Bilateral Lipoadenoma of the Adrenal Glands and Humeral Brown Tumor: An Unusual Association.

Adrenal adenolipomas are rare lipomatous adrenal tumors that can be either functional or not. Only 7 cases have been reported in the English literature so far. However, brown tumors are benign, rare, historical lesions, with histological similarity to giant tumors that can be encountered in 1% of all primary hyperparathyroidism cases. We report the case of an unusual association of bilateral lipoadenoma of the adrenal glands and humeral brown tumor in a 35-year-old patient. He presented to the emergency department with a pathological fracture of the left humerus secondary to a brown tumor. The medical investigations have concluded to primary hyperparathyroidism. The screening for multiple endocrine neoplasia type 1 revealed the presence of bilateral nonsecreting adrenal masses whose anatomopathological study concluded adenolipomas. Adrenal tumors may constitute a part of multiple endocrine neoplasia type 1 in 20 to 40% of cases. In this view, it is necessary to check for the presence of other endocrine gland tumor locations such as primary hyperparathyroidism, neuroendocrine tumors of the duodenum and pancreas, or pituitary adenomas.

Melanotic progonoma of temporal and occipital bones: A case report.

Melanotic progonoma is a rare tumor that primarily affects the maxilla of infants during the first year of life. Involvement in the skull is rare and can mimick other benign or malignant tumors affecting the infant's skull. The authors report a case of melanotic progonoma of right occipital and temporal bones in a 7-months' girl and discuss the histological features, immunohistochemistry study, differential diagnosis and management of this tumor.

[Role of the pathologist in the diagnosis of palpebral keratoacanthoma: case report and literature review]. / Rôle du pathologiste dans le diagnostic du kératoacanthome de la paupière : à travers un cas et une revue de la littérature.

We report the case of a keratoacanthoma of the lower lid in a 14-year-old girl posing diagnostic difficulty with differentiating the lesion from a verrucous squamous cell carcinoma on histologic examination of an incisional biopsy. It was only upon complete excision of the tumor that the diagnosis of keratoacanthoma could be made and that of malignancy ruled out. We discuss the clinical and histologic diagnostic criteria for keratoacanthoma and assert that wide biopsy is essential for adequate morphologic study to enable better therapeutic management.

A rare case of adult medulloblastoma with spinal metastasis.

Medulloblastoma is a relatively common malignant brain tumor of childhood and relatively rare in adulthood, with a propensity for neuraxial spread via cerebrospinal fluid pathways. Osseous extraneural metastasis is uncommon and when it happens, radiologic findings are of sclerotic (60%), lytic (35%), and mixed patterns (5%) (Algra et al. (1992)). In this paper, we present a case of medulloblastoma metastiaszing to the lumbar spine and describe the magnetic resonance appearance, with emphasis on the image findings mimicking spondylodiscitis.

[Cerebral atypical rhabdoid/teratoid tumor: report of two cases]. / La tumeur tératoïde rhabdoïde atypique cérébrale : à propos de deux cas.

The cerebral atypical rhabdoid/teratoid tumor constitutes one of the most aggressive tumors of children. Such tumors are equally characterized by a critically and speedly mortal development. The optimal management of such tumors remains unknown. The authors report two cases of cerebral atypical rhabdoid teratoid tumor in 8- and 10-year girls and discuss the histological features, immunochemistry study, prognosis and treatment of these rare tumors.

Epidemiology of pediatric primary tumors of the nervous system: a retrospective study of 633 cases from a single Moroccan institution.

BACKGROUND AND PURPOSE: There are several reports regarding the epidemiology of pediatric brain tumors. However, little is known about the profile of pediatric brain tumors in Africa especially in Morocco in particular. The authors report the results of epidemiological analysis of a retrospective review of childhood primary brain tumors in a single institution. METHODS: A retrospective review of all cases of primary brain from 1 month to 15 years diagnosed at the Department of Pathology of the Hospital of Specialities of Rabat between January 1991 and December 2009 was performed. RESULTS: Six hundred and thirty-three primary tumors of the central nervous system were reviewed with a mean of 33.31 cases per year. According to the gender, 55% of the tumors occurred in males and 45% in females. The mean age was 8.36 years. Of all the tumors, 47% were situated in the supratentorial compartment, 48% in the infratentorial compartment, and 5% in spinal cord. In the infratentorial compartment, 82% of tumors are located in the cerebellum, 15% in the fourth ventricle, 2% in the brain stem and 1% in the cerebellar pontine angle. In the supratentorial compartment, two third of the tumors were located in the cerebral hemispheres and the sellar region. Thus 39% of tumors are located in the cerebral hemispheres followed by the sellar/suprasellar region (30%), lateral and third ventricles (11%), pineal region (8%), meninges (5%), choroid plexus (4%), and optic chiasma/tracts (3%). The most common types of tumors diagnosed were pilocytic astrocytoma and medulloblastoma together accounting for nearly half of the cases (46%) (23.1% and 22.9% respectively), followed by craniopharyngiomas (9%), ependymomas grade II (6.5%), glioblastomas (6%), astrocytomas grade II (4.4%), ependymomas grade III (3.9%). The other tumors represent 22.6%. CONCLUSION: We think that our results reflect fairly well the incidence of tumors of the nervous system in children due to the fact that this study was performed through many years in a single institution with a homogeneous neuropathological approach.

