1. Cancer Surveillance data of Rare Epithelial Breast cancers in females-A Report from Population Based Cancer Registries in India.
Dr Shakuntala TS, Dr Meesha Chaturvedi, Sathish Kumar K, Priyanka Das, Sudarshan KL, Teena Sajan, Vinodh Nallasamy, Anish John and Dr Prashant Mathur

2. Background
Rare Breast cancer is a divergent group of diseases which presents with varied clinical presentations, morphological characteristics and clinical management.
This study aims to identify the various rare breast tumors based on morphological characteristics through the Population Based Registries and Hospital Based Registries for the period of in India.
This will help in classifying them according to their relevant features regarding rare cancers.

3. Methods
The data from five Population Based Cancer Registries (PBCRs) and Hospital Based Registries(HBCRs) under National Cancer Registry Programme (NCRP) in India was used to identify the rare epithelial tumors based on the morphological subtypes.
The analysis was done for a collective period of from the following PBCRs- Bangalore ( ), Bhopal ( ), Chennai ( ), Mumbai ( ) and Delhi ( ) and the various HBCRs, data was stratified based on morphologies, using the codes from International Classification of Diseases for Oncology, 3rd Edition (ICD-O-3).
The Crude Rate and Age Adjusted Incidence Rates (AAR per 1, 00, 000 population) was calculated using in-house developed PBCRDM 2.1 software.
The HBCR Data was analyzed to determine the correlation between the Clinical Extent of the Disease and the Treatment Patterns that are Followed respectively.

4. Data from 5 PBCRs registered under NCRP-NCDIR was analyzed for the period of 1982-2014
Incidence of <6 per year per lakh population was identified and the selected PHMs for the 4 Major types of Breast tumors was used for further analysis
CR & AAR was Calculated for the 4 Major types of Breast tumors and for the respective registries
Bangalore ( )
Bhopal ( )
Chennai ( )
Mumbai ( )
Delhi ( )
Rare tumors of Breast:
Mammary Pagets
Special types of Adenocarcinoma
Metaplastic Carcinoma
Salivary Gland Type Tumors of Breast

5. Data from various HBCRs registered under NCRP-NCDIR was analyzed for the period of 1982-2014
Incidence of <6 per year per lakh population was identified and the selected PHMs for the 4 Major types of Breast tumors was used for further analysis
Proportion for Clinical Extent of the disease and Treatment Patterns Followed were compared
Rare tumors of Breast:
Mammary Pagets
Special types of Adenocarcinoma
Metaplastic Carcinoma
Salivary Gland Type Tumors of Breast

6. 1. Mammary Paget’s disease of breast
Results
1. Mammary Paget’s disease of breast
Morphology Code
Description
8540
Paget disease, mammary
Paget disease of breast
8541
Paget disease and infiltrating duct carcinoma of breast
8543
Paget disease and intraductal carcinoma of breast

7. 1. Mammary Paget’s disease of breast
Age Group
Numbers
Population
ASPR
0-4
0.0000
5-9
10-14
15-19
20-24 1
0.0028
25-29 6
0.0171
30-34 9
0.0325
35-39 15
0.0582
40-44
0.0777
45-49 31
0.1844
50-54
0.2400
55-59 33
0.3397
60-64
0.3548
65-69 32
0.5447
70-74 10
0.2413
75+ 17
0.3431
Unk.
Total
231
0.0697
AAR

8. 2. Special types of adenocarcinoma of breast
Morphology Code
Description
8050
Papillary carcinoma, NOS
8201
Ductal carcinoma, cribriform type
8211
Tubular adenocarcinoma
8260
Papillary adenocarcinoma, NOS
8290
Oxyphilic adenocarcinoma
8314
Lipid-rich carcinoma
8315
Glycogen-rich carcinoma
8401
Apocrine adenocarcinoma
8480
Mucinous adenocarcinoma
8481
Mucin-producing adenocarcinoma
8502
Secretory carcinoma of breast
Juvenile carcinoma of breast
8510
Medullary carcinoma, NOS

