytic markers. Definitive diagnosis of PEComas relies largely on histological examination with characteristic morphological and immunohistochemical (IHC) findings. However, diagnosing PEComas may be difficult in cases with poor differentiation or atypical immunophenotypes. Genetic studies have recently demonstrated that most PEComas harbor either TSC1 or TSC2 alterations, both of which result in the activation of the mammalian target of rapamycin (mTOR) signaling pathway and tumor proliferation . In addiWhile the majority of PEComas are indolent, some can display a malignant clinical course. Malignant PEComas are exceedingly rare with an incidence of < 1/1,000,000 and their management is actively evolving , 5. RadiA 26-year-old male with a history of asthma initially presented to the emergency department with two weeks of progressively worsening constipation, early satiety, and a twenty-pound unintentional weight loss over less than 3 months. On presentation, patient was found to be febrile and tachycardic. Further workup showed severe iron deficiency anemia with hemoglobin below 5 gm/dl. Computed tomography (CT) imaging demonstrated a 20 × 10 × 15 cm abdominopelvic mass and extensive peritoneal disease  was placed to bypass the obstructed bowel and was successful. The patient was then able to tolerate a normal diet.TP53-p.R337C missense mutation, with an allele frequency of 52.5% . In addition to the TP53 mutation observed by the in-house gene mutational panel, the results from whole exome sequencing showed a pathologic TSC2-p.K1165fs mutation with an allele frequency of 58%  as mesenchymal neoplasms composed of perivascular epithelioid cells that are closely associated with blood vessel walls. These tumors co-express melanocytic and smooth muscle markers . The PECAlthough most cases follow a benign clinical course, a small subset of PEComas do behave aggressively , 15, 16.TSC1 or TSC2 alterations and those with TFE3 fusions [TSC2 locus, such as deletion of 16p or TSC2 mutations, resulting in loss of function of the tuberin protein are commonly found in PEComas [TSC1, hamartin, which functions as a tumor suppressor by inhibiting the mTOR signaling pathway. Loss of tuberin function or hamartin results in the uncontrolled activation of mTOR signaling and potential tumorigenesis. Concurrent TP53 mutations are found in up to 63% of TSC2-mutated PEComas, the significance of which remains to be further delineated [TFE3 rearrangements [TFE3-rearranged PEComas tend to occur in younger patients and display strong nuclear TFE3 expression. TFE3 is a transcription factor that forms a homodimer or heterodimer commonly with MiT-related proteins, such as TFEB, to promote cellular growth and proliferation [Two major molecular subgroups have recently been identified for PEComas: those with  fusions , 18. Int fusions . Loss ofngements . These Tferation . Furtherferation .Our patient’s malignant PEComa is unusual in many aspects. Firstly, it occurred in a young 26- year-old male whereas the majority of PEComas (> 80% reported in the literature) are found in females . There wTP53-p.R337C and TSC2-p.K1165fs mutations, and showed no BRAF, NRAS, CKIT, and PDGFRA hotspot mutations. These results rendered melanoma and conventional GIST highly unlikely and confirmed the diagnosis of a PEComa. Lack of TFE3 nuclear immunostaining and TFE3 fusions by our in-house gene fusion panel further ruled out a TFE3-rearranged PEComa. Although frame-shift mutations in TSC2 are common in multiple disease states, the pathologic TSC2-p.K1165fs frame-shift mutation involving exon 30 seen in this tumor has not been previously reported in literature.The molecular findings in this case were instrumental in confirming the diagnosis. Gene sequencing identified pathologic TSC2 mutation not only confirmed the diagnosis, but also suggested that our patient may benefit from the recently FDA-approved mTOR inhibitor, nanoparticle albumin-bound (nab) sirolimus. Intravenous nab-sirolimus was shown in pre-clinical studies to have higher intra-tumoral drug accumulation, mTOR suppression, and tumor growth inhibition compared to its oral counterparts, such as sirolimus and everolimus [TSC2 mutation, whereas it was 13% without. Indeed, treatment of our patient’s unusually aggressive malignant PEComa with nab-sirolimus showed initial objective evidence of disease control by cross-sectional/nuclear imaging [Detection of a erolimus , 38. The imaging .Limitations of this case report include the retrospective nature of this review, short duration of follow-up and requirement for more patients to validate the diagnostic and treatment strategy presented herein. Although generalities are limited in a case report, our case highlights the importance of molecular tumor profiling in conjunction with light microscopy and IHC findings for the definitive diagnosis and identification of novel actionable targets in malignant PEComas.Additional file 1: Supplemental Figure 1. Molecular findings. (A) Results from in-house hotspot gene mutational panel reporting a TP53-pR337C mutation. (B) Summary of the Caris Molecular Intelligence Tumor Profiling report highlighting the TSC2 p.K1165fs mutation.


Here, we discuss a case of a 42-year-old premenopausal female who presented with chronic pelvic pain and recurrent small bowel obstruction during menstruation. The patient reported a nine-year history of pelvic pain and a four-year history of episodic small bowel obstruction requiring multiple prior inpatient admissions. During these admissions, the obstruction was managed conservatively with bowel rest and nasogastric tube placement; however, symptoms would recur with subsequent menstrual cycles. Computed tomography showed diffusely dilated loops of small bowel with a transition point in the central anterior pelvis, and magnetic resonance enterography revealed a mass-like area involving small bowel loops in the mid pelvis. The patient underwent laparoscopic surgical intervention including bowel resection with re-anastomosis, hysterectomy, bilateral salpingectomy, and left oophorectomy. Intraoperative findings included severe distention of the proximal bowel with a discrete deep endometriosis lesion of the terminal ileum which was confirmed on final pathologic examination. This case emphasizes the importance of considering endometriosis as the etiology of recurrent catamenial small bowel obstruction, particularly in premenopausal women. Endometriosis is a complex disease defined by the presence of endometrial-like tissue outside of the uterus. It is a chronic inflammatory condition associated with pelvic pain and infertility most commonly in reproductive-age women . Three pA 42-year-old G0P0 female with a known history of endometriosis presented for evaluation of chronic pelvic pain in the setting of recurrent small bowel obstruction. The patient reported a nine-year history of pelvic pain for which she previously underwent laparoscopy at an outside facility, the documentation of which was not available at the time of consultation, and was reportedly diagnosed with endometriosis. She subsequently attempted medical management with multiple hormonal therapies including combined oral contraceptives, gonadotropin-releasing hormone (GnRH) analogues, and progestin-only pills; however, she was unable to achieve symptom relief. At the time of presentation, the patient had not been on hormonal therapy for two years. She had also developed recurrent small bowel obstruction requiring multiple inpatient admissions during the preceding four years. During these admissions, conservative management with bowel decompression with nasogastric tube and bowel rest with NPO status resulted in relief of obstruction, but these episodes were recurrent with her subsequent menstrual cycle. Computed tomography (CT) scan showed diffusely dilated loops of small bowel with a transition point in the central anterior pelvis Figure . Prior mThe diagnosis of endometriosis is complicated by variations in patient presentation and poor correlation between phenotype, location of lesions, and symptoms. Additionally, response to treatment is highly variable and appears to differ based on the endometriosis subtype. While the recommended management of peritoneal endometriosis is less clear, surgical excision is generally the treatment of choice for endometriomas and DE . SurgeryDE of the small bowel is a rare phenomenon that requires a high level of clinical suspicion. Clinicians should consider bowel DE as a possible etiology of small bowel obstruction in premenopausal patients, particularly in the setting of chronic pelvic pain, dysmenorrhea, or known endometriosis. Additional workups may include transvaginal or transrectal ultrasound as well as magnetic resonance imaging and/or enterography. Increased clinical awareness and imaging may decrease delays in diagnosis and surgical management thereby reducing patient morbidity and improving quality of life.


