A maxillary location of extra-nodal Rosai Dorfman disease: a case report.

Introduction: Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferation, characterized by a group of clinical symptoms. This report presents a case of extranodal RDD manifesting as a progressively enlarging left maxillary mass in a 42-year-old woman. Surgical exploration and biopsy confirmed the diagnosis of RDD, with characteristic histopathological features including emperipolesis. Treatment involved corticotherapy, resulting in controlled maxillary pain and improvement of the disease after one year. This case underscores the potential for extra-nodal RDD presentations, posing diagnostic challenges and emphasizing the importance of considering RDD in the differential diagnosis of maxillary masses.

Navigating diagnostic challenges-distinguishing malignant melanoma and clear cell sarcoma of soft tissues: a case report and review of the literature.

BACKGROUND: Within the spectrum of melanocytic-differentiated tumors, the challenge faced by pathologists is discerning accurate diagnoses, with clear cell sarcoma of soft tissues standing out as a rare and aggressive neoplasm originating from the neural crest. Accounting for 1% of all soft tissue sarcomas, clear cell sarcoma of soft tissues poses diagnostic complexities, often misidentified owing to its phenotypic resemblance to malignant melanoma. This chapter delves into the intricacies of clear cell sarcoma of soft tissues, its epidemiology, characteristic manifestations, and the imperative need for a comprehensive diagnostic approach involving immunohistochemical and molecular analyses. CASE PRESENTATION: A compelling case unfolds as a 25-year-old male from Morocco, initially misdiagnosed with malignant melanoma, experiences tumor recurrence on the second toe. With no history of trauma or familial neoplasia, the patient's clinical journey is explored, emphasizing the importance of detailed clinical examinations and radiological assessments. The chapter elucidates the histopathological findings, immunohistochemical spectrum, and the correlation between clinical parameters and diagnostic inference, ultimately leading to metatarsal amputation. This clinical vignette highlights the multidimensional diagnostic process in soft tissue neoplasms, emphasizing the synergistic role of clinical, radiological, and histopathological insights. CONCLUSION: The diagnostic challenges inherent in melanocytic-differentiated tumors, exemplified by the rarity of soft tissue clear cell sarcoma, underscore the essential role of an integrated diagnostic approach. This concluding chapter emphasizes the perpetual collaboration required across pathology, clinical medicine, and radiology for nuanced diagnostic precision and tailored therapeutic strategies. The rarity of these soft tissue malignancies necessitates ongoing interdisciplinary engagement, ensuring the optimization of prognosis and treatment modalities through a comprehensive understanding of the diagnostic intricacies presented by clear cell sarcoma of soft tissues.

A tonsillar location of a malignant schwannoma: a case report.

INTRODUCTION: Malignant schwannoma is a malignant tumor of differentiation of Schwann cells or perineural cells. OBSERVATION: The patient was a 74-year-old woman with no particular pathological history. She presented swallowing difficulty of solids and odynophagia, evolving for 1 year. Physical examination revealed a budding tumor of the left palatine tonsil without cervical adenopathy. The CT scan confirmed the lesions and the absence of tumor extensions. Histological and immunohistochemical examination of the biopsy sample of the tonsil tumor concluded to be a malignant schwannoma. The patient underwent a tonsillectomy with postoperative follow-up. DISCUSSION: Malignant schwannomas are aggressive tumors. They usually occur in young adults. They mainly affect nerves and soft tissues. Occurrence in the amygdala is rare. CONCLUSION: The association of malignant schwannoma of the palatine tonsil and advanced age is rare.

A cartilage-forming tumor of the mandibular angle: a case report.

