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A complex poroma-like adnexal adenoma.

Hidroacanthoma simplex, eccrine poroma, and dermal duct tumor are benign adenomas that develop from excretory ducts of eccrine glands and all three are variants of eccrine acrospiroma. To date, counterparts in apocrine or sebaceous glands have not been reported, but in this study we describe an adnexal, poroma-like adenoma that showed apocrine and sebaceous differentiations. Apocrine structures have the same embryonic origin as does the pilosebaceous system; both are derived from the primary epithelial germ. We suggest that the lesion we describe is truly a sebaceous and apocrine poroma. It must be distinguished from an infundibular adenoma whose pattern reproduces that of follicular poroma.

Adenoma↗

Complex adnexal tumor of the primary epithelial germ with distinct patterns of superficial epithelioma with sebaceous differentiation, immature trichoepithelioma, and apocrine adenocarcinoma.

A 60-year-old man came for treatment of a sharply outlined erythematous plaque on the gluteal area (45 x 20 mm) of 20 years' duration. Eccentrically located on the plaque was a nodule, 20 mm in diameter. Histological study of the plaque showed a superficial platelike tumor with basaloid bland cytology and sebaceous gland differentiation. Histologic study of the nodule found an undifferentiated adenocarcinoma whose ductlike glandular structures opened to the skin surface and infiltrated the whole depth of the dermis. Study of other areas of the lesion detected two more neoplasms. A nodule of squamous cell carcinoma was found within the superficial band of the benign sebaceous tumor. The fourth neoplastic pattern consisted of epithelial islands composed of basaloid cells within a fibroblastic stroma. There was prominent palisading of epithelial cell nuclei at the periphery of the islands, which usually were surrounded by a sheath of mesenchymal cells. In this complex adnexal tumor of the primary epithelial germ, sebaceous and follicular differentiation both simulate neoplastic patterns recently described as separate entities: superficial epithelioma with sebaceous differentiation and immature trichoepithelioma. The undifferentiated adenocarcinoma may represent differentiation toward the third component of the germ, that is, the apocrine gland.

Adenocarcinoma↗

Eccrine or apocrine poroma? Six poromas with divergent adnexal differentiation.

We describe six cases of benign eccrine poroma-like neoplasms with divergent adnexal differentiation. Four cases exhibited sebaceous differentiation in the form of individual or clustered sebocytes with or without sebaceous ducts. One case showed both sebaceous and hair follicle differentiation, and one case showed sebaceous and possible apocrine secretory differentiation. Clinically, most were skin-colored, red, or purple papules or nodules. One patient had a preoperative diagnosis of Bowen's disease, with an erythematous plaque. None recurred following biopsy. Previous reports of similar lesions have suggested a possible role for human papilloma virus (HPV) in their pathogenesis; however, immunohistochemical staining for HPV structural antigens was negative in all six of these cases. Similarities to previously reported cases of eccrine poroma-like neoplasms with sebaceous differentiation are discussed. Given the evidence of sebaceous and follicular differentiation seen in this study and the common embryologic origin of follicular, sebaceous, and apocrine structures, it follows that at least some benign neoplastic proliferations with histopathologic features of "eccrine" poroma could be of apocrine origin.

Acrospiroma↗

Cutaneous adnexal carcinoma arising within a solitary cylindroma-spiradenoma.

Malignant transformation within an adnexal neoplasm with features of spiradenoma and cylindroma is reported in a 60-year-old woman. The malignant component of the tumor consisted of areas of solid carcinoma, with focal squamous metaplasia. In the benign component, apart from the features of spiradenoma and cylindroma, sebaceous differentiation was present adjacent to a cystic space lined by apocrine epithelium. The morphologic composition of this unusual neoplasm supports the concept of a histogenetic relationship between cylindromas and spiradenomas, including their differentiation toward the pilosebaceous-apocrine unit.

Adenoma, Sweat Gland↗

Microcystic adnexal carcinoma associated with primary immunodeficiency, recurrent diffuse herpes simplex virus infection, and cutaneous T-cell lymphoma.

Cutaneous microcystic adnexal carcinoma (MAC) is a rare and poorly understood tumor that predominantly occurs in the head and neck. MAC usually affects people in their fourth and fifth decades. Some patients have had a history of radiation. We present a case of MAC occurring in the left antecubital fossa of an 18-year-old white woman with an unusual immunodeficiency syndrome. The patient also developed a squamous cell carcinoma, a cutaneous T-cell malignancy, and a perigastric leiomyoma. A congenital infection of herpes simplex virus (HSV) persisted throughout her life. The association of HSV infection with MAC and squamous cell carcinoma and that of peripheral T-cell lymphoma with Epstein-Barr virus is discussed in relation to her immunodeficiency.

Adolescent↗

Microcystic adnexal carcinoma following radiotherapy in childhood.

A 36-year-old man was treated by radiotherapy for tinea capitis many years before discovery of microcystic adnexal carcinoma (MAC). Because of patient's refusal of any surgical intervention, we were able to follow the natural course of this tumor for 13 years. This case emphasizes the typical slow development of (MAC). The implication of the association of MAC and radiotherapy are discussed.

