Extramedullary plasmacytoma of the orbit complicating the evolution of multiple myeloma in complete remission.

Orbital plasmacytoma is rare and has only been reported in the context of the initial diagnosis of multiple myeloma. Moreover, isolated orbital plasmacytoma without any signs of multiple myeloma is extremely rare. We report the case of a 59-year-old female patient diagnosed with IgA Kappa multiple myeloma. It was stage I ISS (International Staging System) and stage I R-ISS (Revised ISS). According to the Tunisian national protocol, the patient was included in the standard-risk group and was eligible for four cycles of CTD (Cyclophosphamide, Thalidomide, Dexamethasone) followed by autologous stem cell transplantation. Taking into account the partial response after the CTD cycles, the patient has benefited from two VTD cycles (Bortezomib, Thalidomide, Dexamethasone). Thus, complete remission was obtained. The patient refused autologous stem cell transplantation. Therefore, maintenance treatment based on Thalidomide only was started and received over a twelve-month period. Five months after the end of maintenance treatment, she reported frontal headaches that were resistant to symptomatic treatment, with ptosis in the right eye in physical examination. Brain MRI revealed the presence of a right cranio-orbital tissue mass with intra-orbital and extra-axial cerebral components. The mass measured 32/36 mm on axial sections and 47 mm in height. The patient underwent a complete resection of the cranio-orbital mass with cranioplasty. The histopathological examination of the mass with Immunohistochemistry staining confirmed the diagnosis of orbital plasmocytoma. An update of the multiple myeloma assessment did not reveal any biological, cytological or radiological signs in favor of multiple myeloma. Therefore the diagnosis of isolated orbital plasmacytoma without signs of multiple myeloma was made. Post-operative brain MRI showed complete disappearance of the right cranio-orbital tissue lesion. There was only a persistent meningeal enhancement of the dura mater at the surgical site, suggestive of post-operative changes. The patient was then referred for cranio-orbital radiotherapy.

Diffuse Kaposi sarcoma with oral involvement in a person diagnosed with human immunodeficiency virus/acquired immune deficiency syndrome: A case report.

Kaposi's sarcoma is a well-known multifocal vascular tumor initially described by pathologist Moritz Kaposi. This report presents the case of a 38-year-old bisexual male who consulted the infectious diseases department with non-itchy maculopapular and purpuric lesions on the skin with the involvement of the oral mucosa, suggesting a diagnosis of Kaposi's sarcoma. Biological and radiological investigations were performed. The patient was found to be human immunodeficiency virus positive with a viral load of 251.000/mL and a CD4 count of 182/mm3. In addition, secondary syphilis was diagnosed. Histopathological examinations confirmed HHV-8 KS. The patient was treated with Extencilline® and antiretroviral therapy. After 10 months of chemotherapy, a favorable outcome was noted with complete resolution of skin and oral mucosa lesions.

Small Intestine Gastrointestinal Clear Cell Sarcoma: A Case Report and Review of the Literature.

Gastrointestinal clear cell sarcoma (GICCS)/malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare form of cancer with aggressive clinical behavior. It has distinct pathological, immunohistochemical, ultrastructural, and molecular features. Herein, we present the case of a 20-year-old woman with no notable medical history who presented to the outpatient department with complaints of abdominal pain and vomiting. Symptoms had been evolving for 3 months. The physical examination revealed slight abdominal tenderness and melena. Biological investigations revealed iron-deficiency anemia. The upper and lower endoscopies showed no abnormalities. Magnetic resonance enterography revealed small bowel wall thickening of 15 mm × 2 mm. Exploratory laparotomy revealed an ileal mass with mesenteric lymphadenopathy. A wide resection of the mass was then performed. The final pathological report confirmed the diagnosis of small bowel GICCS/GNET. After 11 months of follow-up, the patient presented with mesenteric lymph node metastases.

Olfactory cleft mass as the first presentation of asymptomatic metastatic renal cell carcinoma: A case report and review of the literature.

INTRODUCTION AND IMPORTANCE: Renal cell carcinoma metastasis in the sinonasal cavities is rare. They account for less than 1 % of all metastases of these renal cancers. CASE PRESENTATION: We report the case of a patient with an unremarkable pathological history, who consulted for recurrent right epistaxis. Nasal endoscopy revealed a reddish mass located medial to the right middle turbinate. Computed tomography and magnetic resonance imaging showed a lesion located at the level of the olfactory cleft. The patient had a complete removal of the mass and the anatomopathological examination concluded to a metastasis of a clear cell carcinoma of renal origin. Taking into account these results, a thoraco-abdomino-pelvic CT scan was performed and it discovered a left renal tumor. CLINICAL DISCUSSION: The most frequent functional sign of Renal cell carcinoma metastases is epistaxis. This is explained by the rich vascularity of these metastases. The imaging data are not specific and do not allow differentiation between primary tumor and metastasis. The definitive diagnosis can be confirmed only by histologic examination. CONCLUSION: Faced with any hypervascularized tumor of the nasal cavity, the ENT physician must evoke a metastasis of a renal cancer, even in the absence of history and symptoms evoking this cancer.

