Naourez Gouiaa

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

Leiomyosarcoma of the Kidney

Case Report A 65-year-old woman with arterial hypertension was admitted to our hospital with a presentation of intermittent right flank pain. Physical examination revealed a firm, mildly tender mass in the right lumber region. Laboratory data were within normal limits. Abdominal ultrasonography showed a large heterogeneous mass 13 11 cm. A computed tomographic scan revealed a large right renal solid ass 13 11 10 cm, with necrotic changes infiltrating the perienal fat without any vascular or hepatic metastasis (Figure 1). Radical nephrectomy was performed and a well-encapsulated ass confined to the right renal parenchyma was seen at macroscopic xamination. The renal artery and vein were free of tumor. There was o lymphadenopathy.

Sarcome intimal de l’aorte : une observation

Primary aortic tumours are very rare. To our knowledge, only 120 cases had been reported in the world literature. Their clinical presentation is not specific. They are usually diagnosed at aortic surgery or by post-mortem examination. Intimal sarcoma is the most frequent histological type. We report a case of intimal sarcoma of the thoracic aorta diagnosed on autopsy of a 48-year-old man victim of sudden death. The main problem raised by intimal sarcoma lies in its poor differentiation or lack of differentiation. Thus, the pathologist must perform a phenotypic analysis as complete as possible of this tumor.

Sarcomatoid Collecting-Duct Carcinoma of the Kidney

Collecting duct carcinoma is a distinct entity among renal cell carcinoma, which is characterized by an aggressive behavior. Sarcomatoid variants of this tumor are rarely described. We report the case of a 62-year-old woman who presented with a left kidney tumor. A radical nephrectomy was performed. The tumor was firm with a white appearance, located in the medulla with infiltrating margins. Pathological examination showed a biphasic tumor with carcinomatous areas consisting of a collecting-duct carcinoma and sarcomatous areas with malignant fibrous histiocytoma-like appearance. Immunohistochemical studies confirmed the diagnosis of sarcomatoid collecting-duct carcinoma. The patient died 8 months after the diagnosis.

Cas anatomocliniqueSarcome intimal de l’aorte : une observationIntimal sarcoma of the aorta: A case report

Primary aortic tumours are very rare. To our knowledge, only 120 cases had been reported in the world literature. Their clinical presentation is not specific. They are usually diagnosed at aortic surgery or by post-mortem examination. Intimal sarcoma is the most frequent histological type. We report a case of intimal sarcoma of the thoracic aorta diagnosed on autopsy of a 48-year-old man victim of sudden death. The main problem raised by intimal sarcoma lies in its poor differentiation or lack of differentiation. Thus, the pathologist must perform a phenotypic analysis as complete as possible of this tumor.

Adénome tubulo-papillaire apocrine associé à un syringocystadénome papillifère

Tubulo-papillary apocrine adenoma (TAA) is a very rare sweat gland tumor. TAA in association with syringocystadenoma papilliferum (SCP) is exceptional. A 2-year-old Tunisian child developed a mixed tumor on the scalp: TAA in association with SCP. Histologically, the tumor consisted of dilated duct-like areas with some apocrine gland-like areas. The superficial part of the tumor was connected to the epidermis and showed the characteristics of SCP. The characteristics and differences in histopathologic and immunohistochemical findings in this mixed tumor are described.

Cas anatomocliniqueAdénome tubulo-papillaire apocrine associé à un syringocystadénome papillifèreTubulo-papillary apocrine adenoma in association with syringocystadenoma papilliferum

Tubulo-papillary apocrine adenoma (TAA) is a very rare sweat gland tumor. TAA in association with syringocystadenoma papilliferum (SCP) is exceptional. A 2-year-old Tunisian child developed a mixed tumor on the scalp: TAA in association with SCP. Histologically, the tumor consisted of dilated duct-like areas with some apocrine gland-like areas. The superficial part of the tumor was connected to the epidermis and showed the characteristics of SCP. The characteristics and differences in histopathologic and immunohistochemical findings in this mixed tumor are described.

