[Pure colloid carcinoma of the breast: anatomoclinical study of seven cases]. / Carcinome colloïde pur du sein : étude anatomoclinique de sept cas.

PURPOSE: Breast mucinous carcinoma is a particular histological form characterized by the extracellular production of mucus. The pure form is rarely reported and its prognostic is better than the other types of the breast cancer. The aim of the present study was to discuss the various anatomoclinical, therapeutic aspects and the prognostic factors of the pure mucinous carcinoma of the breast. PATIENTS AND METHODS: We report seven cases diagnosed over a period of 11 years (1993-2003) in the laboratory of anatomy and pathological cytology of the university hospital of Sfax. A review of the clinical files with immunohistochemistry study (hormonal receptor, synaptophysine, chromogranine, Bcl2, Ki67, P53 and Her-2/neu (C-erbB-2)) were carried out for all the cases. RESULTS: pure colloid carcinoma of the breast accounted for 0,5% of the whole of the breast cancers. The average age of patients was 69 years. At the time of the diagnosis, four tumours were classified T4, one T3, two T2, two N1 and one N3; no patient had presented metastasis (M0) according to pTNM classification. The echomammography showed regular contours mass in four cases. The anatomopathological study showed that the neuroendocrine differentiation was found in two cases, all the tumours had presented positivity for the hormonal receptors (oestrogen and progesterone) and negativity for the other antibodies, two cases exhibited the Bcl2 positivity and negativity for the other antibodies. An adjuvant radiotherapy was carried out for all the patients. The average duration of follow-up was of 33 months; the evolution was marked by the occurrence of metastases in two cases. CONCLUSION: The pure colloid carcinoma of the breast constitutes a particular morphological entity which deserves to be individualized; neuroendocrine differentiation of these tumours must be required. Their forecast is relatively favourable.

[Mucinous cystadenoma of the kidney]. / Le cystadénome mucineux du rein.

Mucinous tumors of the kidney are rare. The pathogenesis of these lesions is controversial. We report the observation of a 31-year-old woman presented with right-flank pain and hematuria. Abdominal ultrasonography and computed tomography revealed a multicystic mass of the right kidney. A total nephrectomy was performed. The pathological exam concluded on a mucinous cystadenoma of the right kidney. Mucinous cystadenoma of the kidney with a multicystic presentation has not been previously reported. In this work, we present the different pathogenic hypothesis and the means of treatment of this rare entity.

[Frantz's tumor: anatomoclinical study of six Tunisian cases]. / La tumeur de Frantz: etude anatomoclinique de six cas tunisiens.

Solid and pseudopapillary tumour (Frantz's tumour) is a rare low-grade neoplasm of the pancreas. We report six new cases. Our objective is to specify clinical and pathological characteristics of this rare neoplasm and to discuss its histogenesis. A retrospective review was considered on six Tunisan patients who had solid and pseudopapillary tumor of the pancreas. A review of medical registries and morphological analysis with immunohistochemical study were carried out in all cases. Four patients were female and two patients were male with a median age of 27,5 years (range: 14 - 68 years). Abdominal pain was the most common initial symptoms (5 cases/6). Abdominal computed tomography and/or ultrasonography was used in all the cases. The tumour was in the tail of the pancreas in 4 patients and in the body of the pancreas in one patient; one tumor involved all the pancreas. The median diameter of the tumour was 16,8 cm (range: 8 - 35 cm). Three tumours had an extrapancreatic extension. All patients underwent surgical resection. No adjuvant therapy was recommended. The mean follow up period was 24 months (range: 5 - 78 months). Only one patient died during the surgery. Except for this patient, none experienced tumor recurrence or tumor-related mortality during the follow up period. Solid and pseudopapillary tumour of the pancreas is an uncommon neoplasm which shows distinct clinicopathologically characteristics. Despite diverse studies, its histogenesis remains undetermined. This tumor should be distinguished from other pancreatic neoplasms because its prognosis is excellent after surgical resection.

[Mammary hydatid cyst diagnosed by fine needle aspiration]. / Kyste hydatique du sein diagnostiqué par cytoponction.