Introduction: Low-grade myofibroblastic sarcoma (LGMS) is a rare tumor entity which occurs in the subcutaneous and deep soft tissues; it is less common in the bone with a predilection for the extremities and the head and neck region. As confirming the diagnosis is difficult and treatment strategies are not standardized, we aimed to identify patient and tumor characteristics, and to summarize treatment strategies and their clinical outcomes to guide surgeons. Methods: Included were full articles reporting patients with histology of LGMS in the extremities, excluding tumors of the trunk. All patients underwent surgery but with different extend, from marginal to wide resection. Included studies should inform about local recurrence, metastasis, or evidence of disease, depending on the surgical treatment. We conducted a structured search using MEDLINE (via PubMed), Web of Science, EMBASE and Cochrane Central Register of Controlled Trials (CENTRAL) to identify studies on low-grade myofibroblastic sarcoma of the extremities. Study designs like randomized controlled trials, systematic reviews, prospective trials, retrospective studies, and case reports were included. Prospective studies and comparative studies were not available at all. Therefore, meta-analysis was not possible and statistical analysis was purely descriptive. Results: Of the 789 studies identified from our initial search, 17 studies including 59 cases reported LGMS of the extremities with the surgical treatment and clinical outcome and were therefore analyzed. In addition, we present the rare case and surgical management of a 28-year-old male patient with residual LGMS of the thumb after an initial incomplete resection. The current literature suggests that a wide excision with R0 margins should be considered the standard treatment for LGMS. In cases where surgery leads to significant functional impairment, individual options like free tissue transfer from a donor site have to be considered. Therefore, we also present an illustrative case. For all selected case series and case reports, a high risk of confounding, selection bias, information bias, and reporting bias must be anticipated. Nevertheless, this systematic review provides a comprehensive overview on surgical treatment and clinical outcomes in LGMS surgery of the extremities. Low-grade myofibroblastic sarcoma (LGMS) is an infrequent, well differentiated, and atypical neoplasia characterized by a growth pattern reminiscent of fibromatosis. Although a sarcoma displaying ultrastructural myofibroblastic features was initially described by Vasudev and Harris in 1978 , its defImaging diagnostics for LGMS typically include X-ray, MRI , and CT scans. Imaging features of the non-specific soft tissue mass can vary depending on the tumor’s location. In addition to calcifications or ossifications, lesions with bone involvement may appear as osteolytic and destructive masses . On MRI,Due to all the limiting factors in the histopathological diagnostic routine described above and the challenge in distinguishing between tumor subentities with similar benign, intermediate, and malignant potential, the distinct clinical behavior of most soft tissue and bone sarcomas serves as a crucial determinant for devising treatment protocols ,19. In tIn this systematic review, we identified studies that elucidate and evaluate the surgical management and clinical outcomes associated with LGMS affecting the extremities for patients who underwent surgery, varying from marginal to wide resection. The included studies inform about local recurrence, metastasis, or evidence of disease depending on the surgical treatment. Study designs like randomized controlled trials, systematic reviews, prospective trials, retrospective studies, and case reports were all accepted for inclusion. This review complies with the recommendations of the Cochrane Handbook for Systematic Reviews of Interventions , and is We systematically screened MEDLINE (via PubMed), Web of Science, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) from 1946 to 2023 to identify studies on LGMS of the extremities. The search strategy contained the subject heading low-grade myofibroblastic sarcoma. MESH terms included fibrosarcoma/surgery and extremities. The full search strategy is displayed in After removing duplicates we used the eligibility criteria  for abstA total of 1404 studies was obtained from the systematic literature search. After removing duplicates and using the eligibility criteria  for abstExisting literature predominantly comprises case series, case reports, a limited number of retrospective observational studies, analytical investigations utilizing the Surveillance, Epidemiology, and End Results (SEER) database, and a retrospective multicenter study conducted by the Japanese Musculoskeletal Oncology Group. The mean age of the patients was 43.6 years, with a range spanning from 6 to 86 years. A balanced male-to-female ratio of 1:1 was observed, as indicated in the available studies. Detailed patient and tumor characteristics are summarized in Concerning extremities, radiologic findings showed, in some cases, bone-destructive lytic lesions and calcification within the tumor ,22,27,29All 59 documented cases underwent surgical intervention as part of their treatment regimen. The spectrum of treatment modalities encompassed a range from local excision to wide resection, though without a clearly defined safety margin. Among these cases, wide resection was performed in 20 cases (34%), with an additional 5 cases (8%) subsequently undergoing radiotherapy, and 2 cases (3%) receiving adjuvant chemotherapy. In two cases, prosthetic implantation was employed following wide resection of the tumor. Marginal or local excision, including intralesional resection, was noted in 15 cases (25%), with 2 cases supplemented by adjuvant radiotherapy. Notably, three cases culminated in amputation . In 12 instances (20%), the specific surgical procedure was not explicitly detailed. In total, 12 patients (20%) received adjunctive therapy, either radiotherapy or chemotherapy, subsequent to their surgical intervention.Follow-up information was obtained from 47 patients, with a mean follow-up time of 51 months (range 6–192 months). In total, 13 (22%) suffered from local recurrence, while 1 patient even had eight recurrences in 8 years (case 58). In one case, local recurrence occurred after wide resection and chemotherapy, and was treated by radiotherapy (case 7). One case mentioned transformation of IMT into LGMS after three recurrences (case 19). Another local recurrence after 70 months led to amputation of the leg. In case 45 of this study, another amputation was performed after local recurrence in the tibia. Five cases (10%) showed distant metastasis in the follow-up time from 8–56 months, while one case with metastasis was identified at time of diagnosis (case 54). Three of them occurred in the lung  and one in the bones (case 57). One case described a tumor-related death with cardiac metastasis (case 15).A 28-year-old male patient presented at our clinic with a residual thumb tumor. Initially, he noticed a gradually growing mass on his thumb in September 2019 and subsequently sought medical attention in October at an external clinic due to the presence of a tumor mass on the distal phalanx of the thumb. There was no history of prior skin lesions or trauma. An X-ray examination of the thumb did not reveal any evidence of bone infiltration, and an initial MRI was not conducted. The tumor was excised without prior biopsy. In December 2019, two months post-surgery, the patient presented at our hospital for the first time, reporting recurrent swelling at the surgical site. A comprehensive examination indicated that the patient was in good general health and denied any tobacco or alcohol usage. The thumb displayed an elastic nodular tumor mass on the distal ulnar aspect of the phalanx A. The phOur presented case (case 33) showed an approx. 0.9 × 0.8 × 0.6 cm measuring recurrent tumor with a yellowish-beige cut surface and diffuse borders to the surrounding soft tissue. It was identified in the deeper dermis and subcutis of the finger partial amputate within the macroscopic evaluation at the Institute of Pathology, University Hospital Heidelberg. Histologically, the recurrent tumor showed a diffusely invasive growth pattern with perineural tumor growth and enclosure of intradermal Vater-Pacini bodies, as well as a focal relation to epidermis. It consisted of atypical, compactly packed spindle cells with an arrangement in shorter fascicles, and was embedded in a hyalinized collagenous matrix . The monTFE3 was observed immunohistochemically, no translocation of the TFE3 gene locus was identified by fluorescence in situ hybridization (FISH) using dual color break apart gene probes. Thus, the diagnosis of the alveolar soft part sarcoma was cancelled. Finally, the FISH investigating the chromosome 22q13 could not verify the presence of a PDGFB translocation. In the synopsis of this result, as well as considering the proof of the fusion COL1A2::GNS (exon 6/exon 4) by RNA sequencing, which has not been described in literature so far, the diagnosis of dermatofibrosarcoma protuberans seemed improbable, and was supported only by microfocal weak immunoreactivity against CD34 and the lack of plaque-like neuroid neoplastic areas. Despite a weak diffuse positivity for CD34 (in ca. 10% of tumor cells), negativity for STAT6, several nuclear atypia and the lack of patternless haphazard arrangement of the neoplastic cells, and the lack of NAB2::STAT6 fusion transcripts, the diagnosis of a solitary fibrous tumor (SFT) could also not be confirmed.The primary tumor , located in the deeper dermis and subcutis, showed a similar histological aspect, however, including a central tumor necrosis and several mitoses up to highly pleomorphic nuclei (ca. 5 mitoses/HPF). Due to the combined nuclear pleomorphism and infiltrative growth pattern, the differential diagnoses of fibromatosis and nodular fasciitis could be excluded. In the context of confirming the local recurrence, the fact that the surgical margins of the primary tumor resection were not free of tumor is to be emphasized. Extensive immunohistochemical staining of the primary tumor was performed at the Department of Pathology, University Hospital of Muenster. The neoplastic cell population showed a homogenous expression of the myogenic markers smooth muscle actin (SMA) and desmin , accompanied by a weaker immunoreactivity of CD10, as well as vascular markers ERG (strong), CD34 , and D2-40. The INI1 expression was preserved. The immunohistochemical staining was negative for several other markers . The proConsidering the intermediate-grade biological behavior of the tumor, with early recurrent growth after an initially incomplete resection, the young age of the patient, and the importance of the thumb for the hand function, a two-stage surgical intervention was indicated in our case. In the first step, a wide excision was performed to obtain clear resection margins. Soft tissue was resected, including the ulnar half of the bony distal phalanx A. TemporLow-grade myofibroblastic sarcoma (LGMS) represents a particularly uncommon and often underestimated tumor type that can manifest within the extremities. The reported incidence of this tumor is thought to surpass previous estimations due to the inherent challenges associated with its precise diagnosis. Given the limited number of small-scale clinical studies and individual case reports in existence, it becomes imperative to convey the primary insights concerning diagnosis, surgical management, and outcomes through a comprehensive review of the available collective experiences. This endeavor serves a dual purpose: enhancing clinical practices within the context of rare diseases, and furnishing a more comprehensive informational foundation for clinicians to make informed treatment decisions ,4,14,33.In the current study, we have systematically extracted and amalgamated the principal demographic and clinical attributes characterizing LGMS occurrences in the extremities from the extant body of literature. Additionally, we share our own encounter with an exceedingly rare instance of LGMS affecting the thumb. To the best of our knowledge, no publication has undertaken the task of aggregating reported instances of LGMS within the extremities in a quantitatively structured manner.p < 0.05). Moreover, Chan et al. noticed a significantly different tumor size comparing head and neck LGMS and non-head and neck LGMS, the latter having a significantly greater number of cases with tumor size > 4 cm [Different literature reviews describe the location distribution as follows: most cases occur in the head and neck region, followed by the trunk and extremities , but LGMe > 4 cm . Kito ete > 4 cm  showed aEstablishing uniform diagnostic criteria for this rare tumor, using both MRI and histopathological evaluation, has remained a challenge. However, the inclusion of preoperative MRI is crucial in the evaluation of soft tissue sarcoma . It can The complexity of arriving at a definitive diagnosis necessitates a combined approach involving histopathological and immunohistochemical analyses. Strict determination of the grade of nuclear pleomorphism in each tumor is difficult because of the wide range of histologic features ,23.During the diagnostic process, consideration should be given to incisional biopsy as it facilitates an accurate diagnosis and justifies the invasiveness associated with subsequent surgical interventions . For lesThe most common treatment for LGMS in the studies described here was wide excision. Most studies emphasize excising the tumor with an adequately wide margin ,26. But In this study, we found a local relapse of 22% concerning LGMS of the extremities. Concerning LGMS of different locations, we find a range of recurrence from 13.3–44.4% in the literature ,22,27. WWhile some practitioners have employed radiotherapy or chemotherapy as adjunctive treatments , a comprDistinguishing LGMS of the hand, particularly the thumb, from other tumorous lesions within the extremities, is imperative due to the anatomical proximity to critical structures, resulting in the need for meticulous resection to achieve adequate margins, often requiring subsequent tissue reconstruction. In the context of hand LGMS, only three case reports have been documented case 13, 30, and 48). Initially, the painless nature of the tumor can lead to misdiagnosis as a benign lesion, subsequently resulting in incomplete resection and residual tumor, thus contributing to local recurrence, as illustrated in our presented case. The rapid occurrence of local recurrence within two months of the initial resection indicates the potential for an inadequate initial procedure. In our approach, we employed a two-stage surgical intervention to ensure negative margins. The presented Holevich’s neuro vascular flap is a common and reliable choice at this anatomical region to simultaneously provide defect coverage and preserve local sensibility without compromising surgical oncologic principles . Most im, and 48.Some important limitations should be noted. Firstly, LGMS is a rare tumor entity and evidence on therapy and prognosis of the disease is sparse. Except for one multicenter retrospective study, only small retrospective studies, case reports and case series could be included in this systematic review. Prospective studies and comparative studies are not available at all. Therefore, meta-analysis was not possible and statistical analysis was purely descriptive. For quality assessment, we used the critical evaluation checklist by Moola et al.  for caseLGMS is a rare neoplasm seldomly located in the extremities. Imaging and clinical appearance frequently lead to misdiagnoses as a benign lesion, with consequent insufficient resection and local recurrence but rarely metastasis. Therefore, initial complete resection with negative margins should be the primary oncological goal. Concerning tumors of the hand and limb, function preserving surgery is desirable. Neurovascular flap reconstruction can be used to sufficiently cover the defect and preserve sensibility.Prospective studies are necessary to provide more information about standardized surgical therapy.


