|Year : 2023 | Volume
| Issue : 2 | Page : 496-499
Palpebral adenocarcinoma of moll
Carmen A Porcar Plana1, Emilio Abecia Martínez2, Lavilla García Laura1
1 Ophthalmology Department, Hospital Universitario Miguel Servet, Zaragoza, Spain
2 Pathology Department, Hospital Universitario Miguel Servet, Zaragoza, Spain
|Date of Submission||16-Oct-2022|
|Date of Acceptance||20-Jan-2023|
|Date of Web Publication||28-Apr-2023|
Lavilla García Laura
Plaza de los Sitios, 10, 6º Dcha, Zaragoza
Source of Support: None, Conflict of Interest: None
Adenocarcinoma of Moll is an unusual but dangerous tumor that rarely affects apocrine palpebral glands. In this work, we report an atypical presentation of palpebral Moll adenocarcinoma and discuss the anatomopathological diagnostic criteria. As its prognosis is poor owing to its capacity to invade other tissues, we emphasize the importance of performing an extension study on this patient. Expanding the sample size of reported tumors will be useful in defining their clinical characteristics and establishing guidelines for follow-up and treatment. Additionally, we present a review of the cases reported in the literature.
Keywords: Adenocarcinoma of Moll, apocrine carcinoma, eyelid carcinoma
|How to cite this article:|
Plana CA, Martínez EA, Laura LG. Palpebral adenocarcinoma of moll. Indian J Ophthalmol Case Rep 2023;3:496-9
Sweat gland carcinomas (SGC) are an exceedingly rare variety of eyelid and orbital tumors, including mucinous carcinoma (32%), microcystic adnexal carcinoma (18%), endocrine mucin-producing SGC (18%), eccrine carcinoma (9%), poorly differentiated adnexal carcinoma (9%), and apocrine carcinoma (9%).
In this work, we report a palpebral Moll adenocarcinoma (MAC) case with an unusual presentation and discuss the anatomopathological criteria that define this type of tumor. In addition, we present a review of the 26 cases reported in the literature that truly meet the current diagnostic criteria for MAC.
| Case Report|| |
A 27-year-old Arab woman, 12 weeks pregnant, with no other systemic or ophthalmologic history of interest, refers to our department with progressive growth of a lesion on the right lower eyelid. Examination reveals a 3 × 2-mm tumor of fibroelastic consistency and brownish coloration in the middle third of the right lower eyelid, with local madarosis [Figure 1]a. An incisional biopsy is performed with a suspicious result of apocrine adenocarcinoma. A wide-margin pentagonal wedge with direct closure is achieved. To minimize palpebral malpositioning risk, canthotomy and cantholysis are associated [Figure 1]b and [Figure 1]c. Upon histological examination, a modular and non-capsulated tumor is observed, which invades the surrounding striated muscle [Figure 2]a. Among neoplastic cells, there are normal Moll's apocrine glands, both of them showing clear decapitation secretion [Figure 2]b. The neoplastic glands display a tubular and ductal pattern [Figure 2]b, forming eventual intraluminal papillae. Moreover, apocrine glands with patchy dysplasia and pseudopapillary are focally found [Figure 2]c. Tumoral cells show mild dysplasia features and mitosis [Figure 2]d. Histochemical analysis shows PAS-positive cytoplasm with granules positive for colloidal iron [Figure 2]e. The cells are positive for CK7, CK8, CK18, and CK19, and GCDFP15 [Figure 2]f. Estrogen and progesterone receptors are negative. A diagnosis of MAC is made, with margins free of tumor involvement. Given the gestational state of the patient, monthly clinical and ultrasound follow-up is performed. At the end of the gestational period, the extension study is completed six months later using PET-CT with no evidence of local recurrence. However, latero-cervical lymph nodes with non-specific characteristics are observed; therefore, FNAP is performed with negative results. No evidence of local recurrence or metastasis is found after six months of follow-up.
