Primary Skin Tumors Research Articles

Chondroid Syringoma of the Hypothenar Region.

To the Editor, Chondroid syringoma (CS) is a benign adnexal tumor of eccrine/apocrine sweat glands. CS constitutes of 0.01–0.1% of primary skin tumors (1). CS is usually seen in elderly males as a painless, slowly growing, well demarcated subcutaneous/intradermal nodular lesion originating from the skin of the region of the head and neck, especially the cheek, nose and lip (2). Rare localization areas are the axillary region, abdomen, trunk, genitalia and extremities (1,2). There are a few case reports defining the cytological characteristics of CS in the English language literature (2–5). A 30 year-old female patient was admitted because of a slowly growing painless swelling in the right hypothenar region for 5 years (Figure 1). The swelling was in a character of well demarcated subcutaneous nodular lesion. Aspiration smears had moderate cellularity with mild nuclear pleomorphism. Single or small groups of tumor cells with slightly eccentric nuclei on chondromyxoid matrix were noticed (Figure 2). The cytological findings reminded benign mixed tumor of salivary glands. We reported the case as a benign tumoral lesion with chondroid matrix. The excised lesion was a well demarcated and partially capsulated nodular lesion with bright, solid gray-white colored cut-section. The lesion had dimensions of 2.5×2×1.5 cm. Microscopy showed an intradermal/subcutaneous tumoral lesion consisting of small clusters or short chords of benign epithelial or myoepithelial cells in chondromyxoid matrix. There were no findings of necrosis, atypical mitoses, meaningful nuclear pleomorphism and vascular invasion. We diagnosed CS on the basis of the histopathological findings. No recurrence was observed 3 years after the excision. Informed consent was obtained from the patient. FIG. 1. A subcutaneous nodular lesion with overlying intact skin on right hypothenar region FIG. 2. a, b. Groups of the cells with monomorphic and mildly eccentric nuclei, amphophilic staining cytoplasm in chondromyxoid stroma on aspiration smears (MGG, ×20) (a), tumor cell cords and groups intermingled with myxoid stroma (HE, ×15) (b) ... Despite CS usually being benign tumors, malignant forms have been also reported (1,2). Malignant forms usually localize on the trunk and extremities and are mostly seen in young women. They are rapidly growing masses and their diameters are larger than 3 cm. Erosion/ulceration of the overlying skin is frequent. Mortality rates are high due to metastasis to the lung in malignant forms. Histological evidences of malignancy and satellite nodules favor the diagnosis of malignant CS (1,2). Chondroid syringoma should be differentiated from other subcutaneous tumoral lesions. Some of these lesions are dermoid cysts, sebaceous cysts, peripheral nerve sheet tumors (PNST), lipoma, hemangioma, dermatofibroma and pilomatrixoma, myxoid liposarcoma, extraskeletal myxoid chondrosarcoma, myxofibrosarcoma and malignant PNST (1,2). The diagnosis is usually made by histopathological examination. However, there are only a few reports describing the cytological characteristics of CS (2–5). The presence of uniform plasmacytoid cells and myoepithelial cells in the chondromyxoid matrix favor the cytological diagnosis of CS (2). These findings may also be seen on aspiration smears obtained from mesenchymal tumors, especially chondroid tissue tumors. Cytological discrimination between malignant CS and sarcomas is very difficult. The age of our case and localization of the mass were not usual for benign CS. However, there were no histological/cytological and clinical evidences of malignancy. Some cases may be overlooked in routine practice. Hence, if aspiration smears from a subcutaneous nodular lesion show plasmacytoid cells in a chondromyxoid matrix, CS should be considered in the spectrum of differential diagnosis.