A 24-year-old woman complained of intermittent dysphagia for 2 years. She reported a previous medical history of asthma, hyperthyroidism, and myocarditis. Physical examination revealed no special abnormalities. Computed tomography detected a 5.2 × 4.2-cm mass with a clear boundary in the esophagus . EndoscVideo 1 Endoscopic resection for an esophageal inclusion cyst.Esophageal cysts are rare, with an incidence of one in 8200Endoscopy_UCTN_Code_TTT_1AO_2AG


Dentinogenic ghost cell tumor is a rare benign tumor that accounts for less than 3% of all cases and consists of the stellate reticulum, which is made up of enamel epithelioid and basaloid cells. Although DGCT is a benign tumor, the local infiltration of the odontogenic epithelium or recurrences have been reported, and its detailed pathology and treatments remain unclear.This report describes the case of a 60-year-old Japanese male diagnosed with a maxillary dentinogenic ghost cell tumor. Images showed well-circumscribed, multilocular cystic lesions with a calcified substance in the interior. Marsupialization was performed along with biopsy to prevent the expansion of the lesion, and a partial maxillectomy was performed 2 years after the initial examination. Histopathological findings showed ameloblastomatous proliferation containing clusters of ghost cells and dentinoid materials, resulting in the diagnosis of dentinogenic ghost cell tumor. This article also reviews recently reported cases of dentinogenic ghost cell tumor.It is important to perform marsupialization, proper resection, and postoperative follow-up because of possible recurrence. Dentinogenic ghost cell tumors (DGCT) are composed of stellate reticulum consisting of enamel epithelioid and basaloid cells; these tumors also include ghost cells with multiple characteristics and osteodentines. Although DGCT is a benign tumor, the local infiltration of the odontogenic epithelium has been observed . DGCT waIn this study, we achieved good outcomes by combining marsupialization and radical resection for a case of DGCT that developed on the maxilla and report herein with a review of previous cases reported as DGCT.A 60-year-old Japanese man presented at our hospital in April 2018. The patient, who had a medical history of angina pectoris and no family history, complained of protrusion of the left maxilla and a sensation of nasal obstruction.The patient had noticed a bulge in the left maxillary gingiva for several years before the first examination. However, the bulge was ignored, as it was not painful. Although the patient’s face deformed gradually, the patient did not undergo treatment for financial reasons. Thus, the patient developed a sensation of nasal obstruction that started several months before the first examination and then visited our department.For extraoral findings, the facial asymmetry was due to bulging of the left maxillary body from the left nasal ala to the left upper lip. No hypoesthesia was observed of the infraorbital region. For intraoral findings, an approximately 60 × 40 × 30-mm well-circumscribed elongated bone-like bulge with a flat and smooth surface was observed from the labial and buccal gingiva to the buccal mucosa corresponding to the left upper central incisor of the second molar Fig. . No vitaThe first panoramic X-ray image showed radiolucent multilocular cystoid images around the left maxillary sinus and the disappearance of the border of the maxillary sinus. Knife-cut-like resorption of the dental roots of the maxillary teeth extending from the right central incisor to the left second molar was noted Fig. . ComputeFollowing these findings, we made a clinical diagnosis of a left maxillary bone neoplasm and considered the treatment course. Clinically, the progression of the tumor was suspected and indications for surgery were explained to the patient. However, the patient did not consent to surgery as an initial treatment for financial reasons and the need to continue providing long-term care for his ill wife. Concurrently, the patient complained of a sensation of left nasal obstruction, and, to avoid further progression of the tumor, marsupialization and biopsy were performed. Under local anesthesia, a fine needle aspiration was performed and a dark red serous fluid was obtained Fig. . A 30 × After marsupialization, the patient was fitted with an obturator and buccal protrusion gradually decreased. CT scans performed in December 2018 and 2019 showed the growth of radiopaque bone-like tissue in the interior of the tumor without metastases to the surrounding tissues Fig. . As the To confirm the absence of new lesions in the surrounding tissues, an MRI was performed immediately before surgery. Lesions with mixed low- and high-signals observed from the left alveolar ridge to the left maxillary sinus on T2-weighted imaging were observed, but no clear metastases in the surrounding tissues Fig. . Under tA Weber-Ferguson incision was made from the left filtrum to the nasal ala to secure the operative field. The buccal aspects of the left maxilla and the tumor were exposed, and radical resection with a safety margin of approximately ≥ 5 mm was planned. The oral mucosa was cut to include tumor marsupialization Fig. a. PartiaHistopathological findings: the excised specimen revealed that a collagenous fibrous capsule encapsulated the lesion, and no invasive proliferation was observed in the adjacent tissues Fig. A, B. Theet al. in 1981. They suggested this name because the formation of dentinoid in relation to the epithelial islands was a conspicuous feature and ghost cells were found to a varying degrees [et al. [et al. [et al. [The term DGCT was first proposed by Praetorius  degrees . Later,  degrees . Further degrees . To date [et al.  summariz [et al.  summarizThe 27 cases we searched for in this review consisted of 3 retrospective studies –11 and 1Many of the DGCTs were well circumscribed in imaging findings with mixed radiolucency and radiopacity, as was the case with our patient. Simultaneously, DGCT can sometimes be observed as a simple radiolucent lesion. This difference is attributed to the level of calcification of the interior of the lesion . MoreoveHence, the DGCT diagnosis should be made on the basis of the findings of numerous ghost cells and the presence of osteodentin and solid characteristics observed on histopathological evaluation. In this study, benign odontogenic tumors, such as ameloblastic fibrodentinoma, were suspected because the biopsy performed during marsupialization showed dentin and interstitial cyst formation. However, only part of the lesion was observed, which did not provide adequate evidence to make a definitive diagnosis Fig. . StellatAlthough discussions on optimal treatment are ongoing, intraosseous DGCT has been noted to behave more aggressively than peripheral DGCT , 27, 34.Despite that, it is possible to encounter relatively enlarged lesions as well as patients for whom resection cannot be indicated smoothly owing to a lack of patient compliance, and so on. In this study, radical resection could not be performed as an initial treatment because of the patient’s financial and personal status. Therefore, marsupialization was performed first, and no further enlargement of the lesion was observed after careful subsequent follow-up, which allowed us to perform surgical removal by partial maxillectomy 2 years after the first examination. This review only includes one case , making We reported a case of DGCT that showed a good prognosis following radical resection after marsupialization and reviewed previous reports. Although reports of DGCT are few, a comprehensive diagnosis based on clinical, imaging, and pathological findings is imperative. As the possibility of local recurrence cannot be ruled out, radical resection of the tumor is necessary, even if marsupialization is performed as the first-line of treatment, and the patient should be followed up with.


Renal primitive neuroectodermal tumor (rPNET) has the characteristics of a difficult preoperative diagnosis, a high degree of malignancy, easy early metastasis or postoperative recurrence, a poor prognosis, and so on. However, rPNET that has no metastasis before surgery can have a good survival prognosis only after radical surgical resection.We report the case of a 14-year-old male patient with a renal tumor who underwent open radical left nephrectomy without radiotherapy or chemotherapy before or after surgery, as confirmed by postoperative pathological results. The prognosis was followed up by a regular review of the chest and whole abdomen on CT, hematuria analysis, renal function, and electrolytes according to the guidelines for renal cancer.Postoperative pathological results confirmed rPNET; no adjuvant radiotherapy or chemotherapy were performed after surgery; no tumor recurrence or metastasis were observed during the follow-up of nearly 5 years.Despite the high degree of rPNET malignancy, patients without metastases before surgery can still obtain a good survival prognosis through timely radical surgery. Primitive neuroectodermal tumor (PNET) is a highly malignant small round-cell tumor with neurogenic differentiation . With thA 14-year-old male patient was admitted to the hospital with left-sided lumbago and abdominal pain, a 1-week fever, and no relevant medical history. At physical examination, a large mass could be felt under the costal margin of the left waist, which was hard in quality and fixed in position without tenderness or pulsation. The lower end of the umbilicus was flat, and there were no other obvious abnormalities. Auxiliary examination through a color ultrasound of the urinary system showed a mixed echo (13.2 cm × 10.9 cm) of the lower pole of the left kidney, of unknown nature and a clear border. Urinary CTU + CTA revealed a large mass of soft tissue density shadow (13.2 cm × 10.6 cm) in the lower pole of the left kidney. The mass showed uneven enhancement, but there was no enhancement in cystic lesion or necrotic area. The mass had coarse supplying arteries, and the CT value ranged from approximately 22 to 44 HU . A chestPNET is a highly malignant small-round-cell tumor with neurogenic differentiation. PNET occurs more rarely in the kidney, although its clinical manifestations are similar to those of renal cell carcinoma: abdominal pain or lumbago, hematuria, fever, night sweats, and so on, even though the primary symptoms were the presence of an abdominal mass and weight loss. Risi  reportedThe size of PNETs varies. Clinically, all rPNET tumor bodies are large, with a maximum diameter of 12.5 cm and above , the tumIn recent years, reports on the molecular genetics of rPNET have been increasing, with more than 90% of these predicting that translocation of t  q24; q12) will occur. The EWS gene is found on chromosome 22q12 and has 17 exons, with most breakpoints occurring in exon 7. The FLI-1 gene found on chromosome 11q24 belongs to the ETS proto-oncogene family and acts as a transcriptional activating factor. Further research showed that the t   translocation leads to an EWS/FLI-1 radical fusion  will occ, the proAt present, there is no unified standard for the treatment of rPNET, which mainly includes surgical treatment, chemotherapy, and radiotherapy. Surgical excision is mainly applicable to cases without metastasis, and radical nephrectomy is usually adopted. There have also been reported cases of nephrectomy with nephron-sparing partial nephrectomy and postoperative adjuvant chemotherapy, but no metastasis was found after 2 years of follow-up . It has The prognosis of rPNET is generally poor, with an overall 5-year survival rate of 45%–55% . ThyavihDespite the high prevalence of rPNET malignancy, patients without metastasis before surgery can still obtain a good survival prognosis through timely radical resection.


