Angiomyofibroblastoma of the spermatic cord: a case report.

Angiomyofibroblastoma (AMF) is a rare benign mesenchymal tumor with tendency to arise in the lower genital tract of middleaged women, predominately in the vulva. A few cases of AMF in males have been reported involving the scrotum, perineum or spermatic cord. We report a new case of AMF arising in the right inguinal region of a 27-year-old man. The tumor was well-circumscribed, myxoid and measured 30 mm in maximum dimension. On microscopic examination, the tumor was composed of spindle cells without atypia and with less than one mitosis figure per 10 high-power fields. Multinucleated cells and mast cells were observed. The stroma was myxoid and edematous with abundant capillary-sized blood vessels. Immunohistochemical staining showed a strong immunoreactivity for desmin and smooth muscle actin. The tumor cells were negative for estrogen receptors and focally positive for progesterone receptors with a low proliferative index of Ki67 (< 5%). This unusual neoplasm should be distinguished from aggressive angiomyxoma and other myxoid malignant tumors.

Unilateral orbital mass as an unusual presentation of IgG4-related disease.

IgG4-related diseases are a recently recognized systemic syndrome characterized by mass-forming lesions, in mainly exocrine tissue, that consist of lymphoplasmacytic infiltrates and sclerosis, which may mimic malignant neoplasm due to clinical and imaging features. We report an unusual case of a 62-year-old woman who presented with a left orbital mass, which histologically revealed to be an IgG4-sclerosing disease.

Papillary haemangioma: a case report of multiple facial location.

Papillary haemangiomas were recently defined as morphologically distinct and benign cutaneous haemangiomas showing a predominantly intravascular capillary proliferation within dilated thin-walled dermal blood vessels. We describe the case of a 45-year-old woman who presented with multiple eruptive red-bluish raised papules and nodules distributed over the skin of the chin that were related to a papillary haemangioma.

[Cervical node fine needle aspiration: factors influencing the failure rate]. / Cytoponction ganglionnaire cervicale : facteurs influençant le taux d'échec.

INTRODUCTION: We had for aim to study the factors influencing the rate of non satisfactory (NS) cervical node fine needle aspirations (CNFNA). MATERIALS AND METHODS: We prospectively included 272 CNFNA, performed over 2 years (2010-2012). NS results were studied according to the following criteria: age, size and location of the node, number of punctures performed, and the operating physician's experience. RESULTS: Fifty-six (20.6%) of the 272 CNFNA were NS because they were acellular or paucicellular. The rate of NS CNFNA was not correlated with the patient's age: 63.6% for small lymph nodes (≤1 cm: P=0.01). Submandibular and jugulodigastric locations were observed in 28% of NS CNFNA and in 5% of satisfactory ones (P=0.001). The rate of NS CNFNA was 67% if 1 or 2 punctures were performed; it decreased to 18% for 3 or more punctures (P=0.01). This rate was 77% for the first study semester, and 8% for the fourth semester (P=0.001). CNFNA has been repeated for 19 patients. The second CNFNA was contributive for 14 patients. Repeating the CNFNA increased its efficiency by 73.7% (14/19). DISCUSSION: The rate of NS CNFNA depends on the size and location of the lymph node, and the operating physician's experience. Repeating the CNFNA significantly improves its efficiency.

[Osteoclast-type giant cell tumor of the parotid gland]. / Tumeur à cellules géantes de type ostéoclastique de la parotide.

INTRODUCTION: Osteoclast-type giant cell tumors of the salivary gland are extremely rare; only 23 cases have been reported. Two presentations were observed: isolated tumor or tumor associated with a carcinomatous contingent. CASE REPORT: A 51-year-old female patient consulted for a painless left retro-angulo-mandibular swelling having appeared 2 years before. This was a 2 cm parotid mass without facial nerve palsy or cervical lymphadenopathy. The patient underwent a superficial parotidectomy to remove the nodule. The direct microscopic examination revealed an osteoclastic giant cell tumor without any carcinomatous contingent. At immunohistochemistry, mononuclear cells were diffusely and intensely stained by anti-pancytokeratin, while multinucleated cells were totally negative and CD68 positive. The patient was not given any complementary treatment. She was followed-up 23 months later and did not present any signs of recurrence or metastasis. DISCUSSION: The histogenesis and nosology of osteoclastic giant cell are currently unknown. Although this entity was not integrated into the latest WHO classification, most authors consider it as a variant of carcinoma. Published data on the epithelial or histiocytic nature of multinucleated cells is not consensual. Our case presentation supports the hypothesis of a histiocytic differentiation of giant cells and epithelial mononuclear cells.