BACKGROUND: Mandible can be the site of benign or malignant lesions of different origins, including odontogenic and non-odontogenic lesions. Cartilage-forming tumors have been rarely reported at this site. Chondrosarcoma is a rare malignant cartilage-producing neoplasm that is extremely rare in the mandible. The rarity of cartilage-forming tumor occurrence in the mandible can make diagnosis difficult for pathologists, as they do not expect this type of tumor at this anatomical site. Here we report a case of chondrosarcoma of mandibular angle. CASE PRESENTATION: A 70-year-old Moroccan male patient consulted a dentist for wisdom tooth pain. Wisdom tooth extraction was conducted. After 6 months, the patient reported the recurrence of pain associated with swelling in the mandibular area and paresthesia along the path of the mandibular nerve. A panoramic radiograph demonstrated a mixed radiolucent-opaque lesion involving the mandibular angle. Computed tomography showed a large osteolytic spontaneously hypointense and multilobulated lesion. A biopsy was done. Histopathological examination revealed sheets and irregular lobules of atypical cells presenting cartilaginous differentiation. Tumor cells showed severe nuclear atypia and were located within a hyaline cartilage matrix. Some foci of necrosis were noted. Osteoid deposits were not found. The patient was diagnosed with grade III chondrosarcoma and underwent a right segmental mandibulectomy with submandibular lymph node dissection. Macroscopically, the tumor was localized in the mandibular angle with extension in the mandibular body. Histopathology confirmed the previous diagnosis of grade III chondrosarcoma and did not show any lymph node metastasis. CONCLUSIONS: Owing to many histological similarities, grade III chondrosarcoma must be distinguished from chondroblastic osteosarcoma and metastatic lesions. In addition, chondroblastic osteosarcoma of the jawbones has a worse prognosis than chondrosarcoma, making the distinction between these two malignant tumors the most important concern of the pathologist when dealing with a cartilage-forming tumor at this site. Surgery with wide excision margins remains the best therapeutic approach, while the role of radiotherapy is controversial. The management of mandibular chondrosarcoma requires a multidisciplinary approach involving maxillofacial surgeons, radiologists, pathologists, and oncologists.

The role of humanitarian missions in surgical training for maxillofacial surgery residents: SOS Face Marrakesh experience.

Resident´s participation in humanitarian work has been controversial, while it´s recognized by some authors to bring significant value to the resident´s education. Herein, we aim to provide an evidence of the role of humanitarian missions in the surgical training as part of residency program, through report of a 10 years experience of SOS FACE Marrakesh, a non-benefit association within maxillofacial surgery department of Marrakesh. Its operating mode is to organize humanitarian missions coupled to targeted surgical training program, which is framed by educational objectives using a pedagogic portfolio. As a result, 60.6% of the residents felt an improvement in surgical skills, and the evaluation of residents before and after the targeted training showed an increase of 57% in average clinical knowledge, especially the diagnosis part. In conclusion, humanitarian work helps to improve surgical skills in addition to enhancement of human values and we suggest incorporating volunteerism in residency programs.

What Open-Lung Biopsy Teaches Us about ARDS in COVID-19 Patients: Mechanisms, Pathology, and Therapeutic Implications.

Difficulties have risen while managing Acute Respiratory Distress Syndrome (ARDS) caused by COVID-19, although it meets the Berlin definition. Severe hypoxemia with near-normal compliance was noted along with coagulopathy. Understanding the precise pathophysiology of this atypical ARDS will assist researchers and physicians in improving their therapeutic approach. Previous work is limited to postmortem studies, while our report addresses patients under protective lung mechanical ventilation. An open-lung minithoracotomy was performed in 3 patients who developed ARDS related to COVID-19 and were admitted to the intensive care unit to carry out a pathological and microbiological analysis on lung tissue biopsy. Diffused alveolar damage with hyaline membranes was found, as well as plurifocal fibrin microthrombi and vascular congestion in all patients' specimens. Microbiological cultures were negative, whereas qualitative Reversed Transcriptase Polymerase Chain Reaction (RT-PCR) detected SARS-CoV-2 in the pulmonary parenchyma and pleural fluid in two patients. COVID-19 causes progressive ARDS with onset of severe hypoxemia, underlying a dual mechanism: shunt effect through diffused alveolar damage and dead space effect through thrombotic injuries in microvascular beds. It seems reasonable to manage this ventilation-perfusion ratio mismatch using a high dose of anticoagulant combined with glucocorticoids.