Management of supernumerary teeth fused to the labial surface of permanent maxillary central incisors would require a multidisciplinary approach comprising of endodontic treatment, periodontal recontouring, and cosmetic composite restoration.The reported cases of supernumerary teeth fused to the labial surface of maxillary central incisors are rare. Such cases need multidisciplinary approaches. Herein, management of a supernumerary tooth fused to the labial surface of a maxillary central incisor is reported. Due to the presence of a communication path between the root canal systems of the two fused teeth, root canal therapy was performed first for the maxillary left central incisor and the supernumerary tooth. The crown of the supernumerary tooth was then removed in a surgical setting while preserving the root to maintain the thin covering of alveolar bone and prevent future periodontal problems. Subsequently, an esthetic composite restoration was performed.  The patient had a non‐contributory medical history with no accompanying physical or mental abnormalities. She did not report any dental treatment in the affected area. No previous history of traumatic injury to the jaws or teeth was reported in the affected area. Intraoral examination revealed a conical supernumerary tooth in close contact with the labial surface of the maxillary left central incisor Figure , which rAfter the completion of endodontic treatment, periodontal surgery was performed to remove the supernumerary tooth and reshape the involved region. A full‐thickness mucoperiosteal flap was elevated, and the surgical area was exposed. Since there was a thin layer of bone covering the radicular portion of the supernumerary tooth Figure , only thAt the 6‐month follow‐up, the periradicular tissues appeared normal. The probing depth was within the normal range, and the patient was satisfied with the esthetic outcome Figure .3Fusion is a rare dental anomaly that may occur in primary, permanent, or even supernumerary teeth. Its diagnosis can be challenging since it may be mistaken with gemination when it coincides with hypodontia or hyperdontia. Fusion of a permanent and a supernumerary tooth is an infrequent occurrence compared to fusion of primary teeth. The incidence of fusion of a permanent and a supernumerary tooth is reported to be 0.1%, and it generally involves the maxillary anterior teeth.The best treatment plan depends on the nature of the fusion, the age of the patient, and the patient's overall dental health. Here are some of the interdisciplinary treatment methods that have been proposed; Orthodontic treatment can be used to correct the alignment of fused teeth and improve the patient's bite. This treatment may involve the use of braces, aligners, or other orthodontic appliances.Several materials have been proposed for root canal obturation, including calcium silicate cements, gutta‐percha, and different types of sealers.Additionally, the coronal extension of the root canal filling material was set 2 mm apical to the cementoenamel junction to avoid discoloration due to penetration of root filling particles into the dentinal tubules in the cervical region of the access cavity.Another major concern while using MTA is its washout potential following exposure to fluid flow.Hadi Assadian: Investigation; methodology; writing – review and editing. Behnam Bolhari: Investigation; methodology. Mehrfam Khoshkhounejad: Investigation; supervision; writing – original draft. Nazanin Chitsaz: Supervision; validation. Maryam Babaahmadi: Methodology; writing – original draft.None.The authors deny any conflict of interest.For clinical cases, the local ethics committee considers that the patient's consent is sufficient.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


Schnitzler’s metastasis occurs due to the deposition of the tumor cells in the submucosa of the rectum, leading to rectal stenosis. We present a 60-year-old female who presented with abdominal pain, distension, and vomiting. Abdominal examination showed a distended abdomen and palpable bowel loops, and per rectal examination showed rectal stenosis. Imaging studies suggest rectal stenosis with carcinoma of the pancreas head. The patient was diagnosed with Schnitzler’s metastasis with carcinoma of the pancreas head, which has not been reported in the literature. The patient underwent a diversion sigmoid colostomy and was planned for palliative chemotherapy after stenting the common bile duct. Schnitzler’s metastasis (SM) is rare in diffuse gastric cancer. They occur due to peritoneal or hematogenous spread ,2. SM isA 60-year-old female patient complained of abdominal pain and distention for 10 days. The patient also had a history of vomiting for three days. The patient’s neighbors noticed she had jaundice for two weeks before visiting the hospital. There was no history of fever, hematochezia, or hematemesis. On examination, she had a pulse rate of 90 beats per minute and a blood pressure of 90/60 mm Hg. There was jaundice and mild pitting pedal edema. There was no lymphadenopathy in the neck, axilla, or inguinal region.Abdominal examination showed a distended abdomen. There was mild generalized tenderness but no guarding or rigidity. Bowel loops were palpable with visible peristalsis. There was no evidence of hepatomegaly or splenomegaly. Per rectal examination showed a severely stenosed rectum, unable to administer a finger across the stenosed rectum. There was no palpable growth within the rectum. Rectum was empty without any stool staining. Routine investigations showed hemoglobin of 8 g/dl and mild leucocytosis. The renal function test (RFT) was normal. The liver function test (LFT) showed increased total bilirubin of 16 mg/dL, direct bilirubin of 12 mg/dL, and alkaline phosphatase (ALP) of 495 IU/L. Tumor markers like carcinoembryonic antigen (CEA) were 2 ng/mL, and carbohydrate antigen 19-9 (CA19-9) was more than 200 U/mL.Contrast-enhanced CT showed a strictured rectum with complete narrowing, without any pelvic or abdominal deposits, liver metastasis, or lung metastasis. There was evidence of a tumor in the pancreatic head with dilated CBD and intrahepatic biliary radical dilatation (IHBRD). There was no evidence of metastatic deposits in the pelvis or the abdomen. There was evidence of dilated small and large bowel loops Figures -1D.The patient underwent an emergency diversion sigmoid colostomy due to a fully developed intestinal obstruction. We did a per rectal examination intraoperatively and noted similar findings. We also did a per stomal examination from the distal loop of the sigmoid. There was no palpable growth in the rectum. The patient improved well postoperatively, and her symptoms subsided.The patient underwent sigmoidoscopy and was found to have severe rectal stenosis, and the scope could not be negotiated beyond. She also underwent magnetic resonance cholangiopancreatography (MRCP). She was found to have an ill-defined hypointense T2 image and isointense T1 lesion measuring 1.5 cm x 2 cm in the pancreas head with mild homogenous enhancement and restricted diffusion. The pancreatic duct and CBD were dilated with abrupt tapering  and fine needle aspiration (FNA) to diagnose the pancreatic head lesion. The stomach was normal, and there was no evidence of malignancy. EUS also showed the presence of a hypoechoic lesion in the pancreas head, suggesting malignancy. FNA from the lesion showed scattered tumor cells with hyperchromatic pleomorphic nuclei with scant cytoplasm Figures , 3B.These cells were positive for cytokeratin (CK) 7 and CK 19, suggesting a pancreatic-origin adenocarcinoma. Considering all these findings, we diagnosed our patient with carcinoma of the pancreatic head with SM. After CBD stenting, supportive care and palliative chemotherapy were planned for the patient.Metastasis to the rectum from gastrointestinal malignancies is uncommon. SM is a type of rectum metastasis that can be synchronous or metachronous. It is characterized by severe rectal stenosis with circumferential diffuse wall thickening and tumor-free rectal mucosa . Only a Most SM patients were females . PatientX-ray abdomen may reveal features of intestinal obstruction in patients who have developed frank features of intestinal obstruction. However, the etiology of intestinal obstruction cannot be commented on. CT abdomen may show concentric stenosis of the rectum . Few patEndoscopic evaluation of the rectum is the most effective way to assess mucosal lesions. In patients who develop SM, endoscopic evaluation may show normal mucosa with complete narrowing of the rectum lumen. Due to the stenosed rectum, it may not be possible to pass the scope beyond the rectal narrowing . Our patTreatment of patients with SM depends on the clinical presentation of the patients. Those who have developed frank features of intestinal obstruction will need surgical management. Most patients will need a proximal colon diversion . There aSM is considered the rarest type of metastasis associated with diffuse gastric carcinoma. SM occurs through the hematogenous or lymphatic spread. Considering that no pre-existing literature describes SM associated with pancreatic malignancy, our case would be the first to describe such a scenario. Tumor cells are usually deposited in the rectum submucosa. Patients may present with symptoms of intestinal obstruction. MRI may be more beneficial for diagnosis. SM treatment can be tailored according to the clinical presentation of the patient.