Adult↗

Ocular adnexal injury and complications in orbital dog bites.

Orbital dog bites, though statistically uncommon, occur most frequently in children and are associated with severe ocular adnexal injury. Of 16 victims, two-thirds were under 10 and over half under 5 years of age. The wounds consisted of numerous periorbital punctures, and in most cases, full-thickness lid lacerations involving the tear system. There were no serious injuries to the globe. Reversible amblyopia occurred in two children under 3 years of age with damage to the levator muscle. One child suffered a naso-orbital fracture. Because of the obvious nature of the injury, most patients present early and can be managed well with meticulous wound care and primary surgical repair. The use of prophylactic antibiotics, though controversial, appears prudent in such cases. Ophthalmologists treating these injuries must be aware of serious potential complications including occult facial fracture or intracranial penetration in young children, septicemia caused by bacillus DF-2 in patients with prior splenectomy, tetanus, and rabies.

Adolescent↗

Orbital, adnexal, and unusual systemic involvement in Rosai-Dorfman disease.

PURPOSE: To describe the unusual clinical course of a patient with Rosai-Dorfman disease (RDD) affecting the eyelid and orbital tissues and involving the spinal cord. METHODS: Case report. RESULTS: A 68-year-old Indian man first presented in 1994 with a right lower eyelid lump for 1 year. An en bloc excisional biopsy was reported to show "reactive lymphoid hyperplasia with sclerosis." The patient subsequently defaulted follow-up and presented again in 1999 with bilateral lower eyelid masses and proptosis. Computerized tomography showed bilateral orbital, ethmoidal sinus, and frontal sinus soft tissue masses. Bilateral excisional biopsies of the orbital and eyelid masses showed histologic features of RDD. The patient had a history of paraplegia with decompression laminectomy and excision of an epidural mass in 1994. In addition, he underwent excision of soft tissue masses from the abdominal wall in 1993. Retrospective review of the histologic specimens from these two areas showed a histologic picture similar to that of eyelid specimens (in 1994 and 1999). CONCLUSIONS: It is important to consider RDD in addition to lymphoproliferative disorders in a patient with orbital and ocular adnexal masses. The initial histologic presentation may not be pathognomonic.

Aged↗

Guidelines for referral of the patient with an adnexal mass.

Gynecologists have to differentiate between benign and malignant adnexal masses. We review the evidence supporting to the specialty care of a gynecologic oncologist on the basis of the physical examination, imaging studies, family history, and CA 125 determination. We recommend adherence to the ACOG/SGO Joint Opinion guidelines. Specifically, referral to a gynecologic oncologist seems warranted for postmenopausal women with elevated CA 125, nodular or fixed pelvic mass, metastatic disease, ascites, or family history of breast or ovarian cancer. Premenopausal women should be referred if the CA 125 is elevated above 200 U/mL, there is an evidence of metastatic disease or ascites, or strong family history of breast or ovarian cancer.

CA-125 Antigen↗

The incidental postmenopausal adnexal mass.

The postmenopausal ovary continues to produce cysts; the prevalence in an ovarian cancer screening population approaches 18%. Yet 60% to 70% of unilocular cysts resolve spontaneously. Optimal management of an asymptomatic adnexal mass allows surveillance of women at low malignancy risk while triaging intermediate/high-risk women to surgery. Women with unilocular cysts on transvaginal ultrasound (TVS) and a normal CA-125 are monitored with repeat TVS at 3 to 6 months. Those with a complex mass <5 cm and normal CA-125 should have repeat TVS and CA-125 testing in 4 weeks. Surgery is recommended for any women with increasing morphologic complexity or a rising CA-125.

CA-125 Antigen↗

Acute chlamydial salpingitis with ascites and adnexal mass simulating a malignant neoplasm.

Chlamydia trachomatis is a well-known cause of acute and chronic salpingitis, accounting for approximately half of all cases of pelvic inflammatory disease. Typically, patients with acute chlamydial salpingitis present with acute lower abdominal pain, tenderness on bimanual pelvic examination, or vaginal discharge. We describe a case of acute chlamydial salpingitis with marked ascites and an adnexal mass that simulated a malignant neoplasm. Microscopically, a severe lymphofollicular salpingitis and a marked lymphofollicular hyperplasia of the omentum and retroperitoneal lymph nodes were found. Chlamydial inclusions in the fallopian tube epithelium were demonstrated by immunohistochemistry using a mouse monoclonal antibody to a genus-specific outer membrane lipoprotein. Chlamydial infection may cause marked ascites and a palpable adenexal mass and should be considered whenever marked chronic inflammation with a lymphofollicular hyperplasia involves the fallopian tube or other female genital tract sites.

Adnexa Uteri↗

Ascites, elevated CA 125, and a large adnexal mass with an enteroovarian fistula.

