Granulomatous slack skin - seven years follow-up of a case with features of parapsoriasis en plaques, muscle involvement, granulomatous vasculitis, and necrobiotic changes

Vol. 57 No. 2 Suppl., 2016
This supplement was not sponsored by Outside Organizations.

ROMANIAN JOURNAL of MORPHOLOGY and EMBRYOLOGY

Aurel Doru Chirita, Irina Margaritescu

Granulomatous slack skin (GSS) represents an extremely rare variant of mycosis fungoides with only 70 cases reported in the literature to date. It is characterized clinically by the occurrence of bulky, pendulous skinfolds, usually located in flexural areas and histologically by an infiltrate composed of small neoplastic T-lymphocytes joined by granulomatous inflammation with scattered multinucleated giant cells containing nuclei arranged in a wreath-like fashion. Since its first description, very rare cases of GSS with muscle involvement, large vessels involvement, or necrobiotic changes have been reported. We present an extraordinary case of GSS with all these unusual features developing in the lesions of the same patient. The long follow-up of seven years allowed us to document the evolution of each lesion. Some lesions appeared and evolved in a manner very reminiscent of those of parapsoriasis en plaques, others were classical GSS lesions, and still others developed large ulcerated lesions. These ulcerated lesions consistently failed to respond to conventional wound therapy, skin directed therapy [retinoids + psoralen combined with ultraviolet A (PUVA)-therapy], and interferon-alpha therapy. Remarkably, the ulcers completely healed when systemic corticosteroids were added. We hence postulate that the ulcers appeared because of large vessel vasculitis rather than tumoral direct destruction.

Corresponding author: Aurel Doru Chirita, MD, Dipl. ICDP-UEMS; e-mail: dorutchirita@yahoo.com

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ROMANIAN JOURNAL of MORPHOLOGY and EMBRYOLOGY

Corina Lichiardopol, Valeriu Surlin, Maria-Camelia Foarfa, Mirela-Corina Ghilusi, Simona Bondari

Primary squamous cell carcinoma of the thyroid is a very rare thyroid malignancy (less than 1% of thyroid cancers) with unfavorable clinical evolution and median survival less than one year, due to highly local tumor invasiveness with airway obstruction, metastases and treatment complications. We present a 62-year-old female patient with a fixed, rapidly increasing 5 cm right thyroid nodule, generating compressive signs and significant weight loss, resembling anaplastic thyroid carcinoma. Thyroid hormones, antithyroid antibodies and calcitonin were normal. Computed tomography (CT) scan revealed mediastinal extension of the tumor and excluded the presence of lymph nodes or other tumors (T3N0M0). Total thyroidectomy was performed and histopathological evaluation revealed squamous cell carcinoma, which was confirmed by immunohistochemistry, showing diffuse positivity for CK7, CK19, CK34betaE12, galectin-3, EGFR, focal positivity for p63 and negativity for TTF-1 and CD5. Subsequently, the patient underwent chemotherapy (Paclitaxel, Cisplatin, Epirubicin) and radiotherapy (40 Gy), but tumor recurrence was noticed one month after surgical resection and continued to grow despite treatment. Nodal and metastases status remained negative at regular follow-up. The patient died within one year after diagnosis. External radiotherapy and chemotherapy were not efficient in our case. New treatment options are needed to improve outcome in primary squamous cell carcinoma of the thyroid.

Corresponding author: Corina Lichiardopol, MD, PhD; e-mail: corinalich@gmail.com

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