Cutaneous metastatic adenocarcinoma arising from a malignant sacrococcygeal teratoma in an adult
Published Web Locationhttps://doi.org/10.5070/D35vp1n69r
Cutaneous metastatic adenocarcinoma arising from a malignant sacrococcygeal teratoma in an adult1. Department of Dermatology, Adnan Menderes University, Aydın, Turkey. firstname.lastname@example.org
Ekin Şavk1, Aypınar Kolay1, İbrahim Meteoglu2, Alparslan Ünsal3, Hakan Cevikel4, Meltem Uslu1, Göksun Karaman1, Neslihan Sendur1, İbrahim Erdoğdu2
Dermatology Online Journal 14 (6): 3
2. Department of Pathology, Adnan Menderes University, Aydın, Turkey
3. Department of Radiology, Adnan Menderes University, Aydın, Turkey
4. Department of General Surgery, Adnan Menderes University, Aydın, Turkey
We present a patient with cutaneous metastasis caused by an adenocarcinoma arising from a malignant teratoma. A 37-year-old woman seen for the complaint of swelling in the genital region also complained of a draining mass in her gluteal region present since birth. Physical examination showed marked edema in the labia majora, multiple hyperkeratotic papules in the left labium majus, and erythema, induration, and swelling in the left femoral and inguinal regions. A soft tumor that exhibited sinus tracts was palpated in the left gluteus. Excision of the gluteal tumor revealed a teratoma. Vulvar skin biopsy confirmed a mucinous adenocarcinoma which had derived from this teratoma. A tumor that arises from pluripotent germ cells, teratoma rarely shows malignant transformation. The patient presented is a rare example of a cutaneous metastasis originating from a congenital sacrococcygeal teratoma in an adult.
A typical cutaneous metastasis is in the form of a firm, painless papule or nodule. Additional clinical presentations include sclerodermoid, vascular, alopecic and erysipelas-like lesions . Compared with other organs the skin is a relatively uncommon site for metastasis of an internal malignancy. Breast cancer, melanoma, and squamous cell carcinoma of the head and neck region are the malignancies that lead the list of tumors with cutaneous metastasis . In a large retrospective series of 7316 cancer patients, the skin was involved in 5.0 percent of the cases. This involvement was present at the time of presentation in 1.3 percent of patients and was the first sign of cancer in only 0.8 percent . Cutaneous metastases have been reported as the presenting sign of an underlying malignancy in 0.6-7.6 percent of cases [2, 3]. The difficulty in diagnosing a cutaneous metastasis is frequently overcome by the knowledge of the primary malignancy. In this report, we present a patient diagnosed with cutaneous metastasis caused by an adenocarcinoma arising from a congenital sacrococcygeal teratoma, a tumor that rarely shows malignant transformation.
|Figure 1c||Figure 1d|
|Figure 1c. Soft, subcutaneous tumor palpated in the left gluteal area, roughly 4x4 cm in size and with multiple fistulae on
the skin covering the superior part of the tumor|
Figure 1d. Close-up of the sinus tracts
A 37-year-old woman was seen with the complaint of swelling in the genital region. A small papule in the left labium majus had appeared 3 months prior to presentation and was followed by progressive edema and pain in the genital and left inguinal area. She also complained of a draining mass in her gluteal region present since birth and for which she had not been treated previously. The patient had lost some weight in the last few months and felt fatigue. Her personal and family history was otherwise unremarkable. Physical examination showed marked edema in both of the labia majora and multiple hyperkeratotic papules in the left labium majus (Fig. 1a). There was a well demarcated, erythematous, indurated, cool, and painless plaque encompassing the left femoral and inguinal region that was not accompanied with fever (Fig. 1b). A soft, subcutaneous tumor, roughly 4x4 cm in size, that exhibited sinus tracts was palpated in the left gluteal area (Fig. 1c-d). Computerized tomography and magnetic resonance imaging of the patient revealed an ovoid mass measuring 57x60x80 mm, localized in the fatty tissue of the presacral zone of the gluteal region. In addition, one enlarged lymph node in the right inguinal area and multiple enlarged lymph nodes in the left inguinal area, the largest of which measured 3 cm, were observed (Fig. 2a-b).
