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Introduction and Importance: In Morocco, diagnosing Gamma Sarcoglycanopathies mainly relies on histopathological analysis of muscle biopsies due to limited genetic and molecular research access. This study highlights the significance of muscle biopsies and explores potential predictive factors and possible correlation between histopathological abnormalities and clinical phenotypes., Case Presentation: Muscle biopsies of six patients diagnosed with γ-sarcoglycanopathy were collected over two years. Pathological analysis was initially performed on slides stained with Hematoxylin-Eosin and Gomori Trichrome. Additionally, cryosections marked for dystrophin, alpha, beta, and gamma sarcoglycans were reviewed. In the second phase of the analysis, formalin-fixed sections from each biopsy were immunostained for various markers: "anti-CD68" for macrophagic cells, "anti-CD56" for satellite cells, and "anti-CD31" for vascular capillary. These stained sections were then carefully examined., Clinical Discussion: The clinical presentation of the disease was uniform and consistent with Duchenne-like dystrophy. However, the histological abnormalities were heterogeneous and did not correlate with the severity of the clinical phenotype. The Loss of expression of a Sarcoglycan and earlier age of onset appear to be the most significant predictive markers of disease progression. Immuno-staining patterns for CD68, CD56, and CD31 indicated an impairment in the muscle regeneration process, probably, at an early stage of the disease., Conclusion: This study's findings are crucial for understanding pathogenesis and identifying new therapeutic targets. However, because of the small sample size, further confirmation through a larger cohort is necessary., Competing Interests: Declaration of competing interest There are no conflicts of interest for authors in this study., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Competing Interests: None declared.

This paper details a rare case of primary hyperoxaluria type 1 (PH1) identified through a bone marrow biopsy in a 46-year-old female patient with a history of nephrolithiasis and chronic renal failure. Genetic analysis identified the p.Ile244Thr mutation in the AGXT gene, confirming the diagnosis of PH1. The paper aims to highlight this case, focusing on the genetic basis of the disorder, including the identified mutation. It underscores the importance of early diagnosis of infantile and childhood nephrolithiasis, particularly in cases with familial history, to prevent renal loss and systemic oxalosis., Competing Interests: None declared., (© The Author(s) 2024. Published by Oxford University Press.)

Towards the beginning of the twentieth century, inflammatory myofibroblastic tumors were described as neoplasms characterized by myofibroblastic spindle cells with an infiltrate of inflammatory cells. These rare tumors occur mainly in children and young adults with a preferential pulmonary and abdominal location. They have intermediate biological potency with a tendency to local recurrence and rarely metastasize. We describe the case of an adolescent who presented with an inflammatory myofibroblastic tumor of the thigh, a very rare entity that has only been reported in the literature as case reports, and its clinical, radiological, echographic and MRI appearance. The patient underwent complete surgical resection of the mass with healthy margins and did not receive any additional treatment. No sign of recurrence was detected after 6 months of follow-up., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Competing Interests: The authors declare that they have no competing interests.

