V. Boneschi

Weekly paclitaxel for advanced aggressive classic Kaposi sarcoma: experience in 17 cases.

Backgroundu2002 Paclitaxel has proved to be highly effective in the treatment of severe AIDS‐related Kaposi sarcoma (KS), for which it is now considered as a second‐line monotherapy. Taxanes were recently shown to be active also in classic, endemic and post‐transplantation KS.

Intralesional vincristine as first-line therapy for nodular lesions in classic Kaposi sarcoma: a prospective study in 151 patients.

Backgroundu2002 Classic Kaposi sarcoma is a rare angioproliferative neoplasm with varying biological behaviour. Depending on the clinical stage, local or systemic therapy can be used. Vincristine has proven to be effective as systemic chemotherapy and in very few reports as intralesional treatment.

Combination of vinblastine and bleomycin as first line therapy in advanced classic Kaposi's sarcoma.

Backgroundu2002 Classic KS (CKS) mainly affects elderly people, has an irregular course, and is relatively benign for years. However, sometimes the disease may progress rapidly and spread to internal organs, thus necessitating systemic chemotherapy. We therefore decided to carry out a prospective trial using vinblastine and bleomycin, which are active, easy to administer and control, and low cost.

Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma and Castleman disease

SIR, Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia, most commonly of lymphoreticular origin, first described by Anhalt et al. An alternative term, paraneoplastic autoimmune multiorgan syndrome, has since been proposed to define a condition in which patients, in addition to severe, often fatal pulmonary involvement and deposition of autoantibodies in different organs, may display lesions that resemble pemphigoid, erythema multiforme, graft-versus-host-disease and lichen planus as well as classic pemphigus. We report a patient with a lichen planus-like mucocutaneous variant of PNP associated with follicular dendritic cell (FDC) sarcoma and Castleman disease (CD), emphasizing in particular the rarity of PNP in association with such a type of sarcoma. A 53-year-old woman had painful persistent oral erosions accompanied by dry cough, dysphagia and weight loss since December 2002. She had been given topical and systemic corticosteroids at another hospital, with no improvement. In June 2003, when the patient was referred to our institute, she presented with extensive erosions of the oral mucosa (Fig. 1a), tongue and conjunctiva and a 3-week history of lichenoid papules symmetrically distributed on the trunk (Fig. 1b) and extremities. Laboratory examinations revealed mild anaemia (haemoglobin 10Æ7 g dL; normal 12–16) and positive antinuclear antibodies with speckled pattern at a titre of 1 : 160. Histology from a papular area showed a lichenoid interface dermatitis with numerous necrotic keratinocytes (Fig. 2a). Direct immunofluorescence disclosed intercellular deposits of IgG throughout the epidermis, whereas indirect immunofluorescence demonstrated IgG autoantibodies directed to the intercellular substance of stratified epithelium, namely monkey oesophagus, and also of rat bladder transitional cell epithelium. An immunoprecipitation analysis, carried out as reported, identified a complex of three antigens: proteins of 210 and 190 kDa that could represent desmoplakin II ⁄envoplakin and periplakin, respectively, and the as yet uncharacterized 170-kDa antigen (Fig. 2b). A specific enzyme-linked immunosorbent assay, using recombinant desmoglein (Dsg) 1 and Dsg3 proteins, did not detect antibodies to either Dsg1 or Dsg3. A diagnosis of PNP was made and a search for an underlying neoplasm revealed, on abdominal computed tomographic scans, a 9 · 5 · 8 cm soft tissue mass in the right retroperitoneal area. The mass was excised and pathological examination showed histological features of FDC sarcoma in association with residual foci of CD of the hyaline-vascular type. On immunohistochemistry, the tumour cells of the sarcoma had a CD21+, CD35+, nerve growth factor receptor-positive, actinpositive phenotype. Within 3 weeks after resection of the tumour the mucocutaneous manifestations continued to deteriorate and therapy was initiated with intravenous methylprednisolone 80 mg daily and azathioprine 100 mg daily for 15 days, but this was ineffective. Pulsed intravenous methylprednisolone 250 mg daily for 5 days, and subsequently, a cycle of high-dose intravenous immunoglobulins 0Æ6 g kg daily for five consecutive days resulted in moderate clinical improvement. However, she developed ingravescent dyspnoea: respiratory function tests disclosed restrictive pulmonary changes, whereas positron emission tomography of the chest showed features suggestive of secondary lung involvement by sarcoma. She died of respiratory failure in April 2004. In our patient, the association of PNP with FDC sarcoma, a rare malignant neoplasm of follicular dendritic cells, is unique. Notably, the FDC sarcoma arose from CD, as seen in only one previously reported case. Although there is no firm evidence a

