Rhinoscleroma in a 9-year old boy: Rare case report.

Rhinoscleroma is a rare benign granulomatous disease in children, the pathogen of which is Klebsiella rhinoscleromatis. Rhinoscleroma almost always affects the respiratory tract, the nasal cavities are the most common site, but can also be found over the entire extent of the respiratory tree, clinically the patient often presents with nasal obstruction with other non-specific signs, Histology is the basis of the diagnosis of rhinoscleroma. Early and appropriate medical treatment improves the prognosis and prevents progression to disabling sequelae. We present the case of a 9-year-old child who presented to the pediatric emergency department with a 2-month history of nasal obstruction unresponsive to medical treatment (Oxymetazoline). This was accompanied by mild nasal swelling and intermittent episodes of epistaxis. A CT scan of the paranasal sinuses was performed, ruling out a malignant tumor in the nasal cavity due to the absence of bone lysis. A nasal cavity biopsy with histopathological examination was conducted, confirming the diagnosis of rhinoscleroma. This was supported by the presence of an infiltrate rich in histiocytes and plasma cells, along with the identification of Russell bodies during PAS (Periodic Acid Schiff) staining. The patient underwent a medical treatment regimen involving a sulfamide-based antibiotic therapy (trimethoprim/sulfamethoxazole) for 8 weeks, along with a short-term corticosteroid therapy at a dose of 1 mg/kg/24 hours. At the first follow-up after 1 month, there was a noticeable reduction in nasal swelling and an improvement in nasal obstruction. A second follow-up 3 months later showed favorable results, with complete disappearance of both the swelling and nasal obstruction. Considering the possibility of recurrence, an annual follow-up was decided upon.

Malignant peripheral nerve sheath tumors of the sino-nasal tract: about an unusual case report.

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.

Association of trichilemmal and basal cell carcinomas: a case report.

Proliferating trichilemmal tumor (PTT) is a benign tumor arising from the isthmic portion of the hair follicle. Malignant transformation in PPT is very rare and unusual. Indeed, only about sixty well-documented cases have been found in the English literature. We present here the case of a 72-year-old patient with an exceptional combination of malignant trichilemmal carcinoma and basal cell carcinoma, occurring on actinic keratosis lesions. The aim of this work is to describe the diagnostic and therapeutic modalities of this association which is exceptional.

Pseudovascular Squamous Cell Carcinoma of the Oral Cavity: A Diagnostic Pitfall About a Case.

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.

Concurrent Angiomyolipoma and Clear Cell Renal Cell Carcinoma in the Same Kidney: A Rare Finding in a Patient without Tuberous Sclerosis.

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.

Leiomyosarcoma of the Renal Vein Mimicking a Primitive Renal Cell Carcinoma: Case Report of an Unusual Presentation.

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.

[Therapeutic management and prognostic profile of a patient with maxillary sarcomatoid carcinoma (case report)]. / Carcinome sarcomatoïde: prise en charge thérapeutique et profil pronostique à propos d´une localisation maxillaire inhabituelle (à propos d´un cas).

Sarcomatoid carcinoma is a rare, aggressive, malignant tumor with a poor prognosis and a very high frequency of recurrence. Carcinoma of the maxillary sinus is extremely rare. We report the case of a 42-year-old woman with left maxillary process. Biopsy revealed aggressive sarcomatoid carcinoma with a lymph-node metastasis. The patient underwent surgical excision, lymph-node dissection followed by radiotherapy with good outcome. The rarity of this site-specific cancer poses a problem of diagnosis and timely management which is still a controversial topic. However, wide surgical excision is the gold standard treatment. This study highlights the anatomoclinical peculiarities and, in particular, the prognostic features of this tumor.

Pelvic angiomyofibroblastoma: an unusual case report.

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.

Solitary preperitoneal neurofibroma: a case report.

BACKGROUND: Neurofibroma is a rare benign tumor. The isolated presence of such lesions in the preperitoneal space with no evidence of systemic disease has never been reported in the literature. CASE PRESENTATION: A 29-year-old white man presented with a 12 months history of progressive abdominal distension. Clinical examination revealed a bulky hypogastric mass. Abdominal computed tomography and pelvic magnetic resonance imaging showed a large, well defined preperitoneal tumor measuring 18 x 17 cm extending in the pelvis. A computed tomography guided biopsy was performed which revealed a neurofibroma. Exploratory laparotomy showed a well encapsulated elastic soft tumor in the preperitoneal space which measured 17 x 18 cm and weighted 2 Kg. The tumor was completely excised. No recurrence occurred after one year of follow-up. CONCLUSION: Solitary preperitoneal neurofibroma is an extremely rare benign tumor. Its clinical and radiological signs are nonspecific. Preoperative histology can be useful to guide the surgical approach which is the only curative treatment.

Juxtaglomerular cell tumor: a distinct mesenchymal tumor of kidney.

Juxtaglomerular cell tumor (JGCT) is an unusual mesenchymal entity of the kidney. It is a benign renin-secreting tumor causing hypertension and hypokalemia due to secondary hyperaldosteronism. It is curable if it is discovered early and surgically removed, but may cause a fatal outcome usually due to complications of associated hypertension.

Primary endobronchial leiomyosarcoma of the lung: clinical, gross and microscopic findings of two cases.

Primary leiomyosarcoma of the lung is an unusual malignant tumor. Among this entity, the endobronchial form is very rare and the preoperative diagnosis is extremely difficult. We present two different presentations and outcomes of primary endobronchial leiomyosarcoma of the lung. In both cases, the histological study and the immunohistochemical stain, of the surgical resection, provided the final diagnosis. Through those cases we present the diagnostic and therapeutic difficulties encountered.

Calcified multilocular thymic cyst associated with thymoma: a case report.

INTRODUCTION: There are few case reports of thymoma with a thymic cyst. Such an association renders it difficult for any pathologist to differentiate from other neoplasms, such as a cystic thymoma. CASE PRESENTATION: A 50-year-old Berber woman from Morocco was admitted with a chronic cough of more than 10 years duration. Her medical history and physical examination were normal. Anterior chest radiography demonstrated a calcified opacity in her right anterior mediastinum. A chest-computed tomogram revealed a round cystic tumor, with significant calcification in her right anterior mediastinum. A surgical exploration was performed. The tumor seemed to be a well-encapsulated and totally calcified lesion, arising from the right lobe of her thymus. It was removed by partial resection of her thymus. Through histology, the calcified tumor exhibited some areas of multilocular fibrous-wall cysts. These cysts were partially lined by small cuboidal cells with severe chronic inflammation and an AB thymoma that arose from the wall of the cyst. CONCLUSION: Greater attention should be given to multilocular thymic cysts, to exclude the possibility of neoplasm, especially when the cyst wall is thickened.