[Solitary fibrous tumor of the orbit: report of two cases and review of literature]. / Tumeur fibreuse solitaire de l'orbite : à propos de deux cas avec revue de la littérature.

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that most frequently arises in the pleura and uncommonly involves the orbit. There are 54 cases of SFT of the orbit available in the literature. The authors report tow cases of an orbital SFT and discuss the clinical, radiological, pathological features and differential diagnosis. Long-term follow-up of patients is necessary because SFT may recur many years after operation. Complete surgical resection is the most important prognosis factor of this tumor.

[Primitive aspergillosis of the posterior cerebral fossa in immunocompetent patient]. / Aspergillose primitive de la fosse cérébrale postérieure chez un patient immunocompétent.

Cerebral aspergillosis arises in the great majority of cases during an invasive aspergillosis with hematogene scattering from the lung hurts. The cerebral, not rare location is one of the worse criteria forecast during the invasive aspergillosis. We report the case of patient who was hospitalized in the neurosurgery department for syndrome of increased intracranial pressure, hemiparesis and cerebellar syndrome in febrile context. The radiological exploration objectified a collection of the posterior fossa. A stereotactic biopsy was performed. It collected fragments biopsy and pus. The pathological and microbiological analysis allowed the identification of Aspergillus fumigatus. The originality of this observation comes from the rare location in the posterior fossa of aspergillosis and because the patient is immunocompetent and no primary location is found. The patient presents however a viral hepatitis B of fortuitous discovery. He is put under treatment by amphotericin B. The evolution is marked by meningitis comment-diversion. Antibiotics are prescribed, and then the patient is operated for total ablation of the tumor. He dies following an osmolar coma associated with thrombopenia and a secondary renal insufficiency due to his treatment by the amphotericin B.

[Papillary adenocarcinoma on ectopic thyroid tissue]. / Adénocarcinome papillaire sur tissu thyroïdien ectopique.

OBJECTIVES: To report a case of papillary adenocarcinoma occurring on ectopic thyroid tissue in the hyoid bone region. MATERIAL AND METHODS: A 57-year-old patient consulted for a cervical mass appearing 1 year before, with no signs of compression. The thyroid function was normal. RESULTS: The examination showed a firm tumefaction, fixed on the hyoid bone, with undetermined limits, measuring approximately 8 cm. The thyroid gland was in the normal pretracheal position. The cervical tomodensitometry objectified an osteolytic process centered on the hyoid bone with a fleshy zone of tissue density and calcifications. Cervical MRI provided a more precise assessment of the extension of the mass and confirmed the integrity of the other structures, in particular the endo-larynx. The tumor was totally resected via a cervical approach. The pathologic study suggested a papillary adenocarcinoma on ectopic thyroid tissue. The treatment was completed by a total thyroidectomy with normal histological results. CONCLUSIONS: Papillary adenocarcinoma on ectopic thyroid is a very rare situation. Its diagnosis is histological. Its treatment is primarily surgical, sometimes associated with radioiodine I-131 therapy.

[Solitary myofibroma of the mandible: a case report]. / Myofibrome solitaire de la mandibule. A propos d'un cas.

OBJECTIVES: Infantile myofibromatosis (IMF) are integrated in the group of fibromatosis juvenile-type which are benign mesenchymal lesions, characterized morphologically by a proliferation of fibroblasts and myofibroblastes, with perivascular pattern. Through a case of solitary myofibroma of the mandible, we will emphasize anatomo-clinical features and differential diagnosis. MATERIALS AND METHODS: We report a case illustrating solitary myofibroma of the mandible in 18 month old infant. RESULTS: The histological diagnosis was done on the identification of two separate components, a fascicular myofibroblastic pattern at the periphery with a hemangiopericytoma like pattern in the centre. Both components positive for alpha-smooth muscle actin and vimentin. DISCUSSION: Solitary IMF is characterised by a single lesion arising from cutaneous, bony or soft tissues. Morphological and immunohistochemical examination allow the diagnosis of the typical form. The differential diagnosis includes other benign spindle cells tumours. The prognosis is excellent with possibility to regress spontaneously.

[Pleomorphic adenoma of the tongue base]. / Adénome pléomorphe de la base de langue.

INTRODUCTION: Pleomorphic adenoma is the most common lesion of minor salivary glands but is exceptional in the tongue base. REPORT CASE: We report the case of a 70-year-old woman with pleomorphic adenoma of the tongue base. The patient consulted for difficult swallowing, which developed over 4 months. Physical examination and computed tomography revealed a cystic tumour located in the right side of tongue base. The tumor was removed by transoral resection. The histological examination demonstrated a pleomorphic adenoma. DISCUSSION: We will discuss the diagnostic and therapeutically modalities of pleomorphic adenoma of the tongue base with a review of the literature.