9. 2. Special types of Adenocarcinomas
Age Group
Numbers
Population
ASPR
0-4
0.0000
5-9 1
0.0032
10-14
15-19 2
0.0063
20-24 19
0.0535
25-29 67
0.1907
30-34
134
0.4837
35-39
234
0.9084
40-44
273
1.4140
45-49
344
2.0461
50-54
294
2.2761
55-59
296
3.0472
60-64
330
3.7764
65-69
267
4.5451
70-74
225
5.4300
75+
254
5.1263
Unk.
Total
2740
0.8268
AAR

10. 3. Metaplastic carcinoma of breast
Morphology Code
Description
8070
Squamous cell carcinoma, NOS
8560
Adenosquamous carcinoma
Mixed adenocarcinoma and squamous cell carcinoma
Mixed adenocarcinoma and epidermoid carcinoma
8571
Adenocarcinoma with cartilaginous and osseous metaplasia
8572
Adenocarcinoma with spindle cell metaplasia
8575
Metaplastic carcinoma, NOS

11. 3. Metaplastic carcinoma of breast
Age Group
Numbers
Population
ASPR
0-4
0.0000
5-9
10-14
15-19
20-24 2
0.0056
25-29 11
0.0313
30-34 18
0.0650
35-39 26
0.1009
40-44 36
0.1865
45-49 39
0.2320
50-54 44
0.3406
55-59 27
0.2780
60-64 30
0.3433
65-69 28
0.4766
70-74 12
0.2896
75+ 19
0.3835
Unk.
Total
292
0.0881
AAR

12. 4. Salivary gland type tumours of breast
Morphology Code
Description
8200
Adenoid cystic carcinoma
8430
Mucoepidermoid carcinoma
8550
Acinar cell adenocarcinoma
8982
Myoepithelial carcinoma

13. 4. Salivary gland type tumours of breast
Age Group
Numbers
Population
ASPR
0-4
0.0000
5-9
10-14
15-19
20-24
25-29 1
0.0028
30-34
0.0036
35-39 4
0.0155
40-44 7
0.0363
45-49 2
0.0119
50-54
0.0310
55-59
0.0412
60-64 10
0.1144
65-69
0.1702
70-74
0.0483
75+
0.0202
Unk.
Total 46
0.0139
AAR

14. CR and AARs Sub - Types CR AAR Obs Paget’s 0.7 0.091 231
Adenocarcinoma
0.83
1.05
2740
Carcinoma
0.09
0.11
292
Salivary
0.14
0.02 46
CR & AAR
Proportion & Observed Numbers

15. PBCR Registry Data 1. Bangalore PBCR Age Group Numbers Population ASPR
0-4
0.0000
5-9
10-14
15-19
20-24 6
0.0664
25-29 24
0.2604
30-34 40
0.5942
35-39 70
1.1305
40-44 81
1.8900
45-49
110
2.9087
50-54
105
3.6132
55-59 90
4.2916
60-64
102
5.1447
65-69 76
5.9939
70-74 55
895195
6.1439
75+ 57
5.1191
Unk.
Total
816
1.0287
AAR
1.38

16. 2. Mumbai PBCR Age Group Numbers Population ASPR 0-4 13505660 0.0000
0.0000
5-9 1
0.0068
10-14
15-19
0.0071
20-24 8
0.0507
25-29 25
0.1604
30-34 61
0.4758
35-39
112
0.9415
40-44
129
1.4048
45-49
162
2.0365
50-54
149
2.4462
55-59
156
3.4089
60-64
166
4.2093
65-69
5.9251
70-74
111
5.9015
75+
169
7.4727
Unk.
Total
1412
0.9286
AAR

17. 3. Chennai PBCR Age Group Numbers Population ASPR 0-4 5042722 0.0000
0.0000
5-9
10-14
15-19 1
0.0163
20-24 5
0.0723
25-29 24
0.3522
30-34 35
0.6567
35-39 54
1.0566
40-44 55
1.4056
45-49 65
1.8719
50-54 57
2.0566
55-59
2.4994
60-64
2.7470
65-69 41
3.1096
70-74 33
965195
3.4190
75+ 34
3.0334
Unk.
Total
512
0.7941
AAR