Cystic hydatidosis is a parasitic infection caused by the organism Echinococcus Granulosus that is well known to be endemic in the Mediterranean region, eastern Europe and South America and usually presents as hydatid disease of the liver but may affect other organs. The disease occurs when humans become the accidental host through ingestion of the eggs from contaminated food.We present a case of Hydatid disease presenting as hives refractory to medical therapy over 4 years duration which was revealed to be caused by para-rectal hydatid cysts. Patient received Albendazole for a duration of 2.5 months and then underwent laparoscopic resection of the para-rectal cysts.Pelvic Hydatidosis is a very rare condition accounting for 0.7 % of all cases reported. In most cases, it is coexistent with cysts present elsewhere in the body, namely the liver, which is the case in the presented patient. Imaging is used as a modality to establish the diagnosis of cystic hydatidosis namely Ultrasound (US), Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI). The incidental finding of the hydatid cysts in this patient demonstrated the efficiency of a CT scan as a tool for detection and subsequently diagnosis of the disease in the pelvis. Surgery is the treatment of choice for cysts with daughter vesicles that are not candidates for percutaneous drainage, large liver hydatid cysts of more than 10 cm in diameter, cysts with a risk of rupture in case of trauma, and extrahepatic disease such as the lung, bone, brain, kidneys or pelvis.This article reports the rare occurrence of para-rectal hydatid disease which is only described in few case reports in the literature and provides an overview on diagnosis, and management of the disease. •Hydatid disease is endemic in the Mediterranean region, eastern Europe and South America usually affecting the liver.•Hives may be a presentation of Hydatid disease.•CT scan detects liver and concomitant para-rectal hydatid cysts.•Indirect Hemaglutination Assay is used as Serologic evaluation of hydatid disease.•Albendazole is a standard treatment prior to laparoscopic resection. It is cThe work has been reported in line with the SCARE criteria .2This is a case of a 46-year-old male, previously healthy, who previously presented 4 years ago with a 3-month duration of hives which responded to antihistamines. However, his symptoms increased in severity which required additional medical management including Montelukast, a Leukotriene Receptor Antagonist, and Omalizumab, an Anti-IgE monoclonal antibody. The patient underwent a CT scan of the abdomen and pelvis 8 months prior to the discovery of his condition for an episode of flank pain to rule out nephrolithiasis. It showed three calcified lesions raising suspicion for Hydatidosis: a 4.9 × 4 cm calcified mass in the pelvis to the left side of the rectum just superior to the left seminal vesicle causing a mass effect on the adjacent pelvic structures, a 1.5 × 1.1 cm lesion to the right side of the rectum , and a 1Serologic and tissue investigations were done to characterize the lesions: Echinococcus Granulosus Indirect Hemaglutination Assay showed a titer of 1:32 and a CT-guided core biopsy of left pelvic mass showed laminated membranes with calcifications highly suggestive of the diagnosis of Hydatid cyst. Stool cultures were taken and showed no ova or parasites.Patient received a course of Albendazole therapy (400 mg twice daily) for 2.5 months and was reevaluated by CT scan of the abdomen and pelvis which re-demonstrated the three unchanged cystic lesions in the pelvis and liver. Patient had no signs of fever or chills denied any episodes of nausea or vomiting however he reports having persistent hives. Patient then underwent Laparoscopic en-bloc resection of the para-rectal hydatid cysts with part of the abutting omentum without cyst rupture . Through3E. granulosus). The medical literature has recognized two species that cause the disease in humans which are: E. granulosus and E. multilocularis. The layer of the formed cyst called the pericyst is composed of fibrous tissues that become compressed and calcified over time. Whereas, the inner layers form a laminated membrane and germinal center, referred to as the endocyst, which hold the larval stage and may contain daughter cysts. The infection can spread to other organs, or to other parts of the same organ, thereby having secondary echinococcosis [Hydatid disease is a zoonotic infection characterized by the formation of cysts that are caused by the accidental ingestion of the egg stage of the tapeworm Echinococcus Granulosus , Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI). US is mostly useful in detecting cystic membranes, septations, hydatid sand, and allowing the classification of the hydatid cyst into its categories. CT is highly efficient in showing calcifications in the cystic wall. It is the imaging modality of choice to detect seeding in the peritoneal cavity and elsewhere in the body. In addition, CT scan permits accurate detection of osseous lesions, however MRI is superior in demonstrating neural involvement, and the local extent of the lesion in soft tissues . The incApproaches to treating hydatid disease are through medical, percutaneous, or surgical techniques. Albendazole can be used in the management of small cysts without septations and a diameter less than 5 cm as initial management with a maximum dose of 400 mg orally twice daily. However, patients with chronic liver disease or bone marrow suppression should not be treated with Albendazole due to hepatotoxic effect of the drug and the reported risk of cytopenia and agranulocytosis. It is an appropriate therapy for patients who cannot undergo percutaneous treatment or surgery yet it yields significantly favorable results when used as adjuvant therapy by decreasing the risk of recurrence by inactivation of protoscolices and increasing the success rate of the surgery by an odds ratio of 48 (95 % CI: 4–586) as found in a meta-analysis by Velasco-Tirado et al. . In termSurgery is the treatment of choice for cysts with daughter vesicles that are not candidates for percutaneous drainage, large liver hydatid cysts of more than 10 cm in diameter, cysts with a risk of rupture in case of trauma, and extrahepatic disease such as the lung, bone, brain, kidneys or pelvis . LaparosIt is known that hydatid disease may involve the pelvis and its various contents: the bladder, the seminal vesicles, the sigmoid colon and the rectum. There are few case reports published which describe retrovesical hydatid disease which presenting with various lower urinary tract symptoms in males , and ova4In conclusion, pelvic hydatidosis is a rare finding and is more commonly found in areas where zoonotic infections are endemic. Pararectal hydatid disease is described in only a few case reports in the literature. Hydatid disease should be considered in the differential diagnosis of a patient with chronic urticaria, and eosinophilia. The imaging modality for diagnosis of Liver Hydatidosis is Ultrasound, but CT can be used for detection of extrahepatic involvement of the disease specially for calcified lesions. Surgery remains the most used and reliable treatment of Echinococcosis, with Adjuvant Albendazole therapy 1 to 3 months prior to surgery and 1 to 3 months after surgery to prevent recurrence. Hydatid cysts that are large, septated, extrahepatic and that have a high risk of rupture should receive surgical management. However, stable and inactive calcified cysts, should be observed, and followed upon by Ultrasound or CT scan depending on their location. Lastly, small cysts having a single compartment and a diameter less than 5 cm can be treated with Albendazole only with monitoring and follow up in clinic.As this publication is a case report that contains no identifiable content to the patient, this publication was exempt from ethical approval by the Human Research Protection Program (HRPP) and its Institutional Review Board (IRB) at the American University of Beirut Medical Center, Beirut, Lebanon.N/AOussama Nasralla, MDDivision of Urology, Department of Surgery, American University of Beirut Medical Center, Beirut, Lebanonn/aWritten 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.Oussama G. Nasrallah: Resources, Writing – original draft. Arwa ElRifai: Resources, Writing – original draft, Supervision. Shafik Sidani: Writing – review & editing. Faek Jamali: Conceptualization, Writing – review & editing, Supervision.N/A


Extraosseous osteosarcoma is a rare malignant tumor, most commonly occurring in the thigh, upper limbs, and retroperitoneum. However, there are only a few reported cases of renal osteosarcoma. Herein, we present the case of a 54-year-old woman with malignant extraosseous osteosarcoma of the left kidney. CT and MR imaging revealed a soft tissue mass originating from the left kidney. Primary renal osteosarcoma is an uncommon extraosseous malignant tumor that arises in the kidneys. While there have been sporadic cases reported in the literature, there’s still limited understanding of the variations in clinical presentations and prognoses. Our report provides a unique case with specific imaging and genetic findings, adding to the existing pool of knowledge. Primary renal osteosarcoma mostly affects adults and has a poor overall prognosis . NonspecA 54-year-old female farmer presented with a 6-month history of abdominal pain, and 4 months of weight loss of approximately 5 kg. She had left abdominal pain with no tenderness, accompanied by a mass for 6 months. Physical examination revealed no tenderness in the abdomen. Abdominal computed tomography (CT)  is a rare malignant subtype of osteosarcoma that accounts for about 1% of all soft tissue sarcomas . The patThe exact etiology of EOS remains unclear. According to Virchow’s theory , the risCommon symptoms of renal osteosarcoma are flank pain and hematuria, with weight loss and a progressively enlarged soft tissue mass in the hypochondrium. Some patients experience an occult onset and pain. In most cases, by the time patients go to a clinic, the mass would have grown quite large . Hence, Historically, EOS in kidneys has been an elusive diagnosis with only 28 cases documented, including this case reported here. The manifestation in our patient, particularly the imaging and genetic findings, aligns yet somewhat differs from previous reports, emphasizing the heterogeneity of this disease. Flank pain and hematuria, as observed in our patient, are the most common complaints in renal osteosarcoma . HoweverIn general, osteosarcoma in the kidney grows aggressively and is extremely fatal, and the contiguous structures of the kidney, such as the spleen, liver, and adrenal gland, can easily be infiltrated. The prognosis of EOS in the kidney or other locations is poor, with approximately 86% of patients with EOS in the kidney having metastases . In our PIK3CA, CTCF, and RASA1). PIK3CA has been reported in colorectal, ovarian, and breast cancers; a study by Ian G  for the publication of this case report.JD, GW, and JZ: manuscript writing. QZ: pathological review. JC, HL and RZ: manuscript revision. All authors contributed to the article and approved the submitted version.