INTRODUCTION: Echinococcal involvement of the breast is extremely rare and usually not included in the differential diagnosis of breast lumps. OBSERVATION: This is an unreported case of a 56-year-old woman who came with a 3 cm mammary mass. Mammography showed a dense well-circumscribed lesion with calcifications. The diagnosis was established by fine needle aspiration and confirmed by pathological study. CONCLUSION: Hydatid cyst of the breast is rare. The diagnosis by fine needle aspiration is controversial. The treatment is based on complete excision of the cyst.

[Rhabdoid tumours of the central nervous system: five case studies]. / Les tumeurs rhabdoïdes du système nerveux central: à propos de cinq observations.

PURPOSE: The rhabdoid cerebral tumors were first identified by Briner et al. in 1985. Their frequency was estimated to be 2.1% of those affecting children under 18 months. Such tumors are equally characterized by a critically and speedly mortal development. Their historical genesis is still of a controversial issue. The aim of the present study was to discuss the various anatomicoclinical and therapeutic aspects of these rare tumors. PATIENTS AND METHODS: We report five cases diagnosed over a period of eight years (1997-2004) in the Laboratory of Anatomy and Pathological Cytology of the University Hospital of Sfax. RESULTS: The average age of patients was 20 years old, there were children under 14 years and 4 patients were male. Clinical symptomatology showed the predominance of intra-cranial hypertension syndrome. Radiography through magnetic resonance revealed a heterogeneous tumor process localized respectively on the spine (one case), the insula (one case), the temporofrontal lobes (two cases) and the medulla (one case). Histological examination of the tumors also showed a proliferation of giant cells with a hyaline-based cytoplasmic inclusion. These inclusions were positive for vimentin and keratin. An adjuvant radiotherapy treatment was prescribed for two patients. A recurrence of rhabdoid tumors occurred in two cases. All five patients died within eighteen months. CONCLUSION: The cerebral rhabdoid malignant tumor constitutes one of the most aggressive and life-threatening intracranial tumors. The optimal management of such tumors remains unknown.

Telangiectatic osteosarcoma of the rib: a rare entity and a potential diagnostic pitfall.

Osteosarcoma (OS) is a common primary malignant tumor of bones that produces osteoid matrix. Telangiectatic osteosarcoma (TOS) is a rare variant of OS. It affects the long bones especially the lower end of femur and the upper ends of tibia and humerus, a distribution similar to the conventional osteosarcoma. The rib involvement is very infrequent. We present a case of TOS of the rib that posed a diagnostic difficulty owing to its unusual location and to its resemblance to giant cell tumor and aneurysmal bone cyst. Correspondence.

[Malignant struma ovarii: a case report]. / Goitre ovarien malin: une observation.

Malignant transformation of struma ovarii is exceptional (less than 1%). The histolological diagnosis of malignancy is difficult especially in the well differentiated forms. Immunohistochemistry is highly contributive in the anaplastic forms. The prognosis is relatively favorable except for the metastatic and undifferentiated forms. We report a case of malignant struma ovarii with metastasis observed in a 65-year-old woman who died rapidly from her disease. In light of this observation, we discuss the diagnositic, management and outcome features of these particular tumors.

Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension.

INTRODUCTION: Cervical locations of malignant peripheral nerve sheath tumor (MPNST) are rare, at less than 1% of malignant tumors of this region. CASE REPORT: A 53-year-old woman presented with a lateral cervical swelling involving the parotid region. Histology was in favor of MPNST. Adjuvant radiotherapy was indicated because of the infiltrating nature of the tumor. At 2 years' follow-up, there was no recurrence. DISCUSSION: Clinical diagnosis is difficult in cervical MPNST. Only histology with immunohistochemistry can establish the correct diagnosis. Treatment requires complete surgical resection and regular clinical follow-up.

An unusual tumour of the lung.

We report a case of a 51-year-old woman with a solitary mast cell tumour of the lung, a rare neoplasm with only three previously-reported cases reported in the literature. Unlike previous cases, the tumour in the present case was bulky, measuring 14 cm in diameter and budding into the segmental bronchus. Histologically, it showed proliferation of typical metachromatic mast cells intermingled with undifferentiated cells with a ratio of 3:1. The neoplastic mast cells stained strongly with tryptase, CD117, CD68 and CD45, CD14 and CD33; whereas the undifferentiated cells lacked all these markers and expressed EMA and cytokeratin. Histological examination of bone marrow and laboratory data were unremarkable. To our knowledge, this is the fourth case of solitary extracutaneous mastocytoma of the lung. The differentiating features of this neoplasm and a review of literature are presented.