18. 4. Bhopal PBCR Age Group Numbers Population ASPR 0-4 1813691 0.0000
0.0000
5-9
10-14
15-19
20-24
25-29
30-34 5
0.3444
35-39 8
0.6069
40-44 7
994565
0.7038
45-49 13
828472
1.5692
50-54 6
593305
1.0113
55-59
454409
1.7605
60-64 10
437418
2.2861
65-69 9
286495
3.1414
70-74 4
208046
1.9227
75+
238035
2.5206
Unk.
Total 76
0.4161
AAR

19. 5. Delhi PBCR Age Group Numbers Population ASPR 0-4 1734261 0.0000 5-9
0.0000
5-9
10-14
15-19
20-24 4
0.2161
25-29 12
0.7011
30-34 21
1.5326
35-39 34
2.7350
40-44 58
930627
6.2324
45-49 61
775379
7.8671
50-54 56
554801
55-59 51
425434
60-64 65
409068
65-69 47
267428
70-74 41
194312
75+ 25
220903
Unk.
Total
475
2.7479
AAR

20. HBCRs Data- Comparison between Clinical Extent of Disease & Treatment
1. Mammary Paget’s

22. 2. Special Types of Adenocarcinoma

24. 3. Special Types of Adenocarcinoma

26. 4. Salivary Gland Type Tumors of the Breast

28. Summary
The PBCR registries data was analyzed for 4 Broad Categories which includes 20 Subtypes and was classified as Rare Epithelial tumors of the Breast.
A total of 3,309 cases have been reported from the various registries between
The number of observed cases and AARs were
for Mammary Paget’s disease of Breast (231 & 0.09),
for Special types of Adenocarcinoma of Breast (2740 & 1.05),
for Metaplastic Carcinoma of Breast (292 & 0.11) and
for Salivary gland type tumors of the breast (46 & 0.02) respectively.
Based on the 5 registries the Observed Numbers and AAR were
Bangalore PBCR (816 & 1.34),
Mumbai PBCR (1412 & 1.18),
Chennai PBCR (512 & 0.86),
Bhopal PBCR (76 & 0.59),
Delhi PBCR (4575 & 3.97)

29. Conclusion
The present study gives an insight on the indication of type and treatment of rare breast cancer in India, the data was done in Comparison with Rarecare Net for the European population and had similar epidemiological information.
We are proposing on creating a repository for such cases where the data from India will be pooled in as rare tumors.
This exercise will give the necessary information to further understand the sub classification, behaviors and rarity of the tumors in Indian scenario.
The same approach for classification of rare tumors could be utilized for various other sites and a National Repository can be established.

30. Thank you

31. IARC data
Number of New Cases and Deaths per 100,000: The number of new cases of female breast cancer was per 100,000 women per year. The number of deaths was 20.6 per 100,000 women per year. These rates are age-adjusted and based on cases and deaths.
Lifetime Risk of Developing Cancer: Approximately 12.8 percent of women will be diagnosed with female breast cancer at some point during their lifetime, based on data.
Prevalence of This Cancer: In 2016, there were an estimated 3,477,866 women living with female breast cancer in the United States.

32. IARC data
Percent Surviving 5 Years ( )
89.9%

33. Estimated New Cases in 2019 : 268,600
% of All New Cancer Cases :15.2%
Estimated Deaths in 2019 :41,760
% of All Cancer Deaths: 6.9%

34. New Cases, Deaths and 5-Year Relative Survival
New cases come from SEER 9 Incidence. Deaths come from U.S. Mortality , All Races, Females. Rates are Age-Adjusted. Modeled trend lines were calculated from the underlying rates using the Joinpoint Trend Analysis Software.
Year Perc ent Surviving0100
SEER 9 5-Year Relative Survival Percent from , All Races, Females. Modeled trend lines were calculated from the underlying rates using the Joinpoint Survival Model Software.

