Syringocystadenoma papilliferum is a rare benign adnexal hamartoma that is often associated with the nevus sebaceous of Jadassohn. It usually presents on the scalp and malignant transformation is rare. Here we present a case of digital papillary carcinoma on the toe of a teenage girl. The lesion recurred after two prior excisions without biopsy. The biopsy was read as a syringocystadenoma papilliferum with concerns for aggressive digital papillary adenocarcinoma, highlighting the importance of biopsy with excisions of neoplasms of unknown etiology.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare and aggressive malignant adnexal tumor originating from apocrine or pluripotent appendageal glands, often associated with a preceding syringocystadenoma papilliferum (SCAP) or nevus sebaceus (NS). This systematic review rigorously examines SCACP through an analysis of 78 cases documented between 1980 and 2024. The study aims to provide a comprehensive review of the clinical manifestations, diagnosis, treatment modalities, and outcomes associated with SCACP, while also reappraising its associations, particularly with NS. SCACP predominantly affects older adults, with an average age of 66.3 years and a slight male predominance, commonly presenting as ulcerated nodules or plaques on the scalp. This review highlights the aggressive nature of SCACP, evidenced by significant rates of metastasis and recurrence. Treatment is primarily surgical, with Mohs micrographic surgery offering potential benefits in terms of margin control and cosmetic outcomes. The association of SCACP with NS is critically evaluated, suggesting a complex etiopathogenesis and underscoring the importance of recognizing this association for timely diagnosis and management. Our review also briefly discusses potential pitfalls faced by clinicians in the diagnosis of SCACP. Our findings emphasize the need for standardized treatment protocols and further research into targeted therapies to improve patient outcomes in SCACP.

暂无摘要。

Syringocystadenocarcinoma papilliferum (SCACP) is an exceptionally rare cutaneous adnexal tumor that is infrequently encountered by clinicians worldwide. The tumor typically appears in the older population, affecting patients in their fifth and sixth decades of life without male or female predominance. Patients frequently present with a variable-sized hyperpigmented ulcerative lesion containing an exudate that has a long-standing course of progression from its benign counterpart, Syringocystadenoma papilliferum (SCAP). Additionally, the clinical presentation and morphology of the neoplasm can be easily confused with a variety of other skin cancers, such as squamous cell carcinoma (SCC), basal cell carcinoma (BCC), cutaneous lymphoma, and cutaneous metastasis. Therefore, histopathology and tissue analysis play an essential role in establishing an accurate diagnosis. However, the lesion is so rare that no definitive diagnostic markers have been established yet. We present a case of SCACP localized to the scalp of the patient. Our case study highlights the presence of specific tumor markers that could potentially serve as objective criteria for diagnosis.

Malignant tumors of external auditory canal (EAC) constitute less than 0.2% of all head and neck cancers. The incidence of carcinoma of the EAC is estimated to be between one in six per million populations. Majority of cancers of EAC are squamous cell carcinomas and basal cell carcinomas. Some rare and unusual tumors do occur within the ear canal including malignant melanoma, merkel cell carcinoma, angiosarcoma, lymphoma and adnexal carcinomas like ceruminous adenocaricinoma and adenoid cystic carcinoma. Ceruminous glands tumors constitute about 5% of all external auditory canal tumors. Carcinoma of the external auditory canal is a difficult diagnosis unless the tumors presents as a fungating mass protruding from the external auditory canal. Syringocystadenocarcinoma Papilliferum (SCACP) is an extremely rare cutaneous adnexal neoplasm. Syringocystadenoma papilliferum (SCAP) is thought to be precursor of SCACP. About 50 cases of SCACP have been reported in literature all over the body. The diagnosis is difficult and excisional biopsy becomes mandatory for diagnosis and treatment. We present a case of SCACP in the external auditory canal in a middle-aged female. To the best of our knowledge and belief, this is the first case of SCACP in the external auditory canal in the English literature. This prompted us to report this case.

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm of the sweat glands. It is believed to arise from the malignant transformation of syringocystadenoma papilliferum (SCAP). The majority of cases present on the head and neck and up to 17% of cases show metastatic progression. These tumors seldom occur in the anogenital area and, to date, only one case has been reported on the penis. Here, we report a rare case of SCACP in a 65-year-old man who presented with an erythematous, non-healing, ulcerated lesion on the penis.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare cutaneous adnexal tumor thought to originate from its benign counterpart, syringocystadenoma papilliferum. These tumors are predominantly located on the head and neck with their location on the breast extraordinarily reported; mammary localization poses a great diagnostic dilemma to the practicing pathologist. Herein, we report an unusual case of an 85-year-old woman with an outside diagnosis on a core needle biopsy of metaplastic mammary carcinoma. Upon consultative review of the partial mastectomy specimen, SCACP was identified. Herein, we review SCACP and the diagnostic challenge it poses, especially when localized to the breast. Furthermore, we perform a retrospective review of institutional pathology reports and identified four additional cases of SCACP diagnosed at our institution within the last decade. Finally, we briefly review the literature of SCACP. The entity of SCACP should be well known to pathologists to avoid misdiagnosis.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant adnexal neoplasm, which is considered as a malignant counterpart of syringocystadenoma papilliferum (SCAP). Clinically, SCACP appears as a nodule, inflammatory plaque, or tumor. The lesion is usually covered with crusts, which are formed by secretion of the apocrine epithelial cells. Histologically, SCACP resembles SCAP, with cystic papillomatous invaginations connected to the skin surface by funnel-shaped structures lined by infundibular epithelium. The stroma of the tumor consists of a dense inflammatory infiltrate of plasma cells and lymphocytes. SCACP differs from SCAP in terms of the architectural and cytological features of the tumor cells, and is characterized by higher nuclear cytoplasmic ratio, nuclear irregularity, coarse chromatin, and increased mitotic activity. However, the immunohistochemical findings of SCACP vary. Since only 49 cases of SCACP have been reported in the English literature, the clinical and histologic characteristics of SCACP have not been fully established. Further studies on the diagnostic criteria for SCACP are warranted. Here, we report a rare case of SCACP and present a review of other relevant literature.

Carcinosarcomas are biphasic tumors composed of admixed malignant epithelial and mesenchymal components. Numerous terms have been used to name such neoplasms; therefore, terminological confusion is frequent. Most examples of carcinosarcomas are encountered in non-cutaneous sites, with approximately 100 cases of cutaneous carcinosarcomas reported so far in the English literature. Although different theories have been suggested to explain the occurrence of these peculiar neoplasms, histogenetic mechanisms should be better hypothesized depending on each individual case. Even though prognosis tends to be related to the specific components of the lesion, especially the epithelial one, it seems that cases of cutaneous localization usually have a better outcome. We report an exceedingly rare case of syringocystadenocarcinoma papilliferum which showed an atypical stroma with sarcomatoid appearance, and highlight that the terminology used for this spectrum of lesions is disorganized and confusing.

We present a case of Syringocystadenocarcinoma papilliferum that originated in the eyelid and extended into the orbit. These tumors are very rare and have the potential to metastasize. A literature review of all the previous cases has been compiled from the Medline, EMBASE, and PubMed databases. We found that the majority of cases present on the head and neck and up to 17% of cases showed metastatic progression. This is the first case to show orbital involvement and highlights the need to remain vigilant with such lesions, as they have a tendency to become aggressive.