Marcelo Brum Corrêa

Bazex Syndrome (Acrokeratosis Paraneoplastica) Diagnosed in a Patient with Oral Persistent Ulcerations

Paraneoplastic syndromes associated with head and neck cancer are rare and have been reported under dermatological, endocrine, hematological, neurological and rheumatological disorders. Bazex syndrome is an intriguing paraneoplasia that can be associated with head and neck squamous cell carcinomas. A range of symmetrical dermatological manifestations, with a clear predilection to extremities, that encompasses erythematous squamous plaques, skin scaling and nail dystrophy can provide a psoriasiform pattern in Bazex syndrome. In addition to these tricky clinical features, the rarity of the disease and the lack of understanding on Bazex syndrome generally make such cases to be mismanaged as psoriasis or lichen planus, causing an important delay in the diagnosis of the underlying malignancy. The authors describe a case of Bazex syndrome that occurred in a patient with a recently diagnosed tongue squamous cell carcinoma. Clinicians should consider paraneoplasia when assessing skin and/or oral persistent lesions.

Synchronous oral paracoccidioidomycosis and oral squamous cell carcinomas with submandibular enlargement

Oral paracoccidioidomycosis and oral squamous cell carcinoma may occur in the same patient. As both lesions may present similar clinical and histopathological features, the diagnosis is sometimes challenging. This paper describes the case of a 54-year-old male who was a farm worker and heavy alcohol and tobacco user. He developed paracoccidioidomycosis and two lesions of squamous cell carcinoma in the oral cavity. During the follow-up, the patient presented enlargement of the submandibular lymph nodes, which was thought to be regional metastasis but was diagnosed as paracoccidioidomycosis. Therefore, the significance of this association is emphasized and discussed.

Clinico-Pathologic Conference: Case 1

The clinical differential diagnoses for painful swelling of the right mandible in a 44 years old black female patient, could include fracture, infection, and neoplastic conditions. Although it was not mentioned, if besides these two features, loss of function had been observed, osteogenic sarcoma would be a consideration inasmuch as these tumor may present as a well-known triad consisting of pain, swelling and loss of function [1]. The radiological findings of the right mandibular lesion can be summarized as follow: ill-defined margin, increased bony sclerosis, patchy bone destruction, new bone formation, and periosteal reaction with a Codman’s triangle. These alterations could favor the diagnosis of malignancy. Bony sclerosis was found at the periapical and cervical area of the adjacent tooth and the mandibular canal. The radioopacities seen at the periapex can be interpreted as osteomyelitis as the periapex can be the starting point of oral infection [2]. On the other hand, diffuse scattered radioopacities, which encompassed the cervical area of the tooth and the mandibular canal could not be consistent with osteomyelitis. The radio-opacity around the inferior alveolar nerve may be interpreted as sclerotic bone, which could explain the patient’s pain. Looking at the radiographs carefully, other findings can be observed. Multiple radio-opaque and radiolucent areas are present in the right maxilla and left mandible and entities to consider include a fibro-osseous lesion and Pagets disease. On the other hand, the presence of diffuse patchy bone destruction in the right body of mandible is intriguing and has to be emphasized. Although rare, osteolytic change in fibro-osseous lesion or Paget’s disease must be considered. More detailed radiograph can be useful to define the disease. Some of the alterations in the right mandible, such as bone sclerosis with bone destruction are observed in malignant conditions. Another clue for malignancy is periosteal reaction, which is seen in the ascending ramus to condylar head in the panoramic radiographic view. The periosteum is not normally visible on radiographs and the term ‘periosteal reaction’ refers to excess bone produced by periosteum [3]. The occlusal view demonstrates an M. A. Lopes (&) F. V. Mariano P. A. Vargas Áreas de Semiologia e Patologia Oral, Departamento de Diagnóstico Oral, Faculdade de Odontologia de Piracicaba, Universidade Estadual de Campinas (UNICAMP), Av. Limeira 901, CP52, Areião, Piracicaba, São Paulo 13414-903, Brazil e-mail: malopes@fop.unicamp.br

Primary synovial sarcoma involving the submandibular gland

Synovial sarcoma represents 5.6%-10% of all soft-tissue sarcomas. Adolescents and young adults are most frequently affected, mainly in the deep soft tissue of the extremities. Only 10% of synovial sarcomas affect the head and neck region; most of these are biphasic. We describe a case of an 18-year-old man who complained of a mass in the right submandibular region that had been present for approximately 12 months. On surgical removal, microscopic analysis showed a tumor formed by sheets of malignant spindle cells involving the submandibular gland. Immunohistochemistry displayed positivity for AE1/AE3, CK18/8, epithelial membrane antigen, CD99, CD56, and TLE-1. Based on these immunohistochemical and histopathologic features, a diagnosis of monophasic synovial sarcoma was rendered. The patient was treated with adjuvant radiotherapy and after 1 year was free of disease. To the best of our knowledge, this is the first reported case of synovial sarcoma involving the submandibular gland.

Maxillary desmoplastic fibroma with initial symptoms suggestive of sinusitis.

Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm characterized by the formation of abundant collagen fibers. It arises most commonly in the jaws and exhibits local aggressiveness and high recurrence rates after local resection. An uncommon case of expansive DF involving the right maxilla, maxillary sinus, and inferior orbital wall of a 49-year-old man whose initial symptoms were similar to acute sinusitis is presented, and the criteria for diagnosis and clinical management are discussed.

Reactive post-radiotherapy bone formation in the maxilla.

Objective High-dose radiation therapy affects bone metabolism, and therefore post-radiotherapy bone formation is an uncommon finding. This case describes an unusual ossification in the maxillary region identified after head and neck radiotherapy. Results A 45-year-old female patient was submitted to maxillary surgical resection and orbital exenteration due to squamous cell carcinoma. Radiotherapy post-surgery was performed because of compromised surgical margins. After 4 months of the conclusion of radiotherapy, the patient presented a bone exposition at surgical site. Surprisingly, the oral examination and computed tomography revealed a new formation of the right palatine vault and bone formation filling the nasal and orbital cavity. The incisional biopsy discarded residual disease or osteoradionecrosis and showed normal tissue. Conclusions To the best of the authors’ knowledge, this is the first case of wide bone formation as an early effect of head and neck radiotherapy.

Cribriform adenocarcinoma of the tongue and minor salivary glands: importance of reclassification and presentation of atypical behavior in broad follow up

This work shows the importance of the reclassification of a group of Polymorphous low grade adenocarcinoma (PLGA) to Cribriform adenocarcinoma of the tongue and minor salivary glands (CATMSG) and present a report of a case of this entity, with an unknown behavior, with a large following.