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Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma
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ScienceDirect British Journal of Oral and Maxillofacial Surgery xxx (2020) xxx–xxx
Short communication
Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma Anna Sayan a,∗ , Oliver Mitchell b , Saleem Taibjee c , Velupillai Ilankovan a a b c
Poole Hospital NHS Foundation Trust University Hospital Southampton NHS Foundation Trust Dorset County Hospital NHS Foundation Trust
Abstract Signet-ring cell (histocytoid) carcinoma is a rare and aggressive skin neoplasm that most commonly affects elderly men. It typically originates from the upper or lower eyelid, with bilateral involvement only in rare cases, and results in a diffuse and infiltrative appearance clinically, radiologically, and histologically (the “monocle” sign). It is essential to carry out a full investigation to rule out metastasis from an internal origin. Crown Copyright © 2020 Published by Elsevier Ltd on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved. Keywords: Histocytoid; adenocarcinoma; signet-ring cell; monocle sign
Introduction Primary cutaneous signet-ring cell (histocytoid) carcinoma is a rare neoplasm, originally described by Rosen et al,1 that typically presents as painless infiltration and swelling of the eyelid, most commonly in elderly men.2,3 Histopathological examination shows an adenocarcinoma with signet-ring cell and histocytoid morphology.2 We present an unusual example with progressive infiltration to involve a large area of the patient’s face.
Case report An 87-year old man was referred to maxillofacial surgery for assessment of left-sided facial swelling of 12 months duration (Fig. 1).
∗
Corresponding author. Fax: 01202448410. E-mail address: [email protected] (A. Sayan).
On examination, he had a diffuse erythematous swelling that affected his upper lip, left cheek and left lower eyelid, and there was associated cervical lymphadenopathy. There was no associated facial nerve deficit or sensory loss, and he had normal visual acuity and ocular movements. The initial clinical impression was of a possible infiltrative process, with differential diagnosis that included amyloidosis. Magnetic resonance imaging of the head and neck (Fig. 2) confirmed a sizeable, diffuse plaque with soft tissue signal density that involved the subcutaneous tissue of the left side of the face, including the periorbital, maxillary, and nasal regions. Staging computed tomography (CT) of the head, neck, chest, abdomen and pelvis showed an extensive tumour affecting the left side of the face with multiple abnormal lymph nodes seen within the left side of the neck and indeterminate left-sided lung nodules. Positron emission tomographic CT showed low-grade tracer uptake in the soft tissue of the left side of the face, left cervical level II lymph nodes, and additional uptake in two upper nodules of the left lobe of the lung, caecal mesenteric nodule, and sacrum.
https://doi.org/10.1016/j.bjoms.2020.01.023 0266-4356/Crown Copyright © 2020 Published by Elsevier Ltd on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved.
Please cite this article in press as: Sayan A, et al. Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma. Br J Oral Maxillofac Surg (2020), https://doi.org/10.1016/j.bjoms.2020.01.023
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Fig. 1. Diffuse erythematous swelling affecting left lower eyelid, cheek, and upper lip.
The findings were discussed in the multidisciplinary skin cancer meeting and, as a result of the extent of the disease and comorbidities, the patient was referred for palliative care, and eventually died within six months of initial presentation.
Discussion
Fig. 2. Magnetic resonance image of the head and neck: diffuse plaque with soft tissue signal density (arrow) involving the subcutaneous tissue of the left side of the face.
Histopathological study of a deep incisional skin biopsy, including immunohistochemistry, showed features of adenocarcinoma with signet-ring cell morphology (Fig. 3).
