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Periocular microcystic adnexal carcinoma: management and outcome with Mohs' micrographic surgery.

PURPOSE: To report a series of patients with periocular microcystic adnexal carcinoma (MAC) treated with Mohs' micrographic surgery (MMS). DESIGN: Prospective, interventional case series. METHODS: This series included all patients treated with MMS for periocular MAC, who were monitored by the Skin and Cancer Foundation Australia between 1993 and 2002. RESULTS: There were 5 patients (4 males, 1 female) with a mean age of 52 +/- 12 years. Four tumors were located in the medial canthus and one in the lower lid. The tumor was initially misdiagnosed as basal cell carcinoma in 3 patients and squamous cell carcinoma in 1 patient. The average number of excision levels required for complete removal of the tumor during MMS was 2.2 (range 1-6). Perineural invasion was recorded in one patient.Five-year follow-up data was available for 3 patients, and there was no tumor recurrence in any of these cases. CONCLUSION: The low 5-year recurrence rate of periocular MAC with MMS emphasizes the importance of margin-controlled excision of this tumor.

Carcinoma, Skin Appendage↗

Microcystic adnexal carcinoma: report of seven cases including one with lung metastasis.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm, with a high rate of local recurrences. OBJECTIVE: A series of MAC was analyzed and compared to previously published cases. METHODS: Seven cases of MAC were identified in the register of the institution. Medical and pathological records were reviewed. RESULTS: The primary MAC were located on the face in all patients, and 85% were initially misdiagnosed. The mean follow-up duration was 108 months. The recurrence rate was high: 4 patients developed recurrences. In 3 patients, the course of the disease was severe: one of them developed pathologically proven lung metastasis. CONCLUSION: The present study and review of the literature confirm the clinically aggressive evolution of MAC and its rare ability to give rise to metastasis. Long-term clinical follow-ups with imaging investigations are mandatory.

Adult↗

Aspiration cytology of 147 adnexal cysts with histologic correlation.

OBJECTIVE: To assess the usefulness of cytology in the diagnosis of 147 histologically established adnexal cysts. STUDY DESIGN: Retrospective, macro-microscopic study based on fluid aspirated from 132 ovarian and 15 extraovarian cysts and projected as a cytohistologic correlation. RESULTS: Typical macroscopic features were identified in 76% of endometriotic cysts, in 53% of mucinous neoplasms and in 67% of dermoid cysts. Cytology helped to identify 67% of nonneoplastic and 56% of neoplastic cysts. The lowest diagnostic sensitivities were observed in functional cysts and benign serous neoplasms (50%), while the highest were shown by endometriotic cysts (76%) and malignant epithelial neoplasms (71%). Inadequate samples were obtained from all types of cysts, even malignant ones (two mucinous cystadenocarcinomas). Diagnostic cytology was useless in extraovarian cysts (33% sensitivity). An adult granulosa cell tumor was erroneously diagnosed as a follicular cyst by cytologic examination. CONCLUSION: Examination of the cyst fluids obtained by aspiration demonstrated low sensitivity, with 43% of inadequate samples obtained from all types of cysts. Malignant cystic neoplasms may be overlooked in inadequate samples. Our study also revealed that specificity in this type of analysis is high in inadequate samples, provided that the technique is carried out correctly.

Adenofibroma↗

Angiography in uterine and adnexal tumors.

A series of 106 patients with uterine or adnexal lesions is presented. Bilateral selective internal iliac angiography proved to be valuable in the preoperative diagnosis of gynecologic tumors regarding their origin and in the differential diagnosis between malignant and benign tumors.

Adenocarcinoma↗

Eccrine acrospiroma of the eyelid with oncocytic, apocrine and sebaceous differentiation. Further evidence for pluripotentiality of the adnexal epithelia.

A wide variety of sweat gland tumors occur in the eyelids. We used light and electron microscopy to investigate an eccrine acrospiroma showing areas of oncocytic, apocrine and sebaceous differentiation. To our knowledge this is the first report of an oncocytic lesion arising from an eccrine sweat gland, and only the second report of such a lesion arising from the skin. Furthermore, apocrine and sebaceous differentiation are atypical in lesions originating from sweat glands. The wide spectrum of differentiation in this adenoma lends further support to the concept of pluripotentiality of the adnexal glandular epithelia.

Adenoma, Sweat Gland↗

Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci.

