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PubMed2006

Malignant adnexal neoplasms.

Malignant cutaneous adnexal neoplasms are one of the most challenging areas of dermatopathology. Tumors of the pilosebaceous apparatus can occur as single-lineage neoplasms or may manifest as complex proliferations with multilineal differentiation patterns including not only the germinative component of the hair bulb, the inner or outer root sheath epithelium and the sebaceous gland and duct, but also the sweat duct components that relate to the apocrine secretory apparatus which empties into the follicle near the follicular bulge. Eccrine and apocrine neoplasms present a bewildering array of morphologies, which often defy precise classification. The purpose of this review is to discuss in detail the malignant neoplasms of the cutaneous adnexae and their benign and prognostically indeterminate mimics.

Diagnosis, Differential↗
PubMed1999

The use of the 1.0 mm handpiece in high energy, pulsed CO2 laser destruction of facial adnexal tumors.

BACKGROUND: The treatment of syringoma and trichoepithelioma has included punch and shave biopsy, excision, electrodessication, as well as continuous wave and superpulsed carbon dioxide laser ablation. More recently, high-energy pulsed CO2 lasers have been reported to be effective with standard available handpieces that deliver collimated beams. OBJECTIVE: To report our experience using a focusing handpiece (1.0 mm spot at focus) with a high energy pulsed CO2 laser. METHODS: Four patients with syringoma and two with multiple trichoepithelioma were treated with a high energy pulsed CO2 laser using a 1 mm spot size focusing handpiece. Pulse energies ranged from 125 to 250 mJ. All patients were followed 2 weeks after treatment and then for variable periods ranging from 8 to 18 months (mean=13.3 months). RESULTS: The 1 mm spot focusing handpiece permitted rapid tumor ablation with optimal matching of lesion size and laser spot diameter. Recurrence of tumor was associated with superficial ablation while complications such as hypopigmentation and atrophy were associated with deeper ablation. CONCLUSION: Facial adnexal tumors such as syringoma and trichoepithelioma can be successfully treated with the 1.0 mm handpiece in tandem with high energy pulsed CO2 lasers.

Adult↗
PubMed2001

Microcystic adnexal carcinoma: report of 13 cases and review of the literature.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare tumor of the skin. Clinically it often masquerades as a firm, subcutaneous nodule on the head and neck regions. Microscopically it extends far beyond assessed clinical margins spreading locally in the dermal, subcutaneous, and perineural tissue planes. The local recurrence rate by standard excision is about 50%. Recent preliminary reports indicate more favorable cure rates with Mohs micrographic surgery (MMS). OBJECTIVE: To present our data on 13 cases (12 patients) of MAC treated by MMS. In addition, we reviewed the medical literature to summarize the accumulated experience of MMS treatment in the management of MAC. We also present a case of bilateral MAC of the face and describe a renal transplant recipient on immunosuppressive therapy who developed MAC of the nasal bridge. METHODS: We reviewed and updated our series of MAC cases treated by MMS over the last 9 years. A total of 13 cases of MAC are reviewed. We also searched the literature for MAC treated by MMS with a follow-up of more than 2-years. RESULTS: One patient had bilateral MAC of the nose and cheek. Another patient developed a MAC of the nasal bridge 20 years after renal transplantation. In this patient predisposing factors were radiation for teenage acne and immunosuppression therapy. A total of 13 cases of MAC were treated by MMS with no recurrences, with a mean follow-up of 5.0 years (range 1.1-8.0 years). CONCLUSION: We update the medical literature with 13 MAC cases treated by MMS. To our knowledge there have been 148 cases of MAC reported in the world literature. Including our series, there have been 73 cases of MAC treated with MMS. There were only four treatment failures. Regional and/or distant metastasis from MAC is rare, with only one reported death. Following MMS, the 2-year success rate was 89.7% (35 of 39). The accumulated data continue to confirm that when MAC is discovered early and is readily accessible to excision by MMS and other subspecialty support, a favorable outcome can be expected.

