Development of squamous cell carcinoma into basal cell carcinoma under treatment with Vismodegib.

BACKGROUND: Basal cell carcinoma (BCC) is the most common cancer in humans. Vismodegib, a Hedgehog pathway inhibitor, has proved its effectiveness in treating non-resectable advanced BCC. AIM: However, its action on squamous cell carcinoma (SCC) is unknown. We present three SCC cases developed into BCC in vismodegib-treated patients. MATERIAL AND METHODS: We have described three cases of patients developing SCC during treatment by vismodegib for BCC. RESULTS: Patient 1 was treated with vismodegib for five facial BCC. Due to the progression of one of the lesions at month 3 (M3), a biopsy was performed and showed SCC. Patient 2 was treated with vismodegib for a large facial BCC. A biopsy was performed at M2 on a BCC area not responding to treatment and showed SCC. Patient 3 was treated with vismodegib for a BCC on the nose. Due to vismodegib ineffectiveness, a biopsy was performed and showed SCC. DISCUSSION: Two similar cases have been described in the literature. This could be due to the appearance of the squamous contingent of a metatypical BCC or to the squamous differentiation of stem cells through inhibition of the hedgehog pathway. CONCLUSION: In practice, any dissociated response of a BCC to vismodegib should be biopsied.

[Lymphomatoid granulomatosis revealed by cutaneous lesions]. / Granulomatose lymphomatoïde de révélation cutanée.

BACKGROUND: Lymphomatoid granulomatosis is a rare Epstein Barr virus (EBV)-related lymphoproliferative disorder. It most frequently involves the lungs, skin and central nervous system and arises preferentially in patients with immune disorders. Here we report a case revealed by cutaneous lesions in an immunocompetent patient. CASE REPORT: A 56-year-old man consulted for erythematous nodules of the trunk associated with malaise and marked weight loss (14kg). In a few days the nodules became necrotic. Two weeks later a cough appeared and the chest computerized tomography showed multiple poorly defined nodular opacities with a peribronchovascular distribution. Cutaneous and pulmonary biopsies showed an infiltrate composed of medium-sized atypical lymphocytes T and B. EBV was present in the infiltrate (in situ hybridization) with a high EBV load in plasma. All of these data helped confirm the diagnosis of lymphoid granulomatosis. Despite aggressive treatment with polychemotherapy, the patient died after 2 months. DISCUSSION: Lymphomatoid granulomatosis represents a diagnostic challenge. In most cases, the presenting symptoms are not specific: malaise, weight loss, fever and cough. Moreover histology is difficult because of the T-cell-rich background. It is essential to consider this diagnosis in cases of cutaneous and pulmonary symptoms.

[Paraneoplastic multicentric reticulohistiocytosis]. / Histiocytose multicentrique paranéoplasique.

BACKGROUND: Multicentric histiocytosis (MH) is a rare form of histiocytosis of unknown aetiology and is associated with a malignant neoplasm in 25% of cases. Herein, we report the case of a female patient in whom HM enabled us to diagnose gastric carcinoma. OBSERVATION: A 74-year-old woman consulted for papules and nodules on the hands associated with destructive polyarthralgia of the interphalangeal joints and asthenia. Biopsy of a nodule revealed infiltration of the superficial dermis by numerous multinucleated giant cells containing eosinophilic cytoplasm; immunohistochemical analysis showed positive staining for CD68, confirming the diagnosis of MH. Additional screening tests for malignancy enabled us to diagnose gastric carcinoma. The cutaneous lesions disappeared several weeks after gastrectomy and no recurrence of HM or malignancy was seen in the ensuing 7 years. DISCUSSION: Fewer than 200 cases of HM have been reported. This non-Langerhans proliferative histiocytosis is a rare systemic disorder of unknown aetiology characterized clinically by papules and nodules associated with destructive polyarthralgia, and histologically by dermal proliferation of histiocytic multinucleated giant cells of skin, mucous membrane and synovial membrane. Routine screening for neoplasia should be performed following the discovery of HM as an association is seen in 25% of cases. However, the two diseases do not generally progress in parallel. In our case, the rapid disappearance of lesions within a few weeks of surgery suggests that HM was a true paraneoplastic syndrome.

[Systemic necrobiotic xanthogranuloma with initial pericardial and pulmonary involvement]. / Xanthogranulome nécrobiotique révélé par une atteinte péricardique et pulmonaire.

INTRODUCTION: Necrobiotic xanthogranuloma is a rare disease, usually associated with a monoclonal gammapathy. We report a case with pericardial and pulmonary involvement that preceded the appearance of the cutaneous lesions. OBSERVATION: A 58 year-old woman was hospitalized for cardiac tamponade. She has a past history of a ductal carcinoma of the right breast that had been completely cured. Pericardial and pulmonary samples showed a predominantly histiocyte monomorphous infiltrate. Twelve months later, indurated papular-like cutaneous lesions appeared around the eye orbits and on the thorax. The histological examination of the skin revealed a necrobiotic xanthogranuloma and the diagnosis of cutaneous and visceral necrobiotic xanthogranuloma was retained. The patient exhibited an IgG Kappa monoclonal gammapathy of undetermined significance. Sequential treatment with melphalan and general corticosteroids moderately improved the cutaneous lesions but did not modify the monoclonal peak. DISCUSSION: To our knowledge, this is the first case of pericardial involvement of a necrobiotic xanthogranuloma. The discovery of the disease, revealed by a visceral manifestation at the onset is uncommon. The subsequent appearance of typical cutaneous lesions permits the retrospective diagnosis of systemic necrobiotic xanthogranuloma. Because its diagnosis is difficult in the absence of concomitant cutaneous involvement, visceral localizations of the disease must be recognized.

[Acute thrombocytopenic purpura associated with fenoprofene (author's transl)]. / Purpura thrombopénique aigu associé au fénoprofène.

A 73-year-old woman, taking daily 3 to 4 tablets (900 to 1200 mg) of fenoprofene for 2 weeks, had a purpura related to an acute peripheric thrombocytopenia (platelet count of 24 000/mm3) with fast regression when we have stopped therapy. It is the second case of literature. This drug is also capable of causing acute agranulocytosis.