The clinical presentation of primary cutaneous signet-ring cell carcinoma is insidious and initially non-specific, which may cause a diagnostic dilemma. The gradual thickening and swelling of the eyelid, or lids, and surrounding areas, with absent neurological deficit, may give the false impression of an inflammatory process such as orbital cellulitis or cutaneous deposition such as amyloidosis. Eyelid location is described in most cases, with occasional cases of axillary involvement.1 It is more common in men, with a mean age of 63 years.3 The histopathology is characteristic. The epidermis is unaffected with Grenz zone of spared superficial dermis. The underlying dermis and subcutaneous tissue comprise diffuse cords of histiocytoid cells interspersed between collagen bundles, which are arranged like a “stack of pennies” with the characteristic signet-ring cells.2–4 MUC6 positivity on immunohistochemistry suggests histogenesis from the glands of Moll.5 The differential diagnosis includes metastatic adenocarcinoma, particularly lobular breast carcinoma, or that arising from the gastrointestinal tract or lung.6 There are no defini-
Please cite this article in press as: Sayan A, et al. Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma. Br J Oral Maxillofac Surg (2020), https://doi.org/10.1016/j.bjoms.2020.01.023
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No. of Pages 3
A. Sayan et al. / British Journal of Oral and Maxillofacial Surgery xxx (2020) xxx–xxx
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Fig. 3. Histopathological slides of the skin: A) Diffuse infiltrate of tumour cells involving the full thickness of the biopsy (haematoxylin and eosin, original magnification x 20); B) Signet-ring cells (arrows) (haematoxylin and eosin, original magnification x 400); C) Tumour cells that have infiltrated collagen, with “stack of pennies” configuration (arrows); (haematoxylin and eosin, original magnification x 200); D) Tumour cells that have stained positive for cytokeratin 7.
tive markers to reliably discriminate between a primary or metastatic disease.2–4 Imaging had excluded metastasis in our patient. The recommended treatments include wide local excision, and in some cases exenteration of the orbit may be required. There is a substantial tendency to local recurrence or metastasis, or both. Adjuvant radiotherapy, as well as anti-oestrogen or anti-androgen therapy, depending on the tumour markers expressed, are also described. 2,7 We report this case to highlight the unusual but characteristic clinical presentation of such insidious eyelid swelling in an elderly male, which is also termed the “monocle” sign. Conflict of interest We have no conflicts of interest.
References 1. Tanboon J, Uiprasertkul M, Luemsamran P. Signet-ring cell/histiocytoid carcinoma of the eyelid: a case report and review of the literature. Am J Dermatopathol 2013;35:e1–5. 2. Bernárdez C, Macías Del Toro E, Ramírez Bellver JL, et al. Primary signet¨ ring cell/histiocytoid carcinoma of the eyelid: a binocle¨ presentation of the “monocle tumor”. Am J Dermatopathol 2016;38:623–7. 3. Requena L, Prieto VG, Requena C, et al. Primary signet-ring cell/histiocytoid carcinoma of the eyelid: a clinicopathologic study of 5 cases and review of the literature. Am J Surg Pathol 2011;35:378–91. 4. Mortensen AL, Heegaard S, Clemmensen O, et al. Signet ring cell carcinoma of the eyelid — the monocle tumour. APMIS 2008;116:326–32. 5. Iwaya M, Uehara T, Yoshizawa A, et al. A case of primary signet-ring cell/histiocytoid carcinoma of the eyelid: immunohistochemical comparison with the normal sweat gland and review of the literature. Am J Dermatopathol 2012;34:139–45. 6. Boyd AS. Pulmonary signet-ring cell adenocarcinoma metastatic to the skin. Am J Dermatopathol 2017;39:e66–8. 7. Sakamoto K, Ito T, Tanioka F, et al. Primary signet-ring cell/histiocytoid carcinoma of the eyelid expressing androgen receptors and treated with bicalutamide. J Dermatol 2017;44:e230–1.
Ethics statement/confirmation of patient’s permission Ethics approval not required. Consent has been obtained.
