Vaginal rhabdomyoma: a case report of an uncommon and misleading neoplasm.

Vaginal rhabdomyoma is an extremely rare tumor which presents as a vaginal polypoid masses. It is essential to differentiate it from benign and malignant mimickers so that appropriate therapy may be provided. The present report describes a vaginal wall nodule of a symptomatic 24-year-old woman. Local excision and subsequent pathological examination were performed. The final diagnosis was vaginal rhabdomyoma. The literature is reviewed and differential diagnosis are discussed.

Endometrial stromal sarcoma presenting as a cystic abdominal mass.

Endometrial stromal sarcoma (ESS) is rarely localized in extrauterine sites if metastasis or local extension of the primary uterine tumour are excluded, and diagnosis can be delayed because of the unusual site. We report a case of abdominal ESS in a 45-year-old woman who presented with an abdominal complaint. Ultrasound of the abdomen showed a large multiloculated cystic mass. The complete excision of the tumour revealed ESS arising in endometriosis. The tumour expressed hormonal receptors and the patient was administered hormonal therapy. ESS has a better prognosis than the sarcoma that is part of differential diagnosis, and is associated with endometriosis in about one-half of cases.

Primary CD30/ALK-1 positive anaplastic large cell lymphoma of the skeletal muscle in a child.

Anaplastic large cell lymphoma (ALCL) represents approximately 10 to 30% of all childhood non-Hodgkin lymphomas. It frequently involves both lymph nodes and extranodal sites whereas primary or secondary muscular involvements are quite uncommon. We describe a case of an 8-year-old boy presented with one month progressively swelling right buttock mass without association of lymphadenopathy or skin extension. Biopsy of the lesion showed large anaplastic cells with voluminous and abundant cytoplasm as well as folded nuclei. The tumour cells were positive for CD30, CD3, EMA and ALK-1. Chemotherapy resulted in durable remission status. This case emphasizes the occurrence of anaplastic large cell lymphoma in the soft tissue and the favourable outcome of ALK-positive anaplastic large cell lymphoma.

[Malignant degeneration of benign cystic teratoma of the ovary with synchronous mediastinal teratoma. A case report]. / Association synchrone d'un tératome de l'ovaire cancérisé associé à un tératome du médiastin. A propos d'un cas.

Squamous cell carcinoma arising in mature cystic teratoma of the ovary is an uncommon complication occurring usually in elderly woman. We present an unusual case of a post-menopausal woman with a synchronous mediastinum teratoma. While ovary tumor was symptomatic, mediastinal teratoma was an incidental finding. To the best of our knowledge, this association is the first to be reported in literature.

Desmoplastic spitz nevus: report of a case and review of the literature.

Desmoplastic spitz nevus (DSN) is an uncommon melanocytic lesion. The histologic features of this benign tumour may mimic those of certain benign (dermatofibroma and desmoplastic cellular blue nevus) or malignant (metastatic carcinoma and malignant melanoma) neoplasms. We report the case of a male with a DSN, and also review the clinical characteristics, histologic features and differential diagnosis of this extremely rare lesion. The lesion is identified as an asymptomatic reddish-brown dome-shaped papule on the forearm. Microscopic examination showed a symmetric lesion in the dermis. The neoplastic cells were large and epithelioid-shaped, and were either isolated as individual cells or arranged in small nests in a paucicellular hyalinized stroma. Nuclei were large and vesicular with small nucleoli and no mitoses. Diffuse expression of S100 and the absence of staining with antibodies to melan-A and HMB45 was observed. A diagnosis of DSN with free margins was made. The histologic presentation of this benign lesion mimics both benign and malignant neoplasms. The immunohistochemical profile (S100 positive, HMB-45 negative and cytokeratin negative) may be helpful to differentiate it from other lesions.

Invasive mucinous carcinoma arising within breast fibroadenoma.