Scalp metastasis from follicular thyroid carcinoma diagnosed by fine needle aspiration cytology: A case series

The scalp metastasis of follicular thyroid carcinoma is a rare condition and the lesion should be differentiated from primary skin tumors. It account for 10–20% of all thyroid malignancies and is most commonly seen in female of 40–50 years of age. We present a case series of four cases showing cutaneous metastases from follicular thyroid carcinoma in patients presenting with a nodular thyroid mass and scalp swelling. Fine needle aspiration cytology (FNAC) of thyroid gland and scalp nodule, both shows repetitive microfollicles and clusters of follicular epithelial cells. The cases were diagnosed as scalp metastasis from follicular thyroid carcinoma on FNAC.

Tensor fascia lata flap is a workhorse for defects after inguinal lymph node block dissection

Enlarged inguinal lymph nodes very often present a site of metastatic disease. Inguinal lymph node block dissection is a demanding procedure, which usually requires at least one of reconstructive modalities. Among different reconstruction options we selected the tensor fascia lata (TFL) musculocutaneous flap. The paper aims at presenting a series of inguinal block dissections, followed by immediate reconstruction, using the TFL flap, and evaluation of tumor type, flap dimension, complication rate and the duration of hospital stay. We present a consecutive case series of 25 conducted block dissections. The defects were reconstructed using TFL flap, because of the extent and site of the tissue defects, reliability of the flap, and potentially primarily infected exulcerated tumors. The reconstruction was successful in all cases, the incidence of surgical complications was 16%, no further complications, such as lymphedema or gait disturbances, were noted. Primary skin tumors were predominant (13 cases), followed by genitalia tumors (four cases). The male sex was more frequently affected (14 vs. 11 cases). Having in mind that TFL presents as a flap of adjustable size, length, shape, and volume, with negligible donor site morbidity, and after comparing of our results to those of other authors, we advise broader use of TFL flap. As a reliable flap, not too difficult to harvest, with a low complication rate, it must be taken into consideration regarding the benefits for the patient, and, on the other hand, the surgery cost and duration.

LINFOMA DE CENTRO FOLICULAR CUTÂNEO PRIMÁRIO – UMA ENTIDADE A CONSIDERAR

Introdução: O linfoma de centro folicular cutâneo primário (LCFCP) é o subtipo mais comum de linfoma de células B primário.Caso clínico: Doente do sexo feminino, 69 anos, assintomática até há 1 ano, altura em que refere o aparecimento de um nódulo, indolor, na região frontal, tendo sido observada várias vezes no Serviço de Urgência. Ao exame objetivo, observava-se um nódulo, de coloração rosada, localizado na região frontal direita, com 3x2 cm, circundado por pequenas pápulas. Efetuou-se biópsia do nódulo e a histologia foi sugestiva de linfoma folicular.Imunofenotipagem: CD20+, CD3-, CD5-, CD10+, Bcl2+ em raras células. A doente foi enviada para a consulta de Hemato-Oncologia, tendo realizado estadiamento, que foi negativo e tratamento com R-CHOP, seguido de radioterapia. No follow-up de 6 meses não se diagnosticaram recidivas.Conclusão: Apresenta-se este caso para enfatizar a importância de alertar as outras especialidades para esta entidade, para a diagnosticar atempadamente.

Somatostatin Receptors 2A and 5 Are Expressed in Merkel Cell Carcinoma with No Association with Disease Severity

Background/Aims: Merkel cell carcinoma (MCC) is a rare high-grade neuroendocrine tumour of the skin. It has been speculated that MCCs express somatostatin receptors (SSTRs), but this has never been assessed in a large series of MCCs. The main aim of this study was to assess the expression of SSTR2A and SSTR5 in MCC tumours. The secondary aims were to assess whether expression of SSTR was associated with the Ki67 proliferative index, Merkel cell polyomavirus (MCPyV) status, clinical characteristics and outcome. Methods: Clinical data and tumours were collected from an ongoing cohort of French patients with MCC. Immunohistochemistry was performed with anti-SSTR2A and anti-SSTR5 monoclonal antibodies, and tumours were classified into 3 groups: ‘no expression', ‘low expression' and ‘moderate expression' using an SSTR staining score. Results: SSTR expression was assessed for 105 MCC tissue samples from 98 patients, and clinical characteristics were available for 87 of them. SSTR expression was consistent between the primary skin tumour and the corresponding metastases for SSTR2A and SSTR5 in 3/7 and 6/7 cases, respectively. SSTR2A and SSTR5 were expressed in 58 cases (59.2%) and in 44 cases (44.9%), respectively. Overall, at least one SSTR was expressed in 75 tumours (76.5%). SSTR expression was not associated with clinical characteristics, Ki67 proliferative index, recurrence-free survival or MCC-specific survival. Expression of SSTR2A was associated with MCPyV status in MCC tumours but not SSTR5. Conclusion: SSTRs were expressed in a high proportion of MCCs, although expression was heterogeneous between tumours and was not associated with disease severity.

