Rapidly fatal metastatic cutaneous angiosarcoma initially mimicking a furuncle in a middle-aged male.

Cutaneous angiosarcomas are rare but typically occur in three distinct clinical settings and are most commonly found on the scalp or face of elderly men. Positive prognostic factors include tumor size less than 5 cm, primary tumor location below the head, negative margins after excision, resectability, and younger age. Metastases drastically reduce survival and the most common metastatic site is lung. We present a 43-year-old man who had primary cutaneous angiosarcoma that initially mimicked a furuncle and eventuated in multiple metastases. The metastatic disease included brain involvement, which has rarely been reported, especially in a relatively young person without known predisposing conditions. This unique case also highlights the need for early diagnosis followed by advanced imaging, given the limitations of current therapies and high metastatic potential of angiosarcoma.

Primary Cutaneous Coccidioidomycosis Presenting as a Recurrent Preauricular Cyst.

A 31-year-old Filipino active duty marine presented with a 2-year history of a waxing and waning nodule on his left cheek that had been incised and drained on multiple occasions. The patient had no significant medical history other than a positive purified protein derivative test with negative chest x-ray finding treated with a 9-month course of isoniazid in 2010. He denied cough, fever, chills, night sweats, weight loss, joint/bone pain, or prior trauma to the area. On initial examination, there was a 1×1-cm erythematous indurated nodule associated with an overlying violaceous scar on his left preauricular cheek. Since the lesion was presumed to be an inflamed epidermal cyst, it was initially treated with 0.1 cc of interlesional triamcinolone acetonide (10 mg/cc). At 1-month follow-up, the lesion was slightly less indurated, but an excisional biopsy was performed to remove the residual nodule. The biopsy showed an essentially normal epidermis with focal dermal fibrosis below which were multiple collections of histiocytes and multinucleated giant cells surrounded by a dense lymphoplasmacytic infiltrate with numerous eosinophils (Figure 1). A few multinucleated giant cells contained large thick-walled spherules, some with endospores, consistent with Coccidioides immitis (Figure 2). Serological tests showed positive serum for C immitis IgG antibodies with low levels of complement-fixing antibodies (1:2). IgM antibodies were negative. Findings from chest x-ray and bone scan failed to reveal evidence of systemic disease. Although the infectious disease physician felt that the patient most likely had primary cutaneous coccidioidomycosis (PCC), since the duration of the infection was unknown and the patient was Filipino, thereby increasing his risk of dissemination, he was placed on a daily regimen of 400 mg of oral fluconazole until his complement fixation titers became undetectable.

Intradermal proliferative fasciitis on the finger.

Intradermal nodular fasciitis is a rare but not extraordinary pathological finding with approximately 100 cases reported since originally described in 1990. Intradermal proliferative fasciitis, however, has only been reported twice and both arose in the postauricular region. Herein, we report a third case of intradermal proliferative fasciitis and the first to occur on the finger. This case was also unusual in its slow growth, and complete lack of smooth muscle actin expression that has only been reported once before.

Multiple clustered and focally atrophic dermatofibromas (DF).

Multiple clustered dermatofibromas describes a confluence of dermatofibromas in one anatomic location. We describe a 32-year-old man who presented for evaluation owing to skin papules and plaques and concerns about malignancy. Repeat histopathological evaluation found no evidence for dermatofibrosarcoma protuberans. Our case presents this relatively rare condition and discusses observation along with potential treatment options.

Syringocystadenocarcinoma papilliferum with locoregional metastases.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant counterpart of syringocystadenoma papilliferum (SCAP). To date, less than 30 cases have been reported, and of those, only three had locoregional metastases [1,2]. Herein, we report a case of a 42-year-old man with a rapidly growing nodule on his right parietal scalp ultimately diagnosed as SCACP arising in association with a preexisting SCAP and nevus sebaceus. This case differs from prior reports in the tumor's rapid growth and aggressive course with the development of locoregional metastases within 5-weeks from initial presentation.

Cutaneous metastasis of a transitional cell carcinoma.

Cutaneous metastases are infrequently encountered in dermatology. We describe a rare case of a urolethial carcinoma metastatic to the skin in an elderly female. The metastasis expanded rapidly and was thought to possibly be infectious on her initial evaluation by her primary team. We were called in as consultants and biopsied this mass to confirm our concern for metastatic disease. In this case there was an unknown primary tumor. Histologic staining patterns were used to suggest that the tumor may have originated from her urinary bladder.

Carcinosarcoma: a primary cutaneous tumor with biphasic differentiation.

We present a case of a primary cutaneous carcinosarcoma (PCS). Histopathologically, PCS is defined as a biphasic tumor composed of malignant epithelial (carcinoma) and mesenchymal (sarcoma) elements. The diagnosis of PCS can be challenging, not only because of its rarity but also because superficial biopsies can result in sampling errors. Accurate diagnosis is essential, as PCS carries a higher recurrence and metastatic rate than epithelial carcinomas, thus requiring wider excision or Mohs micrographic surgery as well as closer clinical follow-up.

Hereditary hidradenitis suppurativa restricted to the auricular region.

Hidradenitis suppurativa (HS) is part of the follicular occlusion tetrad, which typically presents as recurrent deep-seated nodules, abscesses, and communicating sinus tracts with a predilection for the axilla and genital region. There is only one prior report of HS restricted to the auricular region. Herein the first familial case of HS restricted to the auricular region is reported.

Metastatic atypical fibroxanthoma.

Atypical fibroxanthoma (AFX) is an undifferentiated pleomorphic sarcoma that typically has low-grade malignant potential. Although recurrences do occur, only approximately 25 cases of metastasizing AFX have been reported. Herein a patient with an AFX that metastasized and eventuated in death is described. Although AFX remains a diagnosis of exclusion, through this pedagogic case, the clinical, immunohistochemical, and genetic features that can assist in diagnosis are discussed.

Signet ring-like cells: no longer a "signature" of glandular differentiation.

The term "signet ring" is used to describe a cell with an eccentric nucleus that is compressed to the periphery of the cell by a large cytoplasmic vacuole or by a substance accumulated within the cytoplasm. In pathology, these cells have been traditionally associated with adenocarcinomas. We describe two cases of cutaneous tumors that contained signet ring-like cells. Because of the morphology of the cells and the fact that one patient had a past history of lung cancer, the possibility of metastatic adenocarcinoma was considered. Upon further investigation, the tumors proved to be a primary cutaneous squamous cell carcinoma and a basal cell carcinoma; surgical management was curative. Because signet ring changes may pose a diagnostic challenge, we review the various benign and malignant cutaneous lesions that have been reported to contain signet ring cells and we explore the possible causative mechanisms of this phenomenon.