[Dientamoeba fragilis: pathogenic flagellate?]. / Dientamoeba fragilis: flagelle pathogène?

INTRODUCTION: D. fragilis is an intestinal protozoa whose pathogenic characteristics are increasingly recognized. The aim of this study is to specify the epidemiologic, biological and clinical aspects of this protozoa. MATERIAL USED AND METHODOLOGY: Survey conducted on 27,058 parasitological test of stools in parasitology-mycology laboratory of the Sfax University Hospital over a period of 5 years. RESULTS: 11,254 parasitological test of stools were positive (41.6%) of which 89.3% comprised protozoa. D. fragilis was found in 1497 cases (13.3% of the positive cases). In 65% of these cases, it was associated with other intestinal parasites in particular Blastocystis hominis (40.3%), Endolimax nanus (24%), Entamoeba coli (6%), Giardia intestinalis (5.7%) and Enterobius vermicularis (5%). Those patients having a parasitism with isolated D. fragilis were predominantly female and young subjects (< 20 years). Clinical signs included abdominal pain (88.5%), anorexia (50%), alternating diarrhoea and constipation (40.4%) and diarrhoea (21%) with mucus in 7.6%. DISCUSSION: D. fragilis is today classified in the group of flagellates and we share the opinion of the majority of the authors as to its real pathogenic capacity.

[Malignant mesothelioma of the vulva. About one case]. / Mésothéliome malin de la vulve. A propos d'un cas.

Most malignant mesotheliomas are pleural; they rarely occur within the peritoneum and their location in the vulva is exceptional. A case of a malignant mesothelioma of the vulva is reported in a 47-year-old woman. The patient was operated for a tumour of the vulva. The preoperative diagnosis was that of a cyst of the Bartholin's gland or a fibroma. The microscopic and immunohistochemical exam confirmed the diagnosis of a biphasic malignant mesothelioma of the vulva. The computed tomography scan of the chest and the abdomen was normal. A local recurrence of the tumour occurred in spite of the postoperative radiotherapy. Our objective about this particular case is to discuss the clinical and pathologic aspects and the diagnostic problems of this exceptional tumour of the vulva.

[Solid cystic papillary tumor of the pancreas]. / Tumeur papillaire solide et kystique du pancréas.

Solid cystic papillary tumors of the pancreas are rare; they occur most commonly in young women. Despite their characteristic microscopic appearance, their immunophenotype is not specific. Their prognosis is excellent after complete surgical resection. The study aim was to report two cases in female patients who were 15 and 20 years old; the first tumor was discovered fortuitously and the second girl presented with abdominal pain and vomiting. Both tumors were encapsulated and located in the tail of the pancreas. The histological study showed the papillary architecture mixed with solid areas. Immunohistochemical staining was positive only for vimentin in one case and positive for cytokeratin, chromogranin, synaptophysin, neuron specific enolase, vimentin and protein S100 in the second case.

[Colonic granulocytic sarcoma: a case report]. / Sarcome granulocytique colique: une observation.

Granulocytic sarcoma is a rare tumor composed of immature cells of the granulocytic series which usually occurs as a secondary manifestation of acute leukaemia. We report the case of a 60 years old woman without particular previous pathologies who was hospitalised for chronic diarrhea developed in a context of health impairment state. The blood cell count revealed severe leucopenia and thrombopenia; an emergency right colectomy was accomplished. The histologic examination showed granulocytic sarcoma of the ascending colon. The death occurred rapidly as a consequence of a toxic shock. This observation seems to be the sixth case report of the granulocytic large bowel sarcoma in the literature which likely complicated a pre-existant and unknown myeloid leukaemia.

[Giant-cell tumor of the patella with lung metastases: a case report]. / Tumeur à cellules géantes de la rotule avec métastases pulmonaires: à propos d'un cas.