Introduction: Adult Head and neck Rhabdomyosarcomas (HNRMS) are exceedingly rare and remain challenging for pathologists., Cases Presentation: Five cases of adult HNRMS (≥19 years) were retrieved from the archives of the Department of Pathology of Hospital of Specialities in Rabat (HSR) in Morocco, over 5 years. Clinical and pathologic findings from hematoxylin and eosin slides and immunohistochemistry for Desmin and Myogenin were reviewed., Clinical Discussion: The median age was 33, with a men's predominance (3 M/2F). Histological analysis revealed three cases of Alveolar Rhabdomyosarcoma (RMS), one Pleomorphic RMS, and one spindle cell/sclerosing RMS. In addition to the typical histology observed in each RMS, we found tricky growth patterns that could be a source of misdiagnosis. All five cases demonstrated variable positivity for Desmin and Myogenin., Conclusion: HNRMS cases have different pathological features than pediatric RMS cases. We identified rare subtypes such as pleomorphic and spindle cell/sclerotic RMS, which exhibit unusual morphological patterns., Competing Interests: Conflict of interest statement There are no conflicts of interest for authors in this study., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Pancreatic primary squamous cell carcinoma (PPSCC) is very uncommon. The major diagnostic method is histology, and it requires the exclusion of a metastasis from a different primary location (lung, esophagus…). Herein, we describe two cases of a PPSCC (one in the head and the other one in the tail and the body of the pancreas) with a brief review of literature. When it comes to the poorly differentiated PPSCC, immunohistochemistry (IHC) is crucial. Regretfully, there is currently no unanimity on treatment, and the outcome is dismal., Competing Interests: The authors declare that they have no competing interests., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Paratesticular mesothelioma is a very rare tumour, accounting for 0.3 to 1.4% of all mesotheliomas. Mesothelioma arising from the spermatic cord is extremely rare with only a few cases reported in the literature. We report a case of spermatic cord mesothelioma in a 70-year-old man who presented with a right inguinal mass and pain., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Chondroblastic osteosarcoma of the nasal cavity is a rare and aggressive tumor that presents diagnostic challenges due to its variable clinical and radiological features. Histological analysis is crucial for an accurate diagnosis, despite morphological similarities with chondrosarcoma. We present a case of primary craniofacial chondroblastic osteosarcoma originating from the nasal cavity in a 17-year-old adolescent. The tumor was characterized by cartilaginous lobules and spindle cell proliferation with osteoid matrix deposition. Immunohistochemical analysis supported the diagnosis. This case highlights the rarity of this particular presentation and emphasizes the significance of accurate diagnosis through histopathological evaluation to achieve optimal management., Competing Interests: None declared., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Primary angiosarcoma of the oral cavity is a rare malignant vascular neoplasm variably recapitulating endothelial cells and is generally associated with a worse prognosis. The epithelioid subtype is even uncommon in this localization. To our knowledge, only seven cases of primary oral epithelioid angiosarcoma have been reported until 2021. This histopathological variant is characterized by solid and sheet-like growth patterns that may be misinterpreted as other lesions with epithelioid cells. Herein, we present a new case of primary epithelioid angiosarcoma of the mandibular gingiva to discuss histopathological differential diagnoses and potential diagnostic pitfalls., Competing Interests: The authors declare that they have no competing interests., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Abdominal tuberculosis (TB) remains a significant health concern globally, particularly in regions with high endemicity such as North Africa and Morocco. Despite advances in diagnostic modalities, the nonspecific presentation of abdominal TB poses challenges for timely diagnosis and management. Here, we report a case of abdominal TB in a critically state of a young man from Morocco, presenting with acute abdominal pain and signs of sepsis. Radiological investigations revealed features suggestive of intestinal perforation complicating peritoneal TB. Urgent laparotomy confirmed the diagnosis, yet the patient succumbed to advanced sepsis postoperatively. This case underscores the complexity of abdominal TB diagnosis and management, necessitating a high index of suspicion and multidisciplinary collaboration. With evolving surgical techniques and ongoing research efforts, optimizing strategies for early detection and treatment of abdominal TB remains imperative, particularly in endemic regions., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Uncommon in nature, retroperitoneal ganglioneuroma represents a neuroblastic benign tumor, predominantly manifesting in young adults, with a notable predilection for females. Often asymptomatic, the condition is frequently diagnosed incidentally due to delayed growth. Clinical manifestations arise primarily from the compression exerted on neighboring organs and vessels. The exclusive curative recourse lies in surgical intervention, underscoring the challenging task of achieving complete tumor excision, particularly when the ganglioneuroma attains considerable development and encapsulates significant retroperitoneal vessels. In this instance, we elucidate a case involving a 33-year-old woman, who had previously undergone a triple valve replacement due to rheumatic valvular disease, presenting persistent