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CASE REPORT
Year : 2023 | Volume
: 31
| Issue : 1 | Page : 27-30
Primary cutaneous apocrine carcinoma of scrotum with pagetoid phenomenon
Phong Jhiew Khoo1, Adzim Poh Yuen Wen2, Siew Cheng Chai3
1 Department of Plastic and Reconstructive Surgery, Hospital Kuala Lumpur; Department of Surgery, Plastic and Reconstructive Surgery Unit, Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
2 Department of Surgery, Plastic and Reconstructive Surgery Unit, Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
3 Department of Plastic and Reconstructive Surgery, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
Correspondence Address:
Dr. Phong Jhiew Khoo
55, Jalan SJ 17B, Taman Selayang Bahagia, 68100 Batu Caves, Selangor
Malaysia
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/tjps.tjps_46_22
Primary cutaneous apocrine carcinoma (PCAC) of the scrotum is an unwonted neoplasm that predominantly occurs in areas with high-density apocrine glands. However, the incidence of scrotal PCAC is uncommon. Further, the pagetoid phenomenon is a rare feature of PCAC involving malignant cells infiltrating the epidermis. We present a rare case of scrotal PCAC in an elderly patient who presented with a slow-growing scrotal lesion. A physical examination identified an ulcerated globular mass at the right scrotum with ipsilateral inguinal lymphadenopathy, and a biopsy confirmed the diagnosis of apocrine carcinoma. In addition, imaging studies revealed features of lymph nodes and bone metastasis. Subsequently, a wide local excision was performed. The clinical features of PCAC are not distinctive; thus, histopathology and immunohistochemistry are paramount to confirm the diagnosis. The ideal treatment to achieve clear margins is tumor excision, while other treatment modalities such as chemotherapy, targeted antibody therapy, and radiotherapy are not established.
Keywords: Pagetoid phenomenon, primary cutaneous apocrine carcinoma, scrotal carcinoma
Primary cutaneous apocrine carcinoma (PCAC) is a rare cutaneous adenocarcinoma predominantly arising from the axilla and anogenital region.[1],[2],[3],[4],[5] The incidence of PCAC is between 0.0049 and 0.0173 per 100,000 people per year.[3],[6] However, only sparse case reports have been published about scrotal PCAC.[1],[2],[3],[4],[5] Further, the clinical manifestation of PCAC is nonspecific.[3] The pagetoid phenomenon or pagetoid spread is uncommon in PCAC but occurs when tumor cells exhibit intraepidermal spread.[1],[2],[3] The primary treatment for PCAC is a wide local excision of the lesion.[1],[3],[5],[7],[8] We present a case of scrotal PCAC with the pagetoid phenomenon in an elderly patient who presented with a slow-growing scrotal lesion.
Case ReportWe present the case of an 85-year-old male with scrotal PCAC. He has complained of a painless, slow-growing right scrotal lesion for a year. He has a history of ischemic heart disease and chronic kidney disease.
On examination, we noted a multilobulated globular mass at the anterior superior aspect of the right scrotum measuring 4 cm × 4 cm × 3 cm [Figure 1]. The irregular pedunculated skin lesion had an extensive surface ulceration and was covered with slough. The painless firm mass was not attached to the subcutaneous structures. In addition, a solitary lymph node measuring approximately 2 cm × 2 cm was palpable at the right inguinal region.
Figure 1: Photograph of the right scrotal primary cutaneous apocrine carcinomaAn incisional biopsy of the lesion confirmed the diagnosis of apocrine carcinoma. Microscopically, the neoplastic cells with eosinophilic cytoplasm were arranged in solid nests and cords [Figure 2], and the pagetoid spread of neoplastic cells was present [Figure 3]. Immunohistochemical studies were positive for gross cystic disease fluid protein-15 (GCDFP-15), GATA binding protein 3 (GATA3), and cytokeratin 7 (CK7); however, the estrogen receptor (ER) and progesterone receptor (PR) were negative [Figure 4]. A staging, contrast-enhanced computed tomographic scan revealed right inguinal lymphadenopathy and multiple sclerotic bone lesions at the left acetabulum, as well as at the fifth thoracic and fourth lumbar vertebral bodies.
Figure 2: Histopathological examination revealing neoplastic cells with abundant eosinophilic cytoplasm
Figure 3: Histopathological examination demonstrating a cutaneous lesion with surface ulceration and the pagetoid spread of malignant cells (green circle)
Figure 4: Immunohistochemistry study demonstrating that the malignant cells are positive for cytokeratin 7A multidisciplinary conference was called. The diagnosis of right scrotal PCAC with possible inguinal lymph nodes and bone metastasis was established. Subsequently, we proceeded with a wide local excision of the mass [Figure 5]. However, the patient refused to undergo inguinal lymph node resection, radiotherapy, or chemotherapy. The final histopathological examination demonstrated a clear surgical margin [Figure 6]. Six months after surgery, no new lymph node metastasis was found clinically, and the solitary inguinal lymph node had not grown. Frequent clinical and radiological surveillance are planned for the patient.