Rosai-Dorfman disease of the oral cavity

ABSTRACT First described by J Rosai and R F Dorfman in 1969, Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. It is usually seen in the first two decades of life. The most frequent clinical presentation is painless, bilateral cervical lymphadenopathy accompanied by fever, weight loss, and an elevated ESR. However, RDD without nodal involvement is extremely rare, and the most common extranodal location is the head and neck region, mainly affecting the nasal cavity, pharynx, and paranasal sinuses. Oral location of RDD is occasional; according to our knowledge, only 17 cases of oral Rosai-Dorfman disease without lymph node involvement have been found in the literature. Because of the rarity of these isolated oral presentations, the clinical and radiological aspects need to be more studied. This article aims to present a rare case of oral Rosai-Dorfman disease without nodal involvement, detail the clinical and radiological signs, and the treatment strategy used in our patient.

Primary retroperitoneal mucinous cystadenoma: A case report with review of literature.

Introduction and importance: Primary Retroperitoneal mucinous cystadenoma (PRMC) is an extremely rare benign tumor, predominantly occurring in women, with unclear pathogenesis. Case presentation: A 31-year-old woman, with no medical or surgical history, presented with left flank pain. Clinical discussion: An abdominal computed tomography (CT) scan revealed an 11cm retroperitoneal cyst. Due to its large size, percutaneous CT-guided drainage followed by a laparotomy surgical resection, were performed. Post-operative course was uneventful. Histological and immunohistochemical findings were consistent with PRMC. The patient was disease-free after a 6-month follow-up. Conclusion: Mucinous cystadenoma is a very odd finding in the retroperitoneum. Multiple differential diagnoses are to be considered beforehand, as most of cystic lesions in this anatomical region are malignant and require a different surgical approach. Radical resection, by laparotomy or laparoscopy, is the treatment of choice.

Case Report: Mitral valve obstruction by metastatic malignant phyllodes tumor.

Cardiac metastases from phyllodes tumors (PTs) are rare. Herein, we report a case of a 37-year-old female patient with a history of borderline breast PTs, admitted to the cardiology department for acute cardiac failure revealing concomitant cardiac and pulmonary metastases of malignant PTs. Cardiac metastasis occurred through direct extension from pulmonary metastasis to the left atrium via the right inferior pulmonary vein, causing severe mitral valve obstruction. Although the metastasis was surgically removed, the patient had an uncommon complication, which led to acute heart failure and huge relapse resulting in her death.

Asymptomatic ameloblastoma of the maxilla with infratemporal fossa involvement: A case report.

INTRODUCTION AND IMPORTANCE: Diagnosis of odontogenic tumors can be challenging due to their rarity and diverse morphology. The clinical diagnosis could be suspected when it had raised near the tooth. But, when their location is not typical, like inside the paranasal sinuses, the diagnosis is less easy. Maxillary ameloblastomas are rare with only sparse information on their epidemiological, histological and effective management. CASE PRESENTATION: A 54-year-old woman presented with ameloblastoma of the left maxillary sinus. Intraoral examination revealed partially edentulous with a 2 cm painless ulceration of the left alveolar process of the maxilla. A biopsy was performed which showed a follicular ameloblastoma. The treatment was surgical. She had a combined endoscopic and transoral resection of the tumor. The patient was diagnosed with recurrence within 3 months from surgery. DISCUSSION: although rarely reported, clinicians should still suspect and know how to manage such as rare and locally invasive tumor as a maxillary ameloblastoma. CONCLUSIONS: As odontogenic tumors are rare, some entities are infrequently encountered, making the diagnosis more difficult. Clinicians, oral and maxillofacial surgeons, and oral pathologists should be familiar with the ameloblastoma and its differential diagnosis for accurate diagnosis and management.

Clear cell carcinoma of the abdominal wall: A case report with a review of the literature.

Introduction and importance: Clear cell carcinoma of abdominal wall is a very rare and aggressive disease. It is mostly related to malignant transformation of abdominal wall endometriosis. This paper provides a new case report and a literature review of primitive abdominal wall clear cell carcinoma. Case presentation: A 45-year-old woman with a history of a two previous caesarian section presented to the outpatient department with a tumor mass evolving since 10 years in the lower right quadrant of her abdomen. Imaging studies revealed a voluminous subcutaneous mass developing at the expense of the anterior abdominal wall. Surgical resection of the mass was performed. Histopathological examination along with immunohistochemical analysis were consistent with clear cell carcinoma. Biopsies of the endometrium and ovaries were performed and were negative for malignancy. The patient underwent therefore a hysterectomy with bilateral salpingo-oophorectomy which did not reveal any disease. The diagnosis of primitive clear cell carcinoma of the abdominal wall was then confirmed. Clinical discussion: Primitive clear cell carcinoma of the abdominal wall is an extremely rare form of cancer with usually poor prognosis. Clinicians must be aware of the possibility of malignancy of any swelling mass occurring near or within a caesarean section scar. Conclusion: Reporting more such cases is still needed to further progress in the understanding of this malignancy in addition to the development of treatment strategies.