[Ileocolic intussusception from metastatic renal cell carcinoma]. / Localisation iléale d'un carcinome à cellules rénales révélée par une invagination iléo-cæcale.

The lung, the liver, the bone tissue and the brain are the most frequent sites for renal cell carcinoma metastasis. Small bowel metastasis from renal cell carcinoma is rare, with only few cases published. We report the case of ileal metastasis from operated kidney cancer revealed by ileocolic intussusception and causing intestinal obstruction in a 32-year-old woman.

Pinkus tumour: an unusual case.

Fibroepithelioma of Pinkus is a rare cutaneous tumour. Its classification is controversial and is considered as a variant of either basal cell carcinoma or trichoblastoma. Its presentation as a multiple tumour is rare. We are reporting such a case occurring in a 55-year-old man presenting with multiple seborrheic keratosis-like lesions corresponding histologically to Pinkus tumours. The clinical diagnosis of Pinkus tumour represents a challenge. Histological examination is extremely useful in aiding in the diagnosis of difficult cases.

The course and prognosis of pemphigus in 47 Tunisian patients.

BACKGROUND: Pemphigus is a severe and life-threatening autoimmune bullous dermatosis. OBJECTIVE: We have analysed parameters that may influence prognosis of pemphigus (P). METHODS: It was a retrospective study (2002-2010), with pemphigus considered as severe if body surface involvement ≥ 30%. Disease control and relapse-free survival (Kaplan-Meier) were analysed and compared according to several parameters (P < 0.05). RESULTS: 47 cases of pemphigus were collected, mean age 51 years ± 16.8 (F/H = 3.27). There were 30 pemphigus profundus and 17 superficial pemphigus. The median remission period was of 9 months (1.2 months-5 years). The mean healing time was of 40 days (6 days-4 months), which did not depend on type of P, its severity or infectious complications, whereas it was shorter in aged patient (≥ 65 years) compared to non aged ones (P = 0.018). 36.2% of patients had relapsed. Relapses were significantly more frequently observed only in the presence of mucosal involvement at presentation (P = 0.015). The median overall 1st relapse-free survival was of 2.33 years. Only mucosal involvement at presentation was associated with a shorter median 1st relapse-free survival time (1.28 years vs. 3 years) (P = 0.0017). Mortality rate was of 10.6% (n = 5); in four patients the death was directly related to pemphigus and occurred rapidly after the onset of lesions. CONCLUSION: Our study illustrates the poor prognosis of pemphigus by a long duration to disease control, a high initial dose of oral steroid, a high rate of relapse and a short remission period. Only mucosal involvement at presentation was identified as a poor prognostic factor.

Malignant proliferating trichilemmal cyst of the scalp: histological aspects and nosology.

BACKGROUND: Malignant proliferating trichilemmal cyst is a rare tumour usually located on the scalp of elderly women. About 40 cases of malignant proliferating trichilemmal tumour have been documented. CASE REPORT: We report a case of a malignant proliferating trichilemmal cyst of the scalp in a 57-year-old woman. On the vertex she had a voluminous vegetated and multinodular tumour measuring 7 x 5 cm with spontaneous and abundant bleeding, and another lesion of the scalp corresponding to a trichilemmal cyst. Based on histopathological findings, the case was diagnosed as malignant proliferating trichilemmal cyst. CONCLUSION: Diagnostic, clinicoprognostic and histological features of this tumour are discussed. Treatment is not yet standardized given its rarity.

[Pediatric mandibular ameloblastic fibro-odontoma]. / Fibrome odonto-améloblastique de l'enfant.

INTRODUCTION: Ameloblastic fibro-odontoma (AFO) is a very rare mixed odontogenic tumor (2% of all odontogenic tumors). It is considered as a non-extensive non-aggressive tumor but recurrence and malignant transformation remain possible. We report a voluminous AFO in a child. CASE REPORT: A 7-year-old boy presented with a right mandibular and facial swelling. The panoramic radiograph and CT scan revealed a voluminous unilocular radiolucent lesion (10 cm) with an impacted tooth that involved the ramus area and the angle of the right mandible. Enucleation and curettage was performed with a good outcome. There was no recurrence at ten months. DISCUSSION: This case is exceptional because of the young patient's age, the large tumor diameter, and the good outcome after ten months. This tumor occurs more often in the posterior region of the mandible and is frequently seen in the first two decades of life. The diagnosis is made on radio-clinical data and may be confirmed by histology. A surgical treatment with complete tumor removal is recommended to prevent recurrence.