The prognostic significance of indoleamine-2,3-dioxygenase and the receptors for transforming growth factor β and interferon γ in metastatic lymph nodes in malignant melanoma.

We analyzed the prognostic significance of indoleamine-2,3-dioxygenase (IDO) and type 1 receptors for transforming growth factor beta (TGF-βR1) and interferon gamma (IFN-γR1) in resected nodal metastases of 48 malignant melanoma patients. In 32 cases the corresponding skin tumors were available. We used immunohistochemical (IHC) staining which was assessed by pathologists and by a computer-aided algorithm that yielded quantitative results, both absolute and relative. We correlated the results with the patient outcome. We identified absolute computer-assessed IDO levels as positively correlated with increased risk of death in a multivariate model (HR = 1.02; 95% CI: 1.002-1.04; p = 0.03). In univariate analysis, patients with IDO levels below the median had a better overall survival time (30.3 vs. 17.5 months; p = 0.03). TGF-βR1 and IFN-γR1 expression was modestly correlated (R = 0.34; p lt; 0.05) and TGF-βR1 expression was lower in lymph nodes than in matched primary skin tumors (Z = 2.87; p = 0.004). The pathologists' and computer-aided IHC assessment demonstrated high correlation levels (R = 0.61, R = 0.74 and R = 0.88 for IDO, TGF-βR1 and IFN-γR1, respectively). Indoleamine-2,3-dioxygenase is prognostic for the patient outcome in melanoma with nodal involvement and should be investigated prospectively for its predictive significance. IHC assessment by computer-aided methods is recommended as its gives IHC more objectivity and reproducibility. ecting mismatch repair deficiency. Association of CDX2 and PMS2 in the present study is necessary to conduct further genetic and pathological studies focusing on these two markers together.

Poorly Differentiated Squamous Cell Carcinoma in an Atomic Bomb Survivor.

