Maria José Julião

Prediction of Sentinel Node Status and Clinical Outcome in a Melanoma Centre

Background. Sentinel lymph node biopsy (SLNB) is a standard procedure for patients with localized cutaneous melanoma. The National Comprehensive Cancer Network (NCCN) Melanoma Panel has reinforced the status of the sentinel lymph node (SLN) as an important prognostic factor for melanoma survival. We sought to identify predictive factors associated with a positive SLNB and overall survival in our population. Methods. We performed a retrospective chart review of 221 patients who have done a successful SLNB for melanoma between 2004 and 2010 at our department. Univariate and multivariate analyses were done. Results. The SLNB was positive in 48 patients (21.7%). Univariate analysis showed that male gender, increasing Breslow thickness, tumor type, and absence of tumor-infiltrating lymphocytes were significantly associated with a positive SLNB. Multivariate analysis confirmed that Breslow thickness and the absence of tumor-infiltrating lymphocytes are independently predictive of SLN metastasis. The 5-year survival rates were 53.1% for SLN positive patients and 88.2% for SLN negative patients. Breslow thickness and the SLN status independently predict overall survival. Conclusions. The risk factors for a positive SLNB are consistent with those found in the previous literature. In addition, the SLN status is a major determinant of survival, which highlights its importance in melanoma management.

Pityriasis Lichenoides et Varioliformis Acuta: Case Report and Review of the Literature

We report a case of a 63-year-old man hospitalized for a polymorphous generalized eruption consisting of maculopapules with peripheral scaling, vesicopustules, and ulceronecrotic and crusted lesions measuring 5–20 mm, localized on his trunk and extremities, particularly exuberant in the flexural area. Histopathology showed necrotic keratinocytes with exocytosis of red blood cells and lymphocytes and a dermal perivascular and periadnexal inflammatory infiltrate, composed of CD8+/CD4–/CD30– T cells, indicating the clinical diagnosis of pityriasis lichenoides et varioliformis acuta. He was treated with erythromycin and methylprednisolone reduced gradually over 5 months, with a slow but complete response; the patient was without lesions after 2 years of follow-up. The authors want to remind of this rare entity which may present difficulties in diagnosis and therapy.

Melanoma: when dermoscopy is a pitfall

References 1 Fischer J. Autosomal recessive congenital ichthyosis. J Invest Dermatol 2009; 129: 1319–1321. 2 Akiyama M, Takizawa Y, Kokaji T, et al. Novel mutations of TGM1 in a child with congenital ichthyosiform erythroderma. Br J Dermatol 2001; 144: 401–407. 3 Herman ML, Farasat S, Steinbach PJ, et al. Transglutaminase-1 gene mutations in autosomal recessive congenital ichthyosis: summary of mutations (including 23 novel) and modeling of TGase-1. Hum Mutat 2009; 30: 537–547.

Epithelioid leiomyosarcoma arising in the subcutis--a new case report of a rare variant.

To the Editor: Most cases of epithelioid leiomyosarcomas (LMSs) reported to date are located in the uterus or in the gastrointestinal tract, being rarely described in somatic soft tissue. A 42-year-old woman presented with a firm erythematous nodular lesion, 30 mm in diameter, strongly adherent to the adjacent tissue planes, with central ulceration, located on her left lower leg. The lesion had developed progressively for 2 years, and it had become painful 1 month previously to our observation. Histopathological examination showed a proliferation composed predominantly of epithelioid cells with eosinophilic cytoplasm, irregular vesicular nuclei, and marked pleomorphism, occupying the dermis and extending to the subcutaneous fat (Figs. 1A–C). Focally, lesional cells had a more spindled morphology. Mitotic figures were frequent (32 per 10 high-power fields). Macroscopically, a vessel was identified and confirmed microscopically to be a medium-sized vessel infiltrated by the tumor, with atypical cells seen in probable continuity with the muscular layer (Figs. 1D, 2A, D). Immunohistochemical study revealed diffuse positivity for smooth muscle actin, desmin, and muscle-specific actin (HHF35), and focal staining for cytokeratin cocktail MNF-116 (Figs. 2B, C), and very weak and focal positivity for epithelial membrane antigen (EMA). Ki-67 revealed a proliferation fraction of approximately 50%. Immunohistochemistry was negative for S100, HMB45, CD31, CD34, ERG, and CD68. Expression of INI-1 was preserved. Based on these findings, the diagnosis of high-grade epithelioid LMS, likely of vascular origin, was established. The patient was submitted to wide excision of the primary tumor with a 2-cm lateral margin down to fascia. Imaging studies (ultrasonography of the inguinal lymph node basin and thoracoabdomino-pelvic computed tomography) did not show any evidence of secondary lesions. Treatment was complemented with adjuvant radiotherapy. After 2 years, the patient remains disease free and is still under follow-up. LMSs account for approximately 10% of soft tissue sarcomas, with predilection for the lower limbs. LMSs that may involve the skin are usually divided in cutaneous (or dermal) and subcutaneous, with distinct features regarding origin, location, and prognosis. Cutaneous LMSs are thought to arise from the arrector pili muscles, mainly on the neck and head (50%). However, the deep subcutaneous type arises from the muscular layer of vessel walls, often on the lower extremities and trunk. Some authors believe that the proximity of the tumor to the vessel wall facilitates hematogenous spread of the tumor, which occurs in up to 30% of cases. This is clearly distinct from the more benign nature of the dermal subtype, which rarely

