Retroperitoneal round‐cell liposarcoma associated with paraneoplastic pemphigus presenting as lichen planus pemphigoides‐like eruption

Abstract

A 59‐year old Caucasian woman refugee from Bosnia had been in good health until a year before admission, when she developed painful erosions and ulcerated lesions in the mouth, anogenital area, hands, and feet. Treatment with topical and systemic antibiotics, acyclovir, and antifungal creams was ineffective. Three months before admission, her skin and mucosal lesions had worsened, resulting in the limitation of walking and her daily activities. She had also experienced significant weight loss. On admission to the Department of Dermatology, University of Belgrade, the patient was noted to be thin, distressed, and quite depressed. Physical examination reveaied ulceration that involved the floor of the mouth, ventral and laterai tongue, buccai mucosa, and hard and soft palate (Fig. 1). The vermiiion surfaces were fissured, eroded, and crusted with shiny vioiaceous scaiy papules dispersed across the cutaneous edge of both upper and iower lip (Fig. 1). Skin examination disciosed scattered vioiaceous scaiy lesions on the fiank, wrists, dorsa of the hands and feet, and lower legs. There were numerous erosions with livid erythema on the palms and soles, fingers and toes, with fissured naiis, partial and totai onycholysis, and pterygium formation. On the vulvar and vaginai mucosa, as well as the anal and perianal area, numerous erosions with erythema and white exudate were aiso present. On the skin of the anterior abdomen, both axillas, and right groin, large tense blisters were noticed. In the right lower quadrant of the abdomen, a firm, painful, ill‐defined mass was palpated, approximateiy 15 cm beiow the right costal margin. A pelvic examination confirmed a hard, immobile, painful, abdominai mass, with the suggested origin from the right adnexai (tubo‐ovarial) region. Abdominal and peivic sonogram, as well as CT scan revealed a 10x9 cm, right‐sided retroperitoneal tumor. Chest rentgenogram, and CTscan of the lungs were within normal limits. Laboratory studies revealed normocytic, normochromic anemia with hypoalbuminemia. The following analyses were negative or within normal limits: antinuclear antibodies, cryogiobulins, VDRL, cultures for Candida and Herpes virus from oral and perianal mucosa. A biopsy specimen from the orai mucosa demonstrated acantholytic blister with keratinocyte necrosis and vacuolar interface changes. A biopsy of the violaceous papules from the wrist, finger and lower legs showed hyperkeratosis, hypergranulosis, dyskeratotic keratinocytes, prominent vacuolar interface changes, lichenoid infiltrate with exocytosis of mononuclear celis and superficial, subcorneal clefting in one specimen (Fig. 2). Biopsy of the tense blister from the anterior abdomen disciosed subepidermal bulla consistent with bullous pemphigoid. Direct immunofluorescence (DIF) of perilesional skin and oral mucosa revealed intercellular binding of IgG as well as diffuse deposition of IgG and C3 along with dermoepidermal junction. Indirect immunofluorescence (IIF) studies of the patient serum revealed strong intercellular binding on...

Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

“…From the literature search, we included an additional 124 articles, reporting 208 patients with PAMS published between January 1, 1996, and August 1, 2012 . A single article reporting patients from our institution was excluded from analysis to avoid duplicate reporting .…”

“…Table outlines the number of patients who underwent diagnostic imaging studies, and the purpose and clinical utilization of the diagnostic imaging studies. Of all the patients studied in the literature review, 106 (51.0%) were reported to undergo a form of diagnostic imaging . Of these patients, 88 (83.0%) had an underlying malignancy detected or confirmed by diagnostic imaging (CT, n = 82; MR, n = 12; chest radiograph, n = 10; 18F‐FDG PET/CT, n = 4).…”

Retroperitoneal liposarcoma associated with small plaque parapsoriasis

“…Bosco et al ., described a unique case of relapsing undifferentiated liposarcoma with biochemical, immunoistochemichal and molecular evidence of alpha-fetoprotein (AFP) ectopic production [4]. Sporadic cases of liposarcoma associated with paraneoplastic pemphigus and leucocytosis are also reported [5,6]. …”

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“…The Nonhematologic neoplasms associated with PNP include follicular dendritic cell sarcoma [5,42,46,47,72,84,85], inflammatory myofibroblastic tumor [86], leiomyosarcoma [87], liposarcoma [88], malignant nerve sheath tumor [89], inflammatory fibrosarcoma [90], reticulum cell sarcoma [91], and malignant melanoma [92], squamous cell carcinoma of the vagina [93], and adenocarcinoma of the colon [55], prostate [94,95], pancreas [88,93], breast [36], and lung [96,97] (Table 1). Follicular Dendritic Cell Sarcoma (FDCS) is a rare tumor associated with paraneoplastic pemphigus.…”