pelvic pain, unearthing a substantial asymptomatic retroperitoneal ganglioneuroma concomitant with an ovarian endometrioma. A laparotomy procedure was conducted, and to achieve a comprehensive excision of the mass, a meticulous intratumoral circular dissection of the prominent vessels, notably the superior mesenteric artery and celiac trunk, was undertaken. No local recurrence has been reported, 6 months after surgery. The significance of an experienced and well-trained surgical staff is underscored in addressing the complexities associated with this condition., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Pseudomyogenic hemangioendothelioma (PMHE) is a rare vascular neoplasm with an intermediate to low-grade malignant potential. Only 5% of PMHEs occur in the head and neck. This tumor exhibits different histological patterns and mimics other vascular tumors, myoid tumors, or carcinomas. The distinction between these tumors can be a very challenging situation for pathologists. In this article, we report the first case, to our knowledge, of PMHE arising in the maxillary sinus, to highlight this uncommon entity and discuss differential diagnoses., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Eosinophilic gastroenteritis is a rare disease with an unknown cause, which can manifest independently or as part of a hyper-eosinophilic syndrome. The severity of the condition depends on the extent of eosinophilic infiltration and damage to the digestive tract. Diagnosis relies on histological examination, which reveals a significant presence of eosinophilic polymorphonuclear leukocytes in the digestive wall. The authors present a new case of eosinophilic gastroenteritis in a 28-year-old patient who exhibited obstructive symptoms but lacked peripheral eosinophilia. Esophagogastroduodenoscopy showed no abnormalities, but barium transit imaging revealed gastro-duodeno-jejunal dilation upstream of a tight jejunal stenosis. Surgical examination of the affected area confirmed a diffuse and transparietal eosinophilic infiltrate, with no evidence of parasitic or granulomatous lesions. Fortunately, the patient had a swift recovery following surgery. Biopsies conducted at other locations, including the gastric, hepatic, and medullary levels, produced negative results, indicating the localized nature of the condition., Competing Interests: None declared., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Introduction and Importance: Inflammatory pseudotumor (IPT) of the spleen is a rare entity that can be difficult distinguishing it from malignancies, both in clinical presentation and radiological imaging., Case Presentation: We present the case of a 43-year-old female presented with 15-cm left hypochondrial mass, Initial imaging studies raised concerns of malignancy, leading to a splenectomy. However, the final pathological examination determined that the patient had IPT of the spleen with focal expression of Smooth Muscle Antibody (SMA)., Clinical Discussion: This case highlights the importance of considering IPT as a potential diagnosis of splenic masses that was difficult to diagnose before surgery., Conclusion: The uniqueness of the case under consideration lies in the rarity and the atypical localization of IPT of the spleen., Competing Interests: Declaration of competing interest N/A, (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Wünderlich's syndrome, a rare and life-threatening condition involving spontaneous renal hemorrhage, is commonly associated with renal angiomyolipoma (rAML). Management options for rAML include surveillance, mTOR inhibitors, selective arterial embolization (SAE), and surgery. When complicated by Wünderlich's syndrome, prompt and adequate interventional treatment is necessary to prevent fatal outcomes. We present a unique case of a young female patient with Tuberous Sclerosis Complex who experienced a massive ruptured rAML, leading to Wünderlich's syndrome. Our approach involved a multidisciplinary hybrid strategy, combining SAE and nephrectomy, highlighting SAE's valuable role even when radical treatment is required., Competing Interests: Declaration of Competing Interest The authors have no conflict of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction and Importance: Syringocystadenoma papiliferum (SCAP) is an infrequent, benign neoplasm originating from the apocrine or, less frequently, eccrine sweat glands. This condition predominantly manifests in regions such as the head, face, neck, and trunk. Notably, it is frequently associated with hamartomas of endocrine, sebaceous, or follicular origin, as well as with sebaceous nevi., Case Presentation: In the context of this study, we present a clinical case involving a 65-year-old patient who exhibited an atypical anatomical presentation of syringocystadenoma papiliferum., Clinical Discussion: This case highlights the importance of considering SCAP as a potential diagnosis of perineal cystic masses that need a surgical excision due to the risk of malignancy transformation., Conclusion: The uniqueness of the case under consideration lies in the rarity of syringocystadenoma papiliferum (SCAP), the atypical perineal localization, and its occurrence at an advanced age (65 years)., Competing Interests: Conflict of interest statement N/A., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Juvenile xanthogranuloma (JXG) is a rare, benign non-Langerhans cell histiocytosis that primarily affects the skin, with infrequent extracutaneous manifestations. Lesions typically emerge during early childhood and often resolve spontaneously, obviating the need for treatment. This paper details the case of a child diagnosed with a solitary JXG on the sole, necessitating surgical excision due to its functional impairment, specifically a delay in walking and weight bearing.

