Saloua Krichen Makni

Pigmented lipofibromatosis in unusual location: case report and review of the literature.

Dear Editor, Lipofibromatosis is a rare, recently reported, fibrofatty pediatric tumour which affects mainly extremities [1, 6]. Histopathological features of this tumour must be distinguished from fibrous hamartoma of child, fibromatosis, calcifying aponevrotic fibroma and lipoblastoma [1]. Pigmented lipofibromatosis is a rare histological variant of lipofibromatosis which is not yet clearly distinguished. We describe a case of pigmented lipofibromatosis in an unusual location (back). We report the case of a 14-year-old female who presented with several years history of a slowly enlarging, painless mass of the back. Physical examination demonstrated a subcutaneous, firm, nodule measuring 3.5 × 2.5cm. The nodule seems to be fixed at deep tissues without abnormality of the overlying skin. She was otherwise healthy and no family history of cutaneous abnormalities was noted. Any radiological exam was done. An excisional biopsy was performed. A 4 × 3 × 3cm ill-defined mass was excised. Grossly, the tumour presented an adipose appearance without encapsulation. The cut surface presented some black pigmented areas. Hematoxylin and eosin stained sections showed small lobules of mature adipose tissue, dissected by moderately cellular bundles, of spindle elements without atypia (Fig. 1). Some scattered cells were pigmented (Fig. 2) and Fontana–Masson stain was positive on these pigmented cells. On immunohistochemistry study, spindle cells were diffusely positive for vimentin and α smooth muscle actin (Fig. 3). Pigmented cells were positive for S100 protein and Melan A and negative for human melanoma black (HMB)-45 (Fig. 4). At 15 months of follow-up, there was no evidence of recurrence. Lipofibromatosis is a distinctive entity of rare benign lesion recently described by Fetsch et al. [1, 4] in 2000 and recognised as a distinctive fibrofatty tumour of childhood in the World Health Organisation classification of tumours in 2002. Since this report, which includes 45 cases, only four other cases of lipofibromatosis were published [1–6]. Lipofibromatosis is different and must be equally distinguished from the entity designated as “lipofibromatosis,” observed in cases of congenital macrodactyly and local gigantism [1]. The tumour commonly occurs in the extremities, rarely in the hands, arms, legs and feet [1, 6]. Chest, abdomen or head and neck are rarely affected [1–5]. The present observation is the first report of lipofibromatosis in the back. There is an over 2:1 male predominance. The median age for initial surgery is 1 year (range from 11 days to 12 year) [1]. The lesion is typically ill defined, painless and slow growing [1]. Macroscopically, it is yellowish or tan–whitish in appearance with a prominent fatty component. Very rare tumour have exceeded 5 cm [1, 3–6]. Histologically, the tumour is poorly marginated, often composed of more than 70% of adipose tissue. Fascicles of Virchows Arch (2008) 452:465–467 DOI 10.1007/s00428-007-0565-6

Cystic undifferentiated sarcoma of liver in children: a pitfall diagnosis in endemic hydatidosis areas

Undifferentiated (embryonal) sarcoma (UES) of the liver is a very rare pediatric neoplasm with an aggressive behavior. Multimodal treatment including complete surgical resection and chemotherapy has improved survival. We report a case of UES occurring in a 15-year-old female. Radiographic findings showed a multicystic mass appearance. The diagnosis of hydatid cyst was initially presumed. A complete tumor resection after 3 cycles of chemotherapy was performed. Histologically, only 20% of tumor necrosis was noted. Adjuvant chemotherapy was instituted. She remained well at 11 months of follow-up. Cystic appearance is a usual finding in UES. Differential diagnosis with hydatid cyst may be problematic in endemic hydatidosis areas.

Gliosarcoma with osteosarcomatous features: a short illustrated review

IntroductionGliosarcoma is a rare, malignant, biphasic brain tumor formed by both glioblastoma and sarcomatous components. Various lines of differentiation are described in the latter component, but most commonly fibrosarcomatous and pleomophic sarcoma are present. Osteosarcomatous features are exceedingly rare.ObjectiveWe report a case of gliosarcoma with osteosarcomatous features in a 33-year-old woman.MethodsHistologically, the sarcomatous portion displayed a typical pattern of fibrosarcoma associated with areas of osteoid formation.Results and conclusionImmunohistochemical glial fibrillary acid protein (GFAP) expression was seen only in the glioblastoma portion. Clinicopathological characteristics and radiological data of this rare condition were reviewed. Possible differential diagnoses and potential histogenesis were also discussed.