Malignant neoplasms arising in the epithelial component of breast fibroadenomas are rare. The most frequent types are lobular and ductal intra-epithelial carcinomas, with a minority of infiltrating carcinoma. We report a case of 36-year-old patient with invasive mucinous carcinoma (30 x 30 mm) arising in a complex breast fibroadenoma (130 x 60 x 30 mm). The patient underwent mastectomy with dissection of the axillary lymph nodes, which were free of tumour. The patient is alive without disease five years later. To the best of our knowledge, this is the first report of an invasive mucinous carcinoma arising within breast fibroadenoma. Our case provides information about the clinicopathologic characteristic of this unusual tumour.

Smooth muscle differentiation in ovarian granulosa-cell tumours: a new case report.

Smooth muscle differentiation in stromal ovarian tissue has rarely been described in normal and tumoural ovaries, especially in granulosa-cell tumours. A moderately differentiated adult granulosa-cell tumour in an 83-year-old-woman is reported. Tumoural stroma included clusters of regular smooth muscle cells stained positively for smooth muscle actin. The presence of smooth muscle differentiation in an ovarian granulosa-cell tumour should be taken into consideration during diagnosis.

[An unusual renal neoplasm: mucinous tubular and spindle cell carcinoma]. / Une tumeur rénale à connaître: le carcinome tubulomucineux et fusiforme.

Mucinous tubular and spindle cell carcinoma is a rare and newly described type of renal cell carcinoma (RCC) with a relatively indolent behaviour. We report a case of 53-year-old woman who had macroscopic hematuria and flank pain. A right radical nephrectomy was performed showing a well circumscribed tumour confined to the kidney and measuring 17cm. The histologic findings consisted of tightly packed, small, elongated tubules separated by pale mucinous stroma. The tumour cells were cuboidal to spindled with eosinophilic cytoplasm and lower nuclear grade. Tumour was immunoreactive for cytokeratin CK 7, CK19 and epithelial membrane antigen (EMA). The morphological and immunohistochemical features were consistent with mucinous tubular and spindle cell carcinoma of kidney.

[Desmoplastic fibroblastoma of the foot]. / Le fibroblastome desmoplastique du pied A propos d'un cas.

Desmoplastic fibroblastoma is a benign, rare, slow-growing soft tissue tumor which is found in a wide anatomic distribution, predominantly in adult males. The characteristic gross aspect is that of a typical cartilage-like tumor which histologically presents regular fibroblastic proliferation, often in a stellar shape within a dense collagen or myxo-collagen stroma. We report a new case observed in the foot and study the anatomic and clinical aspects of this rare entity.

[Sclerosing epithelioid fibrosarcoma. A case report]. / A propos d'un cas de fibrosarcome épithélioïde sclérosant.

Sclerosing epithelioid fibrosarcoma is a rare variant of fibrosarcomas, which was recently identified as a separate entity due to specific histologic and immunohistochemistry features and its poor prognosis. We report a case of sclerosing epithelioid fibrosarcoma of soft tissues, which developed in a 37-year-old woman who presented a tumor involving the posteromedial aspect of the left knee and which progressed in size for one year. Imaging revealed a well-delimited tumor process measuring 8 cm in its largest diameter and situated in the medial compartment of the left knee. Histology of the tumorectomy specimen and the immunohistochemistry study led to the diagnosis of sclerosing epithelioid fibrosarcoma of soft tissues. This new case illustrates the characteristic features of this tumor and recalls the difficult pathological diagnosis.

[Orbital metastasis of liposarcoma]. / Métastase orbitaire d'un liposarcome.

INTRODUCTION: Liposarcoma, the most common soft tissue sarcoma in adults, rarely involves the orbit. Primary orbital liposarcomas are extremely rare, with less than 30 cases previously reported. Metastatic orbital liposarcomas are also extremely rare, with only a few cases documented in the literature. We report a new case of an orbital metastatic liposarcoma and review the literature. OBSERVATION: A 57-year-old man with a history 5 years before of myxoid liposarcoma on the right calf, completely surgically resected, was admitted for recent proptosis of the left eye. A computed tomographic scan revealed a heterogeneously enhancing, left orbital mass measuring 30x23x20 mm. The mass adhered to the internal medial muscle without extension to the optic nerve. A biopsy was taken, which showed typical aspects of myxoid liposarcoma. The patient underwent an orbital left exenteration after first refusing any proposed treatment. CONCLUSION: Liposarcoma metastatic to the orbit is exceptional. It should be suspected in a patient with exophthalmia caused by a space-occupying lesion and a history of liposarcoma.

