Primary Signet-ring Cell/Histiocytoid Carcinoma of the Eyelid

Requena, Luis; Prieto, Víctor G.; Requena, Celia; Sarasa, José Luis Andrès; Manzano, Rosa María Sánchez; Seco, Miguel; Rütten, Arno; Kazakov, Dmitry V.; Cerroni, Lorenzo; Kutzner, Heinz

doi:10.1097/pas.0b013e318209cac7

Cited by 63 publications

(25 citation statements)

References 21 publications

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“…In regards to histopathology, primary cutaneous signet-ring cell/histiocytoid carcinoma is characterized by diffuse infiltration of the dermis by single cells, cords or rows (single file pattern) arranged between collagen fibers [ 1 ]. There is a relative sparing of the epidermis in all cases located in the eyelid.…”

Section: Discussionmentioning

confidence: 99%

“…Currently, there are 32 published cases of primary cutaneous signet-ring cell carcinoma or histiocytoid carcinoma of the eyelid; the term used for diagnosis depends on the major cellular component of the lesion. The histogenesis of this tumour is controversial, however, most features point towards an apocrine differentiation [ 1 , 2 ]. The diagnosis is established by the exclusion of metastases from other primary sites especially the breast, gastrointestinal tract and urinary tract.…”

Section: Introductionmentioning

confidence: 99%

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Diagnostic and treatment challenges of a case of primary cutaneous signet-ring cell/histiocytoid carcinoma of the eyelid

2020

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Background Primary cutaneous signet-ring cell/histiocytoid carcinoma of the eyelid is an extremely rare but aggressive neoplasm diagnosed primarily in elderly men. Until now there are 32 published cases of signet-ring cell carcinoma or histiocytoid carcinoma of the eyelid. We report the clinical, radiographic and histological features of the 33rd reported case of PCSRCC in the eyelid of a 73-year-old male, and review diagnostic and treatment challenges of this rare entity. Case presentation Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. Upon assessment, he was noted to have an upper lateral orbital rim mass. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers; the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor stain and GATA3 positive. Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma. Further imaging failed to identify a distant primary malignancy or metastatic disease. The decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Superficial biopsies of the right periorbital region were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. Due to positive margins on final permanent sections, the patient underwent further wide resection with free muscle-skin flap reconstruction followed by adjuvant radiation treatment. Conclusion Our case represents the 33rd case of primary signet-ring cell/histiocytoid carcinoma of the eyelid in a 73-year-old male, the first documented case with GATA3 positivity and the second documented case with androgen receptor stain positivity.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Diagnostic and treatment challenges of a case of primary cutaneous signet-ring cell/histiocytoid carcinoma of the eyelid

2020

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“…2 In the eyelid, immunostains may be particularly useful for distinguishing mastocytosis from primary signet-ring cell/histiocytoid carcinoma, which has a predilection for the eyelids. 17 Neoplastic mast cells are immunophenotypically similar to normal mast cells but coexpress CD2 or CD2 and CD25. 2 Membrane expression of CD25 on cutaneous mast cells in adults may be predictive of systemic mastocytosis, 18 but a consensus threshold for a positive result remains to be established.…”

Section: Discussionmentioning

confidence: 99%

Solitary mastocytoma in the eyelid of an adult

McKinnon

Rand

Proia

2018

American Journal of Ophthalmology Case Reports

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PurposeTo describe the ophthalmic symptoms and histopathological findings in a rare case of an eyelid mastocytoma in an adult.ObservationsA man in his early 60s developed a painless, non-tender, non-pruritic, mobile nodule on the right lower eyelid beneath the inferior orbital rim. The lesion grew to 15 × 9 mm over eleven months. Biopsy revealed a diffuse infiltrate of histiocytoid and spindle-shaped mast cells forming cords and small nests between collagen fibers in the superficial and deep dermis. Mast cell lineage was confirmed by immunohistochemistry. Physical examination revealed no other cutaneous lesions and no evidence of systemic disease. Serum tryptase level was normal. Annual full-body examination by a dermatologist for 4.5 years has revealed neither recurrence in the eyelid nor cutaneous involvement at other sites.Conclusions and importanceMast cell tumors limited to the human eyelid are extremely uncommon with only four previously reported cases, including one in an adult. This case highlights the rare possibility of a solitary mastocytoma presenting in the eyelid of an adult.