Carcinome adénoïde kystique primitif du poumon : à propos d’un cas

IntroductionPrimary adenoid cystic carcinoma of the lung is an uncommon disease, accounting for only 0.09–0.2% of all lung cancers. The speed of growth is slow and the clinical course is relatively long, so it is generally considered tobe a low-grade malignancy.Case reportA 30-year-old man was admitted to hospital for investigation of haemoptysis. Bronchofibroscopy showed a polypoid tumour in the left lower bronchus. A CT scan of the chest showed that this tumour measured 4 cm in diameter and extended to the left lower pulmonary vessels. A complete excision was achieved by a left total pneumonectomy. The diagnosis of adenoid cystic carcinoma was made.DiscussionDiagnosis of this unusual tumour is confirmed by pathologic examination. Its optimal treatment is a complete surgical resection whenever possible. However, postoperative radiotherapymay be the treatment of choice to control residual lesions and provide long-term survival in the case of incomplete resection.RésuméIntroductionLe carcinome adénoïde kystique primitif du poumon est une tumeur maligne rare, de bas grade, repré sentant 0,09 à 0,2 % de tous les cancers pulmonaires. Elle est d’évolution longtemps locale, mais tend à s’infiltrer le long des voies aériennes.ObservationIl s’agit d’un homme âgé de 30 ans, hospitalisé pour hémoptysie de faible abondance. La fibroscopie bronchique avait révé lé une formation tumorale de la bronche lobaire inférieure gauche. À la tomodensitométrie thoracique, la tumeur mesurait 4 cm de diamètre, engainait l’artère pulmonaire inférieure gauche et les branches de la veine pulmonaire inférieure gauche. Le traitement était purement chirurgical. Le diagnostic retenu était celui de carcinome adénoïde kystique primitif du poumon gauche.DiscussionLe diagnostic du carcinome adénoïde kystique du poumon est anatomopathologique; son traitement est essentiellement chirurgical. La radiothérapie est indiquée quand la chirurgie est incomplète ou impossible.

Une tumeur gastrique d'aspect inhabituel

Un homme, âgé de 51 ans, sans antécédent pathologique particulier, avait consulté pour des épigastralgies postprandiales associées à un amaigrissement récent de 10 kg ; la fibroscopie œsogastroduodénale avait montré une perte de substance suspecte à bourrelets protubérants et à fond nodulaire de l’antre gastrique. La biopsie avait montré une infiltration de la muqueuse gastrique antrale par des cellules carcinomateuses indépendantes de taille moyenne à noyau refoulé par une vacuole de sécrétion alcianophile donnant un aspect en bague à chaton caractéristique ; une gastrectomie totale avec omentectomie étaient réalisées et l’examen macroscopique de la pièce opératoire avait montré dans la région antrale, une ulcération de 3 cm de diamètre et un épaississement pariétal circonférentiel avec un aspect translucide de la tranche de section. L’étude histopathologique avait retrouvé les cellules tumorales mucosecrétantes en bague à chaton ; ces dernières étaient mêlées à plusieurs cellules géantes multinucléées à noyaux réguliers ; ces cellules géantes étaient retrouvées même au niveau des images de lymphangite carcinomateuse (Fig. 1) et au niveau des ganglions métastatiques. Les prélèvements fundiques ne montraient pas d’infiltration tumorale et les limites chirurgicales duodénale et œsophagienne étaient saines. La tumeur gastrique était classée p T3 N2 M0. À l’examen immunohistochimique, les cellules géantes étaient positives pour le CD68 (Fig. 2) et négatives pour les cytokératines (clones AE1/AE3). Les suites opératoires étaient simples ; un traitement adjuvant par chimiothérapie était entrepris ; le recul actuel est de 15 mois avec un état général conservé sans récidive.