Cardiac masses are currently studied using multimodality imaging. For diagnosis, different imaging techniques that can provide complementary information are used. Cardiac magnetic resonance imaging (MRI) has become a fundamental tool for this type of pathology owing to its ability to provide tissue characterization, spatial accuracy, and the anatomic relationships of the different structures. This study presents a series of four clinical cases with an initial diagnosis of a cardiac mass. All cases were evaluated at a single center, and patients were aged 57 to 72 years. An etiological study was conducted on all patients using different imaging techniques, including MRI. This study describes the diagnostic and therapeutic procedures of the four cases, which included two intracardiac metastases and two benign tumors. Cardiac MRI was decisive in the diagnostic process, determining the clinical decision-making in all four cases. Cardiac MRI has emerged as a pivotal technique in the diagnosis of cardiac masses. It can provide a highly accurate histological diagnosis without the need for invasive techniques. Although cardiac tumors are rare, with an incidence in autopsies ranging from 0.001% to 0.030% [A biopsy is used only in cases of diagnostic doubt that could affect therapeutic management. Histological confirmation in the laboratory, both in biopsies and autopsies, helps to assess agreement with imaging techniques, thereby providing a continuous learning process for the ability to perform a differential diagnosis between cardiac masses.We present here a series of cases with a recent diagnosis of cardiac masses in our department and studied by MRI in our center .A 58-year-old woman with a history of smoking, hypertension, and type 2 diabetes was examined for dyspnea on exertion after months of evolution. A TTE was performed, revealing a large mass in the left atrium. Two- and three-dimensional TEEs a showed MRI, performed for better characterization b, showedFinally, the patient has been followed up for six months after discharge. She remained asymptomatic. Subsequent echocardiographic studies describe the absence of left atrial mass, with preserved left ventricular ejection fraction and absence of valvular heart disease.A 57-year-old woman who was a former smoker, hypertensive, and dyslipidemic with a history of left-sided breast cancer was diagnosed in 2016 and treated with mastectomy and neoadjuvant chemotherapy. Cancer was detected in the contralateral breast during follow-up with bone metastases in the lumbar region. She underwent a right mastectomy and received adjuvant radiotherapy and chemotherapy using a permanent central venous catheter with a reservoir. Subsequent follow-up examinations showed no progression of the metastatic disease.One year after the diagnosis of metastasis, she was admitted for an acute inferior myocardial infarction and underwent revascularization of an acute thrombotic occlusion in the mid-right coronary artery using primary angioplasty and implantation of a drug-eluting stent. Her convalescence was uneventful.During hospitalization, TTE was performed and a 23 × 17 mm echodense mass attached to the free wall of the right atrium was detected incidentally. TEE showed a mass with a broad base of implantation that followed the movement of the atrial wall itself, independently of the central venous catheter, without protruding into the cavity or compromising the flow. These findings indicated a neoplastic origin, with a differential diagnosis of primary or metastatic tumor given the patient’s cancer history. On MRI , the lesThe patient was referred to the Oncology Department for further extension studies. Given the progression of her oncological disease and the incidental diagnosis of the atrial mass, it was decided to not submit the patient to surgical tumor resection at the time but to continue with chemotherapy and follow the evolution of the cardiac mass using imaging techniques. In the six-month follow-up cardiac MRI, the mass persisted at the atrial level but with similar dimensions to those previously described. She continues with chemotherapy treatment with palliative intent.A 72-year-old man with no relevant history was admitted for chest pain and diagnosed with anterior acute myocardial infarction. Emergency coronary angiography with drug-eluting stent implantation in the mid-left anterior descending artery was performed. TTE revealed mild left ventricular dysfunction due to an apical contractile disorder, and a heterogeneous mass was incidentally found in the left atrium without compromising mitral valve function. An initial differential diagnosis of thrombus or primary neoplasia was considered, with myxoma as the main suspicion given the echocardiographic appearance and location of the mass. Anticoagulation was maintained and TEE was performed, revealing a large mass of 40 × 33 mm that was not very mobile and heterogeneous, with areas of cavitation and calcification and a wide pedicle attached to the interatrial septum in the anterosuperior region.MRI  showed aIn a multidisciplinary approach, jointly with the Medical Oncology Department, a full-body CT scan was performed , which sA 62-year-old man who was a smoker, dyslipidemic, and undergoing treatment for bipolar disorder was diagnosed with stage IV prostate cancer 4 months earlier, pending treatment. He was admitted with psychotic symptoms and respiratory failure requiring further study. Given the suspicion of pulmonary thromboembolism, CT angiography was performed, which ruled it out, but a solid lesion was incidentally detected in the right atrium.The study continued with TTE, which did not reveal a lesion. MRI confirmed the presence of a 52 × 42 mm heterogeneous mass in the right atrium, which had slightly hyperintense areas on T2 and T1 images, and heterogeneous and intense enhancement after gadolinium administration, with a hypoenhancing center that was probably necrotic. The mass protruded intraluminally and into the pericardium, with mild pericardial effusion. Given these findings, it was suspected to be a malignant lesion, possibly cardiac metastasis or angiosarcoma. .PET-CT showed a slightly heterogeneous uptake of the radiotracer in the prostate in relation to the primary neoformation and foci with increased uptake in the bone at different levels, attributed to secondary neoformation. In addition, a heterogeneous cardiac mass (61 × 45 mm) with a necrotic area infiltrating the wall of the right atrium and pericardium was found with a maximum standardized uptake value of 9.23, suggestive of malignancy (a biomarker of cell proliferation). Given the psychiatric–social context of the patient and the poor oncological prognosis (primary tumor with bone and cardiac metastases), palliative treatment was indicated. The patient died a few weeks after diagnosis.The differential diagnosis of cardiac masses ranges from anatomical variants considered nonpathological and pseudomasses, such as Chiari networks, Eustachian valves, or lipomatous atrial septal hypertrophy, to true lesions, divided into neoplastic and non-neoplastic lesions. Neoplastic lesions are classified as benign and malignant, with this subgroup including both primary and metastatic lesions. Examples of non-neoplastic lesions are intracavitary thrombi, vegetations, caseous necrosis, or annular calcifications. Fortunately, most lesions are non-neoplastic or pseudomasses, and cardiac tumors are rare. Within the group of tumors, the most frequent are metastatic tumors. Among primary tumors, most are benign, accounting for approximately 90% of all excised tumors . They afThe clinical context of each patient is especially relevant for establishing the likelihood of having one type of cardiac mass over another. However, the clinical presentation is highly variable, but most often occurs asymptomatically and is found incidentally. Occasionally, a cardiac mass can cause dyspnea on exertion, fever, weight loss, systemic embolic events such as stroke or peripheral arterial disease, pulmonary embolism in the case of right heart chamber tumors, arrhythmias, cardiac tamponade, and coronary involvement . In moreRegarding electrocardiography, it has been suggested that certain electrical tracings could be related to cardiac tumors, such as sinus tachycardia, low voltages, extrasystoles, atrial fibrillation, atrial flutter, atrioventricular blocks, axis deviation, bundle branch block, or nonspecific repolarization abnormalities. However, studies have failed to find a specific electrocardiographic pattern. Some authors have associated some cardiac tumors with electrical signals of left and right ventricular hypertrophy . Others TTE is the gold-standard test for the diagnosis of cardiac masses due to its wide availability, safety, and high sensitivity (93–94%) . Howeverp = 0.0001), correctly identifying 100% of thrombi, 67% of non-neoplastic masses, 79% of benign tumors, and 62% of malignant tumors [MRI has been a valuable imaging technique for the study of cardiac tumors since the 1970s . Differet tumors . The hisOne of the most relevant objectives of MRI is to determine, as accurately as possible, whether a cardiac tumor is benign or malignant. This implies drastic changes in the attitude towards these patients as it implies deciding whether to adopt an aggressive or conservative approach. Several descriptive studies conducted in different cohorts have assessed the ability to distinguish benign from malignant tumors using MRI, whereas others focus on distinguishing neoplastic from non-neoplastic masses. The ability to determine whether a cardiac mass is a thrombus or a tumor is especially relevant given the different clinical management of these entities. Anticoagulation is the treatment for most intracardiac thrombi. In contrast, cardiac tumors are evaluated in order to decide between surgical resection or a watch-and-wait approach based on clinical needs and characteristics of benignity or malignancy. Some recent studies of cohorts of patients diagnosed with intracardiac masses have been able to distinguish thrombi from intracardiac tumors using cardiac MRI with an accuracy of approximately 95%. They conclude that thrombi are more frequent in patients with previous coronary revascularization, myocardial scarring visualized by late gadolinium enhancement (LGE) sequences, and lower ejection fractions. In addition, thrombi have been reported to be significantly smaller, homogeneous, and less mobile than tumors . T1-weighted turbo spin-echo sequences are not very useful to distinguish between the two entities. However, tumors are statistically more hyperintense than thrombi when T2-weighted (T2W) sequences are used. This sequence quantifies the amount of water and helps distinguish edematous or cystic tissues from other tissues such as fat. Similarly, late gadolinium contrast uptake, which demonstrates the presence of an extracellular matrix, is associated with tumor processes .Although tumors are highly vascularized tissues, thrombi are typically avascular, except for some chronic thrombi that undergo vascularization processes . Only 9%LGE is useful for studying the embolic origin of patients presenting with cardioembolic stroke and intracardiac mass. It provides a differential diagnosis between thrombus and tumor when an intracardiac mass has been detected by echocardiography but without a definitive diagnosis .Descriptive studies of a series of cardiac masses have found significant differences between malignant and benign tumors in terms of size, location, vascularization, infiltration, and evaluation by different MRI sequences ,21,22,23Malignant tumors are, on average, larger than benign tumors (4.8 vs. 3.2 cm). Similarly, masses found in the left heart chamber are more frequently benign than those in the right heart chambers, where more secondary metastatic tumors are found, or in the pericardium. The main pathophysiological reason for the higher prevalence of secondary metastatic tumors in the right cardiac chambers is the neoplastic spread through the venous return system, similar to right-sided endocarditis in predisposed patients. Differences are also found when analyzing tumors based on hypo-, iso-, and hyperintensity on T2W sequences. Benign tumors are