Rupture of splenic angiosarcoma: a rare cause of spontaneous haemoperitoneum

Primary splenic angiosarcoma, a very rare mesenchymal tumour of endothelial cell origin, comprises 2.6% of all cases of angiosarcoma and 10% of all primitive splenic tumours. Clinical presentation is usually unspecific, with abdominal pain and anaemia. Rupture is a rare complication and should prompt emergency splenectomy. Prognosis is usually poor because of liver, lung or bone metastases. We describe the case of an 80-year-old woman admitted to the emergency room with syncope, hypotension and vomiting. She stabilised after fluid resuscitation. Investigations showed anaemia, a large, heterogeneous spleen and free fluid in the abdominal cavity. She underwent emergency splenectomy. Pathology revealed primary splenic angiosarcoma. The postoperative period was complicated by respiratory failure but the patient made an otherwise uneventful course and was discharged 2 weeks after surgery. Six months after the operation she remains free of disease with no adjuvant treatment.

Acantholytic-Like Pattern in a Recurrent Melanoma Mimicking Acantholytic Squamous Cell Carcinoma: A Further Example Highlighting the Potential for Misdiagnosis.

219. 5. Juarez-Ortega M, Hernandez VG, Arce-Paredes P, et al. Induction and treatment of anergy in murine leprosy. Int J Exppathol. 2015;96:31–41. 6. Chander R, Jabeen M, Malik M. Molluscum contagiosum-like lesions in histoid leprosy in a 10-year-old Indian boy. Pediatr Dermatol. 2013;30:e261–e262. 7. Fakhouri R, Sotto MN, Manini MI, et al. Nodular leprosy of childhood and tuberculoid leprosy: a comparative, morphologic, immunopathologic and quantitative study of skin tissue reaction. Int J Lepr Other Mycobact Dis. 2003; 71:218–226. 8. Worobec SM. Treatment of leprosy/Hansen’s disease in the early 21st century. Dermatol Ther. 2009;22:518–537. 9. Harada K, Suzuki K. Periodic acid-ethanol gelatinamethenamine silver stain for demonstrating M lepra and myelin in peripheral nerve fibres of leprosy patients. Int J Lepr Other Mycobact Dis. 1986;54:84–87. 10. Ridley MJ. The degradation of Mycobacterium leprae by a comparison of its staining properties. Int J Lepr Other Mycobact Dis. 1983;51:211–218. Acantholytic-Like Pattern in a Recurrent Melanoma Mimicking Acantholytic Squamous Cell Carcinoma: A Further Example Highlighting the Potential for Misdiagnosis

BIOPSIA DE GÂNGLIO SENTINELA EM PORTUGAL – EXPERIÊNCIA DE UM CENTRO DE REFERÊNCIA

Background: Sentinel lymph node biopsy is undertaken as a standard procedure for patients with localized cutaneous melanoma. The National Comprehensive Cancer Network (NCCN) melanoma panel has reinforced the status of Sentinel lymph node biopsy as the most important prognostic factor for disease survival.Objectives: We sought to identify predictive factors associated with positive SLN biopsy and correlation with overall survival in our population.Methods: A retrospective chart review of 221 patients who had undergone successful Sentinel lymph node biopsy for melanoma between 2004 and 2010 at our department was done. Univariate and multivariate analyzes were performed.Results: Sixty-one point five percent of patients were women and the mean age was 59.3 years. Most of the primary lesions were located on the lower limbs (43%), mainly on the feet (21.3%). The Sentinel lymph node biopsy was positive in 48 patients (21.7%). Univariate analysis showed that male gender, increasing Breslow thickness, tumor type, and presence of tumor-infiltrating lymphocytes were significantly associated with a positive Sentinel lymph node biopsy. Multivariate analysis confirmed that Breslow thickness and the presence of tumor-infiltrating lymphocytes are independently predictive of Sentinel lymph node metastasis. The 5-year survival rates were 53.1% for Sentinel lymph node positive patients and 88.2% for SLN negative patients. Breslow thickness and the Sentinel lymph node status independently predict overall survival.Conclusions: The risk factors for positive Sentinel lymph node biopsy in our study were consistent with those previous found in the literature. In addition, Sentinel lymph node status is a major determinant for survival, which highlights its importance in melanoma management.