Leila Abid

Granulocytic sarcoma of the small intestine in a child without leukemia: report of a case with cytologic findings and immunophenotyping pitfalls.

BACKGROUND Granulocytic sarcoma is a rare tumor that is often misdiagnosed as it can be confused with lymphoma. It has unique cytologic features independent of the site of the tumor and can be identified on fine needle aspiration. CASE A 13-year old girl without a relevant medical history presented with an abdominal mass. Investigation revealed a tumor infiltrate in the small intestine and mesentery. The fine needle aspirate contained myeloid blasts with cytoplasmic granules. Immunohistochemistry on subsequent biopsy confirmed myeloid differentiation. There was no evidence of blood or bone marrow involvement suggestive of acute leukemia. The patient was well after 27 months of follow-up. CONCLUSION Granulocytic sarcoma should be included in the differential diagnosis of any small intestine infiltrate. Cytomorphology is accurate and efficient for the diagnosis in conjunction with complete immunocytochemistry study.

L’histiocytofibrome bénin profond: À propos d’un cas

Bening fibrous histiocytoma is one of the most frequent benign tumors. Most tumors are found in the skin, particularly on the limbs. Tumor size is usually small. According to the WHO, a deep location is found for less than 1% of all benign fibrous histiocytomas. Most deep tumors occur in the subcutaneous tissue. Deep locations in other organs have also been reported. We report a case and discuss the pathological and clinical aspects of this rare tumor.

Le fibrome desmoplastique de la côte : À propos de deux observations

Resume Le fibrome desmoplastique est une tumeur primitive tres rare de l’os, equivalente a la tumeur desmoide des parties molles. Bien qu’elle soit consideree comme une lesion benigne, elle peut etre tres agressive localement avec un haut risque de recidives locales apres exerese chirurgicale. En l’absence de specificite radiologique, elle peut simuler un osteosarcome, une dysplasie fibreuse ou un fibrosarcome. L’atteinte costale par un fibrome desmoplastique est extremement rare. Nous rapportons deux cas illustrant cette localisation exceptionnelle avec lyse costale et extension endothoracique. L’examen anatomo-pathologique apres exerese chirurgicale montrait une proliferation tumorale peu dense de petites cellules fusiformes s’entremelant a des faisceaux collagenes et resorbant les travees osseuses. Il n’a pas ete observe d’atypies ni de mitoses ni de necrose. Nous discuterons les aspects radiologiques, cliniques et morphologiques ainsi que les diagnostics differentiels de cette tumeur dont la reconnaissance est importante afin d’assurer un traitement chirurgical adapte.Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. Although considered a benign lesion, it can be very aggressive locally and has a high rate of local recurrence after incomplete surgical excision. Radiologically, the lesion shows no distinctive features, often simulating osteosarcoma, fibrous dysplasia, or fibrosarcoma. Rib involvement by desmoplastic fibroma is extremely rare. We present two cases of desmoplastic fibroma involving this unusual location with lytic costal lesion and chest wall extension. Histological examination after surgical resection revealed that the tumor consisted of spindle cells with small, elongated nuclei in a background of numerous collagen fibers and infiltrating lamellar bone. There was no evidence of nuclear atypia, mitotic activity, or necrosis. We also discuss histological differential diagnosis as well as clinical features and the radiological and pathologic findings of this rare disease. Recognition of this entity is important to ensure proper surgical treatment.

Carcinome mucineux primitif cutané : Une observation

Primary mucinous carcinoma of the skin is a rare adnexal neoplasm differentiating toward sweat gland, most of which is the eccrine type. The neoplasm shows a high local recurrence rate and a low incidence of metastasis. Diagnosis depends on clinical, histological, histochemical features, and exclusion of metastasis from other sites of mucinous carcinoma. We present a case of primary mucinous carcinoma of the skin, treated with conventional excision. There were no signs of recurrence after 21 months of follow-up.

Apport de la cytoponction dans le diagnostic des tumeurs germinales extragonadiques

Resume Les tumeurs germinales extragonadiques associent frequemment plusieurs composantes tumorales dont les modalites therapeutiques sont differentes. En particulier chez l’enfant, devant une tumeur sacro-coccygienne metastatique qui evoque un teratome immature, le dosage de l’αfoeto-proteine serique et une ponction cytologique peuvent permettre d’identifier un contingent de type sinus endodermique. Nous rapportons l’observation d’une fillette âgee de 25 mois qui presente une tumeur sacro-coccygienne d’emblee metastatique (os et ganglions). Il s’agit d’un teratome sacrococcygien secretant (alphafoetoproteine a 34 100 μg/ml). Une ponction ganglionnaire a mis en evidence une tumeur du sinus endodermique constituee d’une proliferation de structures glandulo-papillaires et de morules, caracterisees par des cellules pâles assez monomorphes, au noyau vesiculeux et nucleole saillant constant et contenant quelques boules cytoplasmiques. Lors de l’exerese de la tumeur primitive sacro-coccygienne, apres chimiotherapie, ce contingent n’a pas ete identifie au sein du teratome immature.