Paraganglioma: report of a rare case with ovarian involvement.

We report a well-documented case of paraganglioma involving right ovary, which was initially misdiagnosed as a Sertoli-Leydig cell tumor and recurred one year later. The right ovarian tumor measured 105x90x60 mm and was associated to a subdiaphragmatic tumor measuring 80x60x35 mm, a peritoneal and a preureteral nodules measuring 10 mm either. Microscopically, tumor cells were arranged in trabeculae and cords separated by a delicate stroma. Their cytoplasm was abundant granular and eosinophilic. Their nuclei were enlarged and regular in size with coarse chromatine and a large nucleolus. The tumor expressed neuroendocrine markers (chromogranin, synaptophysin) epithelial membrane antigen and focally cytokeratin 7 and E-cadherin. Pathological ovarian paraganglioma diagnosis could be difficult but one should be aware of its bona fide existence. The clinical course is favourable in most of the cases.

Small cell malignant melanoma: an unusual morphologic variant.

Small cell melanoma is a recognized rare variant of malignant melanoma. We report a case of a brown, ulcerated papule on the left third finger of an 80-year-old woman. Microscopic examination revealed the presence within the epidermis of diffuse sheets of monomorphic small to medium-sized cells. The nuclei were round or oval, and hyperchromatic with inconspicuous nucleoli. Melanin pigment was either absent or minimal. This case report draws attention to the difficulties encountered in the histological diagnosis of this rare variant of malignant melanoma.

Myxoid tumor of soft tissue.

Focal myxoid change is a well-recognised feature of synovial sarcoma, but the presence of a predominantly myxoid stroma is rare. We describe a new case of myxoid synovial sarcoma in which marked myxoid change initially obscured the diagnosis leading to confusion with malignant peripheral nerve sheath tumor. The patient was a 16 year old man who presented with a left dorsal foot tumor. The diagnosis of synovial sarcoma was suspected on histological and immunohistochemical studies and confirmed with cytogenetic analysis. Recognition of this rare histologic variant of synovial sarcoma is important because it can easily be mistaken for other myxoid spindle cell neoplasms, potentially resulting in suboptimal therapy.

[Conjunctival dirofilariasis due to Dirofilaria Repens. A new Tunisian case]. / Dirofilariose conjonctivale à Dirofilaria repens. A propos d'un nouveau cas tunisien.

INTRODUCTION: Dirofilariasis due to Dirofilaria repens is a parasitic disease affecting domestic animals and transmitted to man by mosquito bites. Human infestation is rare and involves benign subcutaneous and ocular manifestations. The diagnosis is made by excisional biopsy, which is also the sole recommended treatment. There are few reports of human infection in Tunisia. CASE REPORT: We report herein the second case of conjunctival dirofilariasis in a 21-year-old woman residing in Tunis (Tunisia). The clinical presentation evoked orbital cellulites. Clinical and radiological investigations did not provide a diagnosis. Surgical excision and histological examination established the diagnosis by the presence of an adult female worm identified as Dirofilaria repens. Parasite extraction allowed successful treatment. CONCLUSION: Dirofilariasis is rare in Tunisia, with only seven cases reported. The true incidence of dirofilariasis is probably greater than recognized. This can be explained by the apparent benign nature of the lesions that may not warrant excision.

[Soft tissue epithelioid hemangioendothelioma: a case report]. / Hémangio-endothéliome épithélioïde des tissus mous. A propos d'un cas.