Treatment of classic Kaposi's sarcoma-associated lymphedema with elastic stockings

Lymphedema of the lower extremities is a frequent complication of Kaposis sarcoma (KS). Compressive therapy is the basis of treatment for lymphatic disorders, but to the authors’ knowledge, there are no controlled trials to evaluate its effectiveness in KS‐related lymphedema. Sixty‐five patients with classic KS‐associated lymphedema limited to below the knee were studied. Fifty patients received below‐knee elastic stockings, whereas the remaining 15 did not use any compressive device. Among treated patients, 60% (30/50) experienced a limb volume reduction, while 40% (20/50) had an increase of limb volume. In contrast, all patients (15/15) of the untreated group had an increase of limb volume. No correlation between lymphedema reduction and systemic or local chemotherapy was observed, supporting compressive therapy as the major strategy for the treatment of this condition. Our results suggest that elastic stockings may be important tools for the management of lymphedema associated to classic KS.

Human herpesvirus‐8 infection among heterosexual partners of patients with classical Kaposi’s sarcoma

Backgroundu2002DNA sequences of human herpesvirus‐8 (HHV8) are found in lesions of Kaposi’s sarcoma (KS).

Primary giant cell tumor of soft tissue of the groin – a case of 46 years duration

Background: Soft tissue giant cell tumor (GCT‐ST) of low malignant potential is an uncommon neoplasm, considered the soft tissue counterpart of giant cell tumor of bone. GCT‐ST mainly affects young to middle‐age adults and presents as a painless growing mass mainly located in the lower extremities and trunk. Histologically, this tumor is characterized by a mixture of uniformly scattered osteoclast‐like multinucleated giant cells intimately admixed with short fascicles of spindled cells. Complete excision with negative surgical margins is associated with a benign clinical course in most cases.

Oral etoposide for Kaposi's Mediterranean sarcoma.

22 patients affected by locally aggressive or generalized form of Kaposis Mediterranean sarcoma were treated with oral etoposide (VP16) as single-drug therapeutic regimen. Of the 17 evaluable patients, 10 were pretreated with other chemotherapeutic regimens. VP16 was administered at the dose of 100 mg daily for 3-5 days every 3 weeks for 3 times during induction, then every 4 weeks for 10-12 times during maintenance. Hematological (35.2%) and gastrointestinal (64.7%) toxicities were always mild and swiftly reversible. Good percentages of objective responses were achieved in both nonpretreated (85.6%) and pretreated (70%) patients. The chemotherapeutic regimen employed, the way of drug administration, the results as well as the comparison to another study with vinblastine are discussed.

Allergic reactions to topical desoxymethasone and oral triamcinolone.

Associated immediate and delayed contact allergy has been reported to nickel, cinnamon, para-aminophenylarnine, epoxy resin, penicillin and protein, to caterpillar bite, and to fish and chicken meat, but not previously to mercuric chloride (2, 3, 6, 7). In the histological examinations previously reported (4, 5), eczematous changes (subcorneal vesicle, spongiosis, lymphocytic infiltration) were seen as early as 30 min. Skin reactions on the symptom-free skin of our patient showed a more observable continuity from urticaria to eczema, with vasculitis seen in clinicallyeczematous skin after 48 h, suggesting that mixed sensitivity involves IgG as well as IgE antibodies (8, 9), perhaps together with a cytolytic effect (3). The histological signs of immediate urticarial reactions are followed by histological characteristics of delayed hypersensitivity reactions, mixed with vascular involvement. SHORT COMMUNICATIONS

Intralesional alpha 2b recombinant interferon for basal cell carcinomas.

Basal cell carcinoma (BCC) is a rather common skin neoplasm, particularly in white patients. It grows slowly, has a locally malignant behavior, and metas‐tases occur only exceptionally. It is considered to be derived from pluripotential epithelial cells that can partially differentiate towards adnexal structures and sebaceous, apocrine, and sometimes eccrine glands. Prolonged exposure to the sun, a fair and freckled complexion, x‐rays, burn scars, and arsenic by mouth are all predisposing factors for basal cell epithelioma. The face and the trunk are the parts most often affected. Some patients, especially those with a fair complexion, may have dozens of BCCs at one time.