Please cite this article in press as: Sayan A, et al. Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma. Br J Oral Maxillofac Surg (2020), https://doi.org/10.1016/j.bjoms.2020.01.023
ARTICLE IN PRESS
No. of Pages 3
Available online at www.sciencedirect.com
ScienceDirect British Journal of Oral and Maxillofacial Surgery xxx (2020) xxx–xxx
Short communication
Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma Anna Sayan a,∗ , Oliver Mitchell b , Saleem Taibjee c , Velupillai Ilankovan a a b c
Poole Hospital NHS Foundation Trust University Hospital Southampton NHS Foundation Trust Dorset County Hospital NHS Foundation Trust
Abstract Signet-ring cell (histocytoid) carcinoma is a rare and aggressive skin neoplasm that most commonly affects elderly men. It typically originates from the upper or lower eyelid, with bilateral involvement only in rare cases, and results in a diffuse and infiltrative appearance clinically, radiologically, and histologically (the “monocle” sign). It is essential to carry out a full investigation to rule out metastasis from an internal origin. Crown Copyright © 2020 Published by Elsevier Ltd on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved. Keywords: Histocytoid; adenocarcinoma; signet-ring cell; monocle sign
Introduction Primary cutaneous signet-ring cell (histocytoid) carcinoma is a rare neoplasm, originally described by Rosen et al,1 that typically presents as painless infiltration and swelling of the eyelid, most commonly in elderly men.2,3 Histopathological examination shows an adenocarcinoma with signet-ring cell and histocytoid morphology.2 We present an unusual example with progressive infiltration to involve a large area of the patient’s face.
Case report An 87-year old man was referred to maxillofacial surgery for assessment of left-sided facial swelling of 12 months duration (Fig. 1).
∗
Corresponding author. Fax: 01202448410. E-mail address: [email protected] (A. Sayan).
On examination, he had a diffuse erythematous swelling that affected his upper lip, left cheek and left lower eyelid, and there was associated cervical lymphadenopathy. There was no associated facial nerve deficit or sensory loss, and he had normal visual acuity and ocular movements. The initial clinical impression was of a possible infiltrative process, with differential diagnosis that included amyloidosis. Magnetic resonance imaging of the head and neck (Fig. 2) confirmed a sizeable, diffuse plaque with soft tissue signal density that involved the subcutaneous tissue of the left side of the face, including the periorbital, maxillary, and nasal regions. Staging computed tomography (CT) of the head, neck, chest, abdomen and pelvis showed an extensive tumour affecting the left side of the face with multiple abnormal lymph nodes seen within the left side of the neck and indeterminate left-sided lung nodules. Positron emission tomographic CT showed low-grade tracer uptake in the soft tissue of the left side of the face, left cervical level II lymph nodes, and additional uptake in two upper nodules of the left lobe of the lung, caecal mesenteric nodule, and sacrum.
https://doi.org/10.1016/j.bjoms.2020.01.023 0266-4356/Crown Copyright © 2020 Published by Elsevier Ltd on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved.
Please cite this article in press as: Sayan A, et al. Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma. Br J Oral Maxillofac Surg (2020), https://doi.org/10.1016/j.bjoms.2020.01.023
YBJOM-5906; 2
No. of Pages 3
ARTICLE IN PRESS A. Sayan et al. / British Journal of Oral and Maxillofacial Surgery xxx (2020) xxx–xxx
Fig. 1. Diffuse erythematous swelling affecting left lower eyelid, cheek, and upper lip.
The findings were discussed in the multidisciplinary skin cancer meeting and, as a result of the extent of the disease and comorbidities, the patient was referred for palliative care, and eventually died within six months of initial presentation.
Discussion
Fig. 2. Magnetic resonance image of the head and neck: diffuse plaque with soft tissue signal density (arrow) involving the subcutaneous tissue of the left side of the face.
Histopathological study of a deep incisional skin biopsy, including immunohistochemistry, showed features of adenocarcinoma with signet-ring cell morphology (Fig. 3).