Dear Editor: Many epidemiological studies of atomic bomb survivors in Japan demonstrate that exposure to radiation increases the risk of cancers, especially leukemia, in most organs1. The risk of non-melanoma skin carcinoma in atomic bomb survivors is elevated2, and the most common primary skin tumors among 152 skin tumors among patients exposed to atomic bomb radiation were basal cell carcinoma (42%), followed by squamous cell carcinoma (SCC) (26%), Bowen's disease (13%), and malignant melanoma (6%)3. Here, we report a case of poorly differentiated SCC in an atomic bomb survivor. An 86-year-old Japanese woman visited our hospital with a one-month history of subcutaneous tumor in the inguinal region. She noticed a tumor on her the left side of waist when she was 46 years old. She was exposed to atomic bomb radiation in Hiroshima at the age of 19 years. Physical examination revealed a 32×40-mm reddish dome-shaped tumor on her the left side of the waist (Fig. 1A) and a 65×72-mm painful subcutaneous tumor on her left inguinal region (Fig. 1B). Blood examination revealed no abnormal changes. Whole-body computed tomography revealed no remarkable changes. Fluorodeoxyglucose-positron emission tomography revealed abnormal uptake in the tumor of on the waist and left inguinal lymph nodes. Histopathological examination of the waist tumor demonstrated numerous tumor cells throughout the dermis and subcutis (Fig. 1C). Atypical large tumor cells had irregular nuclear contours and prominent nucleoli (Fig. 1D). Immunohistochemistry showed the tumor cells were positive for keratin, CK7, p63, p40, and NSE but negative for CK20, chromogranin A, synaptophysin, thyroid transcription factor-1 (TTF-1), CD56, Melan-A, S-100, and EBER (Fig. 2A~E). The results of the histological and immunohistochemical examinations of the inguinal tumor were same. On the basis of these findings, she was diagnosed with poorly differentiated SCC in the waist and metastasis in the inguinal lymph nodes. Tumors in the waist and inguinal regions were resected, and she was subsequently treated with electron beam radiation (total dose: 59 Gy). Eight months later, a metastatic lesion in the left lung was observed, but she refused additional treatment. Fig. 1 (A) Reddish dome-shaped tumor on the left side of the waist. (B) Painful subcutaneous tumor on the left inguinal region. (C, D) Histopathological examination of the waist tumor (HE C: ×40, D: ×1,000). (C) Numerous tumor cells ... Fig. 2 Immunohistochemical examination of the waist tumor. Tumor cells stained positive for (A) keratin and (B) CK7, and negative for (C) chromogranin A, (D) CK20, and (E) thyroid transcription factor-1 (A~E: ×400). Tumor cells were positive for keratin as well as squamous cell markers p63 and p40. The common markers of neuroendocrine carcinomas, including Merkel cell carcinoma, CK20, chromogranin A and synaptophysin, were negative. TTF-1, a marker of primary thyroid and lung carcinoma, was negative. These immunohistochemical results and the low differentiation pattern of tumors on hematoxylin & eosin staining lead to a final diagnosis of poorly differentiated SCC. Regarding the relationship between the extent of tumor differentiation and atomic bomb radiation exposure, the frequency of poorly differentiated adenocarcinoma of stomach in atomic bomb survivors is significantly higher than that in non-exposed individuals4. Nevertheless, the association between atomic bomb radiation exposure and poorly differentiated SCC in our patient is speculative. A recent study reports multiple basal cell carcinomas were developed in atomic bomb survivors more than 40 years after radiation exposure5. Thus, exposure to atomic bomb radiation might influence the risk of cancers; therefore, dermatologists should perform long-term surveillance for skin carcinomas in atomic bomb survivors.

Abstract 5352: Ultraviolet radiation induces carcinogenesis in a novel transgenic mouse model of melanoma

Abstract Melanomagenesis is caused by environmental factors, such as ultraviolet (UV) radiation, as well as genetic factors. The Nucleotide Excision Repair (NER) pathway repairs photoproducts induced by UV radiation. In humans, NER pathway disruption leads to the development of skin cancers, including melanoma. Upon UV exposure, mice deficient in Xpc, an important protein in the NER pathway, develop skin cancer; but not melanoma. The Endothelin 3 (Edn3) signaling pathway is essential for melanocyte precursor cell development. In humans, this pathway has also been associated with melanoma progression and its metastatic potential. The purpose of this study is the development of a UV-dependent melanoma mouse model that combines Xpc deficiency with the over-activation of the Edn3 pathway. Transgenic mice over-expressing Edn3 under the control of the keratin 5 promoter (K5-Edn3) and carrying a targeted mutation in Xpc were exposed to a single suberythemal neonatal dose of UV radiation. Immunostaining and histomorphology were used to confirm the melanocytic origin of primary skin tumors. Melanomas were only found in animals with the K5-Edn3 transgene. High penetrance was observed in Xpc null (67%, n=3) and Xpc heterozygous (67%, n=3) mice in comparison to Xpc wild type (33%, n=3) mice. RNA extracted from melanomas of Xpc null (n=2), Xpc heterozygous (n=1) and Xpc wild type (n=1) mice were screened for UV signature mutations in Hras, Kras and Nras. No mutations were found in any of these genes. These results indicate that UV radiation exposure, in conjunction with over-activation of the Edn3 pathway is sufficient to lead to melanomagenesis in mice independently of mutations in Hras, Kras or Nras. Citation Format: Ana Paula Benaduce, Deannys Batista, Gabriel Grilo, Karen Jorge, Diana Cardero, Clara Milikowski, Lidia Kos. Ultraviolet radiation induces carcinogenesis in a novel transgenic mouse model of melanoma. [abstract]. In: Proceedings of the 105th Annual Meeting of the American Association for Cancer Research; 2014 Apr 5-9; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2014;74(19 Suppl):Abstract nr 5352. doi:10.1158/1538-7445.AM2014-5352

SOX2 controls tumour initiation and cancer stem-cell functions in squamous-cell carcinoma.

