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Introduction: Les pseudokystes géants de la surrénale sont exceptionnels. A travers une nouvelle observation, nous en décrivons les aspects épidémiologiques diagnostiques et thérapeutiques. Observation: Un patient de 52 ans, sans antécédents notables, rapportait depuis 15 jours des douleurs à l'hypochondre gauche, des vomissements bilieux, et une altération de l’état général. L'examen clinique révélait une volumineuse masse de l'hypochondre gauche. L’échographie et la tomodensitométrie abdominales montraient une énorme lésion kystique intersplénorénale, refoulant les organes voisins. Le bilan biologique surrénalien était normal et la sérologie hydatique négative. La laparotomie avait confirmé la nature kystique de la masse. L'analyse histopathologique de la paroi du kyste n'a montré ni épithélium; ni endothélium, mais un infiltrat inflammatoire polymorphe. Conclusion: Le pseudokyste géant surrénalien observé était symptômatique et non fonctionnel. Le diagnostic de certitude en a été histopathologique, après exérèse chirurgicale.

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.)