Giant-cell tumors are an infrequent clinical, radiological, and pathological entity observed in 5% of primary bone tumors. They generally occur at the epiphysis of long bones, particularly in the knee area but patellar localization seems very rare. Despite their perfectly benign histological aspect, giant-cell tumors may be aggressive, leading to local recurrence or even distant metastasis to the lung. We report a case of benign giant-cell tumor of the patella with lung metastasis observed in a 23-year-old woman. The aggressive radiological image was suggestive of chondrosarcoma. Histologically the differential diagnosis with chondroblastoma was difficult. The tumor and lung metastasis were treated by surgical resection. Four years later there has been no recurrence. We present the anatomic and clinical aspects of giant-cell tumor of the bone together with the diagnostic approach and the clinical course.

[Verrucous carcinoma of the kidney: report of 2 cases]. / Carcinome verruqueux du rein: à propos de deux cas.

The verrucous carcinoma is an unusual shape of well differentiated squamous cell carcinoma, first described at the ORL region; the kidney location is rare; the risk factors are represented essentially by lithiasis and/or urinary infection; the clinical symptom is not specific. Diagnosis is facilitated by radiological investigations and particularly excretory urogram/ultrasound; certainly diagnosis is pathological. The nephro-ureterectomy with collar resection of the bladder is the choice treatment. We report two observations and we clarify clinicopathological aspects of this type of carcinoma and we discuss the prognosis.

[A rare tumor of the parapharyngeal space: myxoid chondrosarcoma]. / Un cas rare de tumeur parapharyngée: chondrosarcome myxoïde.

Extrasqueletal myxoid chondrosarcoma (EMC) is an uncommon soft tissue malignant tumor, locally aggressive with a high incidence of distant metastasis. It has distinctive clinical, immunophenotypic, cytogenetic and ultrastructural features. Most EMC are associated with the translocation t(9;22) (q22;q12). Their occurrence in the parapharyngeal space is extremely rare. Our objective is to discuss the difficulties of the histological diagnosis of EMC and to describe its immunophenotypic, cytogenetic features and clinical behavior. We report a case of a 67 years old woman who presented with a five months history of dysphagia. The oral examination found a mass displacing the posterior and left walls of the pharynx. Surgical resection of the tumor was undertaken. The pathologic examination concluded to the diagnosis of an EMC of the left parapharygeal space. Now, the patient is receiving an adjuvant radiotherapy.

Type 2 autoimmune hepatitis overlapping with primary sclerosing cholangitis in a 10-year-old boy.

BACKGROUND: Overlap syndrome of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) is considered when the patient presents with the diagnostic criteria of both diseases at some stage of the medical history, either simultaneously or consecutively. AIM: To report on a new case of overlap syndrome and describe the clinical presentation, progression, radiological studies, histological characteristics, and therapeutic options of this rare association. CASE REPORT: A 10-year-old boy presented with jaundice and hepatosplenomegaly. Levels of plasma aminotransferases, gamma-glutamyl transferase, serum alkaline phosphatase and gammaglobulins were elevated. Anti-liver cytosol and perinuclear antineutrophilic cytoplasmic antibodies were positive. Liver biopsy showed features of interface hepatitis with ductopenia. Magnetic resonance cholangiography revealed bile duct stenosis and dilations. Serological findings associated with radiological and histological features confirmed the diagnosis of overlap syndrome of AIH with PSC. Treatment with prednisone, azathioprine, and ursodeoxycholic acid led to a good response. CONCLUSION: The possibility of AIH-PSC overlap syndrome should be considered in all children with AIH and, with clinical, biochemical, or histological signs of PSC, complementary investigations should be done to confirm the diagnosis so as to urgently initiate appropriate treatment with immunosuppressive medication and ursodeoxycholic acid.

[Desmoplastic fibroma of the skull in an infant]. / Fibrome desmoplastique du crâne chez un enfant.

Desmoplastic fibromas are uncommon osseous tumors that seldom involve the skull. These tumors are histologically benign but locally aggressive with a propensity for recurrence if resection is partial. To our knowledge, only 16 cases have been reported in the literature, seven of which concerned children. We report a further case of a desmoplastic fibroma of the skull in a 3-year-old boy who presented with a right parietal mass. The CT scan showed a lytic mass with brain compression and cortical destruction. The patient underwent a craniectomy and complete mass resection. Histological diagnosis was desmoplastic fibroma. Postoperative progress was normal without recurrence 6 months later.