[The myofibroblastic inflammatory tumours: A fortuitous association or an unknown postpartum complication]. / Les tumeurs myofibroblastiques inflammatoires : association fortuite ou complication peu connue du post-partum ?

The inflammatory myofibroblastic tumour has clinical, biological or histological features sometimes misleading with a septic condition. Presenting symptoms are variable and arising circumstances remain obscure. We report three cases occurring in a postpartum context. The first patient, a 28-year-old female, had left psoitis with a sepsis the first day postpartum in relation with an inflammatory myofibroblastic tumour of the meso-ovary. The second patient, a 40-year-old woman, had a hepatic inflammatory myofibroblastic tumour revealed by a ruptured sub-capsular haematoma of the liver in the forth day postpartum. The third patient, a 32-year-old woman, had a pulmonary inflammatory myofibroblastic tumour, diagnosed 5 months after a delivery and which recurred 10 years after surgical treatment. These cases illustrate the difficulty to diagnose inflammatory myofibroblastic tumour, particularly in postpartum.

[Cecal perforation in a kidney transplant patient: disseminated histoplasmosis]. / Perforation caecale chez un transplanté rénal.

Disseminated histoplasmosis is a fungal infection caused by Histoplasma capsulatum. It often involves immunodeficient patients and can occur in two forms, i.e., the large- and small-celled variants. The purpose of this report is to describe a case of disseminated histoplasmosis with cutaneous and digestive involvement observed four years after kidney transplantation in a man from Senegal. The patient developed severe sepsis secondary to colonic perforation. Outcome was fatal due to delayed diagnosis and extent of disease.

Rectal leiomyosarcoma: report on two cases and a practical approach to differential diagnosis.

Rectal leiomyosarcoma is an uncommon malignancy. Herein, we describe the clinicopathological features and biological behaviour of these tumours, and provide a practical approach to differential diagnosis, particularly with gastrointestinal stromal tumours (GIST). We report two cases in elderly men. In the first, the lesion was 2 cm from the anal sphincter, while it was located in the rectal ampulla in the second case. Histologically, both tumours were characterized by pleiomorphic, large spindle cells, presenting numerous mitoses and marked nuclear atypia. Immunohistochemical analysis showed that tumour cells coexpressed both actin and desmin, whereas CD117 and S100 protein were negative. The final diagnosis was leiomyosarcoma. One of the patients died of pulmonary metastasis within six months. The second patient had bone metastasis, but was lost to follow up. This report underlines the potential diagnostic problems raised by rectal leiomyosarcoma and emphasizes the role of immunohistochemistry in achieving correct diagnosis, which has important clinical, therapeutic and prognostic consequences.

[Tuberculous lupus]. / Le lupus tuberculeux.

UNLABELLED: Cutaneous tuberculosis accounts for 2% of all extrapulmonary tuberculosis and is one of the multiple clinical presentations of extrapulmonary tuberculosis. We report a case of cutaneous tuberculosis with unusual clinical and histological presentations. CASE REPORT: We report the case of a 29-year-old woman with plurifocal tuberculous lupus. Histological examination of cutaneous lesions revealed the presence of a granuloma with caseum necrosis. Chest X-ray showed a severe pulmonary tuberculosis lesion. The Mantoux test performed by intradermal injection was positive. Skin lesion PCR and sputum culture were negative. Both skin and pulmonary lesions responded to antituberculous treatment. COMMENTARY: Lupus vulgaris is commonly presents on the head and neck. The diagnosis may be difficult when lupus vulgaris occurs at unexpected sites or in unusual clinical presentations. Caseum necrosis observed in our patient was unusual.

Urothelial carcinoma with plasmocytoid component.

Plasmocytoid urothelial carcinoma is a rare subtype of tumour of the urinary bladder. Its clinical and histopathological features have not been well characterized. There are few reports of this type of tumour. We report a case of 65-year-old man who was operated in our department for bladder tumour. The pathological diagnosis was high-grade urothelial carcinoma with plasmocytoid component. The patient died shortly thereafter from liver and bone metastasis.