|Figure 1: Appearance of the lesion (a) Initial. (b) At 7 days after surgery. (c) At 15 days after surgery|
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|Figure 2: Pathologic anatomy. (a) Hematoxylin and eosin (HE), panoramic. Nodular and non-capsulated tumor infiltrating surrounding striated muscle. (b) HE ×20. Neoplastic and normal Moll's apocrine glands, showing clear decapitation secretion. (c) HE ×20. Apocrine glands with patchy dysplasia and pseudopapillary. (d) HE ×40. Mild dysplasia features and mitosis. (e) Colloidal iron ×20. (f) GCDFP15 ×20|
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| Discussion|| |
MAC or apocrine sweat gland carcinoma is an infrequent tumor that was first reported by Stout and Cooley. Currently, fewer than 30 cases of MAC with palpebral involvement have been described in the literature [Table 1]. Analyzing the published cases, the average age of presentation was 64. However, in the present case the patient was an unusually young woman. Probably because the low frequency of these tumors does not provide a representative sample. Therefore, reporting cases of this lesion will help specify their clinical characteristics.
|Table 1: Summary of published cases of Moll's adenocarcinoma with palpebral involvement|
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About clinical presentation, MAC can appear in the areas where these glands are found: the axilla (50%), head and neck (35%), or in other areas such as the ear canal, nipple, or perineum. Specifically, palpebral involvement is very rare and typically presents as a painless, slowly growing mass with skin-like coloration, often confused with chalazion-like lesions. Other possible differential diagnoses for eyelid apocrine carcinoma include apocrine hidrocystoma, apocrine cystadenoma, primary cutaneous mucinous carcinoma, and endocrine mucin-producing sweat gland carcinoma.
The diagnosis of MAC is anatomopathological. Therefore, it is important to biopsy all progressively growing palpebral lesions with signs suggestive of malignancy, such as madarosis in the case presented.
Anatomopathological diagnostic criteria can be defined as (1) morphologically, normal Moll's apocrine glands with decapitation secretion, (2) neoplastic glands with atypia and tubular and ductal pattern,, (3) strongly eosinophilic and PAS-positive cytoplasm, (4) colloidal iron granules positive, (5) low molecular weight cytokeratins positive,,, (6) GCDFP15 positive,,,, and (7) estrogen and progesterone receptors.,,,
Treatment of these tumors is based on sporadic cases and includes surgery,,,,,,,,, radiotherapy,,,,,, and chemotherapy. Regarding surgical treatment, when involvement is limited to the eyelid, as in the case of the patient presented, it is recommended to perform a full-thickness excisional biopsy with 4 mm margins. However, in cases of orbital invasion, exenteration is used.,,,,,,
The prognosis of affected patients depends on tumor invasion in other tissues. Spread can occur by direct orbital (47%), lymphatic (35%), or hematogenous (18%) invasion. Analyzing the published cases, it was observed that almost half of the cases (46%) presented local extension or metastasis. Therefore, performing a complete systemic exploration in the search for adenopathies and an extension study by imaging is highly recommended. In the case presented, given the gestational state of the patient, and since the lesion was smaller than one centimeter, with no regional lymphadenopathies, we decided to perform monthly clinical and ultrasound controls and to complete the extension study with PET-CT at the end of the pregnancy.
| Conclusion|| |
MAC is rare apocrine tumor with infrequent palpebral involvement. The diagnosis of MAC is based on anatomopathological criteria; therefore, it is important to promptly biopsy all palpebral lesions with signs of malignancy. A previous incisional biopsy allows the planning of surgery with a wide safety margin, improving the prognosis of these patients. In general, the prognosis of MAC is poor due to its capacity for recurrence and invasion of other tissues; therefore, it is highly recommended to perform an extension study, even in small tumors. Expanding the sample size of reported tumors would be useful to define their clinical characteristics, and would allow the establishment of guidelines for follow-up and treatment.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]