Cancer stem cells (CSCs) have been reported in various cancers, including in skin squamous-cell carcinoma (SCC). The molecular mechanisms regulating tumour initiation and stemness are still poorly characterized. Here we find that Sox2, a transcription factor expressed in various types of embryonic and adult stem cells, was the most upregulated transcription factor in the CSCs of squamous skin tumours in mice. SOX2 is absent in normal epidermis but begins to be expressed in the vast majority of mouse and human pre-neoplastic skin tumours, and continues to be expressed in a heterogeneous manner in invasive mouse and human SCCs. In contrast to other SCCs, in which SOX2 is frequently genetically amplified, the expression of SOX2 in mouse and human skin SCCs is transcriptionally regulated. Conditional deletion of Sox2 in the mouse epidermis markedly decreases skin tumour formation after chemical-induced carcinogenesis. Using green fluorescent protein (GFP) as a reporter of Sox2 transcriptional expression (SOX2-GFP knock-in mice), we showed that SOX2-expressing cells in invasive SCC are greatly enriched in tumour-propagating cells, which further increase upon serial transplantations. Lineage ablation of SOX2-expressing cells within primary benign and malignant SCCs leads to tumour regression, consistent with the critical role of SOX2-expressing cells in tumour maintenance. Conditional Sox2 deletion in pre-existing skin papilloma and SCC leads to tumour regression and decreases the ability of cancer cells to be propagated upon transplantation into immunodeficient mice, supporting the essential role of SOX2 in regulating CSC functions. Transcriptional profiling of SOX2-GFP-expressing CSCs and of tumour epithelial cells upon Sox2 deletion uncovered a gene network regulated by SOX2 in primary tumour cells in vivo. Chromatin immunoprecipitation identified several direct SOX2 target genes controlling tumour stemness, survival, proliferation, adhesion, invasion and paraneoplastic syndrome. We demonstrate that SOX2, by marking and regulating the functions of skin tumour-initiating cells and CSCs, establishes a continuum between tumour initiation and progression in primary skin tumours.

Use of electrochemotherapy in a case of neck skin metastasis of oral squamous cell carcinoma: Case report and considerations

BackgroundSquamous cell carcinoma (SCC) is the most common oral cavity malignant tumor. Surgery, radiotherapy, and chemotherapy have been the major options for its treatment. Electrochemotherapy (ECT) is a novel local treatment successfully used in secondary or primary skin or subcutaneous tumors. This new cancer treatment is a modality in which a locally applied electrical field enhances cell membrane permeability, thereby allowing greater intracellular accumulation of a chemotherapeutic agent.MethodsWe report a case of a man affected by an ulcerated SCC. The man was treated with ECT with good results.ResultsIn our case, ECT was successful in the management of extensive metastasis of SCC in clinical conditions, whereas other approaches were rejected.ConclusionThis case shows good clinical results; however, other studies are necessary to show that ECT should be considered as a promising treatment option. © 2014 The Authors. Head & Neck Published by Wiley Periodicals, Inc. Head Neck 36: E86–E90, 2014

Postradiation cutaneous pleomorphic rhabdomyosarcoma with extracellular collagen deposits reminiscent of so‐called amianthoid fibers

