A Recalcitrant Case of Senear-Usher Syndrome Treated With Rituximab.

A 51-year-old uninsured, otherwise healthy male who works in the fishing industry presented with a two-month history of pruritic scaly plaques on his face, scalp, and trunk and mild photosensitivity. A biopsy of a scalp lesion revealed acantholysis consistent with pemphigus foliaceus. Laboratory testing demonstrated elevated anti-desmoglein 1, positive antinuclear antibodies (ANA and anti-dsDNA), and elevated Sjögren's anti-SS-A antibodies. The patient was diagnosed with pemphigus erythematosus. The patient was not optimally responsive and was unable to discontinue systemic corticosteroids despite a maximum dosage of mycophenolate mofetil of 3000 mg/day. Hence, rituximab was added as a rescue treatment with the rheumatoid arthritis protocol. Three months after starting rituximab, there was a marked improvement in symptoms with complete resolution of cutaneous lesions.

A Case of Syringocystadenocarcinoma Papilliferum: Not Your Usual Suspect.

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.

Metastatic Pancreatic Acinar Cell Carcinoma: An Unlikely Culprit.

Although acinar cells comprise a large volume of the pancreas, they rarely transform into malignant neoplasms. Once they arise, they rapidly metastasize via hematogenous spread to other organs such as the brain, liver, lung, and skeletal system. Cutaneous involvement, however, is rarely seen in all patients with primary pancreatic neoplasms. The most frequently reported site of cutaneous manifestations is the umbilicus, with the other sites including the trunk, lower extremities, head, and neck. Here, we report a case of metastatic pancreatic acinar cell carcinoma with cutaneous involvement of the patient's scalp.

Tumor-to-tumor metastasis: pathology and neuroimaging considerations.

The phenomenon of tumor-to-tumor metastasis has been reported in the literature for over a century. However, it remains fairly uncommon, with fewer than 100 cases being described during that time. Virtually any benign or malignant tumor can be a recipient, but meningiomas have been implicated as the most common intracranial neoplasm to harbor metastasis. The donor neoplasm is most frequently lung or breast carcinoma, while rare cases of metastasis from other primary tumors have been reported. We report here three examples of such rare metastases. This case series reports the first documented instance involving rectal adenocarcinoma. In addition, we report two cases of metastatic prostate adenocarcinoma to a meningioma; to date of which only three cases have been published. The terms "tumor-to-tumor metastasis" and "collision tumor" are addressed, as are details of the pathology. The limitations of standard radiological imaging techniques, such as standard CT and MR, which cannot reliably identify the presence of metastasis within a meningioma are compared with physiology-based neuroimaging methods, such as perfusion MR and MR spectroscopy, which may be more useful in noninvasively differentiating tumor histology.

Synovial chondromatosis of spine: case report and review of the literature.

Primary synovial chondromatosis is an uncommon, benign entity involving the synovial lining of larger joints. In this metaplastic process, islands of chondrocytes are clustered throughout the synovium, forming nodules that jut into the joint cavity. Clinical manifestations include pain, swelling, and decreased range of motion of associated extremities. Synovial chondromatosis may be confused with other degenerative joint diseases as well as chondrosarcoma. The occurrence of synovial chondromatosis in the spine is rare. Ten cases of this spinal variant have previously been published and this report represents the eleventh case. Here we briefly review the literature and discuss the histopathologic pitfalls and differential diagnosis.

HER2 and EGFR expression in cutaneous spindle squamous cell carcinoma.

BACKGROUND: Epidermal growth factor receptor (EGFR) and HER2, members of the Erb family of transmembrane receptor tyrosine kinases (RTK), are responsible for communicating extracellular signals to the nucleus. Moreover, EGFR and HER2 are implicated in tumorgenesis. Immunohistochemical studies have demonstrated that approximately 80% of cutaneous squamous cell carcinomas (SCC) express EGFR. To date, the expression of EGFR and HER2 has not been evaluated in primary cutaneous spindle squamous cell carcinoma (SSC). In extracutaneous spindle squamous cell carcinoma, there is evidence implicating the expression of RTK as a strong, independent prognostic indicator of potentially poor outcome. The purpose of this study is to investigate the expression of EGFR and HER2 in SSC, using immunohistochemical methods. MATERIALS AND METHODS: Thirteen cases of primary nonmetastatic cutaneous SSC archived at a large veterans' hospital and tertiary referral dermatopathology service were retrieved via a computer-assisted search. Immunohistochemistry was used to evaluate the presence of EGFR and/or HER2 expression. All cases were confirmed before study by a single, board-certified dermatopathologist. RESULTS: All 13 cases (100%) evaluated for the presence of HER2 were nonimmunoreactive for the receptor. Only one of the 13 cases (<8%) stained positive for EGFR. CONCLUSIONS: In several malignancies, the overexpression EGFR and HER2 is associated with clinically aggressive behavior. Despite a limited sample size, EGFR and HER2 are not overexpressed in SSC; this is cognate with the current opinion that SSC possesses limited metastatic potential. With prior reports demonstrating that approximately 80% of cutaneous SCC express EGFR, the negative expression of EGFR and HER2 in SSC presents the potential variable nature of the histologic subtypes of SCC, which can have prognostic and therapeutic implications.