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We describe a case of temporal granulocytic sarcoma in a 26-year-old patient after apparent molecular remission of an acute myeloid leukaemia. He complained of otodynia with hearing loss and facial paralysis on the right side. He was treated with chemotherapy and self-transplant haematopoietic stem cells. He was cured clinically, molecular remission of the haematological processes was achieved, and he remained asymptomatic for three years. Facial paralysis and hearing loss associated with temporal GS should be treated with chemotherapy. Aggressive surgery may complicate the clinical course of the disease and it should be avoided.

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Trigeminal plexiform neurofibromas involving the orbit and the infratemporal fossa are rare tumours. An uncommon case of a plexiform neurofibroma of the trigeminal nerve occurring in a 53-year-old female patient with severe cosmetic deformity is reported. The clinical findings, neuroradiographic features, surgical management, postoperative results and follow-up of trigeminal plexiform neurofibromas are reviewed.

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CONCLUSION: Sentinel lymph node biopsy (SLNB) is a technique that facilitates the study of metastasis in oral and oropharyngeal cancer and reduces morbidity in patients affected by this pathology. OBJECTIVES: Oral and oropharyngeal cancers are common and are frequently associated with spread to cervical lymph nodes. We attempted to confirm the usefulness of SLNB in patients suffering from squamous cell carcinoma of the oral cavity as a prognostic technique for lymph node prediction of micrometastatic invasion. Using surgical parameters, we compared its relevance against a retrospective group without SLNB. PATIENTS AND METHODS: We studied a prospective group of 22 patients, 21 men and 1 woman, aged 40-83 years (mean=57.77, SD=13) in which SLNB was performed and a retrospective group of 22 patients, 19 men and 3 women, aged 34-76 years (mean=52.68, SD=11) in which SLNB was not performed. Both groups presented oral cavity tumors at clinical stages T1-T3 and radiological stage N0. SLNB consisted of preoperative lymphoscintigraphy (LG), intraoperative detection of the sentinel lymph node (SLN), and a histopathological examination following the recommendations of the Association of Directors of Anatomic and Surgical Pathology (ADASP) and the Canniesburn protocol. RESULTS: We observed SLNs in 21 of 22 patients, with a total of 32 SLNs detected at cervical level II (65.63%), I (21.87%), and III (12.5%). SLNB displayed a sensitivity of 73% (confidence interval (CI)=0.51-0.99) and 100% specificity (CI=1). Negative and positive predictive values were 80% and 100%, respectively. SLNB significantly reduced the incidence of radical neck dissection (p=0.003), the need for recovery/resuscitation beds after surgery (p=0.002), surgical complications (p=0.034), and length of hospital stay (p=0.01).

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CONCLUSION: The existence of patients with positive non-sentinel node indicates a risk of misdiagnosis of metastasis in oropharyngeal and oral cancer. OBJECTIVES: We attempted to confirm the usefulness of sentinel lymph node biopsy (SLNB) in oropharyngeal and oral cavity cancer to detect clinically occult metastases. PATIENTS AND METHODS: Twenty-two patients with a mean age of 57 (SD=13) years were studied prospectively. All presented T1-T3 squamous cell carcinoma (SCC) of the oropharynx or oral cavity, and were cN0 on palpation and CT. A preoperative (24 h) lymphoscintigraphy was performed with 99mTc-labelled sulfur colloid injected around the primary tumor. Subsequently, we proceeded with tumor exeresis and detection of SLN following the procedures described in a previous paper. RESULTS: We detected a total of 32 SLNs in 21 of 22 patients. Ipsilateral neck node location was observed in 18 patients and bilateral in 3. We observed 10 patients with true positive SLNs (3 patients had micrometastases), indicating occult metastases. We harvested a total of 454 nodes in our selective neck dissections, 19 of which presented metastatic invasion. We identified four patients with positive non-sentinel nodes.

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A case of inflammatory myofibroblastic tumour of the larynx in a 74-year old man is reported. The lesion presented as a polypoid tumour arising in the left true vocal fold. The patient died of non-tumour-related causes and the autopsy showed persistence of the laryngeal tumour. Multicentricity and distant metastases were not detected. The literature about this entity and its differential diagnosis is reviewed and briefly commented upon.