We report a case of epithelioid hemangioendothelioma observed in soft tissue in a 35-year-old man who presented a painful mass of the right arm which progressed in size for two years. Imaging revealed a 7-cm poorly limited expansive tumor process located in the medial muscle compartment of the right arm. Surgical resection was performed. Histological and immunohistochemical examination led to the diagnosis of soft tissue epithelioid hemangioendothelioma. The tumor recurred in the right arm and the right axillary fossa with development of multiple nodules in the contralateral thoracic wall and pleural effusion. Adjuvant chemotherapy and radiotherapy were performed, but the patient died at two years.

Micropapillary transitional cell carcinoma of the urinary bladder: report of two cases.

Micropapillary carcinoma is an uncommon variant of urothelial carcinoma with apparent high metastatic potential. The reported cases in the literature were associated with high grade and stage of disease at presentation and a poor prognosis. Micropapillary carcinoma is considered a tumor with an aggressive behavior, even though the morphology may be deceptive. The presence of a micropapillary carcinoma component in bladder biopsies should alert the urologists to the potential of higher stage disease and deep biopsies should be obtained. Two cases of micropapillary carcinoma of the urinary bladder were presented. A 71-year-old woman and a 68-year-old man presented with urinary symptoms. Cystoscopy revealed a papillary tumor on the bladder wall in both cases. Pathologic examination of transurethral resection specimen showed an invasive micropapillary carcinoma; small solid nests lying in small clear spaces that were not stained with antibody CD34. Thus, the lacunar histological pattern did not appear to represent invasion of vascular spaces. Only one case showed an association with urothelial carcinoma. No case showed muscle invasion. No recurrence or metastasis were observed after the initial diagnosis in the two cases.

[Epithelioid hemangioendothelioma of bone complicated by femoral fracture]. / Hémangio-endothéliome épithélioïde osseux compliqué d'une fracture du fémur.

A 54-year-old man was seen with a fracture of the left femur. Plain radiographs revealed a 40-mm lytic centromedullary lesion. Magnetic resonance T1- and T2-weighted sequences showed high and low signals. After stabilization of the fracture, the tumor was removed followed by reconstruction with a vascularized fibula. The pathological examination demonstrated proliferation of non atypical CD34 and CD31 positive epithelioid cells with few lumens, accompanied by abundant fibrous stroma, sometimes masking tumor cells. Satisfactory motion was achieved with no recurrence at 20 months follow-up. Bone hemangioendothelioma can simulate metastasis and must be distinguished by immunohistochemistry. Prognosis is a subject of debate as the tumor is considered to exhibit intermediate malignancy by some authors while other consider it to be a malignant tumor.

[Fatal postoperative crisis in acute hereditary porphyria]. / Crise post-operatoire fatale d'une porphyrie aiguë héréditaire.

The acute porphyria is an autosomal dominant disorder of the héme biosynthesis enzyme. The aim of this work is to determin a diagnostic step in order to anticipate porphyric crises. We report the observation of a child which was hospitalized in the pédiatric service three years ago for peripheric arthralgies and myalgies where the diagnosis of an acute porphyria was not posed. He wase admitted in our service for appendicitis, he was operated, the anesthesie was carried out by the thiopenthal and the succinylcholine. At the first post-operative day, a respiratory insufficiency was the cause of death. The rate of coproporphyrines and of uroporphyrines in the urine had confirmed the diagnosis of acute porphyria. The positive diagnostic of acute porphyria is difficult, the association of abdominal, psychological and neurologic signs must suggest the diagnostic, all the more, if the urines have a dark colour. The presence of uroporphyrines and coproporphyrines and certain precursors in the urines or in the high-rated stools confirm the diagnostic. The family survey and dosage of porphyric compounds in the sickness-bearing patient's family members allow to reveal certain asymptomatic forms of the heriditary acute porphyria, it is caused by certain products used in neuropsychiatry, in aneasthesic or in certain circunstances, such as, the infection or the trauma. The treatment of the acute porphyria is an emergency wich requires the transfert of the patient to a unit of intensive care.