Rhabdomyosarcoma is a malignant mesenchymal neoplasm that rarely presents as primary skin tumor. So-called amianthoid fibers are hyalinized collagen mats that have been described in myofibroblastic tumors but not in rhabdomyosarcoma. A 65-year-old male developed a submandibular nodule 9 years after an oral squamous cell carcinoma, which had been treated with chemotherapy and radiotherapy. Histological examination of the nodule revealed a pleomorphic rhabdomyosarcoma with extracellular collagen deposits reminiscent of so-called amianthoid fibers. By immunohistochemistry, the tumor cells were positive for vimentin, desmin, smooth muscle actin (SMA), muscle-specific actin (MSA), CD10, CD56, CD99, β-catenin and D2-40. However, only 15-20% of the tumor cells were positive for myoglobin, MyoD1 and myf-4/myogenin. We describe first so-called amianthoid fibers harboring blood capillaries in a rhabdomyosarcoma, suggesting that they are rigid collagen structures that lead to tumor vascularization. The low expression for myogenic regulatory proteins and strong expression for other markers may be misleading and do not contribute to the diagnosis of rhabdomyosarcoma.

Primary tumor versus metastasis: new experimental models for studies on cancer cell homing and metastasis in melanoma

Primary tumor versus metastasis: new experimental models for studies on cancer cell homing and metastasis in melanoma

Chondroid Syringoma: Report of a Case with Uncommon Location

‘‘The mixed tumor’’ of the skin was originally defined by Billroth in 1859 as an entity having the same histopathologic properties of the mixed tumors of the salivary glands [1]. The term ‘chondroid syringoma’ was first used by Hirsch and Helwig in 1961 to describe this sweat gland tumor, because of the presence of sweat gland elements which are set in a cartilaginous stroma [2]. The reported incidence of CS among primary skin tumor is low, ranging between 0.010.098% [3]. This uncommon eccrine sweat gland tumor clinically presents as a slow-growing, painless, subcutaneous or intracutaneous nodule located usually in the head and neck region, and it affects middle-aged or older men [3,4]. Less commonly, this tumor can develop in the axillary region, penis, vulva, and scalp. We report a rare case of a chondroid syringoma with an atypical location on the back.

Abstract 1276: UV radiation interacts with Endothelin 3 and loss of nucleotide excision repair pathway to promote melanomagenesis.

Abstract Melanomagenesis is influenced by the interaction of environmental (such as UV radiation) and genetic factors. The Xpa gene is important in the Nucleotide Excision Repair (NER) pathway. Similar to humans afflicted with Xeroderma Pigmentosum, which results from mutations in genes that play a role in NER, Xpa deficient mice show high sensitivity to UV light, leading to skin cancer development. However, Xpa deficient mice do not develop melanoma even upon UV exposure. The Endothelin 3 (Edn3) signaling pathway is essential for proliferation, survival and migration of melanocyte precursor cells. In humans, this pathway has also been implicated in melanoma progression and its metastatic potential. The purpose of this study is to develop a UV induced melanoma mouse model that combines Xpa deficiency with the over-activation of the Edn3 pathway. To this end, transgenic mice over-expressing Edn3 under the control of the keratin 5 promoter (K5-Edn3) and carrying a targeted mutation in Xpa were exposed to a single suberythemal neonatal dose of UV radiation. A subgroup of mice was also exposed to a second dose of UV radiation at 6 weeks of age. Histomorphology and immunostaining were used to confirm the melanocytic origin of primary skin tumors and metastases. Melanoma was only found in animals with the K5-Edn3 transgene. High penetrance was observed in animals exposed to one dose of UV radiation that were Xpa null (100%, n=3) when compared to Xpa heterozygous (73%, n=11) or Xpa wild type (43%, n=7). Enlarged and hyperpigmented lymph nodes were present in animals with melanoma skin lesions and these were diagnosed as local metastases. These results indicate that UV radiation exposure, in conjunction with over-activation of the Edn3 pathway is sufficient to lead to melanomagenesis in mice. Citation Format: Ana Paula Benaduce, Deannys Batista, Gabriel Grilo, Karen Jorge, Lidia Kos. UV radiation interacts with Endothelin 3 and loss of nucleotide excision repair pathway to promote melanomagenesis. [abstract]. In: Proceedings of the 104th Annual Meeting of the American Association for Cancer Research; 2013 Apr 6-10; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2013;73(8 Suppl):Abstract nr 1276. doi:10.1158/1538-7445.AM2013-1276