Large splenic epithelial cyst: A rare presentation.

INTRODUCTION AND IMPORTANCE: Splenic cysts are infrequent and most of them are related to parasitic diseases. Splenic epithelial cyst (SEC) is the most common type of primary non-parasitic splenic cysts. Its pathogenesis is not yet clear. Splenic cysts are usually asymptomatic and are detected incidentally during imaging exams or an exploratory laparotomy. CASE PRESENTATION: Our case is about a primary SEC discovered incidentally. An 18-year-old male patient without any personal and family medical history, presented with symptoms of upper urinary tract infection. Renal ultrasound was performed and found incidentally a solitary cystic lesion in the spleen. A laparoscopic partial splenectomy was made then. The specimen was sent for pathological examination and a diagnosis of primary (epidermoid) epithelial splenic cyst was given. CLINICAL DISCUSSION: SEC is a rare pathology that could mimic other splenic cysts like hydatid cyst. The preoperative diagnosis of SEC can be suspected by ultrasonography, computed tomography or magnetic resonance imaging. However, histopathological examination is mandatory to confirm the diagnosis. A wide range of differential diagnoses is considered when we suspect clinically SEC because of its lower incidence. CONCLUSION: Actually, spleen-preserving surgery with minimally invasive methods such as laparoscopy is the gold standard for the treatment of SEC despite the risk of recurrence encountered with these techniques. However, different surgical modalities are discussed depending on the size and the location of the cyst and the patient's age.

A Giant Ureteral Inflammatory Myofibroblastic Tumor in a 4-Year-Old Child: A Case Report and Review of the Literature.

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of children and young adults. It consists of myofibroblastic cells of borderline malignancy admixed with inflammatory cells. According to the WHO classification, of tumors, it is a soft-tissue tumor with an intermediate malignant potential. It may arise within numerous organs. Ureteral location is exceedingly rare. We report the exceptional case of a 4-year-old boy presenting with a giant right ureteral IMT removed during a nephroureterectomy. Histology and immunohistochemistry confirmed the diagnosis of an IMT with Anaplastic Lymphoma Kinase 1 expression. A 7 months follow-up showed no recurrence or distant metastasis. We also reviewed the literature for similar cases of pediatric ureteral IMT.

Basal cell adenoma of the parotid gland: A rare entity.

Basal cell adenoma (BCA) is a rare benign salivary gland tumor accounting for only 1-2% of all salivary gland tumors. We report a case of a 50-year-old man presenting a BCA of the parotid gland. A pleomorphic adenoma was initially suspected based on radiological features and fine needle aspiration cytology findings (FNAC).

Epidermoid cyst of the buccal mucosa-An uncommon entity: Case report and literature review.

Epidermoid cyst of the buccal mucosa is rare. Nevertheless, it must be included in the differential diagnosis of swelling in this area. The diagnosis is based on anatomopathological examination. The surgical enucleation is the gold standard of treatment.

Small bowel and lung histiocytic sarcoma revealed by acute peritonitis: A case report with review of literature.

INTRODUCTION AND IMPORTANCE: Histiocytic sarcoma (HS) is a rare malignant neoplasm showing morphologic and immunohistochemical features of histiocytes. It is characterized typically by extranodal presentation and a poor clinical course, particularly in cases with disseminated disease. CASE PRESENTATION: This report documents a case of bifocal and aggressive HS in small bowel and lung revealed by acute peritonitis in a 63-year-old man. CLINICAL DISCUSSION: Despite its rarity, we believe that the correct diagnosis of HS is crucial for clinical treatment and prognostic prediction. CONCLUSION: The collection of additional cases of HS are important to obtain further progress in prognosis and guide treatment decisions.

Bilateral pleural metastases of a primary central nervous system neuroblastoma in an adult: First case in the world.

Primary central nervous system neuroblastoma (PCNS-NBL) is a rare and aggressive malignant tumour. Pleural metastases of PCNS-NBL have not been documented before. We report a case of a 30-year-old male patient, with a history of PCNS-NBL treated with surgery, radiation and chemotherapy. Three years later, he presented an aggravated dyspnoea with impaired general condition. The different investigations confirmed that his PCNS-NBL has relapsed with bone, lymph nodes and bilateral pleural metastases. Because of the disseminated disease and the poor general condition of the patient, only symptomatic treatment measures were preconized. The patient died 3 months later following cardiorespiratory arrest. To the best of our knowledge, this is the first case reporting bilateral pleural metastases of a PCNS-NBL in a young adult.