Adenoid cystic carcinoma is a rare tumor that typically originates from secretory glands, most commonly found in the salivary glands. However, it can also develop as a primary cutaneous adenoid cystic carcinoma, which appears identical under the microscope to adenoid cystic carcinoma originating in other tissues. Distinguishing between primary cutaneous adenoid cystic carcinoma and extracutaneous adenoid cystic carcinoma with cutaneous metastases is crucial for determining the prognosis and appropriate management of the condition. In this case report, we describe a case of primary cutaneous adenoid cystic carcinoma located on the hand with lung metastases. Proper differentiation, treatment planning and regular clinical follow-up to monitor for any signs of recurrence or metastasis are essential to ensure favorable outcomes for patients with this rare neoplasm., (© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Hydatid disease is still endemic in several regions worldwide including Morocco, and is caused in most cases by the larval form of 2 species of the tapeworm Echinococcus: E. granulosus and E. multilocularis. Primary hydatid disease of the bone without systemic involvement is rare. The disease has a silent clinical evolution until it reaches complicated stages. Complications may include pathological fracture, neural deficit, infection, and fistulization of the abscess. Preoperative diagnosis is based on clinical history, imaging findings, and serological tests, which lack high sensitivity and specificity. Although the interpretation of imaging studies can prove to be very confusing because the bone changes evolve with time, and the nonspecificity of these findings often leads to a mistaken diagnosis. The diagnosis requires a high index of suspicion, especially in patients who reside in or travel to sheep-raising areas where hydatid disease is endemic. A high index of suspicion is necessary for the diagnosis, especially in patients that live in or travel to sheep-raising areas where hydatid disease is endemic. The treatment of choice remains surgical, following the principles of a locally malignant lesion. Chemotherapy (albendazole alone or in combination with praziquantel) is indicated when surgery is not possible or as an adjuvant treatment. The prognosis is often poor. We report the case of a 28-year-old woman with long-standing pain in the left hip joint in which the imaging findings were thought of as being either tuberculous or neoplastic. The result of a CT-guided biopsy concurred with an unexpected diagnosis of a hydatid cyst. This case highlights that in the absence of a high index of suspicion for echinococcal infection, the semblance of imaging findings of hydatid disease in the bone to those of other skeletal pathologies can lead to misinterpretation., (© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Primary leptomeningeal melanoma is an extremely rare type of intracranial melanoma. It cannot be reliably distinguished from metastatic melanoma on neuroimaging and histopathological characteristics alone; its diagnosis is established only after exclusion of secondary metastatic disease from a cutaneous, mucosal or retinal primary. Prognosis is poor, partly due to its high rate of misdiagnosis. Herein, we report a case of a primary meningeal melanoma of the skull base with melanomatosis, in a 31-year-old man, mimicking meningioma. Our aim is to highlight the diagnostic pitfalls and to discuss the histopathological differential diagnoses, especially with other pigmented lesions of central nervous system., Competing Interests: None declared., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2023.)

