Epidemiology: Open Access
Open Access

Rare cancers; Epidemiology; Male bone cancer; Clinic Pathology; Chemotherapy

Introduction

Bone cancer in men is a pretty infrequent complaint and accounts for only 1 of the bone cancer population. Male bone cancer (MaBC), like all other rare diseases, has made it difficult to perform prospective clinical research, as evidenced by multiple clinical trials that were prematurely closed for lack of registration. The historical rejection of male participants in clinical studies for womanish bone cancer (FeBC) has made the issue worse. Our current understanding of male bone cancer is mostly based on limited retrospective studies and frequently single-centre experience; as a result, strategies for treating MaBC are determined by rules for treating FeBC. There is growing evidence that MaBC has discrete clinical characteristics that can be linked to biological circumstances (such as genomes and excrescence subtypes), and various therapeutic modalities may reduce morbidity and death. The epidemiology and risk factors are reviewed, the similarities and differences between MaBC and FeBC at the molecular level (including genomics and excrescence characteristics), the clinical features and individual modalities are described, the treatment guidelines are summarised, and future directions in research are discussed in this study. One of the rarest types of low-grade bone melanoma is secretory melanoma. It most frequently affects people under the age of 30, and it most frequently affects youngsters with bone melanoma [1].

According to McDivitt and Stewart, who first proposed this reality as "Juvenile bone cancer," the average age of the seven instances they recorded in their series was nine times older than typical, with a range of three to fifteen times. Although it was initially seen in youngsters, it is now understood to occur in adults of both sexes and has a higher male to female ratio. The excrescence, which has a balanced translocation that results in an ETV6-NTRK3 gene translocation, is the only epithelial excrescence of the bone [2]. The natural outcome of this translocation is the activation of the Ras- Mek1 and PI3K-Akt pathways, which are critical for bone cell proliferation and survival, through the fusion of the dimerization sphere of a transcriptional controller (ETV6) with a membrane receptor tyrosine kinase (NTRK3). Natural fibrosarcoma and mesoblastic nephroma, two juvenile mesenchymal excrescences without epithelial characteristics, are linked to this particular translocation. Many cytoplasmic grains or transparent vacuolated cytoplasm meet the criterion for separate pieces. Tubule conformation is prevalent and may contain stashing in the lumens. There may be a follicular pattern (thyroid-like) [3].

Cells, lumens, and stroma contain diastase-resistant secretory material that is mucicarmine, alcian blue, and papas positive. There are frequently noticeable stringy bands. Low-grade nuclear cytology, mellow, invariant capitals, and uncommon mitotic numbers are its defining characteristics. Distance-like growth with significantly constricted perimeters and sporadic infiltration foci and in situ elements is typical. On the subject of male gut secretory lymphomas, a whole plethora of writers have conducted immunohistochemistry research [4].

The epithelial membrane antigen, cytokeratin, carcinoembryonic antigen (polyclonal), S-100, and lactalbumin of these excrescences are stated to be positive. Secretory melanoma does metastasis to lymph lumps and recurs after initial excision, despite being seen as an inactive lump. Three characteristics of secretory melanoma, according to Tavassoli and Norris, are indicative of a favourable prognosis: excrescence size less than 2 cm, age less than 20 times at opinion, and excrescence with restricted perimeters. De Bree made the case that men seem to have more aggressive secretory lymphomas. The preferred course of treatment is surgery in the form of a mastectomy with axillary concurrence. Many examples of secretory bone melanoma with distant metastases that were unsuccessfully treated with either single agent or combination chemotherapy have been documented [5]. Medications such as 5-FU, vindesine, mitomycin, prednisone, adriamycin, epirubicin, cyclophosphamide, carboplatin, and even more recent active drugs comparable to docetaxel are among those reported. While all of the instances treated with chemotherapy showed complaint progression while on treatment, these results clearly demonstrate that this mass is not chemo sensitive [6].

Discussion

A remarkably uncommon kind of bone melanoma is secretory melanoma. In a retrospective series of 7038 bone melanoma patients, Tavassoli and Norris reported 4 cases of SC and set up 1 SC case among 3000 bone melanoma cases. The median age at donation is 23 times, although the range is 3 to 66 times. The case described here is incredibly unique because males have rarely been known to have secretory melanoma, especially metastatic secretory melanoma. Only 18 other occurrences in men were related by literature searches. Our patient was 17 times older than the median age reported for secretory melanoma in males. Highlights the key clinical characteristics of the instances of secretory melanoma recorded in males, of which only the case reported by Kuwabara occurred in old age (66 times) [7].

Many histological forms, including solid, microcystic, and ductal, can be seen in secretory lymphomas, and many tumours exhibit all three patterns. The tumour cells are polygonal, with eosinophilic cytoplasm that is granular, and concealment that is papas- and alcianblue positive both inside and outside the cells. Low mitotic effort and minimal or non-existent atypia, According to a study, only 4 and 2 of the 13 patients independently expressed the oestrogen and progesterone receptors, and only two of them were HER2 positive. ER and progesterone receptors were both positive in the tumour in the present case. Clinical donations of asymptomatic mobile masses, which are typically sub areolar, occur most frequently. The excrescence is 1 cm to 16 cm in length, with a 3 cm average periphery. The bulk of our case was 2 2 cm. Given that the lesion was reported to have appeared at least nine times, it is presumed that it had developed slowly and unnoticeably [8].

Other documented cases lend credence to this. Surgery is thought to be the main treatment for secretory melanoma, although no published guidelines for surgical operation are now available due to the failure of documented cases, which have reported a MIB1 labelling indicator of 11.4 (range 1 to 34). Still, numerous studies reported original rush in some cases hence, mastectomy looks to be a sound surgical choice. Although there are currently no data on conservative treatment, this alternative could be investigated, especially in situations where bone formation has not yet finished. In reference to the operation of the axilla, the overall prevalence of axillary lymph knot infiltration is around 30 in children and grown-ups anyway of gender; consequently, axillary lymph knot analysis is supported by some writers for excrescences > 2 cm. Guard knot vivisection, however, may be helpful for secretory lymphomas. This is feasible, as demonstrated by a recent report on a 9-year-old girl who underwent a straightforward mastectomy and axillary guard lymph knot vivisection [9].

Adjuvant chemotherapy and postoperative radiation have both been utilised at least twice. There is currently insufficient evidence to support either recommendation for the treatment of secretory melanoma. Several cases have indicated an initial rush after a protracted period of no complaints; yet, these situations passed with conservative treatment. Only four incidences of distant metastases from secretory melanoma have ever been documented. Despite getting 12 out of 14 positive bumps and skipping adjuvant treatment, another patient was still complaint-free at a 13-month check-up [10].

Conclusion

MaBC is a relatively uncommon problem with increasing prevalence, however it is treated with the most cutting-edge clinical techniques selected based on information in FeBC. We may infer from the information at hand that MaBC differs from FeBC in both its molecular and clinic pathological characteristics, which may call for different treatment strategies. Colourful novel correctives, such as PARP obstructions and anti-androgen remedies that are undergoing tests in FeBC, may also be effective in MaBC. In clinical trials, there has been a recent trend to include MaBC cases in order to build a solid evidence base for the MaBC treatment of unborn children. Ideally, in the near future, cooperative multinational trouble will also smooth the execution of straightforward MaBC prospective studies.

Acknowledgement

None

Conflict of Interest

None

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