[Synovial sarcoma of the head and neck: two cases report]. / Synovialosarcome cervico-facial: à propos de deux observations.

UNLABELLED: Synovial sarcomas are soft tissue tumors that rarely occur in the head and neck The purpose of this report is to accrue data on this sarcoma at a rare site, and to highlight the histopathological differential diagnosis with other cervical tumors. MATERIALS AND METHODS: Two cases of cervico-facial tumors were reported in 26 and 27 year old women. RESULTS: Histologically, these tumors were classified into monophasic and biphasic variants. Immunohistochemistry plays a major part in the differential diagnosis, enabling the demonstration of epithelial differentiation. Radical surgery was the mainstay of treatment with post-operative radiotherapy for residual disease in one case. Local recurrence was developed in the patient who had only surgical treatment. DISCUSSION: Synovial sarcomas are a rare soft tissue malignancies and the head and neck region location accounts for 3-5% of them. The rarity of this tumor in the head and neck and its multitude of his histopathologic features are responsible for frequent initial misdiagnosis. Histologic, immunohistochemic and characteristic chromosomal translocation findings are necessary for diagnosis. The poor prognosis of this sarcoma justified a radical surgery with post-operative radiotherapy.

[Laryngeal neuroendocrine carcinoma: a case report]. / Carcinome neuroendocrine du larynx: a propos d'un cas.

Laryngeal neuroendocrine carcinomas are uncommon and not well known tumors. Three histological subtypes, each of them with a different prognosis and treatment, can be identified. We report a case of a large cell laryngeal neuroendocrine carcinoma in 32 old-year boy who presented a right glotto-subglottic tumoral process. The patient was treated by total laryngectomy associated with bilateral functional neck dissection but without postoperative chemotherapy. A disease recurrence occured three months after surgery consisting on a massive involvment of laterocervical and sus clavicular lymph nodes. The authors discussed the clinical features, the histological and immunohistochemical characteristics, the treatment and the prognosis of laryngeal neuroendocrine carcinoma, according to literature. (full article translated in English available on www.ent-review.com).

[Melanoma of the iris and pregnancy]. / Mélanome de l'iris et grossesse.

Iris melanoma is a malignant melanocytic tumor, making up 1.2%-6.6% of uveal malignant melanomas. The growth of choroidal melanoma during pregnancy is described and a hormonal influence is suggested. We present a case of iris melanoma occurring during pregnancy. A 32-year-old woman, in the 28(th) week of her fifth pregnancy, was referred to the ophthalmologist with a 2-month history of blurred vision and pain in her left eye. The visual symptoms became progressively worse with decreasing vision. Her visual acuity was 20/70 in her left eye, with a temporal, pigmented, prominent iris mass extending into the trabecular meshwork and the corneal endothelium. Elsewhere other pigmented localizations were found in the iris and in the trabecular meshwork. There was secondary glaucoma with intraocular pressure of 36mmHg and C/D=0.9. The right eye was normal. A general physical examination found no pigmented lesions. Chest radiography and hepatic ultrasonography revealed no metastasis. The diagnosis of iris malignant melanoma was made from the diffuse involvement extending into the trabecular meshwork and the advanced secondary glaucoma; an enucleation was performed and histopathological examination confirmed the diagnosis of iris melanoma with involvement of the trabecular meshwork. The patient had no further problems (follow-up of 11 months). From this case report, we discuss the differential diagnosis of iris melanoma and the hormonal influence on its growth.

[Pleuropulmonary blastoma: possible interest of neoadjuvant chemotherapy. A case report]. / Blastome pleuropulmonaire: intérêt possible d'une chimiothérapie néoadjuvante. A propos d'un cas.

Pleuropulmonary blastoma is a very exceptional anatomoclinical and histological entity recently distinguished from adult pneumoblastoma. This tumor, observed in children aged less than 15, can involve the lung, the pleura, or the mediastinum and is characterized by a very poor prognosis. We report the case of a 4-year-old girl who developed pleuropulmonary blastoma which was discovered in a context of respiratory distress. Standard chest x-ray revealed an opacity covering the entire left lung. Histology and immunohistology led to the diagnosis of pleuropulmonary blastoma with several components: blastematous, malignant mesenchymatous with pluridirectional differentiation, and benign epithelial tissue. Treatment consisted in preoperative chemotherapy and radiotherapy to reduce tumor volume. This neoadjuvant treatment is not widely reported and its relatively favorable result allowed tumor resection. This approach might be useful in similar cases.

[Dedifferentiated chordoma of the skull base. A case report]. / Chordome dédifférencié de la base du crâne. A propos d'un cas.

Sarcomatous transformation in chordoma is a very rare condition and has been emphasized as a distinct entity because of its more aggressive clinical course. Here we describe a case of dedifferentiated chordoma arising from the skull base region of an 11-year-old boy, with tumor recurrence within one year. This tumor showed features of pleomorphic cell sarcoma with areas more typical of chordoma. Most of tumor cells expressed cytokeratin, epithelial membrane antigen, vimentin and S-100 protein, thus confirming the diagnosis of dedifferentiated chordoma.