more hyperintense than malignant tumors (60% vs. 36%) and malignant tumors are, in turn, more isointense (32% vs. 8%), with heterogeneous intensity within the same tumor. However, heterogeneity has also been reported in benign tumors, especially myxomas. FPP is more typical in malignant tumors (71%) than in benign tumors (32%) . LGE uptOther characteristics have been shown to be highly suggestive of certain tumors, but because they could not be classified as pathognomonic, they should be used with caution. Examples are malignant hemangiomas and angiosarcomas, indistinguishable from one another as both are highly vascularized lesions with increased uptake . No charAll four cases presented here have the diagnosis of an intracardiac mass in the atrium in common. The information provided by MRI was valuable in all of them for clinical decision-making based on a multimodal approach using different imaging techniques.In case 1, TTE enabled us to identify the origin of the patient’s dyspnea and observe how the intracardiac mass was protruding into the left ventricle and interfering with adequate mitral valve function. Once the etiological diagnosis was established, MRI was able to characterize the intracardiac tumor as benign, and the decision to remove it was finally made due to the hemodynamic repercussions that were causing the patient’s symptoms, but not due to suspicion of malignancy. Knowledge of the tumor’s histology supported the decision to plan coronary surgery and tumor excision during the same surgery. The histological study confirmed the accuracy of the imaging technique.In case 2, routine TTE after acute myocardial infarction revealed the presence of a cardiac mass. Despite initially being an incidental finding, it later had clinical relevance. TTE could not accurately distinguish whether it was a thrombus attached to the catheter or a tumor. TEE provided relevant information as it pointed towards cancer due to the broad base of implantation and followed the movement of the atrial wall itself. Finally, MRI was able to detect characteristics highly suggestive of malignancy such as infiltration of the atrial wall, enhanced uptake after gadolinium administration, heterogeneity, and multiple small calcifications. Although T2 hyperenhancement is more common in benign than in malignant tumors, it is not an exclusive characteristic of the latter. In this case, the image was hyperintense on T2, but the set of characteristics found in the different sequences pointed towards malignancy. This example shows that no characteristic can be used alone to distinguish benign from malignant tumors; therefore, the information should be integrated. This cardiac mass, together with the presence of bone metastases, led to the decision, jointly with the Oncology Department, to continue with chemotherapy and avoid surgical treatment. In case 3, the high likelihood of benignity indicated by this imaging prevented, at first, the subjection of the patient to a high-risk surgical procedure due to the recent infarction. In addition, CT ruled out the presence of satellite images indicative of a primary tumor, further reducing the probability of malignancy. It was decided to postpone surgical excision of the intracardiac mass until after a convalescence period, with initially conservative watch-and-wait management and follow-up. Subsequent studies and, above all, clinical evolution will determine the need for resection of the mass in the future. In the last case, an image suggestive of metastasis in the cardiac region, well characterized by different MRI sequences, provided a poor prognosis for the patient. It was decided, at that time, to start palliative care, avoiding subjecting the patient to more futile treatments. The patient died less than a month after the diagnosis.Despite all the benefits described, MRI use in the study of cardiac masses has certain limitations. These include those specific to the patients, such as carrying metal devices that make it impossible to perform the test, excess abdominal circumference, claustrophobia, inability to collaborate or perform apneas; and those derived from the health system, such as the high costs of its installation, lack of availability or the requirement for highly trained personnel in the interpretation of images, and intrinsic limitations of the technique. There is no feature in any MRI sequence that is capable of determining the diagnosis of a cardiac mass with absolute precision. The combination of characteristics in the different sequences allows us to approach a specific diagnosis, but pathological anatomy provides histological certainty.The currently developed T1 and T2 mapping sequences have been extensively evaluated in the setting of cardiomyopathies; applying these new sequences is helpful in tissue-characterizing cardiac tumors. In these sequences, the intensity of each pixel corresponds to a value of T1 and T2 relaxation times. It brings a proper absolute quantification of diffuse fibrosis (T1) and edema (T2) rather than a simple weighting of the image contrast. Native T1 mapping can offer information without needing LGE, especially in patients with chronic renal failure. Additionally, the extracellular volume can be measured with gadolinium injection.A recent observational study describes the utility of these techniques in the differential diagnosis of cardiac thrombi and masses . The ThrCalcifications with short T1 and short T2.Melanomas or lipomas with short T1 and long T2.Rhabdomyoma Short or similar T1 and long T2.Mixomas and fibroelastoma with long T1 and long T2.Standard values of native T1 and T2 relaxation times remain to be described. There is a crucial inter-observer variability between institutions and different T1 mapping techniques. Larger and multicenter trials will be needed to establish a homogenous clinical application for diagnosing and guiding the treatment of cardiac tumors.MRI has emerged as the most valuable noninvasive technique as it provides more clinical information when a cardiac mass is found. Despite certain technical limitations and the fact that no characteristic can alone provide a reliable diagnosis, the likelihood of a correct diagnosis is increasing every day . In allIntegrating clinical and epidemiological information with advanced imaging techniques helps to further improve accuracy without using unnecessary invasive techniques. Moreover, the ongoing development of new sequences, improvements in the quality of the equipment, and the training of experts in cardiac MRI allow us to look to the future with even more optimism.Despite all the benefits of this technique, its advantages should be considered with caution, always being aware of its limitations and that diagnostic certainty is obtained only by anatomical pathology.


Tumors are rare in neonatal age. Congenital mesoblastic nephroma (CMN) is a usually benign renal tumor observed at birth, or in the first months of life. It may also be identified prenatally and associated with polyhydramnios leading to preterm delivery. Effective treatment is surgical in most cases, consisting in total nephrectomy. In literature, very few studies report on the neonatal management of such a rare disease, and even less are those describing its uncommon complications.+ 1 weeks of gestation (WG) due to premature labor, with prenatal (25 WG) identification of an intra-abdominal fetal mass associated with polyhydramnios. Once obtained the clinical stability, weight gain, instrumental  and histological/molecular characterization of the lesion , a left nephrectomy was performed at 5 weeks of chronological age. The following clinical course was complicated by intestinal obstruction due to bowel adherences formation, then by an enterocutaneous fistula, requiring multiple surgical approaches including transitory ileo- and colostomy, before the conclusive anastomoses intervention. The second patient is a 17-day-old male term baby, coming to our observation due to postnatal evidence of palpable left abdominal mass , feeding difficulties and poor weight gain. An intravenous diuretic treatment was needed due to the developed hypertension and hypercalcemia, which regressed after the nephrectomy  performed at day 26. In neither case was chemotherapy added. Both patients have been included in multidisciplinary follow-up, they presently show regular growth and neuromotor development, normal renal function and no local/systemic recurrences or other gastrointestinal/urinary disorders.We report on two single-center newborns affected with CMN. The first patient is a preterm female baby, born at 30The finding of a fetal abdominal mass should prompt suspicion of CMN, especially if it is associated with polyhydramnios; it should also alert obstetricians and neonatologists to the risk of preterm delivery. Although being a usually benign condition, CMN may be associated with neonatal systemic-metabolic or postoperative complications. High-level surgical expertise, careful neonatological intensive care and histopathological/cytogenetic-molecular definition are the cornerstones for the optimal management of patients. This should also include an individualized follow-up, oriented to the early detection of any possible recurrences or associated anomalies and to a better quality of life of children and their families. Kidney tumors are rare in developmental age, accounting for 6–7% of all children’s neoplasms. Nephroblastoma or Wilms’ tumor (WT) is the most commonly observed throughout the whole pediatric age group, representing the 90% of all cases , while cHerein, we report on two newborns affected with CMN, both observed at the Mother and Child Department of the University Hospital of Palermo. Our study underlines the diagnostic critical issues faced to promptly carry out second level imaging investigations , as well as invasive exams (needle biopsy) before surgery. It also highlights the complex treatment of such a rare disease: both patients underwent surgery at variable times after birth, based on their different gestational age, weight and overall clinical condition, they showed systemic  or abdominal postoperative complications which required additional medical and surgical interventions.+ 1 weeks of gestation (WG), due to premature labor. An intra-abdominal fetal mass with probable origin from the left kidney, along with polyhydramnios, was detected by prenatal ultrasound (US) investigations at 25 WG; the following evaluation at 27 WG showed an increased size and echogenicity of the mass within the left kidney, leading to steroid prophylaxis  in the mother two weeks later, due to the increased risk of preterm delivery. At birth, anthropometric measures were as follows: weight 1,450 g , length 41 cm , occipitofrontal circumference (OFC) 28.3 cm . Postnatally, the newborn manifested mild respiratory distress, which required non-invasive ventilatory support for the first 72 h of life. Chest X-ray examination did not show either elevation of the diaphragm/thoracic compression or signs of pulmonary hypoplasia. Meanwhile, for the first three days, a total parenteral nutrition was given, after which enteral feeding was begun with good tolerance and spontaneous stool emission. Physical examination showed a palpable mass in the left side of the abdomen and no other abnormalities (no hemihypertrophy or other dysmorphic features), which did not lead, then, to perform any genetic investigations  [ETV6-NTRK3 genes translocation was found. Aged 5 weeks (35 weeks of corrected age) the patient underwent left nephrectomy  , 14. Abdndromes) , which w. Chest CT excluded secondary pleuropulmonary lesions , length  and OFC  [CMN is the most frequently diagnosed renal tumor in the first 5 months of life, and it may also be evidenced prenatally . HoweverUrinary catecholamine assay and serum NSE resulted normal in both our patients, they are essential since neuroblastoma represents the most frequent extracerebral solid tumor in newborns and children. Therefore, it must be first included in the differential diagnosis of abdominal masses. Other oncological diseases, from which CMN should be distinguished in the neonatal period, are different kidney tumors (mainly Wilms) and germ cell ones (GCTs) to which teratomas, yolk sac tumors and choriocarcinomas belong. Indeed, other biochemical markers including serum AFP and beta-HCG should be evaluated ; in bothETV6) and Neurotrophic Receptor Tyrosine Kinase 3 (NTRK3) genes; the oncogene resulting from this translocation, encodes for an oncogenic chimeric protein that would be responsible for tumorigenesis, through dysregulation of NTKR3 signal transduction pathways and for chemosensitivity of cancer cells [ETV6-NTRK3 gene fusion is also a key marker which allows to distinguish CMN from other renal tumors, as well as the cellular subtype  frohis form . This rehis form . Also, aIn patient 2, hypercalcemia and arterial hypertension were observed. In literature, both complications are reported associated with CMN, due to secretion of prostaglandins, parathyroid hormone, parathyroid-related and glucagon-like peptides, as well as renin released by tumor cells. Both conditions required medical therapy with furosemide and were promptly resolved after tumor removal, highlighting that their pathogenesis is related with factors secreted by tumor cells (paraneoplastic syndromes) , 29, 34.The present study highlights that, although CMN is usually a benign condition, it may be associated with complications in the neonatal period; they may arise post-surgically or be systemic-metabolic and require different approaches, also in relation with the potential co-occurrence of prematurity/low birth weight and of their related morbidities. Thus, an early  multidisciplinary management, as for other congenital diseases –39, is e