35. Changes Over Time
Keeping track of the number of new cases, deaths, and survival over time (trends) can help scientists understand whether progress is being made and where additional research is needed to address challenges, such as improving screening or finding better treatments.
Using statistical models for analysis, rates for new female breast cancer cases have been rising on average 0.3% each year over the last 10 years. Death rates have been falling on average 1.8% each year over year survival trends are shown below.

36. There are four main female breast cancer subtypes, including the following in order of prevalence:
HR+/HER2- ("Luminal A")
HR-/HER2- ("Triple Negative")
HR+/HER2+ ("Luminal B")
HR-/HER2+ ("HER2-enriched")
HR stands for hormone receptor. HR+ means that tumor cells have receptors for the hormones estrogen or progesterone, which can promote the growth of HR+ tumors. HER2 stands for human epidermal growth factor receptor. HER2+ means that tumor cells make high levels of a protein called HER2/neu, which has been shown to be associated with certain aggressive types of breast cancer.

37. At a Glance
When all subtypes are combined, female breast cancer is fairly common. In 2019, it is estimated that there were 268,600 new cases of female breast cancer.
The breast cancer subtype HR+/HER2- is the most common subtype with an age-adjusted rate of 85.8 new cases per 100,000 women, based on cases.
This is a rate more than six times higher than the triple-negative breast cancer rate of 13.0 and the HR+/HER2+ breast cancer rate of 12.9, and over 15 times higher than HR-/HER2+ breast cancer rate of 5.4.

38. Rate of New Breast Cases per 100,000 Women, SEER 21 2012-2016
Subtype
New Cases
HR+/HER2-
85.8
HR-/HER2-
13.0
HR+/HER2+
12.9
HR-/HER2+
5.4
Unknown
10.4
Total
127.5

39. Percent of Female Breast Cases by Cancer Subtype
Percent of Cases
HR+/HER2- %
HR-/HER2- %
HR+/HER2+ %
HR-/HER2+ %
Unknown %

40. Who Gets This Cancer?
Female breast cancer overall is most common in middle-aged and older women. Although rare, men can develop breast cancer as well. The number of new cases of female breast cancer, with all subtypes combined, was per 100,000 women per year based on cases.
The distribution of breast cancer subtypes among the female population varies by age, race, ethnicity, stage, and more. Compared with women with HR+/HER2− subtype (the most common subtype), those diagnosed with the other three subtypes were somewhat more likely to be younger, belong to minority groups, and be diagnosed with cancer at a later stage. (Howlader 2018)

41. Rate of New Female Breast Cancer Subtypes per 100,000 by Race
Race / Ethnicity
HR+/HER2- Rate
HR-/HER2- Rate
HR+/HER2+ Rate
HR-/HER2+ Rate
Unknown Rate
White
90.2
12.1
13.0
5.1
10.1
Black
70.2
22.3
6.6
11.9
American Indian / Alaska Native
51.7
7.5
8.3
4.8
7.2
Asian or Pacific Islander
66.3
8.4
11.6
5.9
7.9
Hispanic
62.2
10.4
10.6
9.2
Rate of New Female Breast Cancer Subtypes per 100,000 by Race
WhiteBlackAI/ANªAPIᵇHispanicRace Rate of New Cases0100
HR+/HER2- Rate 
HR-/HER2- Rate 
HR+/HER2+ Rate 
HR-/HER2+ Rate 
Unknown Rate
SEER , a American Indian / Alaska Native, b Asian or Pacific Islander
White women have the highest rate of new cases of HR+/HER2− breast cancer. Black women have the highest rate of new cases of HR−/HER2− breast cancer, also known as the triple-negative subtype.

43. For example, among those with
While breast cancer subtype affects survival, stage at diagnosis may be the most powerful factor in determining survival outcome.
For example, among those with
localized disease, the survival rate after 5 years was greater than 91 percent regardless of subtype.
Additionally,
survival for localized triple-negative female breast cancer was 91.0 percent,
but it dropped to 11.2 percent for distant disease.

44. HBCR Data
Mammary Paget’s

45. Special Types of Adenocarcinoma

46. Special Types of Carcinoma

47. Salivary Gland Type Tumors of the Breast