Adult↗
PubMed2001

Neglected microcystic adnexal carcinoma: the second reported case in a black patient.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a histologically aggressive appendageal neoplasm that often pursues a beguilingly indolent clinical course. MAC has previously been reported only once in a black patient. OBJECTIVE: To present the second MAC ever recorded in a black patient. METHODS: We describe a MAC of substantial dimension occurring in a black patient. The literature was subsequently examined for MAC occurring in ethnic patients. RESULTS: This MAC is remarkable not only for its occurrence in a black patient, but also because it is one of the largest MACs ever recorded (8 cm), it occurs in a relatively less common site (scalp), and its latency period before diagnosis is the longest ever definitively documented (31 years). CONCLUSION: MAC is rare among non-Caucasians. Its relatively indolent clinical growth, especially in sites that are poorly accessible to patient observation, may lead to significant delay in diagnosis and treatment.

Carcinoma, Skin Appendage↗
PubMed2002

Microcystic adnexal carcinoma (MAC) of the scalp with extensive pilar differentiation.

BACKGROUND: Microcystic adnexal carcinoma (MAC), or sclerosing sweat duct tumor, is an uncommon, locally aggressive tumor. It typically involves the upper lip or face of middle-aged adults and rarely involves the scalp. It is characterized histologically by both pilar and eccrine differentiation and is associated with frequent perineural invasion. MAC is frequently misdiagnosed because of its bland and asymptomatic clinical presentation. In addition, its defining histologic features may be missed with a superficial biopsy. OBJECTIVE: To describe a case of MAC in the scalp treated with Mohs surgery. We describe a case with extensive pilar differentiation and clear cell changes. In addition, the clinical and histologic characteristics of this rare neoplasm are reviewed. METHODS: Mohs micrographic surgery (MMS) was performed on this neoplasm. RESULTS: The tumor was successfully excised in one surgery (five stages) and is without evidence of recurrence at 18 months. CONCLUSION: We present a case of MAC of the scalp in a 64-year-old white man treated successfully with MMS.

Carcinoma, Skin Appendage↗
PubMed1992

Preoperative discrimination between ovarian carcinoma, non-ovarian gynecological malignancy and benign adnexal masses using serum levels of CA125 and the polymorphic epithelial mucin antigens CASA, OSA and MSA.

Serum levels of the tumor associated antigens CA125, CASA, OSA and MSA were determined preoperatively in a non-consecutive series of patients with: invasive epithelial ovarian cancer (OC, n = 87), ovarian tumors of low malignant potential (LMP, n = 9), benign adnexal masses (BAM, n = 48) and other peritoneal and pelvic malignancies (n = 48). In addition, serum levels of CASA, OSA, and MSA were determined in 3477 asymptomatic well women. Ninety-eight percent of the asymptomatic women had CASA levels < 6.0 U ml-1, OSA levels < 5.5 U ml-1 and MSA levels < 80.0 U ml-1. Serum CA125 levels were> 35 U ml-1 in 89% of OC, in 44% of LMP, and in 23% of BAM. Serum CASA levels were> 6.0 U ml-1 in 58% of OC, in 0% of LMP, and in 0% of BAM. Serum OSA levels were> 5.5 U ml-1 in 61% of OC in 0% of LMP and in 4% of BAM. Serum MSA levels were> 80.0 U ml-1 in 56% of OC, in 11% of LMP, and in 10% of BAM. When cut-off levels were set to exclude all patients with BAM, the best discrimination from OC using a single assay was achieved using CASA (58%). However, a combination of CASA and CA125 gave positive levels in 69% of OC at levels which precluded BAM. All markers were also elevated in some colon cancers, cervical cancers, uterine cancers and other peritoneal malignancies. A combination of CA125 and CASA levels, obtained preoperatively may assist the general gynecologist in avoiding potentially difficult oncologic surgery.

Journal Article↗
PubMed1999

Pretreatment evaluation of adnexal tumors predicting ovarian cancer.