Infantile myofibromatosis (IM) is the most common fibrous disorder of infancy and early childhood. Solitary intracranial involvement is rare and often unrecognized. This makes its early diagnosis and adequate management difficult. The majority of lesions are localized to the skull or dura with variable intracranial extension. Herein, we report a misdiagnosed and aggressive presentation of a solitary IM of the petrous bone. Our aim is to discuss histopathological differential diagnoses and management difficulties., Competing Interests: The authors declare that they have no competing interests., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2023.)

Rosai-Dorfman disease (RDD) is a clonal histiocytic proliferation characterized by large S100 positive histiocytes with variable emperipolesis. Extranodal locations were confirmed with the central nervous system or the meninges involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas in radiological and intra-operative pathological examination. Histopathology and immunohistochemistry are the keys to definitive diagnosis. We present a case of bifocal Rosai-Dorfman disease in a 26-year-old man, mimicking Lymphoplasmacyte-rich Meningioma. This case allows us to demonstrate the diagnosis pitfalls encountered in this localization., Competing Interests: THE author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2023.)

Introduction and Importance: Malign Peripheral Nerve Sheath Tumor is rare and can occur on a solitary neurofibroma or as part of neurofibromatosis type 1. The occurrence of a giant solitary neurofibroma in the thigh is rare and its complication in MPNST is much rarer. The diagnosis is based on a combination of radiological, histological and immunohistochemical evidence., Case Presentation: We present the case of a 55 years old woman who presented a mass of the posterior aspect of the thigh without any other abnormality on systemic examination. MRI showed a T1 hyposignal and a T2 hypersignal and the biopsy performed was in favor of a neurofibroma. After surgical resection, the histological study of the surgical specimen revealed an MPNST developing on a pre-existing neurofibroma. After 5 months, the patient had moderate knee stiffness with thigh amyotrophy., Clinical Discussion: The occurrence of MPNST on a giant solitary neurofibroma of the thigh is extremely rare. MRI allows evoking the diagnosis but radiologists are confronted with a problem of differential diagnosis. Histological examination supports the diagnosis in addition to immunohistochemical examination. Only surgical treatment can give hope for a cure. Recurrences remain frequent with a 5-year survival of 50 %., Conclusion: Early diagnosis is essential to prevent the evolution of the tumor and the occurrence of poor prognostic factors compromising the management, increasing the risk of recurrence and affecting the overall survival of patients., Competing Interests: Declaration of competing interest The authors declare that there is no conflict of interest., (Copyright © 2023 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Estrogen receptor α (ERα) plays a critical role in breast cancer (BC) development. The standard therapeutic strategies for ERα- positive (ERα+) BC consist of impairing ERα signalling pathway by either estrogen competitors blocking its interaction with the ligand binding domain (LBD) or agents inhibiting the production of estrogen. These strategies are limited by many factors that lead to constitutive activation of ERα and consequently, resistance to treatment. Targeting the DNA binding domain (DBD) of ERα instead of its LBD with small-molecule inhibitors could be an alternative to impair ERα's signalling pathway. For this purpose, we conducted a structure based virtual screening of DrugBank against the crystal structure of ERα-DBD (PDB ID: 1HCQ) using the Glide module in standard precision (SP) and extra precision (XP) mode of docking. Molecules with XP Gscore less than -8 kcal/mol were selected and visually inspected to keep only the reasonable docking poses. Subsequently, these molecules were clustered using structural interaction fingerprints analysis and the complexes of the top ranked molecules of each cluster based on XP Gscore were subjected to 200 ns molecular dynamics simulations followed by MM-GBSA binding free energy calculation for the last 100 ns of each complex. In this study, we identified three molecules from DrugBank namely DB03450, DB02593 and DB08001 showing significant stability and strong interaction with the key amino acids during MD simulation suggesting a potential inhibition of the target. These molecules could be used as promising lead compounds to impair the ERα signalisation in hormone therapy-resistant breast cancer.Communicated by Ramaswamy H. Sarma.

Hepatoid adenocarcinoma is a rare type of extrahepatic adenocarcinoma with glandular and hepatocyte differenciation. The tumor can occur in many organs, generally in the stomach, the location of the lung being extremely rare. Despite poor prognosis and few effective treatment options, a timely and accurate histopathological diagnosis is key to optimal clinical management for long-term survival. Given the few reports published to characterized hepatoid adenocarcinoma, the emergence of any new case will contribute to improve understanding of the disease. Very few reports have been published to characterize hepatoid adenocarcinoma cytologically or even histologically. The aim of this work is to focus on the anatomoclinical characteristics of this rare entity. We present a retrospective study of 2 cases of pulmonary hepatoid adenocarcinoma confirmed by histological and immunohistochemical study., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2022.)

Malignant peripheral nerve sheath tumors are defined as malignant tumors arising from or differentiating toward the cells of the peripheral nerve sheath. They occur in about 8-16% within the head and neck region. Morphologically, some malignant tumors look like malignant peripheral nerve sheath tumors, particularly in the head and neck location; however, immunohistochemistry have a great contribution to distinguish between them. This case report is on a 45-year-old woman with a malignant peripheral nerve sheath tumor located in the sino-nasal tract., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2022.)

Squamous cell carcinoma is the most common mucosal malignancy of the head and neck. Adenoid pseudo-angiosarcomatous SCC is a rare variant occurring commonly in the skin of the head and neck. However, involvement of oral cavity is extremely rare, with only few case reports in the English literature. Morphologically, the tumor shows marked acantholysis, with anastomozing channels mimicking vascular neoplasms. This variant represents a diagnostic challenge for pathologists, and immunohistochemical analysis remains essential for an accurate diagnosis. Herein, we describe the case of a pseudovascular SCC of the oral cavity in a 67 years old man with a brief revue of the literature to raise awareness on this rare variant; presenting diagnostic difficulties., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2021.)