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A case of papillary squamous cell carcinoma of the larynx in a 50-year-old man is reported. The lesion was located in the supraglottic region and grossly presented a characteristic finger-like pattern of growth. The tumour pursued an indolent clinical course, and the patient is well and free of disease after 47 months of follow-up. The present paper recapitulates the macro- and microscopic features of this rare neoplasm and stresses its differential diagnosis with other exophytic squamous cell carcinomas of the region.

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The authors present one clinical case of lung epidermoid carcinoma with several concurrent features which are uncommon in relation to this type of neoplasia. Consequently, the age of the patient (22), the lack of known risk factors, the histologic type (little differentiated squamous) and an aggressive biologic behaviour of the tumour, preceded of multiple cutaneous metastasis, are circumstances that make this clinical case exceptional. The more significative aspects present at lung carcinoma included in this age group respect to others are analyzed.

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A case of inverted papilloma of glandular type in the prostatic urethra of a 65 year-old man is reported. The case was asymptomatic and incidentally discovered on histopathologic study of transurethral resection (TUR) specimens from benign prostatic hyperplasia. The literature concerning this rare entity is reviewed and briefly commented.

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A case of atypical carcinoid tumour of the larynx in a 72-year-old woman is presented. The neoplasm was located in the supraglottic region and was first manifest by metastases to the scalp. The presence of amyloid-like material and calcitonin-positive cells were features that resembled medullary carcinoma of the thyroid. Clinically, it pursued an aggressive course that led to death 58 months after diagnosis. The literature is reviewed, particularly that regarding the differential diagnosis.

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A case of meningioangiomatosis occurring in a 15-year-old boy is reported. The patient did not show signs of neurofibromatosis on physical examination, and his medical history included only one previous episode of loss of consciousness, which was accompanied by a self-limited focal seizure. The lesion was associated with an oligodendroglioma and was incidentally discovered during the macroscopic sampling of the neurosurgical specimen. The literature relating to this uncommon entity is reviewed and discussed. To the best of our knowledge, the concurrence of meningioangiomatosis and oligodendroglioma has not been documented previously.

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The presence of the so-called "skeinoid fibers" in stromal tumours of the gastrointestinal tract is a rare event. The exact origin of this puzzling group of neoplasms still remains controversial. Despite all, the recognition of this histologic feature in routine practice is important for its presence seems to carry histogenetic implications. The authors report the histopathologic findings of two of such cases both occurring in the jejunum of a male aged 48 and a woman, 64. Their positivity with CD 34 antigen is enhanced, for this peculiar immunophenotype help to distinguish them from other stromal tumors in the small bowel. Additionally, the literature is reviewed and commented.

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BACKGROUND: The Rothmund-Thomson syndrome is a hereditary dermatosis frequently accompanied by less well-known non dermatologic features including osteogenic sarcoma. CASE REPORT: A girl developed the classical dermatologic features of the Rothmund-Thomson syndrome since the first months of life. When she was 6 years old, she suffered from painful limitation of motion of her left leg. X-rays, MNR imaging and bone scintigraphy showed typical features of osteosarcoma of the distal portion of the femur. Diagnosis was confirmed by histologic examination through open biopsy. The search for metastatic lesions was negative. The patient was given chemotherapy and the tumor was resected 45 days later followed by postoperative chemotherapy. CONCLUSION: About 12 similar cases of osteosarcoma have been reported in patients with the Rothmund-Thomson syndrome. A review of literature allows to recognize some peculiar features of such association.

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A neuroendocrine breast carcinoma occurring in a 79 year-old woman is presented. The case presented typically as a single nodule in the upper and outer quadrant of her right breast and showed a pure histology consisting exclusively of organoid nests of well differentiated cells that mimicked those of carcinoid tumors of the lung. The literature is briefly reviewed and commented. In the light of this review, we conclude that the issue of carcinomas with neuroendocrine differentiation in breast pathology is controversial and its terminology somewhat debatable.

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We describe a patient with chordoma located in the mid-posterior mediastinum whose first clinical symptoms were respiratory. This, together with the patient's age at presentation, made this case unusual.

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