Penetrating trauma to the brain is a rare mode of self-harm in individuals with depressive psychosis. It may have variable presentations ranging from intact neurological status to non-survivable damage and the subjects may be surprisingly apathetic to pain. It is even unusual for such an injury to have an excellent prognosis despite coming late to clinical attention.We report two cases of patients with psychotic depression who attempted suicide by hammering nails into their heads. On imaging, deep penetration within the brain parenchyma was noted; however, neither case had any neurological deficit or symptoms attributable to brain trauma.Self-inflicted penetrating brain injuries with peculiar objects such as nails are rarely encountered in practice. They need prompt management for their removal and addressing the underlying mental health illnesses. Penetrating brain injury is defined as a wound in which an object enters the cranium without making an exit . Such inThe burden of deliberate self-harm and suicide in South Asia, including Pakistan, remains largely unknown and under-reported . Self-inWe report two such cases that we encountered in which the patients presented after driving nails into their heads while attempting suicide.A 26-year-old male was brought to the emergency department by his father when he incidentally noticed nails projecting from his son’s head. The patient had hammered four nails into his head, each measuring 10 cm, with a brick under the influence of alcohol two days before presentation. He continued to function normally while covering them with a cap at home. He also had a prolonged history of untreated depression and alcohol abuse with three attempted suicides in the past.On arrival, he was vitally stable and without any gross neurological deficits. However, he was completely apathetic toward pain. Inspection of the scalp revealed 3 nails over the right mid-parietal region and 1 nail at the left parieto-occipital region. There were several other healed puncture wounds of which the patient had no memory see Fig. .Fig. 1CaEmergency removal of the nails was carried out the same day. A C-shaped incision was given at the right frontoparietal region and the flap was carefully raised around the three right-sided nails. The nails, thus loosened, were then retrieved. A similar craniectomy was performed through a curvilinear incision posteriorly on the left side, and the left nail was removed see Fig. . There wA 24-year-old incarcerated male was brought to the emergency room by the police four hours after he had hammered a 10-cm long nail in the middle of his head by repeatedly striking his head against the cell’s wall. He had a history of a similar attempt 1 month back, which resulted in a deep scalp laceration.On examination, he was fully conscious with intact neurological function. A nail was impacted perpendicularly over the frontal region in the midline. There were no derangements in his laboratory parameters. A non-contrast CT scan revealed a hyperdense linear object at the vertex involving the frontal bone in the midline and extending deep into the superior sagittal sinus and brain parenchyma. On the brain window, an interhemispheric bleed and a left frontal contusion were also appreciated (refer to Fig. Emergency craniectomy and nail removal was subsequently attempted. Bi-coronal flaps were raised, craniectomy was done around the nail, and the foreign body was extracted. Postoperative recovery was unremarkable. The psychiatry team was taken on board and a diagnosis of acute psychotic depression was made. The patient was discharged back to central jail on the 6th postoperative day without any postoperative complications After evaluation, the patient was commenced on anti-depressant and anti-epileptic therapy and scheduled for a follow-up after two weeks but was subsequently lost to follow-up.Penetrating brain injury is very rarely self-imposed and may carry a grave prognosis. Various tools for self-harm have been described in the literature and they range from the commonly used knives to the most bizarre objects such as pencils, chopsticks, nails, metal rods, and screwdrivers –9. In ouAn underlying psychiatric condition has been a frequent accompaniment of such cases of deliberate self-harm. Schizophrenia, bipolar disorder, major depressive disorder, and acute drug-induced psychosis have all been implicated. In one case, a young female attempted suicide by shoving a metal rod in her head after experiencing auditory hallucinations . AnotherIt is very atypical to encounter repeated self-imposed trauma to the head with the same tool, as occurred in our first case. A similar event was narrated by James et al., in which a 44-year-old male had driven 10 nails of various sizes in his head over a span of 3 months. His neurological functions were preserved until the last nail which led to left-sided weakness and gait unsteadiness [Another case by Salar et al. reported a 36-year-old male who was found semi-conscious with 11 nails protruding from his head. While they were successfully removed without any focal neurological deficits, follow-up at 1 year showed impairment of memory, and a diagnosis of frontal lobe syndrome was made . AlthougDepending on the depth of penetration and nearby structures, the foreign bodies can be extracted either under local anesthesia or via craniectomy under general anesthesia. We opted for the latter given the deep-seated location of the nails in both of our patients. In cases where multiple such foreign bodies are found projecting in various directions, it may be wiser to perform separate mini craniotomies instead of a single craniotomy to manipulate the nails. This strategy may minimize the damage caused by nail mobilization and manipulation .Self-inflicted nail injury to the head is rare and may be encountered in patients with depressive psychosis. Despite the gruesome presentation, they may be surprisingly asymptomatic and pain-free. However, they require urgent neurosurgical intervention and prompt psychiatric help, for optimum outcomes, and prevention of future occurrences.


Hemangioma is congenital or traumatic in origin, and it is caused due to atypical build-up of the blood vessel. It is a painless benign condition with typical characteristic clinical features. These generally occur in the first three decades of life with no gender predispositions. A plain soft tissue radiograph can demonstrate phleboliths and aid in diagnosing an intramuscular hemangioma. The present report is a rare deep-seated facial hemangioma involving various facial muscles with multiple phleboliths; characteristics clinical and radiological features. A 22-year-old male patient reported a complaint of swelling on the right side of the jaw. Conventional radiography showed the right cheek’s soft tissue and multiple round, target-like radiopacities of variable sizes. The word "hemangioma" originates from Greek literature where haema is "blood"; angeio means "vessel," and oma is "tumour." The hemangioma can be congenital or traumatic in origin and is an atypical build-up of blood vessels in the skin or internal organs. It is also known as vascular nevus containing unorganized blood vessels connected to the central vein. Intramuscular hemangioma (IMH) has about 1% of cases and is typically seen in the trunk or limb's skeletal muscles . Only 8%Initial diagnosis is commonly considered a parotid tumour due to the location, whereas hemangiomas are barely suspected . The patA 22-year-old male patient reported swelling without pain on the right side of his face since childhood as the chief complaint. On further interrogation, the patient said that the swelling had been growing gradually over the years and has now become of the present size, which was approximately fist-sized. The patient did not recall any history of trauma. The extraoral examination demonstrated a swelling on the face's right side, extending supero-inferiorly from the right eye's outer canthus to the mandible's lower border and anteroposteriorly from the right corner of the mouth to the pre-tragus region Figure .The painless swelling was fluctuant, compressible, and soft in consistency on palpation. There was a slight rise in temperature over the surface of the swelling. On temporomandibular joint examination, the deviation was present towards the left side while closing the jaw without any pain, crepitus, or clicking sound. Intra-oral examination revealed bluish-red discolouration in the posterior part of the right side buccal mucosa extending up to the right side soft palatal region Figure .Maxillo-mandibular bony morphology was within the standard limit in the vicinity of the swelling. On bimanual palpation, the lesion was soft and compressible, non-tender, and had a diffuse periphery with evident pulsation. While palpating, the lesion was exaggerated, and an elevated red-purplish lesion appeared in the posterior part of the mucosa Figure .All the teeth were in good condition. Only extrinsic stains and calculus were there. Panoramic and submentovertex radiographs showed the presence of multiple concentric calcifications in the soft tissue shadow of the lesion suggestive of phleboliths Figures , 5.Assuring the benign nature of the disease and possible definite treatment, the patient was referred to the department of interventional radiology for management.Scott reported a series of 393 IMHs in 1957; 12% were reported in the head and neck region . The masThe Wattle sign that the size of the tumour increases by lowering the position of the head helps in diagnosis was positive in this case. Precautions should be taken while examining the facial region as new vascularised tissue is more torturous and fragile as compared to the native vessels, and this may lead to submucosal bleeding and may lead to subepithelial hematoma formation . We expeThe vascularity of the mass can be confirmed by EchoDoppler, but cannot accurately decide contours and associations with surrounding tissue, which can be done with contrast-enhanced computed tomography and exclusively on magnetic resonance imaging (MRI): these investigations have become essential before the treatment . Biopsy Hemangioma of the face, in this case, was diagnosed based on the typical clinical presentation and using conventional radiography. The presence of small hard nodules that are compressible and diffuse should alert a clinician to the possible presence of an intramuscular hemangioma. X-ray images can show phleboliths and help diagnose them, whereas to identify the exact location of the calcification, non-ionizing techniques such as MRI and ultrasound can be helpful. The management is usually conservative, and surgery is the last treatment of choice.