Synchronous renal cell carcinomas (RCC) and angiomyolipomas (AML) occurring in the same kidney are rare. Cases in the setting of tuberous sclerosis (TS) have been reported in the literature. However, the association of these tumors in the same kidney without TS is even more rare. We report here a case of a clear cell renal cell carcinoma (CCRCC) associated with an AML in the same kidney in a 42 years old female lacking the TS diagnostic criteria. The patient underwent a radical nephrectomy. Six months after surgery, the patient is healthy without signs of tumor recurrence or distant metastasis., Competing Interests: All authors declare no conflicts of interest., (Copyright © 2021 Amal Fekkar et al.)

Competing Interests: There are no conflicts of interest.

Hodgkin lymphoma is a lymphoid malignancy characterized by minority population of neoplastic cells (Reed-Sternberg cells and its variants) within a reactive inflammatory background. It encompasses two entities: classical HL (∼95% of cases) and nodular lymphocyte predominant HL (∼5% of cases). Primary lymphoma of peripheral nerves (PLPN) represent a very rare condition, since only 19 cases have been reported in the English literature to date, all of which are of a non-HL phenotype. A 20-year-old female presented an intramural mass of the ulnar nerve. Histological analysis revealed a Classical Hodgkin lymphoma. Further investigations failed to reveal nodal or extranodal involvement. PLPN is a very rare entity. There is a need for further understanding of this unusual lymphoma presentation., (Published by Oxford University Press and JSCR Publishing Ltd. 2021.)

Primary leiomyosarcomas (LMS) of vascular origin are rare tumors, and more than half of the cases arise in the inferior vena cava (IVC). Primary LMS of the renal vein are extremely rare tumors with only a few cases reported in the literature. Their diagnosis is made only by pathological features. Histologically, they are made of atypical spindle-shaped cells arranged in long intersecting fascicles. Tumor cells stain positive for myogenic markers in immunohistochemistry. Standard treatment consists of radical nephrectomy followed by chemotherapy and/or radiotherapy. Because of insufficient histological data and follow-up, the prognosis factors are not well identified. Overall prognosis of renal vein LMS is poor. We report here an exceptional case of a huge LMS of the right renal vein mimicking a primitive renal cell carcinoma, occurring in a 56-year-old male patient., Competing Interests: All authors declare no conflicts of interest., (Copyright © 2021 Amal Fekkar et al.)

Retroperitoneal leiomyomas are rare benign tumors with smooth muscle differentiation, with only ~1001 cases reported in the English literature to date. Because of its scarcity and non-specific presentation, the preoperative diagnosis might be challenging. On histology, these neoplasms share the same macroscopic, morphological and phenotypic features with uterine leiomyoma; thus, they are referred to as leiomyoma of gynecologic type. Herein, we describe a case of a voluminous retroperitoneal leiomyoma in a 51-year-old woman with a history of total hysterectomy to raise awareness about this condition, as its differential diagnosis might be challenging., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2020.)

Angiomyofibroblastoma is a rare mesenchymal benign tumor that frequently occurs in young- to middle-aged women, arising from the genital tract. There are many overlapping radiological and immunohistochemical features with other stromal cell lesions, making the diagnosis difficult. We report here a case of a 29-year-old woman admitted for a pelvic mass, in whom, the histopathological and immunohistochemical studies led to the diagnosis of angiomyofibroblastoma., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2020.)

Pseudomyxoma peritonei (PMP) is a clinicopathologic syndrome characterized by mucinous ascites and pools of mucin resulting in neoplastic mucinous epithelium in the peritoneal cavity. PMP is uncommon and it is characterized by clinical and unusual pathologic manifestations posing diagnostic and therapeutic problems. Involvement of abdominal viscera and lymph node metastases are rare and sporadic cases have been reported in the literature. We here report the case of a 56-year old patient who had undergone two operations for PMP of appendicular origin presenting with progressive abdominal pain five years after his last treatment. Scanner objectified a recurrence of peritoneal pseudo-myxoma with liver and splenic intraparenchymatous lesions. Anatomopathological examination showed intrasplenic and hepatic recurrence of low-grade peritoneal pseudo-myxoma confirming the metastases.