Linking Helicobacter pylori with gallbladder and biliary tract cancer in Moroccan population using clinical and pathological profiles.

It is of interest to assess the clinical and pathological aspects of Gallbladder and biliary tract carcinomas confirmed by histological data. It is also of further interest to evaluate the link between Helicobacter pylori and biliary tract cancers. Eighty-nine (89) cases (mean age 60±12 years) of Gallbladder and biliary tract cancer confirmed by histological data were enrolled for the study at the Department of Pathology in Mohammed VI University Hospital, in Morocco. The data such as age, sex, clinical and histo pathological features were collected. Bile duct specimens were investigated for H. pylori using Giemsa and immuno histo chemistry staining. Results show that bile duct stones were found in 53.9% of cases. It is known using histological data that adeno carcinoma is common accounting for 70 % of all bile duct tumors. Moreover, Helicobacter pylori was detected in 54% of cases linking with the presence of bile duct stones characterized by the histological subtype, the macroscopic classification and lymph node's presence (p<0.001). Thus, data collected suggest the potential association of Helicobacter pylori with gallbladder cancer possibly through the formation of bile duct stones.

[Brain tumors in children: about 136 cases]. / Tumeurs cérébrales de l'enfant: à propos de 136 cas.

Brain tumors are the most diagnosed solid tumors in children under the age of 15 years worldwide. However, the epidemiological and anatomopathological profile of these tumors has been poorly described in African and, particularly, in Moroccan literature. This study highlights the epidemiological and anatomopathological peculiarities of primary brain tumors in children living in the region of Marrakech (south Morocco). We conducted a retrospective study in the Division of Anatomic Pathology at the Mohammed VI University Hospital, Marrakech from 2004 to 2016. One hundred and thirty-six patients were diagnosed with primary brain tumor, a mean of 11.33 cases per year. The average age of patients was 8.28 years. Sex-ratio (M/F) was 1.6 with a slight male predominance. Infratentorial tumors were found in 61,53% of cases while supratentorial tumors were found in 38.47% of cases. Infratentorial tumors mainly occurred in the cerebellar hemisphere (61.4%). Eighteen histological types were diagnosed. Astrocytoma and medulloblastoma accounted for 46,32% (29.41% and 16.91%, respectively). In our context, the majority of brain tumors in children was predominant in both age groups: 5-9 years and 10-15 years. The epidemiological data of these tumors from south Morocco are mostly consistent with those already published in North Country's literature and in other non-african countries' literature.

Posterior cerebral fossa medulloepithelioma: report of a case.

BACKGROUND: Medulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system, usually developing in childhood. Due to its rarity, the optimal management is still unknown. The prognosis is poor, especially when resection is incomplete. Adjuvant radiochemotherapy is often indicated. CASE PRESENTATION: We report a rare case of infratentorial medulloepithelioma in a 3 year old girl. She presented symptoms of increased intracranial pressure. On examination, she had coordination problems, ptosis and exotropia of the right eye. Magnetic resonance imaging demonstrated a large cerebellar vermix tumor. Immuno-histochemistry revealed a diffuse positivity for Vimentin and focal positivity for the epithelial membrane antigen, but Glial Fibrillary Acidic Protein and Synaptophysin were negative, the MIB-1 antibody was very high. She received postoperative craniospinal irradiation and died 7 months later. CONCLUSION: We describe the features (epidemiological, clinical, histological, immunohistochemical and therapeutic outcomes) of our case and confront it to literature data.

[Central neurocytomas: clinical and radiopathological correlations: about 12 cases]. / Neurocytomes centraux: corrélations cliniques et radiopathologiques à propos de 12 observations.