Kobal B, Rakar S, Ribic-Pucelj M, Tomazevie T, Zaletel-Kragelj L. Pretreatment evaluation of adnexal tumors predicting ovarian cancer. The objective of this study was to determine the ability of tumor marker assessment, gray-scale transvaginal with color Doppler ultrasonography to predict ovarian malignancy. One hundred thirty-four subjects with ovarian masses who entered the study prospectively underwent pelvic examination, tumor marker assessment and gray-scale transvaginal with color flow Doppler ultrasonography preoperatively. Malignancy predictors were statistically evaluated with stepwise multiple logistic regression, and the scores from the model were transformed to probability for having a malignant disease. The presence of neovascularization, intracystic papillary projections, elevated serum CA 125, and age over 45 years were significant predictors for malignancy. Positive predictive value (PPV) for the regression model was 89.0%, and negative predictive value (NPV) was 96.8%. Probability for malignancy ranged from 0.004 to 0.991 depending on which covariates were included. Logistic regression analysis of pretreatment diagnostic gray-scale and color Doppler ultrasonographic characteristics, together with CA 125 enabled a creation of probability assessment scale for individual estimation of ovarian mass, which may contribute to final clinical decision.

Journal Article↗
PubMed2000

Maximizing ovarian salvage when treating idiopathic adnexal torsion.

Idiopathic adnexal torsion threatens ovarian function. The best ways to maximize ovarian salvage have not been stressed widely. The authors describe 2 girls who illustrate the benefit of untwisting the torsion, limiting resection and performing bilateral gonadopexy to prevent both ipsilateral recurrence and contralateral occurrence.

Adolescent↗
PubMed2001

Techniques for surgical correction of adnexal disease.

The adnexa frequently requires the general practitioner to intervene surgically to manage underlying disease or as an ancillary aid in the management of corneal disease. Both acquired and hereditary disorders are encountered. This article reviews general anatomy and physiology of the eyelids and discusses indications for the more commonly performed surgical procedures and their execution. Procedures include tarsorrhaphy, management of eyelid lacerations, eyelid tumor resection, correction of upper and/or lower lid entropion, ectropion, a medial and lateral canthoplasty, colobomatous syndrome in the cat, management of distichiasis, trichiasis and ectopic ciliae, and reduction of a prolapse of the gland of the third eyelid. Finally a surgical pack inventory useful for adnexal surgery is described.

Animals↗
PubMed2004

[Microcystic adnexal carcinoma (malignant syringoma) of the nose: case report and review of the literature].

A case of Malignant Syringoma (syn. = Microcystic Adnexal Carcinoma, Sclerosing Swat Duct Carcinoma) of the nose in a 44 year old female patient is reported. The tumor had been misdiagnosed as a Rhinophyma some 20 years before and had thus been treated under cosmetic aspects. Besides the appearance, the patient did not suffer any complaints and was referred to the ENT-department of the Klinikum Hannover for cosmetic reasons. Histopathological examination after removal revealed a malignant syringoma, which, due to its extensive size and subepithelial growth pattern, made a complete ablation of the entire nose and the adjacent soft tissue of the face necessary. Pathohistologically cellular atypia, invasive growth pattern, perineural and perivascular infiltration was characteristic. Quantitative DNA anaylsis revealed a tumor with a diploid stem line and only few aneuploid cells. Malignant syringoma is a rare differential diagnosis of face skin tumors. The present case is discussed based on a review of the literature.

Adolescent↗
PubMed1987

[Ca 125 values in inflammatory adnexal disease and endometriosis].

The serum concentration of the tumour marker CA 125 was determined during an inflammatory adnexitis of a young woman of 22 years of age, the measured value being 478 kU/l. After the inflammation had subsided, a malignoma was largely excluded by laparoscopy; the CA 125 value had dropped to 70 kU/l. In a woman of 34 years of age who had an endometriosis that had been identified for the last ten years, as well as an acute suppurative fibrinous peritonitis, the CA 125 value was found to be 205 kU/l. On the fourth postoperative day the value was around 165 kU/l, on the 13th and 26th postoperative day 273 kU/l and 168 kU/l, respectively. There were no signs of an inflammation.

Adult↗
PubMed1993

Laparoscopy in adnexal pathology in the child: a study of 28 cases.