Non-Hodgkin lymphomas are among the most common primary tumors occurring in the ocular adnexa. Herein, we present a 14-year single-institution experience in 62 patients with primary ocular adnexal lymphomas (OALs). Association with Chlamydia psittaci infection is examined in 57 tumor specimens. Extranodal marginal zone lymphoma (EMZL) was the most frequent histologic subtype (89%). The majority of patients with EMZL (84%) presented with stage E-extranodal (IE), however only 16% had an advanced stage. All stage IE patients were treated with local radiotherapy, whereas patients with disseminated disease received systemic therapy with or without local irradiation. All but 1 patient with EMZL achieved complete remission (CR). During a median follow-up of 52 months (range, 3-153 months), the estimated 5-year overall survival (OS) and freedom from progression (FFP) were 96% and 79%, respectively. During the follow-up, 22% of patients relapsed, mainly in extranodal sites, and 4% transformed to diffuse large B-cell lymphoma. None of the patients exhibited local orbital failure in the radiation field. None of the OAL specimens harbored C psittaci DNA. Our study demonstrates that EMZLs, accounting for the majority of primary OALs, are characterized by an indolent natural history with frequent, continuous extranodal relapses. In South Florida, OALs are not associated with C psittaci infections.

Adult↗

Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options.

The recent literature shows that interest in ocular adnexal lymphomas and their biologic and clinical characteristics--along with their possible association with Chlamydia psittaci infection and therapeutic management with rituximab or anti-Chlamydia psittaci antibiotic therapy--is considerable. These new data have modified the previously reported features of this disease and have made an updated review of the literature necessary. The aims of this review are to present the current knowledge on the biology of these lymphomas, their clinical features and prognostic factors, and the panel of all available treatment options.

Chlamydia Infections↗

Microcystic adnexal carcinoma: management options based on long-term follow-up.

Microcystic adnexal carcinoma (MAC), a recently described neoplasm that frequently affects the head and neck, presents a confusing problem for the clinician due to its unusual behavior. The individual cells have a bland microscopic appearance, and there is a predilection for neural invasion. Four cases of MAC are reported. All four cases demonstrate the difficulty with pathologic diagnosis. Follow-up of as long as 33 years begins to delineate the protracted nature of MAC. In addition, this paper includes the first report of a case of lymph node metastasis. Although resection may result in a significant defect, negative margins may not be achieved. Despite this, the defect can heal, as demonstrated by the cases described. In addition, MAC may recur many years later, irrespective of the status of the margins at the time of surgery. Given these unusual characteristics and the slowly progressive nature of MAC, strong consideration must be given to less radical surgical procedures, with close follow-up for grossly recurrent disease.

Aged↗

Preventive procedure of dysuria after radical hysterectomy by adnexal flap fixation to the bladder.

To prevent postoperative dysuria, which occurs inevitably after the radical hysterectomy, several surgical procedures have been tried. The principal method is to suture adnexal flaps to the bladder trigone and fundus. Of four procedures so far tried sequentially, the best one was to fix the bladder trigone and to support it with round ligament flaps sutured with the bladder fundus covered by tubal flaps (Type IV). In follow-up studies, Type IV proved more preferable than the other three types or the non-sutured control group in regard to the following aspects: (1) days necessary for the disappearance of residual urine, (2) appearance of urinary sensation, (3) acquirement of urinary sensation, (4) incidence of urinary incontinence, (5) residual urine/bladder capacity ratio, (6) cystometric findings, and others. Effectiveness of our procedures, particularly of Type IV, may be ascribed to the supported bladder trigone and fundus by sutured tissues and to the acquirement of urinary sensation, rather than to the restoration of nervous contact between the bladder and the micturition center in the spinal cord.

Adnexa Uteri↗

A case of female adnexal tumor of probable Wolffian origin: histologically, tumor cells showed three different patterns.

We report a 63-year-old woman with a female adnexal tumor of probable Wolffian origin, which arose within the leaves of the broad ligament, connected to the left ovary and the fallopian tube. She remains alive without evidence of disease 2 years after left salpingo-oophorectomy without subsequent adjuvant therapy. Microscopically, tumor cells were arranged in three different patterns; closely packed tubules, solid, and sieve-like. Cells had uniform round or oval nuclei and mitoses were rare. Although the majority of these tumors are benign, a few cases have shown malignant potential. Therefore, such tumors should receive careful follow-up for possible recurrence and/or metastasis.

Broad Ligament↗

Microcystic adnexal carcinoma involving a large portion of the face: when is surgery not reasonable?

BACKGROUND: We report a case of microcystic adnexal carcinoma (MAC) involving a large portion of the face, one of the largest of any MAC reported thus far in this area, and review the literature regarding the nature of the tumor and available treatments. We also review all of the reported cases of metastases and the possible role of radiation in the etiopathogenesis of this tumor. OBJECTIVE: To review the literature about what is known about therapy for MAC and what options are available to patients who have this disease. MATERIALS AND METHODS: Case report and review of the literature. RESULTS: Of the 274 cases of MAC thus far reported, there are 6 cases of metastases, only 1 of which resulted in death. CONCLUSION: Mohs surgery should be the treatment of choice for this tumor; however, when extirpation entails sufficiently large morbidity, given the low rate of metastases and mortality, observation is a reasonable alternative.