|Figure 3a||Figure 3b|
|Figure 3a. Gross photomicrograph of the specimen|
Figure 3b. Photomicrograph showing differentiated tissue components, cuboidal cells and atypical epithelial cell clusters in large mucinous lakes (x200, hematoxylin-eosin)
An ultrasonography guided tru-cut biopsy was performed on the mass in the gluteal region. Pathologic examination revealed a diagnosis of mucinous adenocarcinoma. No tumoral mass was detected in the lungs, uterus, ovaries, or gastrointestinal tract. The paracoccygeal mass was excised by explorative laparotomy; incisional biopsies were obtained from the skin lesions of the vulvar and inguinal areas simultaneously. Pathologic examination of the mass that measured 9x8.5x5 cm macroscopically showed well-defined margins and multiple fistulae on the skin overlying the superior part of the mass (Fig. 3a). On cross-section these fistulae were seen to be connected to cystic areas that contained papillae with mucinous consistency in their lumen. A solid tumoral mass involving fatty, connective, chondral and osseous tissues was observed adjacent to the cystic areas. Microscopic examination showed mature chondral, osseous, fatty and glial tissues; cystic areas lined by ciliated epithelium; epithelial cell accumulations into large mucinous lakes; and in the adjacent areas, an infiltrative pleomorphic tumor having glandular structures lined by atypical cells (Fig. 3b). The tumor involved the surgical margin in a few microscopic foci and showed extensive lymphatic invasion. Mucinous adenocarcinoma accompanied with lymphatic emboli was observed in the vulvar and inguinal skin lesions (Fig. 3c). Immunhistochemical staining demonstrated positive staining of the tumoral mass for keratin 7, keratin 8 and CEA; whereas TTF-1 staining was positive in the luminal surfaces of glandular structures of the tumor (Fig. 3d). No staining was observed by keratin 20, TAG-72, estrogen, and progesterone receptors. The final diagnosis was cutaneous metastasis of a mucinous adenocarcinoma that had developed from the respiratory epithelium of a teratoma by malignant transformation. The patient was started on chemotherapy.
Teratomas are germ cell tumors derived from pluripotent cells and made up of elements of different types of tissue from 1 or more of the 3 germ cell layers . In adults teratomas are most often found in the ovary or testis, whereas in children, the sacrococcygeal region is the expected site. Although sacroccoccygeal teratomas are the most common solid neoplasms in newborns, with a prevalence of 1 in 35000-40000 live births, they are quite rare in adults; 80 percent of these tumors are diagnosed and treated by the sixth month of life and only fewer than 10 percent are diagnosed after infancy . Reported cases of sacrococcygeal teratomas in adults are few and among these tumors malignant transformation is quite rarely described. Reviews of the literature found a total of 88 reported cases, with only 16 described as malignant . The reported female:male ratio is 2-4:1 . In contrast to the presence of a posterior mass in infants, external findings may be subtle in adults. If not asymptomatic, symptoms such as lower back pain, constipation, and urinary tract infection caused by compression of the organs surrounding the tumor predominate. Some cases may present with recurrent pilonidal cyst infections or chronic fistulas as in our patient. Other less frequent presentations include a growing mass causing skin ulceration and neurologic symptoms [5, 6]. The differential diagnoses of sacrococcygeal teratoma in adults include chordoma, meningocele, neurofibroma, fibrosarcoma, giant tumor of sacrum, pilonidal cysts, osteomyelitis of sacrum, fistula with presacral extension and abscess formation, postinjection granuloma, and tuberculosis . Because malignant sacrococcygeal teratomas are rare tumors there is yet no standard regimen of chemotherapy or radiotherapy, but the best approach appears to be referral to oncology where individualized therapy can be conducted [5, 6, 7]. Our patient is currently on chemotherapy.
Our patient presents a constellation of rarities. Congenital sacrococygeal teratomas rarely present in adulthood and even more rarely exhibit malignant transformation. The fact that the cutaneous metastasis was the presenting complaint is an additional unusual feature.
References1. Ahmed I. Cutaneous metastasis. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. Mosby, Spain, 2003: 1953-6
2. Lookingbill DP, Spangler N, Sexton FM. Skin involvement as the presenting sign of internal carcinoma. A retrospective study of 7316 cancer patients. J Am Acad Dermatol. 1990;22:19-26. PubMed
3. Lookingbill DP, Spangler N, Helm KF. Cutaneous metastases in patients with metastatic carcinoma: a retrospective study of 4020 patients. J Am Acad Dermatol. 1993;29:228-36. PubMed
4. Dorland's Illustrated Medical Dictionary. http://www.mercksource.com/pp/us/cns/cns_hl_dorlands
5. Monteiro M, Cunha TM, Catarino A, Tome V. Case report: sacrococcygeal teratoma with malignant transformation in an adult female:CT and MRI findings. Br J Radiol. 2002;75:620-3. PubMed
6. Tulchinksy H, Tovar A, Gutman H. Adenocarcinoma within a paracoccygeal teratoma in an adult: report of a case. Surg Today. 2005;35:259-62. PubMed
7. Ng EW, Porcu P, Loehrer PJ Sr. Sacrococcygeal teratoma in adults: case reports and a review of the literature. Cancer. 1999;86:1198-202. PubMed
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