Primary vaginal adenocarcinoma of intestinal type arising from an adenoma

Vaginal adenocarcinoma is uncommon, representing 510% of vaginal cancers. Most of the reported cases are of clear cell histology (Heller et al. 2000). Vaginal adenocarcinoma of intestinal type, in the absence of diethylstilboestrol exposure (DES), is an extremely rare neoplasm with few cases reported in the literature. Two of these reported cases arose from a tubulovillous dysplastic polyp (Nagar et al. 1999; Mudhar et al. 2001). We report an additional case which is, to the best of our knowledge, the third reported case in the literature. A 70-year-old Caucasian woman presented to our institution with a history of terminal and intermittent haematuria of 1 year duration, without associated bowel symptoms. Her medical and surgical history was unremarkable with no history of DES exposure. Physical examination revealed a vaginal mass measuring about 40 mm on the anterior lower and middle vagina and involving the area of the urethra. She subsequently had a work-up consisting of chest X-ray, mammography, colonoscopy, abdominal ultrasound examination as well as a cystoscopy with multiple biopsies of the lesion and the bladder. The biopsy of the vagina confirmed the diagnosis, while the other clinical investigations were negative. Microscopic examination of the vaginal biopsy showed, in the first and second fragments, a villous adenoma morphologically identical to its colonic counterpart, characterised by papillary projection with delicate fibrovascular stroma lined by columnar epithelial of intestinal type showing moderate to severe dysplasia (Figure 1). In one area, focal invasion of the stalk was noted. The third fragment showed moderately differentiated adenocarcinoma composed of trabecular, cordonal and glandular cellular infiltration. Cells were cuboidal to cylindrical with atypical nuclei and eosinophilic cytoplasm with few mucin secreting cells (Figure 2). Immunohistochemistry analysis (Dako, Denmark; dilution: 1/50) demonstrated a strong and diffuse positivity for cytokeratin 20 (clone Ks20.8), carcinoembryonic antigen (clone II-7) and epithelial membrane antigen (clone E29). They were negative for cytokeratin 7 (clone OV-TL 12/30). Few cells were positive to Chromogranin (clone DAK-A3). Bladder biopsy revealed intestinal metaplasia characterised by the presence of epithelial cells of colonic type with a goblet cell appearance within the surface epithelium. Subsequently, the patient received external beam and interstitial radiotherapy. Three months later, a clinical examination revealed a 20620 mm firm glistening mass involving the area of the urethra. A biopsy revealed adenocarcinoma. She was advised to undergo an exenteration but she refused further treatment.

La tumeur à cellules claires du poumon: A propos d’un cas avec revue de la littérature

Resume La tumeur a cellules claires du poumon est une entite rare, d’histogenese incertaine et discutee. Cette tumeur se presente typiquement comme une masse asymptomatique, peripherique et grossierement arrondie du poumon. L’examen histologique montre qu’elle est faite de cellules polygonales claires regulieres a cytoplasme rempli de glycogene, au sein d’un riche reseau de capillaires sinusoides. Certaines caracteristiques immunohistochimiques et ultrastructurales permettent de confirmer le diagnostic. Nous rapportons le cas d’une tumeur a cellules claires primitive du poumon chez une jeune femme âgee de 28 ans, avec une revue de la litterature. L’histogenese encore hypothetique de cette tumeur, son potentiel evolutif, et les differentes difficultes diagnostiques souvent rencontrees sont discutes.

Dirofilariose conjonctivale à Dirofilaria repens. À propos d′un nouveau cas tunisien

Introduction La dirofilariose a Dirofilaria repens est une parasitose repandue chez les animaux domestiques et transmis chez l′homme par l′intermediaire de piqures d′insectes vecteurs, surtout les moustiques. L′infestation humaine est rare, et les manifestations cliniques sont benignes a type de nodules sous-cutanes ou oculaires. Observation Nous rapportons le deuxieme cas de dirofilariose conjonctivale chez une femme âgee de 21 ans residente a Tunis (Tunisie). La presentation clinique particuliere faisait evoquer une cellulite orbitaire. Les differentes investigations cliniques et radiologiques n′ont pas permis d′en preciser la nature. L′exerese chirurgicale et l′examen histopathologique ont permis de porter le diagnostic par l′identification d′un ver adulte femelle de type Dirofilaria repens. L′evolution fut favorable apres extraction du ver. Conclusion La dirofilariose est rare en Tunisie, seuls 7 cas ont ete rapportes. Sa frequence est probablement sous-estimee en raison de sa benignite. L′exerese permet de porter le diagnostic et assure le traitement.