Biopsy↗

DF3 (CA15-3) antibody as a marker of cutaneous adnexal tumors.

DF3(CA15-3) monoclonal antibody detects the 20 amino acid sequence of epithelial mucin. In normal cutaneous tissue DF3 antibody exhibits strong and constant positive reactivity with sebaceous and secretory segments of eccrine and apocrine sweat glands. All epidermal tumors are DF3 negative but poorly differentiated squamous cell carcinoma. Various adnexal tumors such as eccrine hidrocystoma, tubular apocrine adenoma, syringocystadenoma papilliferum, eccrine poroma, eccrine spiradenoma, clear cell hidradenoma, mixed cell tumor, eccrine porocarcinoma, extramammary Paget's disease and adenoid cystic carcinoma are DF3 positive, which indicates epithelial mucin production by these tumors.

Adenoma↗

The clinical characteristics and treatment results of ocular adnexal lymphoma.

PURPOSE: To assess the clinical pattern, the histopathological findings, the response to treatments, the recurrence pattern and the prognosis of malignant lymphoma in the ocular adnexa. METHODS: This study was performed on 22 total eyes from 17 patients who were diagnosed with ocular adnexal malignant lymphoma. We retrospectively analyzed the medical records for patient information including the histological classification based on age, the gender of each patient, the symptoms and signs at the initial diagnosis, the presence of binocular invasion, the findings of the surgical biopsy, the clinical stage of each patient's tumor, and the treatment methods used and their effectiveness. The mean follow-up period was 24.8 months. RESULTS: The mean age of patients studied was 46.8 years old. Six females and 11 males were included in the study. Fifteen cases consisting of 20 total eyes represented extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT). Five of seven patients (71.4%) whose lymphoma occurred within the conjunctiva relapsed after irradiation or chemotherapy, and four of the relapsed patients were salvaged with further therapy. CONCLUSIONS: Extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) constituted 88.2% of all lymphomas involving the ocular adnexa. Lymphoma in the ocular adnexa responded well to conventional treatment, but the recurrence rate of lymphoma in the conjunctiva was significantly high.

Adult↗

Eyelid depigmentation following corticosteroid injection for infantile ocular adnexal hemangioma.

We report an unusual case of development of upper eyelid depigmentation in a child 4 months following corticosteroid injection of a periocular capillary hemangioma. Depigmentation has been described with local steroid injections for other dermatologic conditions, but has not been reported previously with treatment of infantile ocular adnexal hemangiomas.

Adrenal Cortex Hormones↗

Steroid injection versus conservative treatment of anisometropia amblyopia in juvenile adnexal hemangioma.

Because of serious side effects, the indications for intralesional steroid injection of adnexal hemangiomas are unclear. Of 23 children with such lesions who were examined over a period of 9 years, 9 had no evidence of amblyopia and needed no intervention. Five required steroids intralesionally and/or systemically because of threatened occlusion of the pupillary axis. The remaining 9 were considered at risk of anisometropic amblyopia because of induced astigmatism: 5 received injections and 4 were treated with glasses and/or patching alone. The visual, refractive, and cosmetic results of the injected and conservatively managed anisometropes were similar. We recommend that steroid injection be reserved for patients with threatened occlusion of the visual axis and for those with severe astigmatism or amblyopia refractory to conservative management.

Amblyopia↗

Receiver operating characteristic curves of transvaginal Doppler blood flow measurements in benign and malignant adnexal tumors.

The role of receiver operating characteristic curves of transvaginal Doppler velocimetry in predicting malignancy was evaluated in 80 patients with benign and 40 patients with malignant adnexal tumors. The mean values of peak systolic velocity did not differ significantly. Malignant tumors had a significantly higher end diastolic velocity and mean flow velocity than benign tumors. Benign tumors had a significantly higher ratio of peak systolic to end diastolic velocity, pulsatility index, and resistive index than malignant tumors. The diagnostic accuracies in predicting malignancy were as follows: peak systolic velocity, 62%; end diastolic velocity, 79%; ratio of peak systolic to end diastolic velocity, 63%; mean flow velocity, 73%; pulsatility index, 91%; resistive index, RI 92%. Doppler velocimetry analysis allows us to predict the presence of malignancy with limited reliability.

Adolescent↗