Chondroid syringoma: A rare lid tumor

Sir, Chondroid syringoma is a rare primary skin tumor (0.01% to 0.1% of primary skin tumors).[1] Though its very common occurrence is in head and the neck region, involvement of eyelids is extremely rare. In English literature we found 27 reported cases of chondroid syringoma involving ocular adnexa.[1,2] We are reporting a case of recurrent chondroid syringoma of upper eyelid in a young male. A 27-year-old male present with a slow growing painless mass in right upper lid since past 6 months duration. The mass was 1.5 cm × 1.5 cm firm, non-tender, sub-cutaneous and not fixed to underlying tissues [Fig. 1]. This was a recurrence of a lesion that had been removed by lamellar excision in the same site 2 years back. The recurrent mass was removed this time by pentagonal full thickness lid excision with re-construction by advancement eyelid flap. Histopathological examination showed tumor with variegated appearance. The epithelial component composed of round to oval cells with large nucleoli with scanty cytoplasm and the cells were arranged in nests and acinar pattern. The coating for epithelium of the glandular elements was two layered. The intervening stroma showed fibrosis and myxoid areas. No evidence of lacrimal gland tissue was noted in multiple sections [Fig. 2]. Figure 1 1.5 cm × 1.5 cm mass involving the right upper lid Figure 2 Histopathology showing glandular elements with two layered lining epithelium in myxoid matrix Chondroid syringoma is a tumor arising from the sweat glands which can be apocrine (found throughout the surface of lid) or eccrine (glands of Moll in association with eyelash follicles) with a mean age of 50 years (22-73 years).[1] Ozdal et al. in a study of 228 benign ocular adnexal tumors reported apocrine/eccrine hydrocystoma as very common and no case of Chondroid Syringoma was found in that series.[3] Tyagi et al. in their study of 207 eyelid tumors had one case of Chondroid syringoma with an incidence of 0.48% in their series.[4] This tumor was first described by Billroth[5] and the term chondroid syringoma was coined by Hirsh and Helwig to characterize the origin from sweat glands[6] though the appearance is like pleomorphic adenoma of lacrimal gland. A proper histopathological examination to rule out presence of normal lacrimal gland tissue external to the tumor capsule helps to differentiate chondroid syringoma.[1] The case being reported had tubular and cystic branching lumina with 2 layered epithelium showing an apocrine origin.[1] Chondroid syringoma is a benign tumor but incomplete excision can undergo malignant transformation.[1] Hence complete excision is advised. This case is being reported for its rarity (this is the 28th case in literature involving ocular adnexa), its occurrence in a young patient and the need to keep this rare entity as a differential diagnosis of eyelid tumors.

Facial Localization of Malignant Chondroid Syringoma: A Rare Case Report

First described by Hirsch and Helwig in 1961, chondroid syringomas (CSs) are rare, benign tumors of the skin arising from the eccrine sweat glands with tumor differentiation in the epithelial and mesenchymal tissues. They most commonly occur in the head and neck, although they may be also found in the axilla, trunk, limbs, and genitalia. The incidence of CS is <0.01% of all primary skin tumors. Malingnant chondroid syringomas (MCS), which are also called malignant mixed tumors of the skin, are extremely uncommon. MCSs commonly involve the limbs and rarely head and neck. In this article, we present a case of malignant chondroid syringoma localized in the face at the left nasolabial region in the light of literature review.

Cardiac fibroma, anomalous pulmonary venous course, and persistent pneumonia in a patient with gorlin syndrome

We report the unusual case of a child referred for persistent pneumonia and possible pulmonary nodules who was found to have a cardiac fibroma and diagnosed with Gorlin syndrome. Pulmonologists should be aware of the potential for vascular anomalies associated with this syndrome as well as the risk for pulmonary metastases from primary skin tumors.