We report our experience with laparoscopy in adnexal pathology in the child. Twenty-eight children (mean age 13 years; range 8 to 16) underwent 30 laparoscopy procedures. Therapeutic laparoscopy was performed in 3 cases: transposition of an ovary before radiotherapy, bilateral gonadal excision for Turner's syndrome and ovarian fixation after contralateral torsion of normal adnexa (TNA). Diagnostic and potentially therapeutic laparoscopy was performed in 25 cases: 12 suspected torsion of adnexa (6 confirmed), 4 possible ovarian or appendicular pathology (1 appendicitis), 4 to confirm the histological nature of an ovarian tumor (2 functional cysts, 1 old TNA, 1 dermoid cyst), 3 suspected salpingitis (2 confirmed), and 2 chronic pelvic pain (1 endometriosis). No pathology was found in 2 cases, and in 1 case pelvic adhesions prevented confirmation of the diagnosis. Thirteen laparoscopically-guided surgical acts were performed: 2 detorsions of adnexa, 2 excisions of necrosed adnexa and 9 punctures with or without biopsy for functional cysts. Intralaparoscopic detorsion of TNA was complicated in one case by fibrinolysis requiring secondary laparotomy. Mean hospitalisation was 3 days (range 1 to 16). the preferential therapeutic indications for laparoscopy in the child are transposition of an ovary and ablation of the gonads in case of sexual ambiguity. It is used diagnostically in cases of sudden pelvic pain. In addition to this diagnostic role, it now allows most treatments to be carried out (detorsion with or without fixation, transparietal cystectomy), including those for associated lesions (appendicectomy). Its morbidity is quite low, which warrants increasing the number of indications in pediatric pathology.

Adolescent↗
PubMed1998

An evaluation of transvaginal ultrasound-guided aspiration of simple adnexal cysts.

We carried out this clinical study to evaluate the benefits the risks of transvaginal ultrasound-guided aspiration in a group of patients. The study group consisted of 22 premenapausal women who were detected as having benign adnexal cysts, based on ultrasound appearances. Before the procedure, all patients were evaluated thoroughly by pelvic examination, transvaginal ultrasound and serum measurements of CA-125. Oestradiol CA-125, follicle stimulating hormone, luteinising hormone and progesterone were analysed in aspirated cystic fluids as well as cytological evaluation. It was concluded that measurements of follicle stimulating hormone, luteinising hormone, oestradiol and progesterone concentrations in cystic fluid had no prognostic significance. But CA-125 levels over 55 iu/ml were likely to predict the recurrence of the cysts. Cytological examination of the aspirated cystic fluids revealed that 21 cysts were benign functional cysts, but one was serous cystadenoma. We observed recurrence following aspiration in five patients (22.7%) who were followed for 6 months. Patients with simple ovarian cysts will benefit from transvaginal ultrasound-guided aspiration by being saved from surgery and its related complications. However, its use should be limited to those masses that appear to be completely cystic with well-defined borders, because of the risk of the intra-abdominal spillage of the contents of complicated cysts.

Journal Article↗
PubMed2006

Ocular adnexal Langerhans cell histiocytosis clinical features and management.

PURPOSE: To review the clinical features, investigations, management, and outcomes of Langerhans cell histiocytosis (LCH) with ocular adnexal involvement. MATERIALS AND METHODS: Retrospective, non-comparative, chart review of 30 patients with LCH involving the ocular adnexa treated at 6 major Australian hospitals. Clinical features, imaging findings, treatment, local and distant recurrence and outcome were evaluated. RESULTS: Twenty-four patients (80%) were male with a mean age of presentation of 9.5 years. Females presented at an earlier age (mean age 4.1 years) with more severe involvement. Eighty percent of the cases presented with periorbital swelling. Computed tomography usually showed destructive osteolytic lesions centred on the frontal bone. Twenty-four patients (80%) had unifocal, unisystem disease. From this group, none of 13 treated with excision alone had recurrence whereas 2 of 6 treated with excision followed by chemotherapy recurred. CONCLUSIONS: Unifocal, unisystem LCH often presents to the ophthalmologist and can usually be diagnosed on clinical and imaging grounds. Computed tomography and magnetic resonance imaging are complementary imaging studies. Biopsy is essential to confirm diagnosis. Unifocal, unisystem disease can be treated with local excision and curettage. Incomplete excision, recurrent disease or multifocal disease may require systemic chemotherapy.