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“…Signet-ring-cell is a malignant histological cell type which is predominantly seen in carcinomas. Signet ring cells have frequently been reported in association with carcinoma of stomach [1] , but signet ring cells have also been reported in carcinomas of other tissues including the prostate [2] , bladder, gallbladder [3] , breast, colon [4] , ovarian stroma [5,6] , eyelid [7,8] . There have also been occasional reports of metastatic signet ring cell carcinoma to the testis, spermatic cord and scrotum.…”

Section: Introductionmentioning

confidence: 99%

“…Only about 3 cases of primary seminoma of testis with signet-ring-cell morphology have been reported in the literature. In general, a number of signet ring cell carcinomas have been reported to be associated with a worse prognosis [8][9][10] . We report a case of seminoma of testis associated with foci of signet-ring-cell morphology with a review of the literature.…”

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confidence: 99%

Primary classical seminoma of testis with foci of signet-ring-cell morphology: A case report and review of the literature including ESMO guidelines on management of seminoma of testis

Venyo

Baiden-Amissah

2012

JBGC

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Background: Signet-ring-cell carcinoma is a rare variant of mucinous adenocarcinoma. Only about 3 cases of primary testicular cancer with signet-ring-cell morphology have been reported so far in the literature. There is lack of knowledge regarding the biological behaviour of this type of tumour affecting the testis. Seminoma of the testis is quite common and its biological behaviour is well known. When a classical seminoma of the testis is found to be contemporaneously associated with signet-ring-cell morphology, in view of the absence of any detailed literature on this rare clinical entity, the biological behaviour of such a tumour cannot be predicted. Objectives:To report a case of Classical Seminoma of the testis interspersed with seminoma cells with signet-ring morphology. To review the literature on signet-ring-cell carcinomas including carcinomas of testis with signet-ring-cell morphology. To review the ESMO guidelines on management of seminoma of testis Results: A 32-year-old man came for urology consultation 11 months after he had noticed a lump in his left testis. He had a family history of testicular cancer. A hard lump clinically suspicious of a tumour was palpable in the lower pole of his left testis. The right testis felt normal on palpation. His serum Beta HCG level was raised. He had ultra-sound scan of testis which showed bilateral microlithiasis with a tumour in the left testis but no tumour in the right testis. He underwent left radical orchidectomy and histology of the testicular tumour revealed a classical testicular seminoma with foci of signet-ring -cell morphology based upon haematoxylin and eosin staining as well as positive immunostaining for CD117 and OCT 3 / 4. Literature review revealed paucity of literature on testicular tumours with signet-ring-cell morphology. Conclusions:There is paucity of literature on primary testicular tumours with signet-ring-cell morphology. In order to establish the biological behaviour of such tumours we would encourage pathologists and urologists to report cases of testicular tumours associated with signet-ring-cell morphology together with the long term biological behaviour of such tumours. They should also report on the percentage of testicular tumours that have signet-ring-cell morphology and their distribution whether patchy or diffuse. Ultrasound-scan of testis was required to establish the diagnosis of testicular cancer 82 in the patient. Ultrasound-scan of testis is useful in the investigation of a testicular lump. Ultrasound and CT scans are also useful for the follow-up of patients with testicular cancer and microlithiasis. IntroductionSignet-ring-cell is a malignant histological cell type which is predominantly seen in carcinomas. Signet ring cells have frequently been reported in association with carcinoma of stomach [1] , but signet ring cells have also been reported in carcinomas of other tissues including the prostate [2] , bladder, gallbladder [3] , breast, colon [4] , ovarian stroma [5,6] , eyelid [7,8] . There have also been occasional rep...

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Primary Signet-ring Cell/Histiocytoid Carcinoma of the Eyelid

Cited by 63 publications

References 21 publications

Diagnostic and treatment challenges of a case of primary cutaneous signet-ring cell/histiocytoid carcinoma of the eyelid

Diagnostic and treatment challenges of a case of primary cutaneous signet-ring cell/histiocytoid carcinoma of the eyelid

Solitary mastocytoma in the eyelid of an adult

Primary classical seminoma of testis with foci of signet-ring-cell morphology: A case report and review of the literature including ESMO guidelines on management of seminoma of testis

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