The clinical presentation of primary cutaneous signet-ring cell carcinoma is insidious and initially non-specific, which may cause a diagnostic dilemma. The gradual thickening and swelling of the eyelid, or lids, and surrounding areas, with absent neurological deficit, may give the false impression of an inflammatory process such as orbital cellulitis or cutaneous deposition such as amyloidosis. Eyelid location is described in most cases, with occasional cases of axillary involvement.1 It is more common in men, with a mean age of 63 years.3 The histopathology is characteristic. The epidermis is unaffected with Grenz zone of spared superficial dermis. The underlying dermis and subcutaneous tissue comprise diffuse cords of histiocytoid cells interspersed between collagen bundles, which are arranged like a “stack of pennies” with the characteristic signet-ring cells.2–4 MUC6 positivity on immunohistochemistry suggests histogenesis from the glands of Moll.5 The differential diagnosis includes metastatic adenocarcinoma, particularly lobular breast carcinoma, or that arising from the gastrointestinal tract or lung.6 There are no defini-
Please cite this article in press as: Sayan A, et al. Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma. Br J Oral Maxillofac Surg (2020), https://doi.org/10.1016/j.bjoms.2020.01.023
YBJOM-5906;
ARTICLE IN PRESS
No. of Pages 3
A. Sayan et al. / British Journal of Oral and Maxillofacial Surgery xxx (2020) xxx–xxx
3
Fig. 3. Histopathological slides of the skin: A) Diffuse infiltrate of tumour cells involving the full thickness of the biopsy (haematoxylin and eosin, original magnification x 20); B) Signet-ring cells (arrows) (haematoxylin and eosin, original magnification x 400); C) Tumour cells that have infiltrated collagen, with “stack of pennies” configuration (arrows); (haematoxylin and eosin, original magnification x 200); D) Tumour cells that have stained positive for cytokeratin 7.
tive markers to reliably discriminate between a primary or metastatic disease.2–4 Imaging had excluded metastasis in our patient. The recommended treatments include wide local excision, and in some cases exenteration of the orbit may be required. There is a substantial tendency to local recurrence or metastasis, or both. Adjuvant radiotherapy, as well as anti-oestrogen or anti-androgen therapy, depending on the tumour markers expressed, are also described. 2,7 We report this case to highlight the unusual but characteristic clinical presentation of such insidious eyelid swelling in an elderly male, which is also termed the “monocle” sign. Conflict of interest We have no conflicts of interest.
References 1. Tanboon J, Uiprasertkul M, Luemsamran P. Signet-ring cell/histiocytoid carcinoma of the eyelid: a case report and review of the literature. Am J Dermatopathol 2013;35:e1–5. 2. Bernárdez C, Macías Del Toro E, Ramírez Bellver JL, et al. Primary signet¨ ring cell/histiocytoid carcinoma of the eyelid: a binocle¨ presentation of the “monocle tumor”. Am J Dermatopathol 2016;38:623–7. 3. Requena L, Prieto VG, Requena C, et al. Primary signet-ring cell/histiocytoid carcinoma of the eyelid: a clinicopathologic study of 5 cases and review of the literature. Am J Surg Pathol 2011;35:378–91. 4. Mortensen AL, Heegaard S, Clemmensen O, et al. Signet ring cell carcinoma of the eyelid — the monocle tumour. APMIS 2008;116:326–32. 5. Iwaya M, Uehara T, Yoshizawa A, et al. A case of primary signet-ring cell/histiocytoid carcinoma of the eyelid: immunohistochemical comparison with the normal sweat gland and review of the literature. Am J Dermatopathol 2012;34:139–45. 6. Boyd AS. Pulmonary signet-ring cell adenocarcinoma metastatic to the skin. Am J Dermatopathol 2017;39:e66–8. 7. Sakamoto K, Ito T, Tanioka F, et al. Primary signet-ring cell/histiocytoid carcinoma of the eyelid expressing androgen receptors and treated with bicalutamide. J Dermatol 2017;44:e230–1.
Ethics statement/confirmation of patient’s permission Ethics approval not required. Consent has been obtained.
Please cite this article in press as: Sayan A, et al. Unusual case of primary cutaneous signet-ring cell (histocytoid) carcinoma. Br J Oral Maxillofac Surg (2020), https://doi.org/10.1016/j.bjoms.2020.01.023