Psycho-oncological aspect of surgery in palliative care: our satisfactory experience using a V-Y island flap

Conclusions: Our reconstruction method using a V-Y island flap was minimally invasive and yielded a satisfactory esthetic result without impairing the patient's postoperative quality of life (QOL). Objective: Malignant skin tumors of the head and neck often affect the patient's appearance and QOL. Therefore, surgery for terminally staged patients with malignant skin tumors may be a treatment of choice for surgeons in a palliative care team to improve and sustain the patient's QOL. Methods: We describe our experience of surgical treatment of a large primary malignant skin tumor in the head performed as part of palliative care in a 38-year-old woman in the terminal stage. We developed a V-Y island flap that did not result in postoperative alopecia. Results: After surgery, the patient did not have alopecia and her hair hid the scar. The patient and her family were greatly satisfied with the result. Although the surgery did not extend her life, it alleviated her depressed condition caused by the uncomfortable primary tumor.

Lymphangiectases Are Common Underlying Warts and in Normal Peritumoral Skin: Histologic Evidence of Decreased Immune Surveillance

Lymphangiectases are a histologic sign of lymphostasis, which is associated with decreased immune cell trafficking and cell-mediated immunity. To determine if latent lymphedema is apparent underlying warts and in skin affected by cutaneous neoplasia. The number and maximal dilation of lymphangiectases were measured in the upper half of the dermis of 51 consecutive biopsies of warts, 230 consecutive normal skin samples from primary skin tumor excisions, and 14 normal skin samples from breast reduction (11) and panniculectomy (3) specimens. All warts had one or more underlying lymphangiectases compared with 79% of peritumor normal skin samples and 50% of cosmetic specimens. The mean number of lymphangiectases and mean maximal dilation were significantly greater in warts than in peritumor skin, which was significantly greater than cosmetic skin samples (3.6 vs. 1.3 vs. 0.12 lymphangiectases per square millimeter and 54 vs. 23 vs. 1 μm, respectively; P = 0.0001). Warts exhibited mild fibrosis significantly more frequently than peritumor skin (57% vs. 5%; P = 0.0001). For peritumor (normal) skin, age, solar elastosis, and adjacent malignancy correlated with greater dilation of lymphatics. Solar elastosis also correlated with increased number of lymphangiectases. Minor trauma and solar elastosis from chronic ultraviolet radiation exposure are likely the etiologic factors in the development of lymphostasis. By decreasing immune surveillance, latent lymphedema ostensibly facilitates human papillomavirus infection and carcinogenesis.

Metastatic vs primary malignant neoplasms affecting the umbilicus: clinicopathologic features of 77 tumors

Periumbilical skin is unique due to its proximity to intra-abdominal and pelvic structures. In addition to primary skin malignancies, it is a site often involved with metastatic disease. We reviewed the clinical and pathologic features of 77 umbilical malignancies occurring at our institution since 1988. Seventy-seven patients were identified (female/male ratio, 4.1:1.0) with the average age for women being 63 years and 55 years for men. Eighty-eight percent of malignancies originated outside the umbilicus and 12% were primary skin tumors. Fifty-eight (85%) patients with metastatic tumors had umbilical involvement from a known primary vs 10 (15%) with unknown primaries. Nine patients with metastatic tumors to the umbilicus would present with solitary umbilical involvement. Of these patients, 56% would not have a primary site assigned to their metastatic disease. In women, the 3 most common primary sites were the ovary, endometrium, and pancreatobiliary tree, whereas for men, it was the genitourinary tract, pancreatobiliary tree, and the gastrointestinal tract. Of the primary umbilical malignancies, 44% of patients were male and 56% female. Malignant melanoma was the most common primary umbilical malignancy. In summary, women are more likely than men to have malignant tumors affecting the umbilicus. Overall, the most likely primary site of a metastatic tumor to the umbilicus is the genitourinary tract. Rarely, patients present with metastatic tumors to the umbilicus, and most of these patients will not have a primary site of tumor origin assigned.