BACKGROUND: Rectovaginal and vesicovaginal fistulae occur commonly in human immunodeficiency virus (HIV)-positive women and typically present with a characteristic vaginal discharge. CASE: We report a case of a jejunal-ovarian fistula presenting as suspected malignancy in a 38-year-old woman with HIV. The patient presented with ascites, moderately elevated CA 125, and a 12-cm adnexal mass. She was expedited to surgery secondary to a persistent pelvic mass and the patient's desire for definitive treatment. We believe the patient's immunocompromised state caused a microperforation in the small bowel, which subsequently formed a fistula to the ovary. The patient's left adnexa was removed, along with a 3-cm portion of small bowel. CONCLUSION: Uncommon entities should be included in the differential diagnosis of pelvic masses in immunocompromised women.

Adnexa Uteri↗

Endometrioid carcinoma of the fallopian tube resembling a female adnexal tumor of probable wolffian origin.

Endometrioid carcinoma of the fallopian tube resembling a female adnexal tumor of probable wolffian origin (FATWO) is a rare, recently delineated, low-grade carcinoma that may be confused with both FATWO and high-grade carcinoma. Only about 20 cases have been reported so far, but it probably represents almost half of the endometrioid carcinomas of the fallopian tube. It has a better prognosis than conventional tubal endometrioid and serous carcinomas. Macroscopically, the tumors form a small, solid, polypoid mass that is usually confined within the tube. Microscopically, the tumors are characterized by proliferations of spindle cells in whorls and closely packed confluent elongated glands with focal tubular, sieve-like, and papillary structures. Unlike FATWO, this variant of endometrioid carcinoma exhibits focal squamous differentiation, intraluminal mucin, mild to moderate nuclear atypia, and occasional mitosis. Unlike FATWO, tumor cells are negative for inhibin-alpha and calretinin.

Carcinoma, Endometrioid↗

Female adnexal tumor of probable wolffian origin: a clinicopathological case report and a possible new treatment.

Female adnexal tumors of probable wolffian origin (FATWOs) are rare tumors arising in the broad ligament from the remnants of the mesonephric duct. We report a case of recurrent disease. A 15-year-old girl who presented with a painful pelvic mass underwent a laparotomy with tumor resection. Pathology findings confirmed a FATWO. The tumor recurred within 2 years and was treated with multiple chemotherapy regimens, including a platinum-based drug, and surgery for progressive disease. The tumor was positive for c-kit oncogene (CD 117). Gleevac therapy, a tyrosine kinase inhibitor, was prescribed, and she developed severe persistent lower abdominal pain 2 months later. She underwent a hysterectomy and debulking of retroperitoneal masses. Pathology showed evidence of tumor necrosis, suggesting a possible beneficial effect, and she was recommenced on Gleevac in an effort to prevent recurrences. She is currently asymptomatic, without evidence of disease 10 months after surgery, continuing on Gleevac therapy. FATWOs are very rare tumors. Most cases are benign but have the potential to recur and metastasize. There is limited knowledge about the optimal treatment for this neoplasm. Our patient's favorable response to Gleevac therapy supports the concept of targeted molecular therapy in patients with c-kit-positive FATWO tumors.

Abdominal Pain↗

Pilary complex carcinoma: an adnexal carcinoma of the skin with differentiation towards the components of the pilary complex.

We report twelve cases with a distinctive form of adnexal carcinoma occurring most commonly over the head and neck of relatively old individuals. The growth was single in all cases, deep dermal in location, and consisted of massive proliferation of small basaloid cells with only occasional connection with the surface epidermis. The basaloid tumor masses showed only a few areas of palisading of their outer cell nuclei and occasional retraction space formation. The neoplasm revealed scattered areas of trichilemmal and epidermoid keratinization, foci of sebaceous, and areas of sweat ductal differentiation indicating participation of various components of the pilary complex.

Adult↗

Synchronous microcystic adnexal carcinoma and gastric cancer with review of the literature.

A microcystic adnexal carcinoma (MAC) on the left lateral chest was synchronously accompanied by both an adenocarcinoma and a granular cell tumor of the stomach in a 70-year-old Japanese male. The MAC lesion had first appeared approximately 20 years earlier and had slowly increased without symptoms. After a definitive diagnosis was made by an excisional biopsy, a second operation was performed with wider excision, followed by split thickness skin grafting. Meanwhile, a punch biopsy from an erosive lesion, which was performed three days before the second operation, demonstrated gastric cancer. Consequently, a gastrectomy was undertaken 20 days later. The patient had since been followed for 2 years and 3 months without any sign of recurrence nor metastasis. Subsequently, soft lymph nodes in two regions of the left axilla were noticed. Three lymph nodes were excised for diagnostic purposes and found to be reactive hyperplasia without metastasis of skin tumor cells. To our knowledge, in the literature, 11 patients have been reported with MAC associated with double or multiple cancers which developed syn- or meta-chronously; ours is the 12th patient.

Aged↗

Microcystic adnexal carcinoma.

A patient is described with a tumour of the upper lip which was proved histologically to be a microcystic adnexal carcinoma, probably derived from the outer hair root sheath. This appears to be the eighth in the literature.

Adult↗