Figure 5: Serial photographs demonstrating the wide local excision of the right scrotal primary cutaneous apocrine carcinoma and the primary closure of the defect
Figure 6: Photograph of the excised right scrotal primary cutaneous apocrine carcinoma with a 1 cm surgical margin Discussion Literature on scrotal PCAC is limited to a few case reports and a case study that Kamanda et al. conducted involving six patients.[1],[2],[3],[4],[5] The clinical spectrum of PCAC might feature dermatitis-like characteristics to a painless, erythematous, slow-growing solid or cystic nodule with focal ulcerations.[1],[2],[3] PCAC causes potential hematogenous and lymphogenous metastasis to regional lymph nodes, liver, lungs, brain, and bones.[1],[3],[5] The overall median survival rate for PCAC is 51.5 months and that of the localized disease is 55 months, but this is reduced to 33 months in lymph node-positive patients.[6] Metastatic disease has the poorest survival rate at 14.5 months.[6]
A diagnosis of PCAC is defined by the presence of histopathological evidence of apocrine differentiation of the neoplastic cells with eosinophilic granular cytoplasm and the decapitation phenomenon.[1],[3] Moreover, the expression of GCDFP-15, GATA3, and CK7 in immunohistochemical studies is characteristic of PCAC.[1],[2],[3],[9] GCDFP-15 is used to differentiate apocrine glands from eccrine glands, where the former expresses GCDFP-15.[7] CK7 is practical in identifying PCAC from metastasis.[7] PCAC is usually negative for ER and PR compared to metastatic mammary adenocarcinoma.[7] Histopathologically, our case displayed neoplastic cells with eosinophilic cytoplasm and pagetoid spread, but the decapitation phenomenon was not identified, possibly due to the solid growth pattern of the tumor, which is similar to the cases that Kamanda et al. reported.[1]
Only four other cases that Kamanda et al. and Zibis et al. published demonstrate the paucity of scrotal PCAC with the pagetoid phenomenon.[1],[3] One of the differential diagnoses of PCAC with the pagetoid phenomenon is extramammary Paget disease (EMPD).[1],[2],[3],[9] The pagetoid phenomenon is caused by a preexisting tumor that infiltrates the epidermis with neoplastic cells, while EMPD involves an intraepithelial lesion with no underlying malignancy.[1],[3] Compared to PCAC with pagetoid spread, lymph node metastasis is rare in EMPD.[1]
Ogata et al. advocated the wide local excision of PCAC, preventive regional lymph node dissection, and adjuvant radiotherapy, especially in locally advanced cases, although PCAC has a questionable response to chemotherapy and radiotherapy.[8] A wider excision margin should be considered in PCAC with pagetoid spread, as intraepidermal neoplastic cell infiltration might proliferate more peripherally beyond the infiltrated border.[3] If regional lymph node metastasis is present, lymph node dissection should be offered.[1],[3],[6] However, standardized chemotherapy and radiotherapy regimens for the treatment of PCAC have not been established.[1],[3],[5] Some authors have described the use of targeted antibody therapy with trastuzumab in human epidermal growth factor receptor 2-positive PCAC patients, but this yielded variable responses.[1],[3] Furudate et al. reported on the receptor activator of a nuclear factor kappa-B ligand-positive scrotal PCAC patient who refused surgery but achieved remission after being treated with chemotherapy using carboplatin and paclitaxel, as well as radiotherapy followed by denosumab.[5] Histologically, PCAC resembles the apocrine subtype of breast cancer.[5] Based on this histological similarity, some authors have suggested that the chemotherapy regimen for breast cancer treatment is effective against PCAC.[5],[10] Ideally, our patient should undergo ipsilateral inguinal lymph node dissection and a wide local excision of the primary tumor, followed by radiotherapy and chemotherapy, subject to the patient's fitness due to his underlying comorbidities.
ConclusionUltimately, multidisciplinary meetings should be held to discuss the treatment plan for individual PCAC patients, as variable factors such as the patient's fitness, the presence of the pagetoid phenomenon, the patient's metastasis status, and immunohistochemical studies play a role in determining the appropriate management of PCAC.
Acknowledgment
Azri I and Suria HMD assisted in histopathology figure editing and configuration.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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