Central neurocytomas are defined as neoplasms composed of round, uniform cells, with neural immunophenotypic profile and low proliferation index. They account for 0.5% of intracranial tumors. They usually occur near the foramen of Monro and can cause obstructive hydrocephaly. We conducted a retrospective data collection from records of 12 patients with central neurocytomas in the Anatomopathological Laboratory at the Mohammed VI University Hospital, Marrakech, between January 2006 and June 2015. This study aimed to report and describe the radiopathological features of this rare histologic type. The male/female sex-ratio was 1.4. The average age at diagnosis was 22.3 years. The revealing symptomatology was dominated by intracranial hypertension associated with decreased visual acuity and diplopia in all patients. Our study concerned about simple biopsy in one case, subtotal resection in seven cases and total resection in four cases. Histopathological examination showed tumor proliferation with endocrine architecture. Tumor cells are more often small and uniform. Mitotic index was low. Tumor proliferation was associated with fibrillary fundus and vascular network with three types of trees. Immunohistochemical examination was identical in all patients. It showed tumor cells positive for anti-synaptophysin antibodies, chromogranine and NSE. In all patients the radio-pathologic correlation suggested central neurocytoma (grade II - WHO 2016). This study highlights the anatomo-clinical, radiological and evolutionary features of these rare tumors.

Primary breast angiosarcoma: a rare presentation of rare tumor - case report.

BACKGROUND: Primary breast angiosarcoma is defined as malignant proliferation showing endothelial differentiation. It is a very rare tumour (0.05% of primary mammary cancers), whose diagnosis can be difficult. CASE PRESENTATION: We report the observation of a patient with no previous history, aged 27 years. The clinical examination finds a right breast discreetly increased in volume. The trucut biopsy was in favour of a lactating tubular adenoma. However, an immunohistochemical complement was requested. An absence of pancytokeratin labelling contrasted with strong expression of CD31, CD34 (endothelial markers) are described. The proliferation index (Ki67) was estimated at 30%. This led to the conclusion that the phenotypic aspect is related to a vascular proliferation that evokes an angiosarcoma. After a multidisciplinary assessment, the patient benefited from an enlarged excision of the tumour. The histopathological examination of the surgical specimen found an infiltrating mesenchymal proliferation made of vessels of variable sizes anastomosed to vascular slits with lesional limits. The immunohistochemical examination on the surgical specimen showed to the same phenotypic profile on biopsy. The final diagnosis was a high-grade mammary angiosarcoma of incomplete excision. The patient refused any additional surgical management; external radiotherapy and close supervision were prescribed. After eight months of evolution, no local or remote recurrence was reported. CONCLUSION: Primary breast angiosarcoma is a mesenchymal malignant tumour of rare vascular origin. Our observation is peculiar by the absence of any prior radiotherapy, its clinical presentation, its morpho-phenotypic characteristics, its management and its evolutive aspects.

[Neuroendocrine carcinoma of the breast: about a case and review of the literature]. / Carcinome neuroendocrine du sein: à propos d'un cas et revue de la literature.

Primitive neuroendocrine carcinomas of the breast are rare tumors. They are now included in the latest WHO classification of tumors of the breast. We report the case of a 39-year old patient with tumor located in the breast. It was a locally advanced tumor that required mastectomy and ipsilateral axillary node dissection. Adjuvant chemotherapy was indicated. The evolution was marked by local progression. The patient died in a state of febrile pancytopenia after a one-year survival.

Curious vascular tumor.

Introduction. Sinusoidal hemangioma is a rare variant of acquired cavernous hemangioma predominantly occurring in females. Very few case reports have been described in the literature. Case Report. We present a case of a 46-year-old woman who noticed a slowly growing, cutaneous nodule on the left breast. Local excision of the lesion was performed and histology allowed to find a sinusoidal hemangioma. No recurrence was noticed. Conclusion. The very few reports of such a lesion in the literature reflect either rarity of such lesions or unfamiliarity of this subset among the pathologists.