Human papillomavirus-related multiphenotypic sinonasal carcinoma: a clinico-pathological dilemma case report.

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), is a new entity that is restricted to the sinonasal tract and is associated with high-risk HPV. This tumor is suggested to have an indolent behavior with a better prognosis than other carcinomas. We report a unique case of HMSC with a locally aggressive behavior. It is about a 61-year-old men presented with 12 months of unilateral progressive olfactory dysfunction accompanied by exophthalmia of the left eye, declining vision and headaches for 6 months. Computed tomography imaging revealed a voluminous mass occupying the ethmoid, maxillary and frontal sinus with bony destruction of the left ethmoidal blade. Histology showed a tumor composed of variably sized nests, separated by thick mucoid stroma. Tumor cells are plasmacytoid with hyperchromatic nuclei and frequent mitoses. Immunohistochemistry revealed that these cells were positive for cytokeratin AE1/AE3, p16 and negative for CK7, CK20, CD117, p40, p63, S100, synaptophysin and chromogranin.

Ossifying fibromyxoid tumor of soft tissue: A case report with review of literature.

INTRODUCTION AND IMPORTANCE: The ossifying fibromyxoid tumor of soft tissue is a rare tumor of intermediate differentiation and uncertain lineage that occurs in adults mostly in the extremities and the trunk. PRESENTATION OF CASE: we present a case of 57 year-old man presenting with a right scapular mass. It was a subcutaneous and painless mass that was largely excised. The diagnosis of ossifying fibromyxoid tumor of the right shoulder was made. The follow up of 1 year was without recurrence and metastasis. CLINICAL DISCUSSION: The ossifying fibromyxoid tumor of soft tissue is exceptional, microscopic diagnosis and management is challenging, considering the scarcity of the tumor. CONCLUSION: More cases and retrospective studies are needed to understand the pathogenesis and to determine optimal treatment regimens.

Cystic form of Actinomycotic mycetoma: A new case with a diagnostic challenge.

Mycetoma, commonly known as Madura foot, is a chronic granulomatous infection caused either by fungi (eumycetoma) known as actinomycete. This disease occurs preferentially in young adults, and it affects the foot in particular. We report a Tunisian case of mycetoma occurring in an old patient, particular by its cystic presentation.

An unusual presentation revealing Peutz-Jeghers syndrome in adult.

INTRODUCTION: Peutz-Jeghers syndrome (PJS) is a rare hereditary disease characterized by hyperpigmentation on the lips and oral cavity and gastrointestinal hamartomatous polyps. The most common complications in PJS patients are bleeding, bowel obstruction and intussusception. PRESENTATION OF CASE: We hereby report a case of a 33-year-old female, without a family history of the disease, who presented to the emergency room with acute abdominal pain, bloating and not passing gas. On abdominal examination, upper abdominal and periumbilical tenderness was found. Computed tomography (CT) of the abdomen demonstrated suspected ascending colon intussusception. The patient underwent a mid-line laparotomy that showed an ileocolic intussusception. Reduction of this intussusception was successfully done with resection of the affected segment that showed presence of two pedunculated polyps. The specimen was sent thereafter to our department for histopathological evaluation, which confirmed the diagnosis of hamartomatous Peutz-Jeghers polyps with no malignancy. Afterwards, the patient was carefully reexamined and the physical examination revealed multiple pigmented spots on the face and lips. Thus, the diagnosis of Peutz-Jeghers syndrome was made. DISCUSSION: PJS is a rare autosomal dominant disorder that often remain undiagnosed for many years. Acute complications such as intestinal obstruction secondary to intussusception is one of infrequent revealing symptoms. CONCLUSION: Early identification, in patients with PJS and family members, as well as close cancer surveillance can improve certainly prognosis in these individuals.

Castelman's disease of the neck: a case report and literature review.

Castleman's disease is a rare pathology, poorly understood. It is considered as a lymphoproliferative disorder, described for the first time in 1954, which may be confused with other causes of lymphadenopathy. We report in this paper the case of a young women presenting with left latero-cervical lymphadenopathy. All the investigations were negative except a large high-vascularized level II cervical lymphadenopathy. We performed a cervicotomy. The extemporaneous histological exam was non-contributive. We decided to perform a complete level II and III left cervical lymphadenectomy. The diagnosis of unicentric Castleman's disease was confirmed based on the final histological study of the specimen, and the absence of other cervical and extra-cervical lymphadenopathy. The patient is free of recurrence at the time of reporting this article.