Adolescent↗
PubMedDate not supplied

Adnexal surgery for severe ocular surface disease.

Secondary causes of ocular surface disease are-to a large extent-due to disorders of the ocular adnexae. The main pathomechanisms involved include exposure, abrasion and malnutrition, resulting from a multitude of disorders such as ec- or entropion (e.g. in cicatrizing conjunctivitis), lid retraction and severe aqueous deficiency. In the presence of these problems, surgical attempts of ocular surface reconstruction frequently fail. Here we review established and evolving new techniques in the field of adnexal surgery to specifically address these problems.

Corneal Diseases↗
PubMed1990

Laparoscopic management of adnexal cystic masses.

Four hundred twenty of 481 patients with adnexal cystic masses (508 cysts) confirmed by laparoscopy were treated by translaparoscopic surgery only. The remaining 61 patients were treated by laparotomy for one of the following reasons: malignancy or suspicion of malignancy (19 cases) and dense adhesions or large cysts (42 cases).

Adolescent↗
PubMed1994

Paravaginal female adnexal tumor of probable wolffian origin.

Female adnexal tumor of wolffian origin (FATPWO) is a lesion that predominantly occurs in the broad ligament. Its occurrence in the right paravaginal area is rare; only one other case has been reported to occur at this site. The authors report a case of paravaginal FATPWO in a 20-year-old woman that recurred twice during a 5-year period. The microscopic and ultrastructural features of this lesion were identical to other cases of FATPWO reported in the literature. This tumor must be recognized, especially at this site, so that it is not confused with vaginal adenocarcinoma related to diethylstilbestrol exposure in utero.

Adenoma↗
PubMed2006

Bacteria-eradicating therapy with doxycycline in ocular adnexal MALT lymphoma: a multicenter prospective trial.

BACKGROUND: An association between ocular adnexal MALT lymphoma (OAL) and Chlamydia psittaci (Cp) infection has been proposed, and recent reports suggest that doxycycline treatment causes tumor regression in patients with Cp-related OAL. The effectiveness of doxycycline treatment in Cp-negative OAL has not been tested. METHODS: In a prospective trial, 27 OAL patients (15 newly diagnosed and 12 having experienced relapse) were given a 3-week course of doxycycline therapy. Objective lymphoma response was assessed by computerized tomography scans or magnetic resonance imaging at 1, 3, and 6 months after the conclusion of therapy and every 6 months during follow-up. Cp infection in patients was determined by touchdown enzyme time-release polymerase chain reaction (TETR-PCR). Statistical tests were two-sided. RESULTS: Eleven patients were Cp DNA-positive and 16 were Cp DNA negative. Doxycycline was well tolerated. At a median follow-up of 14 months, lymphoma regression was complete in six patients, and a partial response (> or = 50% reduction of all measurable lesions) was observed in seven patients (overall response rate [complete and partial responses] = 48%). Lymphoma regression was observed in both Cp DNA-positive patients (seven of 11 experienced regression) and Cp DNA-negative patients (six of 16 experienced regression) (64% versus 38%; P = .25, Fisher's exact test). The three patients with regional lymphadenopathies and three of the five patients with bilateral disease achieved objective response. In relapsed patients, response was observed both in previously irradiated and nonirradiated patients. The 2-year failure-free survival rate among the doxycycline-treated patients was 66% (95% confidence interval = 54 to 78), and 20 of the 27 patients were progression free. CONCLUSIONS: Doxycycline is a fast, safe, and active therapy for Cp DNA-positive OAL that was effective even in patients with multiple failures involving previously irradiated areas or regional lymphadenopathies. The responses observed in PCR-negative OAL may suggest a need for development of more sensitive methods for Cp detection and investigation of the potential role of other doxycycline-sensitive bacteria.

Adult↗