Hidradénocarcinome développé sur un hidradénome préexistant

ne femme, âgée de 60 ans, avait depuis deux ans, ur le genou droit, un petit nodule non douloureux, ans signes inflammatoires locaux. Elle consultait devant ’augmentation récente de la taille du nodule (4 cm de iamètre) et l’apparition d’une ulcération cutanée en egard. L’échographie des parties molles montrait le caracère solide et kystique du nodule. L’étude histologique e la pièce d’exérèse chirurgicale montrait une proliféraion cellulaire intradermique disposée en nappes lobulées ar de fins septa fibreux, hyalinisées et creusées de avités pseudoglandulaires parfois dilatées kystiques. Les ellules tumorales étaient de taille moyenne, cuboïdes, à ytoplasme faiblement éosinophile ou clarifié et à noyau égulier sans atypie. D’autres cellules étaient plus grandes,

Secondary oxalosis due to excess vitamin C intake: a cause of graft loss in a renal transplant recipient.

Renal oxalate deposition can be seen with primary hyperoxaluria, malabsorptive states, ethylene glycol toxicity and, rarely, with excessive vitamin C ingestion. We report a case of secondary hyperoxaluria in which the diagnosis was not considered initially because there was no past history of urinary calculi and no evidence of nephrocalcinosis on plain X-ray of the abdomen and ultrasonography. The disease was detected and diagnosed only after kidney transplantation. Secondary oxalosis can cause graft loss or delayed graft function. Biopsy of the allograft should be carefully examined for oxalate deposits even in the absence of a family history. When oxalosis is diagnosed, intensifying hemodialysis (HD) to eliminate calcium oxalate can help in the recovery of renal function in some cases. Systematic vitamin C supplementation in HD patients should be avoided as it can be a cause of secondary oxalosis.

Fiche thématique/HistologieHidradénocarcinome développé sur un hidradénome préexistantHidradenocarcinoma arising from pre-existing hidradenoma

ne femme, âgée de 60 ans, avait depuis deux ans, ur le genou droit, un petit nodule non douloureux, ans signes inflammatoires locaux. Elle consultait devant ’augmentation récente de la taille du nodule (4 cm de iamètre) et l’apparition d’une ulcération cutanée en egard. L’échographie des parties molles montrait le caracère solide et kystique du nodule. L’étude histologique e la pièce d’exérèse chirurgicale montrait une proliféraion cellulaire intradermique disposée en nappes lobulées ar de fins septa fibreux, hyalinisées et creusées de avités pseudoglandulaires parfois dilatées kystiques. Les ellules tumorales étaient de taille moyenne, cuboïdes, à ytoplasme faiblement éosinophile ou clarifié et à noyau égulier sans atypie. D’autres cellules étaient plus grandes,

BK virus-associated hemophagocytic syndrome in a renal transplant recipient

Hemophagocytic syndrome (HPS) is a life-threatening hematological disorder in immunocompromised patients. Reactive HPS is observed in patients with systemic infection, neoplasia or auto-immune diseases. It is a rare hematological disorder after renal transplantation and must be suspected when fever and pancytopenia are seen in association with viral infections. HPS is usually associated with infection with the Cytomegalovirus and Epstein-Barr viruses. We report here a case of BK-virus-associated HPS.

Métastase ovarienne d’un carcinome bronchique

INTRODUCTION The ovary, being a richly vascular organ, may receive metastases from numerous tissues, particularly of gynecologic origin. Extragenital cancers are dominated by gastro-intestinal and breast tumors. The lung is exceptionally described as a primary site of ovarian metastasis. CASE REPORT We report a 28-year-old woman who was found to have a right perihilar opacity. The diagnosis of small cell bronchial carcinoma was made by bronchial biopsy. A right-sided uterine mass was also identified. This was resected and histopathology identified it as an ovarian metastasis from the pulmonary tumor. CONCLUSION We report, through this case, and present a review of the literature on ovarian metastasis originating from bronchopulmonary neoplasms.

Alport syndrome and diffuse leiomyomatosis

Review on Alport syndrome and diffuse leiomyomatosis, with data on clinics, and the genes involved.

Fibrome desmoïde mandibulaire : difficultés diagnostiques et thérapeutiques

INTRODUCTION The desmoplastic fibroma is a rare bone tumor, characterized by aggressive local infiltration, with frequent recurrence. The most common site is the mandible. Radioclinical signs are not specific and the histological diagnosis may be difficult. CASE A 16 year-old male patient consulted for a painless and hard left mandibular swelling, without inferior alveolar nerve disorders. The tumor extended from tooth 31 to tooth 35, the mucosa was healthy. The panoramic view showed a multilocular osteolytic lesion with dental root resorption. CT scan showed expansion of bony cortex with rupture of the outer cortical. The biopsy indicated a desmoplastic fibroma or a fibrosarcoma. A second histological analysis combined with an immuno-histochemical study proved the diagnosis of desmoplastic fibroma. DISCUSSION The desmoplastic fibroma has a polymorphous symptomatology. Radiological signs are unspecific. Anatomopathology combined with immuno-histochemistry can prove the diagnosis and guide the treatment.