The guidelines cover diagnosis and referral, preoperative investigations, surgical management (local treatment, groin treatment including sentinel lymph node procedure, reconstructive surgery), radiation therapy, chemoradiation, systemic treatment, treatment of recurrent disease (vulvar recurrence, groin recurrence, distant metastases), and follow-up.

No abstract

Vulvar lichen sclerosus (LS), a lymphocyte-mediated chronic skin disease, begins with uncharacteristic symptoms and progresses undiagnosed to atrophy and destructive scarring. Some patients with longstanding advanced LS have an increased risk of vulvar carcinoma. Early LS is treatable, although not curable, if diagnosed early. Therefore, patients with persistent vulvar symptoms should be biopsied to establish the diagnosis. In contrast to advanced LS, the histological features in early LS are quite subtle and often more prominent in adnexal structures than in interfollicular skin. Adnexal structures show acanthosis, luminal hyperkeratosis and hypergranulosis with/without dystrophic hair and basement membrane thickening. The epidermis/mucosa shows mild irregular, occasionally psoriasiform acanthosis and focal basement membrane thickening. Early dermal changes are homogenized collagen and wide ectatic capillaries in dermal papillae immediately beneath the basement membrane. The lymphocytic infiltrate can be sparse or dense, lichenoid or interstitial with epidermal lymphocyte exocytosis and lymphocytic/lymphohistiocytic vasculitis. Dermal melanophages indicate preceding keratinocyte/melanocyte destruction. Biopsy specimens of early LS rarely display all features. Therefore, serial sections and periodic acid-Schiff reactions are necessary for their identification. Recognition and treatment of these early stages of LS may result in longstanding remission. Progression to atrophic stages with their associated morbidity and even to squamous cell carcinoma may be prevented.

Hormonal therapy with progestins, aromatase inhibitors and gonadotropin-releasing hormone analogues has become an effective treatment alternative to radiation and chemotherapy for low-grade endometrial stromal sarcoma patients. Preventive hormonal therapy is of particular interest in the setting of concomitant endometriosis.

No abstract

No abstract

We report an aggressively behaving malignant trichogenic tumor arising in a trichoblastoma (TB) with widespread lymphatic and hematogenous metastases in a 55-year-old man with a concomitant B-cell chronic lymphocytic leukemia. The primary tumor had been present and unchanged for as long as 40 years before excision. Typical trichogenic TB with dystrophic calcification and even ossification was still present peripheral to the malignant transformation. The malignant neoplasm consisted of basaloid cells, spindle cells arranged in fascicles and densely packed rounded nests or "cell balls." The metastases consisted of immature basaloid cells and cell balls, and the recurrences became successively more undifferentiated. The residual TB reacted with antibodies to cytokeratin (CK) 6, 8, 14, and 17 and focally to S-100; the malignant primary tumor reacted uniformly with antibodies to vimentin and only focally with antibodies to CK and S-100. The metastatic tumor had lost epidermal CK expression but maintained expression of S-100 in paraffin-embedded tissues. Trichoblastic differentiation was confirmed in frozen tissues with antibodies to hair keratins. No expression of p53 or bcl-2 was identified, but p-glycoprotein (MDR-1 gene related) was expressed by primary and metastatic tumor cells. We believe that this neoplasm is best classified as a trichoblastic carcinoma arising in a TB in association with a B-cell chronic lymphocytic leukemia. This case illustrates that TBs have the potential for malignant transformation and aggressive behavior.

Carcinomas of the oro/nasopharynx often present with regional lymph node (LN) metastases before the primary tumour is discovered. Some of the cervical LN metastases feature prominent cyst formation. Solitary cystic LN metastases have often been mistaken for primary squamous cell carcinomas (SCC) originating within a branchiogenic lateral neck cyst (LNC), resulting in the commonly used terms 'branchioma' and 'branchiogenic carcinoma ' (von Volkmann, 1882;Wolff et al, 1979;Khafif et al, 1989;Parks and Karmody, 1992;Carroll et al, 1993;Hall and Dexter, 1993;Flanagan et al, 1994;Knobber et al, 1995). For patients with an isolated finding of SCC in a neck LN, a search for a primary carcinoma in the upper aerodigestive tract is mandatory, including US, MRI and CT, as well as extensive biopsies of the base of tongue, nasopharynx and tonsillectomies in the case of clinically occult tumours (Spiro et al, 1983;Flanagan et al, 1994). This clinical practice has provided overwhelming evidence in recent years that isolated cystic SCC in neck LN are metastases from primary SCC of the palatine tonsils, the base of the tongue and the nasopharynx, tissues also collectively referred to as the Waldeyer's ring (Micheau et al, 1974(Micheau et al, , 1990Flanagan et al, 1994;Thompson and Heffner, 1998). Although this explanation has been slow to gain wide recognition and claims of the first true 'branchiogenic carcinoma' have been made rather recently (Micheau et al, 1974;Jones et al, 1993), cystic SCC in cervical LN is now regarded as a typical presentation of metastatic SCC arising in the oro/nasopharynx. At the same time, the idea of a primary branchiogenic carcinoma has become a vanishing concept (Thompson and Heffner, 1998). Despite the body of literature about this topic, there is little information regarding the frequency of cystic LN metastases from SCC of the Waldeyer's ring. Our goal was to establish the frequency of cystic LN metastases in our series of 123 patients with SCC of Waldeyer's ring origin who had been treated primarily surgically along with neck dissections. Furthermore, we discuss the morphological features and clues for establishing a diagnosis of cystic LN metastases rather than 'branchiogenic carcinoma'. 1984 and 1997, 108 patients with biopsy-proven SCC of Waldeyer's ring origin underwent neck dissections at the ORL Department of the University Hospital in Graz, Austria. Fifteen patients were treated at the Elisabethinen Hospital, Graz, Austria, between 1992 and 1997. Archival formalin-fixed, paraffinembedded haematoxylin and eosin (HE) stained sections of all primary SCC and their corresponding neck dissections were reviewed. SCC were separated into (1) tonsillar origin (palatine tonsil), (2) base of tongue origin and (3) nasopharyngeal origin. Extensive tumours involving the entire oropharynx, and multiple or synchronous intraoral carcinomas were not included in this study, because we were not able to determine the exact origin of the SCC in these cases. Initial diagnosis was established by biopsy and fol...